The textbook that is utilized by Prof. Dr. Frederick R Carrick are reference for Autonomic Controls 1-3 is "A Textbook of Clinical Disorders of the Autonomic Nervous System. Oxford, Oxford University Press, 1983" This text is a comprehensive compilation of information that is very strong in application. It should be a standard text in your neurology library. Although this text is dated, its applications are very specific to the modern day neurologist and literature searches listed for these lectures will update any errors in the text.

Prof. Dr. Frederick R Carrick has also recommended that all learners obtain the following 3 papers authored by RH Johnson et al to assist them in their understanding and applications of the autonomic nervous system.

Johnson RH, Spalding JMK: Disorders of the Autonomic Nervous System. Oxford, Blackwell Scientific Publications, 1974

Johnson RH, Lambie DG, Spalding JMK: Neurocardiology: The Interrelationships Between Dysfunction in the Nervous and Cardiovascular Systems. London, W B Saunders, 1984 Johnson RH: Clinical assessment of sympathetic function in man. Methods Find Exp Clin Pharmacol 6:187, 1984

 

 

The following references have been utilized by Prof. Dr. Frederick Carrick in his preparation of his lecture of the Autonomic Nervous system controls #1. Abstracts have been included. Learners may obtain specific papers of interest to them from the Logan College Library or from their own University library.

 

1. Alvarez, E.; Ferrer, T.; Perez Conde, C.; Lopez Terradas, J. M.; Perez

Jimenez, A.; Ramos, M. J. Evaluation of congenital

dysautonomia other than Riley-Day syndrome. Neuropediatrics.

1996 Feb; 27(1): 26-31; ISSN: 0174-304X.

GERMANY. We report on four children, from different families,

who suffer from a congenital autonomic disorder, presumably

inherited. Three of them have a sensory neuropathy but do not

fit any described hereditary sensory and autonomic neuropathy.

All four were examined along with some of their immediate

family members. We assessed the cardiovagal, sympathetic

adrenergic and sympathetic cholinergic functions with a

battery of non-invasive tests. Results demonstrated that

sudomotor and cardiovascular orthostatic regulation exhibited

the greatest abnormalities, pointing to a predominant

impairment of sympathetic components, both cholinergic and

adrenergic. The overall examination showed a heterogeneous

group of congenital dysautonomia, exclusive of Riley-Day or

other recognized hereditary sensory and autonomic

neuropathies. We emphasize the importance of studying whole

family groups to diagnose subclinical impairment and to

provide correct genetic counselling.

2. Araujo, L. M.; Freeman, R.; Broadbridge, C. Cardiovascular

autonomic tests in diabetic patients with gastroparesis. Arq-

Neuropsiquiatr. 1997 Jun; 55(2): 227-30; ISSN: 0004-282X.

BRAZIL. The aim of this report was to study the

cardiovascular autonomic tests in the evaluation of diabetic

patients with gastroparesis. Forty diabetic subjects were

divided into two groups: one group with gastroparesis (GP, n =

20) and another group paired by age and duration of diabetes

without any complaint of autonomic neuropathy (DC, n = 20).

They were evaluated clinically and submitted to a battery of

five cardiovascular autonomic tests. The presence and severity

of autonomic neuropathy were defined according to the number

of normal cardiovascular tests. Each test had a score: zero

(normal), one (borderline) and two (abnormal). The GP group

showed a higher abnormal total score in the cardiovascular

autonomic test than the group without any complaint (6.6 +/-

3.0 vs. 2.7 +/- 1.4, p < 0.01). These data suggest that diabetic

with gastroparesis presents more abnormal cardiovascular

autonomic tests than diabetic without autonomic neuropathy

and these tests should be included in the evaluation of diabetic

patients with gastroparesis.

3. Arden, R. L.; Bahu, S. J.; Zuazu, M. A.; Berguer, R. Reflex

sympathetic dystrophy of the face: current treatment

recommendations. Laryngoscope. 1998 Mar; 108(3): 437-42;

ISSN: 0023-852X.

UNITED-STATES. Reflex sympathetic dystrophy (RSD) of the

face is an infrequently reported clinical pain syndrome

characterized by dysesthesia, hyperalgia, hyperpathia, and

allodynia. Treatment strategies, extrapolated from RSD and

causalgia of the extremities, remain variable and poorly

defined. Sympathetic blockade is generally the diagnostic and

therapeutic treatment of choice; however, the frequency,

timing, and duration of injections; need for neurolytic blocks;

and role of sympathectomy are not well understood. The

objectives of this report are to highlight the clinical behavior

of facial RSD and contrast its essential differences from

extremity RSD in response to standard treatment regimes. The

case studies of two patients with this syndrome, following

vascular surgery in the neck, are retrospectively reviewed

with existent reported cases. Age, gender, etiology, symptoms,

onset, triggers, and examination findings; timing, duration, and

method of treatment; and outcome are summarized, forming

the database for this study. Findings demonstrate an

infrequent association of vasomotor and sudomotor changes

with facial RSD, and lack of progression to a dystrophic or an

atrophic stage, in contrast to extremity RSD. Furthermore,

treatment response to sympathetic blockade is durable and

less critically dependent on timing. The authors conclude that

facial RSD has a favorable prognosis and should be managed

conservatively with nonneurolytic stellate ganglion blocks,

even when initiated as a delayed and repetitive injection

series.. 0; 2180-92-9.

4. Barbut, D.; Gold, J. P.; Heinemann, M. H.; Hinton, R. B.; Trifiletti, R.

R. Horner's syndrome after coronary artery bypass surgery.

Neurology. 1996 Jan; 46(1): 181-4; ISSN: 0028-3878.

UNITED-STATES. We established the frequency of Horner's

syndrome (HS) in 248 elective patients after coronary artery

bypass surgery. Patients were evaluated neurologically pre-

and post-operatively and 6 months after surgery. Nineteen

patients (7.7%) developed unilateral HS postoperatively, 12

involving the left eye. The finding persisted in 10 patients

(4%) at 6 months. When assessed 2 to 6 days, or 6 months,

postoperatively, HS tended to be isolated and not associated

with C8/T1 plexopathy. Among nondiabetic subjects,

hypertensive patients had a higher frequency of HS than

normotensive patients (10.6% versus 2.9%, p = 0.05). Among

normotensive subjects, diabetic patients had a higher

frequency than nondiabetic patients (15% versus 2.9%, p =

0.08). There was no association between HS, age, sex, internal

mammary artery grafting, or length of cardiopulmonary bypass

time. In summary, HS is a common and sometimes persistent

complication of coronary artery bypass surgery. Hypertensive,

and possibly diabetic, patients appear to be at greatest risk

for developing HS.

5. Beard, J. P.; Wade, W. H.; Barber, D. B. Sacral insufficiency stress

fracture as etiology of positional autonomic dysreflexia: case

report. Paraplegia. 1996 Mar; 34(3): 173-5; ISSN: 0031-1758.

ENGLAND. The medical literature is replete with case reports

of the syndrome known as autonomic dysreflexia. Although the

majority of cases are known to be induced by either bladder or

bowel distention. there does exist a small number of cases in

which the inciting stimulus is more obscure. In such cases, a

comprehensive medical evaluation is necessary to ensure

proper identification of the source of irritation and the

appropriate medical management of the patient. We present a

patient with a heretofore unreported suspected etiology of

autonomic dysreflexia, axial loading of a sacral stress

fracture.

6. Benarroch, E. E.; Stotz Potter, E. H. Dysautonomia in fatal familial

insomnia as an indicator of the potential role of the thalamus

in autonomic control. Brain-Pathol. 1998 Jul; 8(3): 527-30;

ISSN: 1015-6305.

SWITZERLAND. Fatal familial insomnia (FFI) is characterized

by insomnia, dysautonomia, disruption of circadian rhythms,

and motor dysfunction. The typical neuropathological findings

in FFI are severe neuronal depletion in the mediodorsal (MD)

and anteroventral nuclei of the thalamus. The interaction

between the thalamus and central autonomic control

mechanisms is poorly understood. The central autonomic areas

include the anterior cingulate and insular cortices; amygdala,

paraventricular nucleus, dorsomedial nucleus, and lateral

hypothalamic area; periaqueductal gray; parabrachial nucleus;

ventrolateral medulla; and nucleus of the solitary tract.

Several nuclei of the thalamus have connections with areas of

the central autonomic network. The paraventricular nucleus

(PVT) projects to the medial prefrontal cortex, and receives

multimodal visceral and somatosensory inputs. The MD nucleus

is connected with several "limbic" areas involved in autonomic

control. The autonomic manifestations of FFI are exaggerated

sympathetic activation with preserved parasympathetic drive

to the cardiovascular system. This reflects an exaggerated

sympathetic drive from supramedullary structures.

Bicuculline, administered into the MD, elicits an increase in

arterial pressure and heart rate. The medial portion of the MD

may share with the PVT a relay function for circuits

controlling autonomic responses. MD involvement in FFI

suggests a role of the thalamus in central autonomic and other

integrative functions.

7. Blaber, A. P.; Bondar, R. L.; Stein, F.; Dunphy, P. T.; Moradshahi, P.;

Kassam, M. S.; Freeman, R. Complexity of middle cerebral

artery blood flow velocity: effects of tilt and autonomic

failure. Am-J-Physiol. 1997 Nov; 273(5 Pt 2): H2209-16; ISSN:

0002-9513.

UNITED-STATES. We examined spectral fractal characteristics

of middle cerebral artery (MCA) mean blood flow velocity

(MFV) and mean arterial blood pressure adjusted to the level of

the brain (MAPbrain) during graded tilt (5 min supine, -10

degrees, 10 degrees, 30 degrees, 60 degrees, -10 degrees,

supine) in eight autonomic failure patients and age- and sex-

matched controls. From supine to 60 degrees, patients had a

larger drop in MAPbrain (62 +/- 4.7 vs. 23 +/- 4.5 mmHg, P <

0.001; means +/- SE) and MFV (16.4 +/- 3.8 vs. 7.0 +/- 2.5

cm/s, P < 0.001) than in controls. From supine to 60 degrees,

there was a trend toward a decrease in the slope of the fractal

component (beta) of MFV (MFV-beta) in both the patients and

the controls, but only the patients had a significant decrease

in MFV-beta (supine: patient = 2.21 +/- 0.18, control = 1.99 +/-

0.60; 60 degrees: patient = 1.46 +/- 0.24, control = 1.62 +/-

0.19). The beta value of MAPbrain (MAPbrain-beta; 2.19 +/-

0.05) was not significantly different between patients and

controls and did not change with tilt. High and low degrees of

regulatory complexity are indicated by values of beta close to

1.0 and 2.0, respectively. The increase in fractal complexity of

cerebral MFV in the patients with tilt suggests an increase in

the degree of autoregulation in the patients. This may be

related to the drop in MAPbrain. The different response of

MFV-beta compared with that of MAPbrain-beta also indicates

that MFV-beta is related to the regulation of cerebral vascular

resistance and not systemic blood pressure.

8. Bleasdale Barr, K. M.; Mathias, C. J. Neck and other muscle pains in

autonomic failure: their association with orthostatic

hypotension. J-R-Soc-Med. 1998 Jul; 91(7): 355-9; ISSN: 0141-

0768.

ENGLAND. Neck pain in the suboccipital and paracervical

region ('coathanger' configuration) is often reported by

patients with autonomic failure and orthostatic hypotension.

The frequency of this pain, along with pains in the buttock and

calf regions, was determined by questionnaire in two major

groups with primary chronic autonomic failure--pure

autonomic failure (PAF) and multiple system atrophy (MSA).

Comparisons were made with Parkinson's disease, cerebellar

degeneration and other disorders in which neurological

symptoms overlap but in which there was neither autonomic

failure nor orthostatic hypotension. Neck pain was present in

93% of patients with PAF, 51% of patients with MSA and 38-

47% of the non-autonomic groups. Buttock pain was present in

smaller but similar proportions (8-19%) of each group, like

calf pain (23-37%). Neck pain in PAF and MSA differed from

that in the other groups in being relieved by sitting or lying

flat and in being associated with factors that lower blood

pressure in these patients. Buttock pain was posturally related

in PAF and MSA; for calf pain there was no difference between

groups. Neck pain was related to the degree of orthostatic

hypotension; in PAF patients, whose postural blood-pressure

fall was greater than that in MSA, there was a greater

frequency of neck pain.

9. Bombana, J. A.; Ferraz, M. P.; Mari, J. J. Neurovegetative dystonia-

-psychiatric evaluation of 40 patients diagnosed by general

physicians in Brazil. J-Psychosom-Res. 1997 Nov; 43(5): 489-

95; ISSN: 0022-3999.

ENGLAND. The diagnosis of neurovegetative dystonia (NVD) is

commonly made by general physicians in Brazil, but its precise

meaning is unclear. Anecdotal evidence suggests that it is

used to describe patients with a wide range of psychological

and physical symptoms and is often used pejoratively, in a

similar way to "crocks" in the USA. Forty patients who had

been diagnosed as having NVD by general physicians working in

a triage department of a general public hospital were

compared with 40 non-NVD patients, matched for age and

gender, from the same department. Patients were evaluated by

a psychiatrist who was blind to the diagnosis that had been

made. The assessment included a structured sociodemographic

questionnaire, the Clinical Interview Schedule (CIS), and a

routine psychiatric interview using DSM-III-R criteria. Using

the CIS, the "reported symptoms" that most distinguished NVD

patients from controls were somatic and anxiety, whereas for

"manifest abnormality" NVD patients displayed more anxiety,

histrionic behavior, hypochondriasis, and depressive thoughts.

A total of 92.5% of NVD patients received diagnoses using

DSM-III-R criteria compared to 37.5% of controls. The relative

risk of NVD patients subsequently receiving a psychiatric

disorder was 8.3 (95% CI = 2.5-43.1, p < 0.001). Although

general physicians correctly identify most patients with

psychiatric disorder they miss many others. Furthermore, they

use an obsolete diagnostic category which has no psychiatric

currency. Medical students and residents need better

psychiatric training so that they can correctly identify

patients in general medical settings who are suffering from

mental disorders and make a diagnosis using accepted

psychiatric terminology.

10. Bordet, R.; Benhadjali, J.; Destee, A.; Hurtevent, J. F.; Bourriez, J.

L.; Guieu, J. D. Sympathetic skin response and R-R interval

variability in multiple system atrophy and idiopathic

Parkinson's disease. Mov-Disord. 1996 May; 11(3): 268-72;

ISSN: 0885-3185.

UNITED-STATES. We compared autonomic function in patients

with multiple system atrophy (MSA) or with idiopathic

Parkinson's disease (IPD) by measuring sympathetic skin

response (SSR) and R-R interval variability (RRIV). SSR was

investigated in 26 patients (13 with MSA and 13 patients with

IPD). RRIV during deep breathing, Valsalva maneuver, and on

standing was investigated in 20 patients (nine with MSA and

11 with IPD). MSA and IPD patients had similar age, illness

duration, and therapy. Abnormal SSR was more frequent in MSA

(69%) than in IPD (7.7%; x2, 10.4; p < 0.002). RRIV during deep

breathing and the Valsalva maneuver was lower in MSA than in

IPD (p = 0.02). RRIV during standing up was not significantly

different in IPD and MSA. These differences between MSA and

IPD may be due to more severe and widespread autonomic

disturbance in MSA, related to more severe neuropathologic

involvement of the autonomic nervous system. SSR and RRIV

may aid in the differential diagnosis of parkinsonism and help

to exclude from clinical trials MSA patients clinically

misdiagnosed as having IPD.

11. Borg, A. A. Reflex sympathetic dystrophy syndrome: diagnosis and

treatment. Disabil-Rehabil. 1996 Apr; 18(4): 174-80; ISSN:

0963-8288.

ENGLAND. The reflex sympathetic dystrophy syndrome is a

very common, poorly recognized syndrome which is associated

with marked disability in some cases. The historical aspects,

current ideas about the pathogenesis and pathophysiology,

clinical features and staging are discussed. Early recognition

and appropriate intervention are the cornerstone of successful

treatment and are also discussed.

12. Braune, H. J. Early detection of diabetic neuropathy: a

neurophysiological study on 100 patients. Electromyogr-Clin-

Neurophysiol. 1997 Oct; 37(7): 399-407; ISSN: 0301-150X.

BELGIUM. As a long-term complication of diabetes mellitus,

autonomic neuropathy has received considerable attention in

the last few years since a tripled 5-year-mortality of

patients with diabetic neuropathy including autonomic

disturbances has been observed. In a total of 100 diabetics

with clinical manifest neuropathy of different stages (N0 = 8,

N1 = 15, N2a = 24, N2b = 28, N3 = 25), 26 of whom were

insulin-dependent and 74 non-insulin-dependent, the validity

of different neurophysiologic and autonomic function test

procedures proposed in literature was assessed. Apart from

clinical examination, nerve conduction velocity measurement

of five nerves as well as amplitude measurement of evoked

sensory and motor actin potentials, electromyography of at

least four muscles of the lower limbs, and measurement of

sympathetic skin response on hands and feet were performed.

Furthermore, heart rate variation at deep periodical breathing

(E/I-ratio), during Valsalva's manoeuver and after standing up

(30/15-ratio) was determined. In addition, the drop in systolic

blood pressure to standing up was measured. The further

developed the clinical picture of neuropathy, the more

pathological were the results obtained in different tests. The

results suggest that most changes leading to pathological

values of nerve conduction velocity and heart rate variation

measurement take place in a clinical stage, in which no or only

very slight clinical signs give evidence of diabetic neuropathy

(N0-N1). Therefore, especially these examinations should be

performed on diabetics with no or only slight clinical signs of

neuropathy in order to reveal those with beginning neuropathic

disturbances. EMG examination is preferable in later stages of

the disease.

13. Braune, S.; Auer, A.; Schulte Monting, J.; Schwerbrock, S.; Lucking,

C. H. Cardiovascular parameters: sensitivity to detect

autonomic dysfunction and influence of age and sex in normal

subjects. Clin-Auton-Res. 1996 Feb; 6(1): 3-15; ISSN: 0959-

9851.

ENGLAND. In 137 healthy volunteers between 18 and 85 years

of age, blood pressure (BP) and heart rate (HR) were measured

continuously with the Finapres device during active change of

posture (ACP), i.e. standing upright, passive tilt (PT, i.e. head-

up tilt), Valsalva manoeuvre (VM), deep breathing (DB),

isometric muscle exercise (IME) and a mental arithmetic task

(MA). Mean HR activation was attenuated with increasing age

in all manoeuvres, but was unrelated to sex. In non-orthostatic

challenge procedures like MA and IME mean BP increases were

independent of age and sex, despite lower increases in HR in

the elderly. This points to a preserved sympathetic efferent

activity. Following a forced fall in BP during ACP, PT and VM,

the initial responses and maintenance values of BP showed a

significant age-related decrease. This finding was strongly

related to lower BP values in males compared with females,

which became more pronounced with increasing age. Further

studies to investigate age-related changes in the activation of

the various components of the cardiovascular regulation need

to consider the mode of activation of the autonomic nervous

system and sex as factors of influence. Normal ranges, and

also some new points in time not previously measurable, were

calculated for all standard autonomic tests based on the

continuous measurement of BP and HR. The minimum length of

time necessary to assess the cardiovascular responses during

ACP and PT was found to be 60 s. The upper time limits for

reaching maximum activation during IME and MA were 3.5 min

and 1 min, respectively. Age had a relevant influence on the

lower limits of normal of all HR parameters and of some BP

measurements during PT, ACP and VM. Sex was found to have

no relevant impact on normal ranges. Over 65 years of age the

normal values for HR activation during VM and DB hardly

exceeded baseline values. The possibility of increasing the

sensitivity of detection of autonomic dysfunction by

measuring BP continuously must be approached with caution,

as sufficient sensitivity was only reached at the lower limits

of normal during late phase II of the VM. The initial increase of

HR after ACP and the BP values after 60 s standing time

proved to be the parameters with the best sensitivity for

detecting an affection of the regulation of HR and BP over the

whole range of age.

14. Bullens, P.; Daemen, M.; Freling, G.; Kitslaar, P.; Van, den

Wildenberg F.; Kurvers, H. Motor dysfunction and reflex

sympathetic dystrophy. Bilateral motor denervation in an

experimental model. Acta-Orthop-Belg. 1998 Jun; 64(2): 218-

23; ISSN: 0001-6462.

BELGIUM. Reflex sympathetic dystrophy (RSD) is a neuropathic

pain condition most often occurring in relation to trauma to,

or surgery on, an extremity. It is characterized among other

things by motor disturbances such as joint stiffness and

tremor. Signs and symptoms can be induced in a rat model

through chronic constriction of a sciatic nerve (CCI-model). In

this study the CCI-model was used to evaluate the extent of

bilateral peripheral motor nerve-fiber involvement in relation

to ligature localization. In 12 Lewis rats, the common sciatic

nerve was loosely ligated with four chromic catgut ligatures

at the midthigh level just proximal to the right sciatic

trifurcation. Acetylcholinesterase (CE) histochemistry of

sciatic (distal and proximal to ligation) and corresponding

contralateral nerve biopsy specimens was performed at 21

days after ligation. An additional 12 rats were sham-operated

and served as controls. As compared to sham-operated

controls or contralateral nonligated sciatic nerves, CE

histochemistry after 21 days revealed a marked decrease of

CE-positive fibers in cross-sections taken from distal and

proximal sciatic nerve biopsies ipsilateral to the ligatures. In

addition, as compared to sham-operated controls, there was a

decrease of CE-positive fibers in cross-sections taken from

contralateral nonligated sciatic nerves. The present findings

indicate profound motor denervation, distal as well as

proximal to the ligatures. Motor denervation also affected the

contralateral nonligated sciatic nerve. The evident usefulness

of the CCI-model for the study of RSD places the present

results in line with the concept of central nervous system

involvement in the pathophysiology of RSD.. EC 3.1.1.7.

15. Burger, A. J.; Charlamb, M.; Weinrauch, L. A.; D'Elia, J. A. Short-

and long-term reproducibility of heart rate variability in

patients with long-standing type I diabetes mellitus. Am-J-

Cardiol. 1997 Nov 1; 80(9): 1198-202; ISSN: 0002-9149.

UNITED-STATES. Heart rate variability (HRV) has been used to

assess cardiac autonomic function noninvasively, understand

the pathophysiologic mechanisms of heart disease, evaluate

therapy, and assess long-term prognosis. We examined both the

short- and long-term reproducibility of the time and frequency

domain HRV parameters in 23 type I diabetics over a 12-month

interval. Entry criteria included juvenile onset diabetes before

age 35 years, >24-year duration of diabetes, diabetes difficult

to control, and albuminuria. Standardized noninvasive

autonomic testing and 24-hour ambulatory

electrocardiographic recordings were obtained. Fifteen men

and 8 women (mean age 36.7 years) were enrolled. Fifty-three

percent of the men and 75% of the women were smokers, and

women had higher cholesterol than men. All HRV parameters

were markedly decreased when compared with normal persons.

Using Pearson correlation, the time domain indicators of

parasympathetic activity demonstrated very strong

correlations at 3 and 6 months compared with baseline, with

good correlations at 1 year. The average SD of all 5-minute RR

intervals maintained a very strong correlation for the entire

year (r >0.94). In the frequency domain, the measures of

parasympathetic and sympathetic activity maintained a solid

correlation for the entire study period. Reproducibility of HRV

was also examined using repeated-measures analysis of

variance. The time and frequency domain parameters

demonstrated very little variation over the study period of 12

months. Thus, our investigation demonstrated that HRV in

long-term diabetics using 24-hour ambulatory recordings is

abnormal and reproducible over a 12-month interval; very

little variation in all HRV parameters, especially in

parameters of parasympathetic activity, occurred during the

study period.

16. Chaudhuri, K. R.; Ellis, C.; Love Jones, S.; Thomaides, T.; Clift, S.;

Mathias, C. J.; Parkes, J. D. Postprandial hypotension and

parkinsonian state in Parkinson's disease. Mov-Disord. 1997

Nov; 12(6): 877-84; ISSN: 0885-3185.

UNITED-STATES. Abnormal postprandial cardiovascular

responses such as postprandial hypotension (PPH) occur in

primary autonomic failure and contribute significantly to

morbidity. The extent and frequency of PPH and its

relationship to the parkinsonian state in idiopathic Parkinson's

disease (IPD) is unknown. By studying 20 patients with IPD

(without autonomic failure) and 16 age-matched controls after

both groups ingested a standard isocaloric balanced liquid

meal, we have shown that supine PPH complicates IPD and is

related to marked worsening of the parkinsonian state as

measured by a cumulative score of tremor, rigidity,

bradykinesia, posture, and gait. Furthermore, significant

postural hypotension is unmasked that results in postural

intolerance due to presyncopal symptoms. Our study indicates

that, in patients with IPD, ingestion of a meal may lead to

abnormal postprandial cardiovascular responses and

aggravation of the parkinsonian stage. The underlying

mechanisms are unclear, although vasodilatory gut peptides

released in response to food ingestion may be contributory.. 0;

11061-68-0.

17. Clinchot, D. M.; Lorch, F. Sympathetic skin response in patients

with reflex sympathetic dystrophy. Am-J-Phys-Med-Rehabil.

1996 Jul; 75(4): 252-6; ISSN: 0894-9115.

UNITED-STATES. The sympathetic skin response (SSR) was

recorded in four patients diagnosed with reflex sympathetic

dystrophy (RSD) in one upper limb using Kozin's clinical

criteria in conjunction with a three-phase bone scan. All

patients had sustained cerebral vascular accidents and were

classified as Stage I RSD. The SSR was recorded in both hands

after each of ten contralateral median nerve surface

stimulations and in both feet after each of ten contralateral

peroneal nerve surface stimulations. Amplitude, onset latency,

and number of phases were recorded for all responses in each

limb. Mean amplitude, onset latency, and the number of phases

of the five largest potentials were then determined. In all

patients, there was a statistically significant difference in

the amplitude and latency of the SSR in the involved limb

compared with the uninvolved limb; mean amplitude of the

involved limb was greater than the mean amplitude of the

uninvolved limb (P < 0.001), and latency to onset of the SSR in

the involved limb was shorter than that of the uninvolved limb

(P < 0.001). There was no statistically significant difference

in mean amplitude and latency between the involved side and

uninvolved side responses as measured at the feet.

18. Daemen, M. A.; Kurvers, H. A.; Kitslaar, P. J.; Slaaf, D. W.; Bullens,

P. H.; Van, den Wildenberg FA. Neurogenic inflammation in an

animal model of neuropathic pain. Neurol-Res. 1998 Jan; 20(1):

41-5; ISSN: 0161-6412.

ENGLAND. Loose ligation of a rat sciatic nerve (chronic

constriction injury (CCI) model) provokes signs and symptoms

like those observed in reflex sympathetic dystrophy (RSD)

patients. Primary afferent nociceptive C-fibers seem to be

involved in an afferent orthodromic as well as in an efferent

antidromic manner. In this study we hypothesize that

consequent to development of antidromic impulses in C-

nociceptive afferents, neuropeptides released from peripheral

endings of these fibers, increase skin blood flow (SBF),

vascular permeability, and tissue accumulation of

polymorphonuclear leukocytes (PMNs). Collectively, these

phenomena have been referred to as neurogenic inflammation.

To investigate the presence of neurogenic inflammation in the

CCI-model, we assessed skin blood flow (SBF) as well as the

level of edema and accumulation of PMNs in muscle tissue

obtained from the affected hindpaw. SBF was measured, by

means of laser Doppler flowmetry, before ligation as well as

at day 4 after ligation. At day 4, SBF measurements were

performed before and after abolition of the capability of C-

fibers to mediate a vasodilator response. To this end,

capsaicin was applied perineurally. Increased vascular

permeability was inferred from the level of edema of muscle

tissue as determined by assessment of wet/dry weight ratios

of muscle biopsies. PMN accumulation was investigated by

enzymatic detection of myeloperoxidase (MPO) activity in

muscle biopsies. Compared with preligation values, at day 4

SBF was increased more than twofold (p < 0.05). The latter

response was annihilated by capsaicin application. Compared

with sham operated controls, wet/dry ratios were higher in

the ligated animals (1.104 vs. 1.068; p < 0.05). Likewise, when

compared with sham operated controls, MPO activity was

found to be increased in the ligated hindpaw (Optic Density

0.15 vs. 0.89; p < 0.001). In conclusion, the findings of this

study indicate that loose ligation of a sciatic nerve induces an

inflammatory response in the ipsilateral hindpaw, which most

likely is mediated by release of neuropeptides from the

peripheral endings of antidromically acting nociceptive C-

fibres.. EC 1.11.1.7.

19. Day, C. J.; Shutt, L. E. Auditory, ocular, and facial complications of

central neural block. A review of possible mechanisms. Reg-

Anesth. 1996 May; 21(3): 197-201; ISSN: 0146-521X.

UNITED-STATES. BACKGROUND AND OBJECTIVES. The purpose

of this review is to draw together a collection of uncommon

complications of central neural block that affect the cranial

nerves. There have been a small number of case reports, some

of which have included a possible mechanism of the nerve

dysfunction, but there is no prior review that collected them

together and discussed the possible mechanisms. METHODS.

Published case reports were identified by searching Medline of

the British Medical Association and the Silver Platter CD-ROM

library. In addition, the Medical Defence Union and Medical

Protection Society were contacted to find cases that were

unpublished but the subject of medical negligence lawsuits.

RESULTS. The authors collected these cases together to

increase awareness of alarming complications. If such cases

are recognized for what they are, then the prognosis is good;

however, delayed diagnosis can make them a cause of great

anxiety and possible litigation. CONCLUSIONS. Increased

awareness of these complications can decrease the likelihood

of litigation by early diagnosis and explanation. Their

mechanisms are not fully understood but the likely

possibilities have been discussed. Further work is needed to

establish the incidence of these problems, as it is likely that

many cases are not diagnosed and not reported.

20. Donaghue, K. C. Autonomic neuropathy: diagnosis and impact on

health in adolescents with diabetes. Horm-Res. 1998; 50 Suppl

1: 33-7; ISSN: 0301-0163.

SWITZERLAND. Symptomatic autonomic neuropathy and

abnormal cardiovascular autonomic tests are associated with

increased mortality due to vascular disease and increased risk

of sudden deaths. Intensive therapy in the DCCT caused a

significant risk reduction of developing autonomic nerve

abnormalities at five years only in the Primary Prevention

Group (4 vs. 9%). At the Royal Alexandra Hospital for Children,

the prevalence of cardiovascular reflex abnormalities is 28%.

Over 5 years, we have found no increase in the rate of single

cardiovascular abnormalities and a low rate of persistence.

Two or more abnormalities developed in 5%. Repeated

measures of pupillary function showed a significant increase

in abnormalities and a higher level of persistently abnormal

tests. Spectral analysis and 24-hour BP monitoring may detect

autonomic dysfunction at earlier stages but longitudinal

studies are not yet available.

21. Drummond, P. D. The site of sympathetic deficit in cluster

headache. Headache. 1996 Jan; 36(1): 3-9; ISSN: 0017-8748.

UNITED-STATES. The pattern of autonomic deficit in the face

of cluster headache patients resembles the deficit in patients

with a postganglionic sympathetic lesion from some other

cause; however, the presence of abnormal cardiac rhythms and

bilateral pupillary reflex deficit in some patients with cluster

headache suggests that the lesion might compromise central

sympathetic drive. To investigate this possibility, the

vasomotor and sudomotor startle reflex was investigated in

the hands of six cluster headache patients with ocular and

thermoregulatory signs of postganglionic sympathetic deficit

in the face; for comparison, responses were also investigated

in 15 patients with a lesion in the cervical sympathetic

pathway from some other cause. The startle reflex was intact

in the hands of the six cluster headache patients, but was

diminished ipsilaterally in patients with a central or

preganglionic sympathetic lesion and also, surprisingly, in

patients with a postganglionic lesion caused by an aneurysm of

the internal carotid artery. Ocular sympathetic deficit was

greater in patients with an aneurysm of the internal carotid

artery than in cluster headache patients or in patients with a

postganglionic sympathetic lesion from some other cause; the

aneurysm may have compromised neurons with projections to

the face and hand, or could have induced transsynaptic

degeneration of preganglionic fibers supplying both regions.

The findings indicate that central sympathetic drive is not

impaired in cluster headache patients; thus, a peripheral

lesion probably induces sympathetic deficit on the

symptomatic side of the face.

22. Durlach, J.; Bac, P.; Durlach, V.; Bara, M.; Guiet Bara, A. Neurotic,

neuromuscular and autonomic nervous form of magnesium

imbalance. Magnes-Res. 1997 Jun; 10(2): 169-95; ISSN: 0953-

1424.

ENGLAND. The nervous form of magnesium imbalance

represents the best documented experimental and clinical

aspects of magnesium disorders. The nervous form of primary

magnesium deficit (MD) in the adult appears as the best

descriptive model for analysis of the symptomatology,

aetiology, physiopathology, diagnosis and therapy of the most

frequent form of MD. Nervous hyperexcitability due to chronic

MD in the adult results in a non-specific clinical pattern with

associated central and peripheral neuromuscular symptoms,

analogous to the symptomatology previously described in

medical literature as latent tetany, hyperventilation

syndrome, spasmophilia, chronic fatigue syndrome,

neurocirculatory asthenia and idiopathic Barlow's disease. On

encountering this non-specific pattern, the signs of

neuromuscular hyperexcitability are of much greater

importance. Trousseau's sign is less sensitive than Chvostek's

sign, but their sensitivities are increased by hyperventilation

(Von Bondsdorff's test). Examination of the precordial area

will be conducted in order to search clinical stigmata of

mitral valve prolapse (MVP) which is a frequent dyskinesia due

to chronic MD (about a quarter to one-third of cases). The

electromyogram (EMG) shows one (or several) trains of

autorhythmic activities beating for more than 2 min of one of

the three tetanic activities (uniplets, multiplets or 'complex

tonicoclonic tracings') during one of the three facilitation

procedures: tourniquet-induced ischaemia lasting 10 min.

post-ischaemia lasting 10 min after the removal of the

tourniquet and hyperventilation over 5 min. A repetitive EMG

constitutes the principal mark of nervous hyperexcitability

(NHE) due to MD. The echocardiogram (ECC) is the best tool for

detecting MVP, the 2-dimensional ECC with pulsed Doppler

being more accurate than time-motion ECC. The routine ionic

investigations comprise five static tests: plasma and

erythrocyte magnesium, plasma calcium and daily magnesiuria

and calciuria. An evaluation of magnesium intake is desirable.

Normal concentrations of magnesium in blood do not rule out

the diagnosis of the nervous form of primary chronic MD. The

histograms of MD group reveal Gaussian type magnesaemias

with significantly lower means and the constituent elements

can be individually hypo- (one-third of cases), normo- (about

two-thirds of cases) and even, exceptionally, hyper-

magnesaemic. The diagnosis of MD requires an oral magnesium

load test. At physiological dose (5 mg of Mg/kg/day), oral

magnesium is totally devoid of the pharmacological effects of

parenteral magnesium. Corrections of symptomatology by this

oral physiological magnesium load is the best proof that it

was due to magnesium deficiency. In particular clinical forms,

more sophisticated studies may be useful: standard and

quantitative electroencephalograms, electropolygraphic

studies of afternoon sleep, electronystagmography,

optokinetic test, skin conductance reflex, psychometric

inventories, standard or monitoring electrocardiogram,

treadmill test, other static and dynamic investigations: e.g.

ionized free Mg2+, lymphocyte Mg, brain Mg, cerebrospinal Mg,

Mg balance, Mg parenteral load test, glucose load, and even

radio-isotope study, the only one able to reveal intestinal

magnesium hypersecretion. Nervous primary chronic MD

progresses by phases of decompensation against a background

of latency. Marginal magnesium deficiency, that is to say an

insufficient magnesium intake which merely requires simple

oral physiological supplementation, is fundamental in the

aetiology of primary magnesium deficit. However a

constitutional homeostatic lability of the nervous system or

of magnesium metabolism such as belonging to the B35 type of

HLA group must be involved. Part of the aetiology of this

magnesium deficit is a magnesium depletion, where the

disorder which induces magnesium deficit is related to a

dysregulation of the control mechanisms of magnesium status

which requires a more or less difficult. 0; 7439-95-4.

23. Evers, S.; Voss, H.; Bauer, B.; Soros, P.; Husstedt, I. W. Peripheral

autonomic potentials in primary headache and drug-induced

headache. Cephalalgia. 1998 May; 18(4): 216-21; ISSN: 0333-

1024.

NORWAY. Autonomic functions of different primary headache

types have been investigated in several studies, most of them

analyzing cardiovascular reflex mechanisms or biochemical

changes. The results are contradictory; only in tension-type

headache and in cluster headache has a sympathetic

hypofunction been shown in a preponderance of studies. We

analyzed the peripheral autonomous potentials (PAPs) in

different primary headache types and in drug-induced headache

and compared the results with those of healthy subjects and of

patients with low back pain. Latencies of PAPs were

significantly increased in all headache types but not in low

back pain; amplitudes of PAPs did not show significant

differences compared to healthy subjects. Patients with a long

duration of drug abuse had increased PAP latencies, whereas

patients with a high number of migraine attacks per year had

decreased latencies. Our data suggest that sympathetic

hypofunction as measured by PAP latencies is a general

phenomenon in headache but not in all pain syndromes. Drug

abuse leads to an increase of this hypofunction. While

measuring PAPs is not an appropriate method by which to

differentiate between headache disorders, it allows

assessment of autonomic disturbances in primary and drug-

induced headache.. 113-15-5.

24. Fleckenstein, J. F.; Frank, S. m.; Thuluvath, P. J. Presence of

autonomic neuropathy is a poor prognostic indicator in

patients with advanced liver disease. Hepatology. 1996 Mar;

23(3): 471-5; ISSN: 0270-9139.

UNITED-STATES. Autonomic neuropathy (AN) is seen in both

alcohol-induced and non-alcohol-induced liver disease, and

when present is an independent predictor of mortality. We

postulated that patients who were awaiting liver

transplantation are likely to have a high prevalence of

autonomic neuropathy with an associated increase in

mortality. To test our hypothesis, we evaluated the presence

of autonomic neuropathy using a battery of tests in 33

patients awaiting liver transplantation and prospectively

followed them to determine their prognosis. Twenty-two of 33

(67%) patients with liver disease had evidence of autonomic

neuropathy; of these, 12 (36%) had evidence of definite and 10

(31%) had early autonomic neuropathy. The prevalence of AN

was similar in alcohol-induced and non-alcohol-induced liver

disease. Using Child-Pugh classification, 14.3% Child A, 31.3%

Child B, and 60% Child C had definite autonomic neuropathy.

Six patients died during a median observation period of 10

months, and all had AN. Kaplan-Meier survival analysis showed

a significantly higher mortality (P=.05) in patients with AN. On

the basis of this observation, we suggest that consideration

should be given for early liver transplantation in patients with

advanced liver disease and autonomic neuropathy.

25. Fournier, R. S.; Holder, L. E. Reflex sympathetic dystrophy:

diagnostic controversies. Semin-Nucl-Med. 1998 Jan; 28(1):

116-23; ISSN: 0001-2998.

UNITED-STATES. Reflex sympathetic dystrophy, (RSD) is a

complex physiologic response of the body to an external

stimulus resulting in sympathetically mediated, usually

nonanatomic pain, which is out of proportion to the inciting

event or expected healing response. This complex entity has

been the focus of much investigation, leading however to

somewhat confusing and conflicting results and theories about

the etiology and pathophysiology. There is even significant

conflict about what characteristics define the clinical entity

called RSD, and if these characteristics vary with the specific

site of involvement. We have examined the current literature

regarding these fundamental conflicts, and in addition we have

evaluated the current controversies surrounding the role of

Three Phase Radionuclide Bone Imaging (TPBI) for diagnosis,

prognosis, and patient management. These controversies

include the role of scintigraphy, the various criteria for

scintigraphic diagnosis, and the reported variations in

sensitivity and specificity of TPBI in RSD. We have examined

several factors that may have affected these results, and

potentially underestimated the value of scintigraphy in the

diagnosis of RSD. In addition to the heterogeneous patient

populations used to establish the diagnosis by different

subspecialty physicians, these factors include duration of

patient's symptoms, age of the patient population evaluated,

location of the disease, and the varying scintigraphic scan

interpretation criteria used.

26. Frattola, A.; Parati, G.; Gamba, P.; Paleari, F.; Mauri, G.; Di Rienzo,

M.; Castiglioni, P.; Mancia, G. Time and frequency domain

estimates of spontaneous baroreflex sensitivity provide early

detection of autonomic dysfunction in diabetes mellitus.

Diabetologia. 1997 Dec; 40(12): 1470-5; ISSN: 0012-186X.

GERMANY. Diabetic autonomic dysfunction is associated with a

high risk of mortality which makes its early identification

clinically important. The aim of our study was to compare the

detection of autonomic dysfunction provided by classical

laboratory autonomic function tests with that obtained

through computer assessment of the spontaneous sensitivity

of the baroreceptor-heart rate reflex (BRS) by time domain and

frequency domain techniques. In 20 normotensive diabetic

patients (mean age +/- SD 41.9 +/- 8.1 years) with no evidence

of autonomic dysfunction on laboratory autonomic testing (D0)

blood pressure (BP) and ECG were continuously monitored over

15 min in the supine position. BRS was assessed as the slope

of the regression line between spontaneous increases or

reductions in systolic BP and linearly related lengthening or

shortening in RR interval over sequences of at least 4

consecutive beats (sequence method), or as the squared ratio

between RR interval and systolic BP spectral powers around

0.1 Hz. We compared the results with those of 32 age-matched

normotensive diabetic patients with abnormal autonomic

function tests (D1) and with those of 24 healthy age-matched

control subjects with normal autonomic function tests (C).

Compared to C, BRS was markedly less in D1 when assessed by

both the slope of the two types of sequences (data pooled) and

by the spectral method (-71.3% and -60.2% respectively, both p

< 0.01). However, BRS was consistently although somewhat

less markedly reduced in D0, the reduction being clearly

evident for all the estimates (-57.0% and -43.5%, both p <

0.01). The effects were more evident than those obtained by

the simple quantification of the RR interval variability. These

data suggest that time and frequency domain estimates of

spontaneous BRS allow earlier detection of diabetic autonomic

dysfunction than classical laboratory autonomic tests. The

estimates can be obtained by short non-invasive recording of

the BP and RR interval signals in the supine patient, i.e. under

conditions suitable for routine outpatient evaluation.

27. Frontoni, M.; Fiorini, M.; Strano, S.; Cerutti, S.; Giubilei, F.; Urani,

C.; Bastianello, S.; Pozzilli, C. Power spectrum analysis

contribution to the detection of cardiovascular dysautonomia

in multiple sclerosis. Acta-Neurol-Scand. 1996 Apr; 93(4):

241-5; ISSN: 0001-6314.

DENMARK. In multiple sclerosis (MS) autonomic cardiovascular

dysfunction is an uncommon, but potentially dangerous event,

to which studies of spectral analysis of heart rate variability

have not been applied, yet. MATERIAL AND METHODS--We

studied 16 patients with definite MS (11 women and 5 men,

mean age 30.3 +/- 7.4 yrs., mean EDSS 2.06 +/- 1.42) and 16

sex- and age-matched healthy controls. Besides cardiovascular

reflex tests (valsalva manoeuvre, deep breathing, lying to

standing, Blood Pressure response to standing and sustained

handgrip), each underwent spectral analysis of the R-R

interval short-term variability at rest and after tilting, to

detect three components: very low frequency (VLF), low

frequency (LF) and high frequency (HF). A recent brain MRI was

obtained from patients, to compare plaque characteristics

with spectral parameters. RESULTS--At cardiovascular

reflexes, only four patients (25%) showed an impairment,

mostly of a mild degree. VLF and LF at rest were lower in MS

subjects than in controls (p < 0.01). No significant correlation

was found between spectral parameters and lesion area or

localization as detected on MRI. CONCLUSIONS--Spectral

analysis could usefully flank reflex tests to detect autonomic

subclinical cardiovascular abnormalities.

28. Gambetti, P.; Lugaresi, E. Conclusions of the symposium. Brain-

Pathol. 1998 Jul; 8(3): 571-5; ISSN: 1015-6305.

SWITZERLAND. On the basis of twenty-one kindreds and three

cases from uninformative families, the Symposium has

confirmed that fatal familial insomnia (FFI) is genotypically

and phenotypically distinct and, likely, the third most common

inherited prion disease. The genotype, characterized by the

D178N mutation on the prion protein (PrP) gene coupled with

the methionine codon at position 129 has been demonstrated in

all cases. The immunoblot pattern of the PrPres associated

with FFI shows a molecular mass of approximately 19kDa for

the core protein and a marked underrepresentation of the

unglycosylated form. The histopathology, characterized by

marked thalamic and inferior olivary atrophy with a variable

degree of cerebral cortical spongiosis has been observed in all

but two cases. The disease duration was found to be

significantly shorter in the FFI subjects homozygous at codon

129 than in the heterozygous subjects. The FFI sleep disorder

is characterized by lack of spindle activity and disruption of

the wake-sleep cycle which can only be established , or

excluded, by polysomnography. Autonomic, endocrine and

cognitive impairments also require careful assessment in each

case. A condition lacking the D178N mutation and

pathologically identical to FFI has been reported. Presence of

sleep, autonomic and endocrine abnormalities needs to be

demonstrated to identify this condition as a sporadic form of

FFI. The pathophysiology of the sleep disorder, the pathogenic

mechanisms, fine and early structural changes, including the

role of apoptosis, and disease penetrance are the major

unresolved issues in FFI.. 0.

29. Geertzen, J. H.; Dijkstra, P. U.; Stewart, R. E.; Groothoff, J. W.; Ten

Duis, H. J.; Eisma, W. H. Variation in measurements of range of

motion: a study in reflex sympathetic dystrophy patients. Clin-

Rehabil. 1998 Jun; 12(3): 254-64; ISSN: 0269-2155.

ENGLAND. OBJECTIVE: To quantify the amount of variation

attributed to different sources of variation in measurement

results of upper extremity range of motion, and to estimate

the smallest detectable difference (SDD) between

measurements in reflex sympathetic dystrophy (RSD) patients.

DESIGN: Two observers each measured in two sessions the

range of motion of several upper extremity joints of RSD

patients participating in an outcome study. SETTING:

Department of Rehabilitation of a university hospital.

SUBJECTS: Twenty-nine upper extremity RSD patients. MAIN

OUTCOME MEASURES: The range of motion of forward flexion

and external rotation of the shoulder, extension, flexion and

supination of the elbow, palmar, dorsiflexion and ulnar, radial

deviation of the wrist of affected and nonaffected sides, using

a two-armed goniometer and an inclinometer. The

measurement results were analysed using an analysis of

variance according to the generalizability theory. RESULTS:

The results indicate that observer and patient-observer were

important sources of variation. The random error was the most

important source of variation. Averaged over all ranges of

motion the observer contributed 3.9% to the total variation,

patient-observer interactions contributed 5.2% and the random

error 20.3%. The SDD was smallest for elbow flexion, 7.1

degrees and 9.6 degrees and was largest for external rotation

of the shoulder, 24.8 degrees and 28.7 degrees. The SDD was

smaller for the nonaffected side as compared to the affected

side for the majority of ranges of motion except elbow

extension, wrist dorsiflexion, and radial and ulnar deviation.

CONCLUSION: Clinically, our results indicate that range-of-

motion measurements in RSD patients are subject to

considerable variation and indicate that results of medical

examinations in order to assess disability on the basis of

range-of-motion measurements are subject to the same

variation.

30. Goldstein, D. S.; McRae, A.; Holmes, C.; Dalakas, M. C. Autoimmune

autonomic failure in a patient with myeloma-associated Shy-

Drager syndrome. Clin-Auton-Res. 1996 Feb; 6(1): 17-21; ISSN:

0959-9851.

ENGLAND. We report here the case of a patient with the Shy-

Drager syndrome and multiple myeloma who had evidence

consistent with a central neural autoimmune basis for

sympathetic autonomic failure. Autonomic function testing

showed no recordable peroneal skeletal muscle

sympathoneural traffic, normal arterial norepinephrine (NE)

spillover during supine rest and no increment in NE spillover

during exposure to lower body negative pressure. The patient's

cerebrospinal fluid and serum contained an immunoglobulin G

that bound to rat locus ceruleus (LC) in an in vitro test system.

The myeloma protein was of the lambda subtype and bound in

the rat LC, without binding in the substantia nigra, as

demonstrated with anti-lambda antiserum. Since in this case

the monoclonal antibody produced by the myeloma bound

specifically to LC cells, the results are consistent with the

hypothesis that in this patient the Shy-Drager syndrome may

have had an immune-mediated basis.. 0; 0; 0.

31. Goris, R. J. Reflex sympathetic dystrophy: model of a severe

regional inflammatory response syndrome. World-J-Surg. 1998

Feb; 22(2): 197-202; ISSN: 0364-2313.

UNITED-STATES. The systemic inflammatory response

syndrome (SIRS) and acute reflex sympathetic dystrophy

syndrome (RSD) share clinical signs of severe inflammation, a

protracted course, and a similar problem of impaired oxygen

utilization. The difference is that SIRS patients have these

signs and symptoms systemically and are severely ill in the

intensive care unit (ICU), whereas acute RSD patients are in

good health and their problems are limited to one extremity.

Both conditions seem to be the result of an exaggerated

inflammatory response. As RSD patients have a healthy

contralateral extremity, they may be their own control in

various flux studies. It is hypothesized that this situation is

exquisitely suitable for studying the pathophysiology of severe

inflammatory responses in humans. Only a few patients are

required to perform studies of, for example, oxygen

metabolism and cytokine or oxygen radical production.

Assessment methods may be utilized, such as nuclear

magnetic resonance spectroscopy, which cannot easily be

performed in ICU patients.

32. Graif, M.; Schweitzer, M. E.; Marks, B.; Matteucci, T.; Mandel, S.

Synovial effusion in reflex sympathetic dystrophy: an

additional sign for diagnosis and staging. Skeletal-Radiol.

1998 May; 27(5): 262-5; ISSN: 0364-2348.

GERMANY. PURPOSE: To improve the present MRI criteria for

diagnosis and staging of reflex sympathetic dystrophy (RSD) by

including increased joint fluid as an additional MRI sign of

RSD. DESIGN AND PATIENTS: One hundred and fourteen

extremities (69 affected and 45 contralateral controls) in 57

consecutive patients with RSD were evaluated using a 1.5-T

unit. T1- and T2-weighted pulse sequences, often with fat

suppression, were used before and after administration of

intravenous contrast enhancement (Gd). Following T2-weighted

image digitization the volume of synovial fluid was measured

with a computer model. RESULTS: Effusions were detected in

61% of the extremities suspected of RSD and in 44% of the

contralateral control joints. The mean fluid quantity measured

in the symptomatic articulation was 201 mm3. MRI diagnosis

of RSD based on previously described criteria was done in 62%

of the patients, yielding a sensitivity of 60%. Effusions were

present in 79% of the false negative MRI cases.

Retrospectively considering the presence of fluid as a

potential positive criterion for RSD increases the sensitivity

by 31% (to 91%). CONCLUSIONS: Joint effusions are probably

associated with early stages of RSD. Adding effusion to the

list of radiological criteria for RSD increases the sensitivity

of MRI from 60% to 91%.. 0; 7440-54-2.

33. Hagay, Z.; Weissman, A. Management of diabetic pregnancy

complicated by coronary artery disease and neuropathy.

Obstet-Gynecol-Clin-North-Am. 1996 Mar; 23(1): 205-20;

ISSN: 0889-8545.

UNITED-STATES. Various manifestations of diabetic

neuropathy may complicate pregnancies of young diabetic

patients. Of all forms of diabetic neuropathy, autonomic

neuropathy, and, in particular, gastropathy, may cause the

most devastating complications. Because neuropathy is a

common abnormality in young asymptomatic diabetic women,

screening for this disorder may be advisable and can be

accomplished by relatively simple and noninvasive tests.

Screening is best performed before conception or early in

pregnancy, because pregnancy itself and its possible

complications later modify the autonomic nervous function

tests and make testing unreliable. Practitioners and

obstetricians who provide care and counseling to young

diabetic patients should be familiar with the risks and

consequences to maternal and fetal health that may be

imposed by the different forms of neuropathy. Moderate-to-

severe autonomic dysfunction may be considered a relative

contraindication to pregnancy, especially if gastropathy is

part of the clinical presentation. The management dilemmas

and high mortality and morbidity associated with symptomatic

diabetic neuropathy may justify the addition of a new

independent class, class N (neuropathy), to the current

classification systems for diabetes in pregnancy.

34. Hassenbusch, S. J.; Stanton Hicks, M.; Schoppa, D.; Walsh, J. G.;

Covington, E. C. Long-term results of peripheral nerve

stimulation for reflex sympathetic dystrophy. J-Neurosurg.

1996 Mar; 84(3): 415-23; ISSN: 0022-3085.

UNITED-STATES. This prospective, consecutive series

describes peripheral nerve stimulation (PNS) for treatment of

severe reflex sympathetic dystrophy (RSD) or complex regional

pain syndrome, in patients with symptoms entirely or mainly

in the distribution of one major peripheral nerve. Plate-type

electrodes were placed surgically on affected nerves and

tested for 2 to 4 days. Programmable generators were

implanted if 50% or more pain reduction and objective

improvement in physical changes were achieved. Patients were

followed for 2 to 4 years and a disinterested third-party

interviewer performed final patient evaluations. Of 32

patients tested, 30 (94%) underwent permanent PNS

placement. Long-term good or fair relief was experienced in

19 (63%) of 30 patients. In successfully treated patients,

allodynic and spontaneous pain was reduced on a scale of 10

from 8.3 +/- 0.3 preimplantation to 3.5 +/- 0.4 (mean +/-

standard error of the mean) at latest follow up (p<0.001).

Changes in vasomotor tone and patient activity levels were

markedly improved but motor weakness and trophic changes

showed less improvement. Six (20%) of the 30 patients

undergoing PNS placement returned to part-time or full-time

work after being unemployed prestimulator implantation.

Initial involvement of more than one major peripheral nerve

correlated with a poor or no relief rating (p<0.01). Operative

modifications that minimize technical complications are

described. This study indicates that PNS can provide good

relief for RSD that is limited to the distribution of one major

nerve.

35. Hilz, M. J.; Kolodny, E. H.; Neuner, I.; Stemper, B.; Axelrod, F. B.

Highly abnormal thermotests in familial dysautonomia suggest

increased cardiac autonomic risk. J-Neurol-Neurosurg-

Psychiatry. 1998 Sep; 65(3): 338-43; ISSN: 0022-3050.

ENGLAND. OBJECTIVE: Patients with familial dysautonomia

have an increased risk of sudden death. In some patients with

familial dysautonomia, sympathetic cardiac dysfunction is

indicated by prolongation of corrected QT (QTc) interval,

especially during stress tests. As many patients do not

tolerate physical stress, additional indices are needed to

predict autonomic risk. In familial dysautonomia there is a

reduction of both sympathetic neurons and peripheral small

nerve fibres which mediate temperature perception.

Consequently, quantitative thermal perception test results

might correlate with QTc values. If this assumption is correct,

quantitative thermotesting could contribute to predicting

increased autonomic risk. METHODS: To test this hypothesis,

QTc intervals were determined in 12 male and eight female

patients with familial dysautonomia, aged 10 to 41 years

(mean 21.7 (SD 10.1) years), in supine and erect positions and

postexercise and correlated with warm and cold perception

thresholds assessed at six body sites using a Thermotest.

RESULTS: Due to orthostatic presyncope, six patients were

unable to undergo erect and postexercise QTc interval

assessment. The QTc interval was prolonged (>440 ms) in two

patients when supine and in two additional patients when

erect and postexercise. Supine QTc intervals correlated

significantly with thermal threshold values at the six body

sites and with the number of sites with abnormal thermal

perception (Spearman's rank correlation p<0.05). Abnormal

Thermotest results were more frequent in the four patients

with QTc prolongation and the six patients with intolerance to

stress tests. CONCLUSION: The results suggest that impaired

thermal perception correlates with cardiac sympathetic

dysfunction in patients with familial dysautonomia. Thus

thermotesting may provide an alternative, albeit indirect,

means of assessing sympathetic dysfunction in autonomic

disorders.

36. Iani, C.; Attanasio, A.; Manfredi, M. Paroxysmal staring and

masticatory automatisms during postural hypotension in a

patient with multiple system atrophy. Epilepsia. 1996 Jul;

37(7): 690-3; ISSN: 0013-9580.

UNITED-STATES. PURPOSE: We studied a 51-year-old man with

multiple system atrophy and autonomic insufficiency. He had

repeated episodes of loss of contact, staring, and masticatory

automatisms. METHODS: Blood pressure during these events

documented a systolic pressure of 60 mm Hg. Cardiovascular

reflex tests provided evidence of autonomic failure. Head

computed tomography (CT) revealed moderate, diffuse cortical

and cerebellar atrophy. RESULTS: These events were strictly

related to blood pressure decreases and could be reproduced

consistently by having the patient sit up after a meal. Ictal

polygraphic recordings showed EEG changes consistent with

anoxia, preceded by sudden hypotension with fixed heart rate.

CONCLUSIONS: Cerebral anoxia during a syncopal attack may

therefore precipitate transient, sudden neurologic dysfunction

that closely mimics complex partial seizures. Masticatory

automatisms may represent a release phenomenon resulting

from inactivation of neocortical structures by cerebral anoxia

or reticular disconnection.

37. Inui, H.; Kitaoku, Y.; Yoneyama, K.; Nakane, M.; Ohue, S.; Yamanaka,

T.; Ueda, T.; Fujita, N.; Miyahara, H.; Matsunaga, T. MR-

angiographic findings of patients with central vestibular

disorders. Acta-Otolaryngol-Suppl-Stockh. 1998; 533: 51-6;

ISSN: 0365-5237.

NORWAY. Magnetic resonance angiography (MRA) is a new,

noninvasive, and useful method to estimate the posterior

circulation in patients with vertigo. From June 1995 to May

1997, 180 patients were examined by magnetic resonance

imaging (MRI) and MRA in our department. One hundred and

forty-seven patients were vertiginous patients. We measured

the displacement angle of the basilar artery with MRA, and

examined the relationship between the findings from some

neurological examinations and MRA findings in patients with

vertigo and dizziness. One hundred and forty-seven patients

with vertigo or dizziness were examined by MRI and MRA. They

were diagnosed with MRI images in addition to several

neurological examinations. MRA was not used for the diagnosis

but rather for measuring the displacement angle of the basilar

artery. Eighty-six cases with central vestibular disorders, 11

cases with vertebrobasilar insufficiency, and 26 cases with

autonomic nerve disorders were recognized. In the cases of

central vestibular disorders, the incidences of hyperlipidemia

and hypotension were higher than the incidence of anemia. The

average displacement angle of the basilar artery (n = 180) was

153.4 degrees +/- 39.4 degrees (mean +/- S.D.). MRA findings

were classified into five categories. Ten patients were

classified as category III, which represented unilateral partial

vertebral artery stenosis. The detection rate for category III

and IV abnormalities by neurological examination was higher

than that for the other categories. MRI and MRA are important

methods to examine patients with central nervous disorders.

Distal vertebral artery stenosis may carry a higher risk of a

stroke than brainstem infarction.

38. Ishizaki, F.; Harada, T.; Yoshinaga, H.; Nakayama, T.; Yamamura, Y.;

Nakamura, S. [Prolonged QTc intervals in Parkinson's disease--

relation to sudden death and autonomic dysfunction]. No-To-

Shinkei. 1996 May; 48(5): 443-8; ISSN: 0006-8969.

JAPAN. Sudden death has been reported in Parkinson's disease

(PD), but the cause of death has not been fully clarified. A

prolonged QT interval on the electrocardiogram (ECG) of

patients without cardiac dysfunction is an independent risk

factor for sudden death regardless of etiology. QT prolongation

is believed to be related to cardiac autonomic dysfunction. We

suspected that QTc intervals, as well as QT intervals, might be

related to the clinical characteristics of PD and to the

function of the autonomic nervous system in PD and also

postulated a relationship between QTc prolongation and sudden

death in PD. We investigated the QTc intervals on the ECGs of

48 PD patients (20 males 28 females) aged 64.5 +/- 9.4 years

and 44 controls aged 60.0 +/- 8.2 years, and excluded patients

with heart disease. QTc intervals were determined by using

ECG-8210, ECAPS12 (Nihon-Kohden). The autonomic nervous

system was evaluated by measuring CVR-R and performing

orthostatic tests. Since the autonomic nervous system is

considered to play an important role in the mechanism of

diurnal blood pressure variation (DBPV), we assessed DBPV in

19 PD patients by determining blood pressure automatically

every 30 minutes for 24 hours with an ambulatory blood

pressure monitor (90202, Space Lab). QTc intervals were

significantly longer in the PD patients (412 +/- 26 msec) than

in the controls (401 +/- 14 msec) (p < 0.02, t-test). QTc

prolongation was significantly correlated with severity

according to Hoehn and Yahr stage (r = 0.509, p < 0.001),

orthostatic hypotension, and decreased CVR-R ratio but not

with duration of PD or treatment. The incidence of QTc

prolongation was higher in the PD patients with non-dipper

type DBPV than in those with the dipper type. Two of the PD

patients died suddenly. Their QTc intervals a year before their

death were 451 msec and 470 msec, respectively, suggesting

that cardiac dysautonomia may have been involved in the cause

of their death. These findings suggest that cardiac autonomic

dysfunction is related to the severity of PD, and that it may

predispose such patients to cardiac disorders including sudden

cardiac death.

39. Karlsson, A. K.; Friberg, P.; Lonnroth, P.; Sullivan, L.; Elam, M.

Regional sympathetic function in high spinal cord injury during

mental stress and autonomic dysreflexia. Brain. 1998 Sep;

121( Pt 9): 1711-9; ISSN: 0006-8950.

ENGLAND. Centrally mediated sympathetic stimulation of

subjects who have suffered a spinal cord injury (SCI) does not

activate the decentralized part of the body below the level of

the lesion, whereas experimental data indicate an exaggerated

response above the level of the lesion. SCI subjects may

exhibit an autonomic dysreflexia reaction following afferent

stimulation below the level of the lesion. In order to

investigate the function of the sympathetic nervous system

above and below the level of the lesion, regional noradrenaline

spillover was measured by means of steady-state isotope

dilution technique above (forearm) and below (leg) the level of

the lesion at baseline, during mental stress and following

bladder stimulation in nine SCI subjects (mean age 41 years;

level of injury C7-T4; mean duration of injury 13.8 years). The

results from the SCI subjects were also compared with those

from 10 weight- and age-matched control subjects, both at

rest and during mental stress. Body composition was

determined by dual energy X-ray absorptiometry scanning and

arm/leg blood flow by occlusion plethysmography. At baseline,

total and regional noradrenaline spillover did not differ

between the groups. Mental stress increased mean arterial

pressure in both groups. Heart rate (76 versus 64 beats/min; P

< 0.05) and arm noradrenaline spillover (2.73 versus 1.71

pmol/min/100 g; P < 0.05) increased more in spinal cord injury

subjects than in control subjects, whereas total body (2826

versus 3783 pmol/min; P < 0.01) and leg noradrenaline

spillover (0.23 versus 0.41 pmol/min/100 g; P < 0.05)

increased only in the control group. During bladder stimulation,

SCI subjects reacted with a marked increase in mean arterial

pressure and leg noradrenaline spillover (from 0.06 to 0.91

pmol/min/100 g; P < 0.05) and their leg blood flow decreased.

Regional and total noradrenaline clearance were similar in the

two groups. In conclusion, peripheral afferent stimulation

below the level of the lesion in spinal cord injury subjects

gives rise to a marked noradrenaline spillover from the

decentralized part of the sympathetic nervous system

suggesting a remaining, but qualitatively altered, neuronal

function. Centrally mediated stimulation induced an

exaggerated response above the level of the lesion.. 51-41-2.

40. Kasdan, M. L.; Johnson, A. L. Reflex sympathetic dystrophy. Occup-

Med. 1998 Jul; 13(3): 521-31; ISSN: 0885-114X.

UNITED-STATES. Reflex sympathetic dystrophy (RSD) is a

diagnosis fraught with clinician disagreement on every facet.

The authors explore the controversy, outline prevailing

opinions, and stress that no patient should be diagnosed with

RSD unless there are strong, objective findings.

41. Kluin, K. J.; Gilman, S.; Lohman, M.; Junck, L. Characteristics of the

dysarthria of multiple system atrophy. Arch-Neurol. 1996 Jun;

53(6): 545-8; ISSN: 0003-9942.

UNITED-STATES. OBJECTIVE: To characterize the dysarthria in

patients with multiple system atrophy (MSA). DESIGN: Motor

speech examinations, consisting of oral motor, oral agility,

and perceptual speech analysis, were performed on 46 patients

with MSA. SETTING: University department of neurology

referral center. RESULTS: All patients had dysarthria with

combinations of hypokinesia, ataxia, or spasticity. Thirty-two

patients had all 3 components, 13 had 2 components, and 1 had

only 1 component. In most patients the hypokinetic components

were the most severe. Hypokinetic components predominated

in 22 patients (48%), whereas ataxic components predominated

in 16 (35%), and spastic components in 5 (11%). In 1 patient

(2%) the hypokinetic and spastic components were equal and

greater than the ataxic components, and in 1 patient (2%) the

hypokinetic and ataxic components were equal and greater than

the spastic components. One patient (2%) had only ataxic

dysarthria. The predominant type of dysarthria corresponded

well to the subtype of MSA. CONCLUSIONS: The finding of a

mixed dysarthria with combinations of hypokinetic, ataxic, and

spastic components is consistent with both the overall

clinical and the neuropathologic changes in MSA. Motor speech

examination can provide helpful information in evaluating

patients who might have MSA.

42. Knappertz, V. A.; Tegeler, C. H.; Hardin, S. J.; McKinney, W. M. Vagus

nerve imaging with ultrasound: anatomic and in vivo validation.

Otolaryngol-Head-Neck-Surg. 1998 Jan; 118(1): 82-5; ISSN:

0194-5998.

UNITED-STATES. To provide the anatomic basis and

demonstrate the reproducibility of ultrasound studies for the

identification of the vagus nerve within its course in the

carotid sheath in the neck, cadaveric and in vivo imaging

studies were conducted. On transverse B-mode images of the

neck, there is a centrally hypoechoic and peripherally

hyperechoic structure between the common carotid artery and

the jugular vein inside the carotid sheath. This structure was

also identified in a fresh, nonpreserved cadaver and was

marked with a hypodermic needle by means of a transdermal

approach. Neck dissection was performed leaving the carotid

sheath intact. B-mode imaging yielded detailed anatomic

information about the structures in the carotid sheath. Further

dissection showed the vagus nerve as the target of the needle.

One hundred consecutive transverse carotid scans were

reviewed, and the characteristic echo patterns of the vagus

nerve were identified in 97 instances. A distinct and

reproducible, round, hypoechoic structure was defined

adjacent to the common carotid artery and jugular vein as the

vagus nerve. On the basis of this study, a new, noninvasive, and

highly reproducible method to locate the vagus nerve in the

carotid sheath is introduced. This may lead to further clinical

application such as presurgical localization or ultrasound-

guided needle studies. Stimulation of the vagus nerve has been

proposed for seizure therapy. The diagnosis of vagus nerve

tumors may be improved.

43. Koike, Y.; Takahashi, A. Autonomic dysfunction in Parkinson's

disease. Eur-Neurol. 1997; 38 Suppl 2: 8-12; ISSN: 0014-3022.

SWITZERLAND. This article is a review of autonomic

dysfunction in idiopathic Parkinson's disease (iPD), as well as

the clinical features of a specific form of PD, i.e. autonomic

failure (AF) with PD, and is based mainly on the results

obtained from our recent studies. Since James Parkinson's

original discription, the definition of autonomic dysfunctions

in iPD and their clinical characteristics have undergone

changes. Autonomic dysfunction is considered to be uncommon

and rarely severe on one hand, while not infrequent but not as

severe as in Shy-Drager syndrome on the other hand. AF with

PD is characterized by severe orthostatic hypotension,

postprandial hypotension, supersensitivity to noradrenaline,

low or absent uptake of m-[123I]iodobenzylguanidine

scintigraphy of the limbs, and preserved arginine vasopressin

response to head-up tilt, suggesting a postganglionic

sympathetic lesion resembling pure AF (PAF). On the other

hand, reduced cortical glucose metabolism in positron

emission tomography study may indicate that AF with PD has

diffuse nervous system lesions resembling diffuse Lewy body

disease.

44. Kribben, A.; Bremer, C.; Fritschka, E.; Koeppen, S.; Ahrens, O.;

Philipp, T. Ambulatory infusion of noradrenaline for long-term

treatment of Shy-Drager syndrome. Kidney-Blood-Press-Res.

1998; 21(1): 70-3; ISSN: 1420-4096.

SWITZERLAND. A 70-year-old female patient with advanced

Shy-Drager syndrome exhibited severe orthostatic

hypotension, low serum catecholamine levels, and autonomic

dysfunction. She was bedridden despite oral medication with

fludrocortisone, etilefrin, dihydroergotamine, L-dopa,

yohimbine, and amezinium methyl sulfate. Only intravenous

application of noradrenaline (30 ng/kg/min) provided complete

mobilization. After implantation of a port-a-cath system,

intravenous noradrenaline treatment could be continued on an

outpatient basis. Over the following 5 years, the patient was

throughout sufficiently mobile and did not show any

significant side effects of this treatment. However, during the

5th year she suffered from nonhemorrhagic brain stem

infarction due to cerebral hypoperfusion after orthostatic

stress in the absence of noradrenaline infusion. We conclude

that ambulatory noradrenaline infusion is a new valuable tool

for long-term treatment of advanced Shy-Drager syndrome.. 0;

51-41-2.

45. Ku, A.; Lachmann, E.; Tunkel, R.; Nagler, W. Upper limb reflex

sympathetic dystrophy associated with occult malignancy.

Arch-Phys-Med-Rehabil. 1996 Jul; 77(7): 726-8; ISSN: 0003-

9993.

UNITED-STATES. Reflex sympathetic dystrophy, characterized

by pain, swelling, vasomotor instability, and trophic changes

in an extremity, has been infrequently described in patients

with occult malignancy. Two cases of reflex sympathetic

dystrophy associated with local tumor involvement are

reported. Both patients had a history of cancer in clinical

remission. Despite aggressive physical therapy measures, the

patients' symptoms persisted. Workup of the first patient

found an apical paravertebral mass in the lung; biopsy revealed

recurrent breast carcinoma. In the second case, workup found

an axillary mass contiguous with the lower brachial plexus.

Biopsy revealed lymphoma, a second primary malignancy. In

both cases, medical treatment of the tumor was instituted,

with consequent improvement of hand and shoulder function.

Both patients required prolonged hospitalization and multiple

procedures that might have been avoided if malignancy had

been suspected. Spontaneous development of reflex

sympathetic dystrophy in patients with a history of cancer

should alert the physician to the possibility of occult

malignancy.

46. Kurvers, H. A.; Hofstra, L.; Jacobs, M. J.; Daemen, M. A.; van, den

Wildenberg FA; Kitslaar, P. J.; Slaaf, D. W.; Reneman, R. S.

Reflex sympathetic dystrophy: does sympathetic dysfunction

originate from peripheral neuropathy? Surgery. 1996 Mar;

119(3): 288-96; ISSN: 0039-6060.

UNITED-STATES. BACKGROUND: Sympathetic dysfunction in

reflex sympathetic dystrophy (RSD) has been purported to

consist of an afferently-induced increase in efferent

sympathetic nerve impulses (somato-sympathetic reflex)

and/or denervation-induced supersensitivity to

catecholamines. In addition, both the central and peripheral

nervous systems have been claimed to be involved. It was the

aim of this study to obtain more insights into these underlying

mechanisms. METHODS: In the affected extremeties of 42

patients with RSD we investigated as indirect measures of

sympathetic (dys)function: (1) skin blood flow and the

vasoconstrictive response to dependency of skin microvessels

by means of laser Doppler flowmetry (distal to the site of

trauma), (2) relative distention of the brachial artery and

changes in relative distention consequent to a cold pressor

test by means of ultrasonic vessel wall tracking (proximal to

the site of trauma), and (3) arterial blood pressures by means

of the Finapres technique. Both provocation tests induce a

sympathetically mediated response. Patients were divided into

three categories according to their perception of skin

temperature in their injured limb (stage I, stationary warmth

sensation; stage II, intermittent warmth and cold sensation; or

stage III, stationary cold sensation). RESULTS: Distal to the

site of trauma, when compared with controls, skin blood flow

was increased at stage I and decreased at stages II and III,

whereas the vasoconstrictive response to dependency was

impaired at all three stages. Proximally, when compared with

controls, relative distention of the brachial artery and its

response to the cold pressor test were decreased at all three

stages. No differences were observed in pulse pressure

between patient groups and controls. CONCLUSIONS: These

results suggest that sympathetic dysfunction in extremities

of patients with RSD distal to the site of trauma consists of

hypersensitivity to catecholamines at stages II and III as a

result of autonomic denervation at stage I, whereas proximal

to the site of trauma sympathetic nerve impulses may be

increased at all three stages.

47. LaFavor, K. M.; Ang, R. Managing autonomic dysreflexia through the

use of clinical practice guidelines. SCI-Nurs. 1997 Sep; 14(3):

83-6; ISSN: 0888-8299.

UNITED-STATES. One of the goals listed in the American

Association of Spinal Cord Injury Nurses (AASCIN) strategic

plan 1993-1998, is to provide strategic vision to advance

professional practice in spinal cord injury (SCI) nursing. In

this quest, along with the development of Standards of Spinal

Cord Injury Nursing Practice, the AASCIN Clinical Practice

Committee is developing Clinical Practice Guidelines to

describe a suggested course of action to address specific

clinical conditions or needs of individuals with spinal cord

injury. The first Clinical Practice Guideline to be completed is

Autonomic Dysreflexia (AD) (Kuric & Hixon, 1996). The purpose

of this article is to review the pathophysiology of AD and then

describe the symptoms, assessment parameters and nursing

interventions suggested in the Clinical Practice Guidelines.

48. Laghi Pasini, F.; Pastorelli, M.; Beermann, U.; de Candia, S.; Gallo,

S.; Blardi, P.; Di Perri, T. Peripheral neuropathy associated

with ischemic vascular disease of the lower limbs. Angiology.

1996 Jun; 47(6): 569-77; ISSN: 0003-3197.

UNITED-STATES. This paper deals with the possible

identification of somatic and autonomic nerve damage in

patients with peripheral obliterative arterial disease (POAD)

at different stages of the disease, with a well-reproducible

technique like electroneurographic evaluation of nerve

conduction. In 64 patients with intermittent claudication, 19

patients with pain at rest, and 7 patients with trophic ulcers,

electroneurographic evaluation of motor (tibial and peroneal)

and sensory (superficial peroneal and sural) nerve conduction

was performed. The median nerve (motor and sensory) was

used as control. A severe impairment of sural and superficial

peroneal nerve velocities was evident in many claudicant

patients and in all patients with pain at rest and trophic

ulcers, with a progression in the conduction abnormalities in

advanced stages of the disease. Motor nerve conduction showed

only minor reductions in patients with claudication and pain at

rest, although some of them did show very poor velocity

values. In 21 patients with intermittent claudication and

sensory nerve abnormalities, the autonomic fibers activity,

evaluated by the skin sympathetic response (SSR) test, was

significantly depressed, thus suggesting an involvement of the

local autonomic system in the ischemic disease. A correlation

exists between the severity of the somatic nerve damage and

the stage of the vascular insufficiency. However, in the group

of claudicant patients, the evidence of similar ischemic

threshold (claudication distance) may be associated with a

marked difference in the amount of somatic nerve damage. The

somatic and autonomic nerve alterations may play a relevant

role in the progression of the disease toward critical limb

ischemia.

49. Lambert, G. W.; Kaye, D. M.; Thompson, J. M.; Turner, A. G.; Cox, H.

S.; Vaz, M.; Jennings, G. L.; Wallin, B. G.; Esler, M. D. Internal

jugular venous spillover of noradrenaline and metabolites and

their association with sympathetic nervous activity. Acta-

Physiol-Scand. 1998 Jun; 163(2): 155-63; ISSN: 0001-6772.

ENGLAND. It is recognized that the brain plays a pivotal role in

the maintenance of blood pressure and the control of

myocardial function. By combining direct sampling of internal

jugular venous blood with a noradrenaline isotope dilution

method, for examining neuronal transmitter release, and

microneurographic nerve recording, we were able to quantify

the release of central nervous system noradrenaline and its

metabolites and investigate their association with efferent

sympathetic nervous outflow in healthy subjects and patients

with pure autonomic failure. To further investigate the

relationship between brain noradrenaline, sympathetic nervous

activity and blood pressure regulation we examined brain

catecholamine turnover, based on the internal jugular venous

overflow of noradrenaline and its principal central nervous

system metabolites, in response to a variety of

pharmacological challenges. A substantial increase was seen

in brain noradrenaline turnover following trimethaphan,

presumably resulting from a compensatory response in

sympathoexcitatory forebrain noradrenergic neurones in the

face of interruption of sympathetic neural traffic and

reduction in arterial blood pressure. In contrast, reduction in

central nervous system noradrenaline turnover accompanied

the blood pressure fall produced by intravenous clonidine

administration, thus representing the blood pressure lowering

action of the drug. Following vasodilatation elicited by

intravenous adrenaline infusion, brain noradrenaline turnover

increased in parallel with elevation in muscle sympathetic

nervous activity. While it is difficult to assess the source of

the noradrenaline and metabolites determined in our studies,

available evidence implicates noradrenergic cell groups of the

posterolateral hypothalamus, amygdala, the A5 region and the

locus coeruleus as being involved in the regulation of

sympathetic outflow and autonomic cardiovascular control.. 0;

0; 0; 4205-90-7; 51-41-2; 51-43-4; 7187-66-8.

50. Langen, K. J.; Ziegler, D.; Weise, F.; Piolot, R.; Boy, C.; Hubinger, A.;

Gries, F. A.; Muller Gartner, H. W. Evaluation of QT interval

length, QT dispersion and myocardial m-iodobenzylguanidine

uptake in insulin-dependent diabetic patients with and without

autonomic neuropathy. Clin-Sci-Colch. 1997 Oct; 93(4): 325-

33; ISSN: 0143-5221.

ENGLAND. 1. An association has been reported between QT

interval abnormalities and cardiovascular autonomic

neuropathy in diabetic patients. The QT interval abnormalities

reflect local inhomogeneities of ventricular recovery time and

may be related to an imbalance in cardiac sympathetic

innervation. Sympathetic innervation of the heart can be

visualized and quantified by single-photon emission-computed

tomography with m-[123I]iodobenzylguanidine. In this study

we evaluated cardiac sympathetic integrity by m-

[123I]iodobenzylguanidine imaging and the relationship

between both QT interval prolongation and QT dispersion from

standard 12-lead ECG variables and m-

[123I]iodobenzylguanidine uptake in insulin-dependent diabetic

patients. 2. Three patient groups were studied, comprising six

healthy control subjects, nine diabetic patients without

cardiovascular autonomic neuropathy (CAN-) and 12 diabetic

patients with cardiovascular neuropathy (CAN+). Resting 12-

lead ECG was recorded for measurement of maximal QT

interval and QT dispersion. The QT interval was heart rate

corrected using Bazett's formula (QTc) and the Karjalainen

approach (QTk). Quantitative measurement (in counts/min per

g) and visual defect pattern of m-[123I]iodobenzylguanidine

uptake were performed using m-[123I]iodobenzylguanidine

single-photo emission-computed tomography. 3. Global

myocardial m-[123I]iodobenzylguanidine uptake was

significantly reduced in both diabetic patient groups compared

with control subjects. The visual defect score of m-

[123I]iodobenzylguanidine uptake was significantly higher in

CAN+ diabetic patients than in control subjects and in CAN-

patients. This score was not significantly different between

control subjects and CAN- patients. QTc interval and QT

dispersion were significantly increased in CAN+ diabetic

patients as compared with control subjects (QTc: 432 +/- 15

ms versus 404 +/- 19 ms, P < 0.05; QT dispersion: 42 +/- 10

versus 28 +/- 8 ms, P < 0.05). QT dispersion was also

significantly longer in CAN- diabetic patients than in control

subjects (41 +/- 9 ms versus 28 +/- 8 ms, P < 0.05). QTc

interval was significantly related to global myocardial m-

[123I]iodobenzylguanidine uptake and defect score in diabetic

patients (r = -0.648, P < 0.01, and r = 0.527, P < 0.05,

respectively). There was no correlation between QT dispersion

and both m-[123I]iodobenzylguanidine uptake measures. 4. In

conclusion, these findings suggest that m-

[123I]iodobenzylguanidine imaging is a valuable tool for the

detection of early alterations in myocardial sympathetic

innervation in long-term diabetic patients without

cardiovascular autonomic neuropathy. Insulin-dependent

diabetic patients with cardiovascular autonomic neuropathy

have a delayed cardiac repolarization and increased variability

of ventricular refractoriness. The cardiac sympathetic nervous

system seems to be one of the determinants of QT interval

lengthening, but does not appear to be involved in dispersion of

ventricular recovery time. It is assumed that QT dispersion is

based on more complex electrophysiological mechanisms

which remain to be elucidated.. 0; 77679-27-7.

51. Lehmann, L. J.; Warfield, C. A.; Bajwa, Z. H. Migraine headache

following stellate ganglion block for reflex sympathetic

dystrophy. Headache. 1996 May; 36(5): 335-7; ISSN: 0017-

8748.

UNITED-STATES. The alteration of extracranial blood flow in

conjunction with clinical signs of autonomic nervous system

dysfunction have led to various explanations concerning the

pathophysiology of migraine headache. Reflex sympathetic

dystrophy, a painful disorder of the sympathetic nervous

system, can be treated by blocking the sympathetic nerves

located in the stellate ganglion, resulting in vasodilation,

ptosis, miosis, and anhydrosis. In theory, these changes could

trigger a migraine headache attack secondary to autonomic

dysfunction reflecting an imbalance between sympathetic and

parasympathetic nervous systems. This may be especially true

in a patient with a previous history of meningitis that may

have resulted in a disorder of cerebrovascular regulation. We

report a 56-year-old man with no previous history of migraine

who developed migraine with aura after a stellate ganglion

block. These episodic headaches occurred with decreasing

frequency and severity for over 6 months, with eventual

complete resolution. This interesting phenomenon has not been

reported in the English literature and may help to better

understand the pathophysiology of migraine.

52. Leira, E. C.; Bendixen, B. H.; Kardon, R. H.; Adams, HP Jr. Brief,

transient Horner's syndrome can be the hallmark of a carotid

artery dissection. Neurology. 1998 Jan; 50(1): 289-90; ISSN:

0028-3878.

UNITED-STATES. We describe a 41-year-old woman in whom

the diagnosis of carotid artery dissection was suspected based

on a recent history of anisocoria and ipsilateral ptosis that

lasted 2 days. She had a normal neurologic examination,

including no clinical evidence of anisocoria or ptosis.

Subsequently, a cocaine test demonstrated pharmacologic

Horner's syndrome. MRI confirmed the carotid dissection. This

patient illustrates that a history of transient pupillary and

eyelid abnormalities can lead to the diagnosis of a carotid

dissection. Specific questioning about transient anisocoria and

ptosis should be considered when a carotid artery dissection

is suspected. Pharmacologic testing may be a useful tool in

such instances.. 0; 50-36-2.

53. Levy, E. I.; Clyde, B.; McLaughlin, M. R.; Jannetta, P. J.

Microvascular decompression of the left lateral medulla

oblongata for severe refractory neurogenic hypertension.

Neurosurgery. 1998 Jul; 43(1): 1-6; discussion 6-9; ISSN:

0148-396X.

UNITED-STATES. OBJECTIVE: To demonstrate that

microvascular decompression of the left medulla oblongata is

a safe and effective modality for treating elevated blood

pressure in patients with severe medically refractory

"essential" hypertension (HTN). METHODS: Twelve patients with

medically intractable HTN with or without autonomic

dysreflexia underwent microvascular decompression of the

left rostral ventrolateral medulla oblongata. Causes such as

pheochromocytoma, carcinoid syndrome, and renal disease

were ruled out before surgery. Indications for surgery included

systolic blood pressures greater than 180 mm Hg refractory to

three or more medications, severe blood pressure lability, or

medically resistant HTN at systolic pressures greater than

160 mm Hg associated with autonomic dysreflexia and/or

magnetic resonance images demonstrating left medullary

compression. The median age and follow-up duration were 51

years and 4.1 years, respectively. RESULTS: Ten of 12 patients

experienced reductions in systolic blood pressure greater than

20 mm Hg. Of these 10 patients, pressure reductions were

temporary (6 mo) in two. Seven of eight patients experienced

improvement in blood pressure lability and/or autonomic

dysreflexia, with five patients showing sustained

improvements. CONCLUSION: Microvascular decompression of

the left rostral ventrolateral medulla oblongata may be an

effective treatment modality for patients suffering from

severe HTN and/or autonomic dysreflexia refractory to

medical management.

54. Lock, G.; Straub, R. H.; Zeuner, M.; Antoniou, E.; Holstege, A.;

Scholmerich, J.; Lang, B. Association of autonomic nervous

dysfunction and esophageal dysmotility in systemic sclerosis.

J-Rheumatol. 1998 Jul; 25(7): 1330-5; ISSN: 0315-162X.

CANADA. OBJECTIVE: The primary event in the pathogenesis of

gastrointestinal involvement in systemic sclerosis (SSc) has

been hypothesized to be an early neural lesion. We investigated

the association of autonomic nervous dysfunction and

esophageal involvement in SSc. METHODS: Thirty-six

consecutive patients with SSc were investigated by

esophageal manometry and autonomic nervous function tests

for cardiovascular and pupillary autonomic dysfunction.

RESULTS: In 27 of 36 patients, esophageal manometry showed

esophageal dysfunction. Twelve patients had either pupillary

(n = 6) or cardiovascular (n = 5) dysfunction or both (n = 1). All

patients with autonomic dysfunction had esophageal

dysfunction. Patients with autonomic dysfunction had

significantly reduced mean distal esophageal contraction

amplitudes compared to patients without autonomic nervous

dysfunction (p < 0.05). The association of autonomic

dysfunction and esophageal dysfunction was significant (p =

0.02). CONCLUSION: Our results support the concept of a role

for neurogenic defects in the development of esophageal

dysfunction in SSc.

55. Longshore, R. C.; O'Brien, D. P.; Johnson, G. C.; Grooters, A. M.;

Kroll, R. A. Dysautonomia in dogs: a retrospective study. J-Vet-

Intern-Med. 1996 May; 10(3): 103-9; ISSN: 0891-6640.

UNITED-STATES. Dysautonomia was diagnosed in 11 young

(median age, 14-months), predominantly medium- to large-

breed dogs from 1988 to 1995. Clinical signs caused by

autonomic dysfunction of the urinary, alimentary, and ocular

systems were most common. Dysuria, mydriasis, absence of

pupillary light reflexes, decreased tear production, dry mucous

membranes, weight loss, and decreased anal tone were present

in over 75% of affected dogs. Ocular pharmacological testing

with a dilute (0.1%) solution of pilocarpine was used to

demonstrate iris sphincter receptor function in all dogs. A

low-dose (0.0375 mg/kg s.c.) bethanechol test and

pharmacological testing with phenylephrine and epinephrine

also demonstrated cholinergic and adrenergic receptor

function in 4 dogs. All dogs died or were euthanized as a

results of autonomic dysfunction. Neuronal depletion, with

associated gliosis and minimal inflammation were noted

histologically in the autonomic ganglia of each dog. The pelvic,

ciliary, celiac, cranial cervical, and cranial and caudal

mesenteric ganglia were affected in all dogs. The cause of

autonomic failure in these dogs was not determined.

56. Lugaresi, E.; Tobler, I.; Gambetti, P.; Montagna, P. The

pathophysiology of fatal familial insomnia. Brain-Pathol. 1998

Jul; 8(3): 521-6; ISSN: 1015-6305.

SWITZERLAND. The key clinical aspects of FFI, i.e.

hypovigilance and attention deficit, inability to generate EEG

sleep patterns, sympathetic hyperactivity and attenuation of

vegetative and hormonal circadian oscillations, are related to

selective atrophy of the anteroventral and mediodorsal

thalamic nuclei. These nuclei constitute the limbic part of the

thalamus interconnecting limbic and paralimbic regions of the

cortex and other subcortical structures in the limbic system

including the hypothalamus. The hypothalamus released from

cortico-limbic control is shifted to a prevalence of activating,

as opposed to deactivating, functions including loss of sleep,

sympathetic hyperactivity and the attendant attenuation of

autonomic circadian and endocrine oscillations. These findings

document that the limbic thalamus has a strategic position in

the central autonomic network running from the limbic

cortical regions to the lower brain stem which regulates the

body's homeostasis in an integrated fashion.

57. Lyons, A. J.; Mills, C. C. Anatomical variants of the cervical

sympathetic chain to be considered during neck dissection. Br-

J-Oral-Maxillofac-Surg. 1998 Jun; 36(3): 180-2; ISSN: 0266-

4356.

SCOTLAND. Horner's syndrome, as a complication of radical

neck dissection, is given little attention in textbooks of head

and neck surgery. To investigate which topographical anatomic

factors, if any, might influence damage to the sympathetic

chain during neck dissection, we undertook a series of 12

cadaveric neck dissections. The axial position of the cervical

sympathetic chain varied. The chain could be clearly delineated

from the carotid sheath except in two cadavers, in which it

was found within the sheath. The presence of cervical ganglia

also varied. We suggest that if the chain is within the sheath,

it may be more likely to be injured during operation.

58. Magnifico, F.; Misra, V. P.; Murray, N. M.; Mathias, C. J. The

sympathetic skin response in peripheral autonomic failure--

evaluation in pure failure, pure cholinergic dysautonomia and

dopamine-beta-hydroxylase deficiency. Clin-Auton-Res. 1998

Jun; 8(3): 133-8; ISSN: 0959-9851.

ENGLAND. The sympathetic skin response (SSR) detects

changes in the electrical potential in the skin in response to

physiological and electrical stimuli and, therefore, may

indicate the integrity of sympathetic cholinergic neural

pathways to sweat glands. This has been evaluated in 21

patients with three forms of peripheral autonomic failure. Of

these, 15 had pure autonomic failure (PAF) without additional

neurological features; investigations indicated both

sympathetic and parasympathetic failure. Four patients had

pure cholinergic dysautonomia (PCD), with clinical and

laboratory features indicating only cholinergic failure. Two

siblings had dopamine-beta-hydroxylase (DBH) deficiency with

only sympathetic adrenergic failure. None was on drugs

affecting cholinergic function. Ten normal individuals were

aged-matched with PAF patients and studied as controls. The

SSR was recorded from the palmar hand and plantar foot

surfaces, using previously described techniques, in response to

physiological (auditory, cough and inspiratory gasp) and

electrical stimuli. Nerve conduction studies excluded an

associated motor or sensory neuropathy. The SSR was present

in all normal individuals, and in both patients with DBH

deficiency who had preserved cholinergic and sudomotor

function, It was absent in all 15 PAF and all four PCD patients

with impaired cholinergic function. Therefore, we conclude

that the SSR reflected sympathetic cholinergic function in

these three different groups with peripheral autonomic

failure.. EC 1.14.17.1.

59. Masur, H.; Schulte Oversohl, U.; Papke, K.; Oberwittler, C.; Vollmer,

J. Involvement of the autonomic nervous system in patients

with syringomyelia--a study with the sympathetic skin

response. Electromyogr-Clin-Neurophysiol. 1996 Jan; 36(1):

43-7; ISSN: 0301-150X.

BELGIUM. In order to determine the function of the autonomic

nervous system in syringomyelia, the sympathetic skin

response (SSR) was performed in 13 patients with

syringomyelia and 20 healthy controls. SSR was recorded from

both palms and soles. In patients with syringomyelia, we found

absent responses, prolonged latencies and reduced amplitudes.

SSRs could be recorded in 15 out of the examined 26 upper

extremities. The latencies were prolonged in 12 of these

cases. In the lower limbs, 11 SSRs could be obtained. In 4 of

these cases the latencies were prolonged. The SSR latencies

recorded from the palms and soles were both significantly

prolonged (p < 0.05) and the amplitudes were reduced (p < 0.05)

as compared to normal persons. Our data strongly suggest

involvement of the autonomic nervous system in syringomyelia

as assessed by the SSR response (in upper and lower

extremities). In our patients, the extent of autonomic

dysfunction was not related to the stage or the duration of

disease.

60. Mathias, C. J. Disorders affecting autonomic function in

parkinsonian patients. Adv-Neurol. 1996; 69: 383-91; ISSN:

0091-3952.

UNITED-STATES. This chapter deals with certain aspects of

autonomic dysfunction in parkinsonian patients. It provides a

classification of autonomic disorders and a brief description

of autonomic manifestations. An outline of autonomic

investigations, including those to diagnose the Shy-Drager

syndrome (multiple system atrophy), is provided. It concludes

with comments on terminology and on whether the

parkinsonian syndromes with autonomic failure comprise

single or multiple entities.

61. Matsumura, H.; Jimbo, Y.; Watanabe, K. Haemodynamic changes in

early phase reflex sympathetic dystrophy. Scand-J-Plast-

Reconstr-Surg-Hand-Surg. 1996 Jun; 30(2): 133-8; ISSN:

0284-4311.

SWEDEN. We studied six patients with early phase reflex

sympathetic dystrophy (RSD). Osteoporotic changes were noted

in the bones of the affected limb. Despite higher temperatures

indicated by thermography, laser speckle image sensing

showed no increase in blood flow on the skin surface. Digital

subtraction angiography showed arteriovenous shunting or

increased density of perfused vessels. Based on these results,

we speculate that in RSD persistent vascular contraction

caused by pain leads to the formation of arteriovenous shunts

in the affected limb with an ischaemic state in the peripheral

subcutaneous tissue which is indicated by pain and swelling.

62. Matthews, J. M.; Wheeler, G. D.; Burnham, R. S.; Malone, L. A.;

Steadward, R. D. The effects of surface anaesthesia on the

autonomic dysreflexia response during functional electrical

stimulation. Spinal-Cord. 1997 Oct; 35(10): 647-51; ISSN:

1362-4393.

ENGLAND. Recently, increases in blood pressure (BP) and

concomitant bradycardia, suggestive of autonomic dysreflexia

(AD), have been documented during functional electrical

stimulation (FES) in individuals with a high spinal cord injury

(SCI). If uncontrolled, this response could preclude the safe

use of FES among such individuals. FES induced pain is partly

related to stimulation of skin nociceptors. Therefore,

measures to reduce skin sensitivity may reduce the risk of AD

during FES. The purpose of this study was to determine if

topical anaesthetic applied over the site of electrical

stimulation could minimize the AD cardiovascular and

hormonal responses to FES in individuals with SCI above the

T6 level. Seven subjects with a SCI above T6 received FES to

the quadriceps muscle of each leg under two conditions on two

different testing days. The two treatment conditions, topical

anaesthetic and placebo creams, were double blinded and

randomized. The cream was administered to an area the size of

the electrode (10 x 10 cm) 1 h prior to stimulation.

Stimulation began at 0 mAmps and increased by 16 mAmps

every 2 min until an intensity of 160 mAmps was achieved. HR

and BP were measured at each stimulation intensity level.

Catecholamines were analyzed three times during the

stimulation protocol (pre, mid and post stimulation

intensities). At the end of the stimulation protocol, FES

induced isometric quadriceps contraction force at 160 mAmps

intensity was measured using a hand held dynamometer. As FES

stimulation intensity increased, significant rises in systolic

and diastolic BP were seen, with a concomitant progressive

drop in HR. The AD response to stimulation was not

significantly different between the topical anaesthetic and

placebo conditions. Serum catecholamine (epinephrine and

norepinephrine) levels tended to rise with increasing FES

intensity levels but did not reach statistical significance. The

two treatment conditions did not significantly affect serum

catecholamine levels or FES-induced quadriceps contraction

force. In summary, FES application to the quadriceps muscle in

high level SCI subjects resulted in significant increases in BP,

decreases in HR (AD-like response), a trend towards elevations

in catecholamine levels, and no difference in quadriceps

muscular strength. However, these responses were unaffected

by the use of topical anaesthetic cream on the skin at the

stimulation site. This suggests that other mechanisms than

skin nociception are operative in FES-induced AD.. 0; 0.

63. Maule, S.; Quadri, R.; Mirante, D.; Pellerito, R. A.; Marucco, E.;

Marinone, C.; Vergani, D.; Chiandussi, L.; Zanone, M. M.

Autonomic nervous dysfunction in systemic lupus

erythematosus (SLE) and rheumatoid arthritis (RA): possible

pathogenic role of autoantibodies to autonomic nervous

structures. Clin-Exp-Immunol. 1997 Dec; 110(3): 423-7; ISSN:

0009-9104.

ENGLAND. Autonomic nervous dysfunction has been previously

reported in SLE, RA and systemic sclerosis, but the

pathogenesis of such a complication is poorly understood. In

the present study, four standard cardiovascular autonomic

function tests were performed in 34 female patients with

connective tissue diseases and in 25 healthy control subjects,

and results expressed as cardiovascular (CV) test scores.

Moreover, in each subject the presence of circulating

complement-fixing autoantibodies directed against

sympathetic and parasympathetic nervous structures,

represented by superior cervical ganglia and vagus nerve,

respectively, was simultaneously assessed by an indirect

immunofluorescent complement-fixation technique, using

rabbit tissue as substrate. None of the patients reported

autonomic symptoms. However, an abnormal CV test score (> or

= 5) was detected in 15% of the patients and in none of the

healthy control subjects, approaching statistical significance

(P = 0.07). No correlation was found between CV test results

and disease duration, type of therapy or presence of

conventional autoantibodies. One or two autoantibodies to

autonomic nervous structures were detected in six patients

(18%) and not in the control subjects (P < 0.05). Values of deep

breathing test were significantly lower in autoantibody-

positive patients compared with those amongst the control

subjects (P < 0.05), and an abnormal CV test score was

significantly associated with the presence of autoantibodies

to autonomic nervous structures (P < 0.05). In conclusion, we

confirm that autonomic nervous function can be impaired in

patients with connective tissue diseases, and suggest that

autoantibodies directed against autonomic nervous system

structures may play a role in the pathogenesis of the

autonomic dysfunction.. 0.

64. McBrien, F.; Spraggs, P. D.; Harcourt, J. P.; Croft, C. B. Abductor

vocal fold palsy in the Shy-Drager syndrome presenting with

snoring and sleep apnoea. J-Laryngol-Otol. 1996 Jul; 110(7):

681-2; ISSN: 0022-2151.

ENGLAND. The case of an elderly male with Shy-Drager

syndrome is presented. His presentation to the Sleep Clinic for

assessment of snoring illustrates bilateral abductor vocal fold

palsy as a rare presentation of the syndrome. This case

emphasizes the need for thorough investigation of all patients

with sleep-related breathing disorders with video and sound

recordings prior to anaesthesia and surgery.

65. McDougall, A. J.; McLeod, J. G. Autonomic neuropathy, I. Clinical

features, investigation, pathophysiology, and treatment. J-

Neurol-Sci. 1996 May; 137(2): 79-88; ISSN: 0022-510X.

NETHERLANDS. Autonomic dysfunction is a common

complication of peripheral neuropathies. It is often of little

clinical importance, but some conditions may cause profound

disturbance of autonomic function, including postural

hypotension, impotence and impairment of heart rate and

bladder and bowel control. Autonomic function can be

evaluated by a number of investigations, some of which can be

performed in a neurophysiology laboratory. Diseases that

primarily affect small nerve fibres or cause acute

demyelination of small myelinated fibres are most likely to

cause autonomic dysfunction. Management includes treating

the underlying cause and symptomatic therapy.. 0.

66. McLachlan, R. S. Vagus nerve stimulation for intractable epilepsy:

a review. J-Clin-Neurophysiol. 1997 Sep; 14(5): 358-68; ISSN:

0736-0258.

UNITED-STATES. Electrical stimulation of the vagus nerve in

the neck by using a programmable stimulator similar to a

cardiac pacemaker is being explored as a treatment for

epilepsy. There is sound rationale based on studies of animal

seizure models for pursuing this treatment modality, and early

clinical trials provide support for efficacy in patients with

intractable epilepsy at least equivalent to that of some of the

new antiepileptic drugs. Safety and tolerability have been

demonstrated in >800 patients worldwide since the first

implant in 1988. Most of these had partial seizures for which

resective epilepsy surgery was not feasible or had failed, but

efficacy of vagal stimulation appears to be the same for both

partial and generalized epilepsy. Specific selection criteria

for this procedure have yet to be established, and further

studies are warranted to determine whether vagal stimulation

becomes an accepted procedure for epilepsy management.

67. Mittal, R.; Khetarpal, R.; Malhotra, R.; Kumar, R. The role of Tc-

99m bone imaging in the management of pain after

complicated total hip replacement. Clin-Nucl-Med. 1997 Sep;

22(9): 593-5; ISSN: 0363-9762.

UNITED-STATES. A case of reflex sympathetic dystrophy (RSD)

after total hip replacement (THR) is reported. It is a very

unusual cause of pain after any arthroplasty procedure. Three-

phase bone imaging diagnosed the condition and sympathetic

blockade was used for confirming and treating the condition.

The role of the bone scan in differentiating RSD from other

causes of pain after THR is highlighted.. 0; 63347-66-0.

68. Mohamed, R.; Forsey, P. R.; Davies, M. K.; Neuberger, J. M. Effect of

liver transplantation on QT interval prolongation and

autonomic dysfunction in end-stage liver disease. Hepatology.

1996 May; 23(5): 1128-34; ISSN: 0270-9139.

UNITED-STATES. Both a prolonged QT interval and disturbance

of autonomic nervous system function are markers of poor

prognosis in patients with diabetes mellitus and alcoholic

liver disease (ALD). We studied the prevalence of abnormal QT

interval and autonomic nervous system dysfunction in 53

consecutive patients with end-stage liver disease before and

after orthotopic liver transplantation (OLT). The maximum QT

interval in any lead (QTmax) was assessed by two independent

observers. The QTmax, corrected for heart rate (QTcmax) was

prolonged in 44 patients (83%), although increased QT

dispersion was not found. There was a significant correlation

between the QTcmax and Child-Pugh score but not with

etiology. Evidence of parasympathetic dysfunction was present

in 41 patients (77%), and sympathetic dysfunction was present

in 20 patients before OLT. Fifty-two patients underwent

transplantation. There was significant improvement in the

QTcmax interval after OLT (P < .001); 32 of the 44 patients

with prolonged QTcmax ( > 440 milliseconds) improved. Repeat

testing was not performed in 7 patients, because they had died

or had not undergone transplantation. Indices of

parasympathetic function improved in 27 patients after OLT,

but no improvement was observed in 8. Improvement in

sympathetic dysfunction was observed in 13 of the 19 patients

tested. There was no association between QTcmax, autonomic

dysfunction, and survival. These results suggest that both

prolonged QTcmax and some tests of autonomic function are

temporary and arise as a consequence of liver dysfunction.

69. Montagna, P.; Cortelli, P.; Avoni, P.; Tinuper, P.; Plazzi, G.;

Gallassi, R.; Portaluppi, F.; Julien, J.; Vital, C.; Delisle, M. B.;

Gambetti, P.; Lugaresi, E. Clinical features of fatal familial

insomnia: phenotypic variability in relation to a polymorphism

at codon 129 of the prion protein gene [see comments]. Brain-

Pathol. 1998 Jul; 8(3): 515-20; ISSN: 1015-6305.

Note: Comment in: Brain Pathol 1998 Jul;8(3):553.

SWITZERLAND. Fatal Familial Insomnia is a hereditary prion

disease characterized by a mutation at codon 178 of the prion

protein gene cosegregating with the methionine polymorphism

at codon 129 of the mutated allele. It is characterized by

disturbances of the wake-sleep cycle, dysautonomia and

somatomotor manifestations (myoclonus, ataxia, dysarthria,

spasticity). PET studies disclose severe thalamic and

additionally cortical hypometabolism. Neuropathology shows

marked neuronal loss and gliosis in the thalamus, especially

the medio-dorsal and anterior-ventral nuclei, olivary

hypertrophy and some spongiosis of the cerebral cortex.

Detailed analysis of 14 cases from 5 unrelated families

showed that patients ran either a short (9.1 +/- 1.1 months) or

a prolonged (30.8 +/- 21.3 months) clinical course according to

whether they were homozygote met/met or heterozygote

met/val at codon 129. Moreover, homozygotes had more

prominent oneiric episodes, insomnia and dysautonomia at

onset, whereas heterozygotes showed ataxia and dysarthria at

onset, earlier sphincter loss and epileptic Grand Mal seizures;

they also displayed more extensive cortical involvement on

PET and at postmortem examination. Our data suggest that the

phenotype expression of Fatal Familial Insomnia is related, at

least partly, to the polymorphism at codon 129 of the prion

protein-gene.. 0.

70. Mueller, P. D.; Korey, W. S. Death by "ecstasy": the serotonin

syndrome? Ann-Emerg-Med. 1998 Sep; 32(3 Pt 1): 377-80;

ISSN: 0196-0644.

UNITED-STATES. "Ecstasy" or 3,4-

methylenedioxymethamphetamine (MDMA) is a popular drug of

abuse and is generally regarded as safe by the lay public. There

are an increasing number of reports of MDMA-induced toxicity

that exhibit features of the serotonin syndrome. We report a

case of severe hyperthermia, altered mental status, and

autonomic dysfunction after a single recreational ingestion of

MDMA.. 0; 0; 42542-10-9.

71. Muramatsu, K.; Kawai, S.; Akino, T.; Sunago, K.; Doi, K. Treatment

of chronic regional pain syndrome using manipulation therapy

and regional anesthesia. J-Trauma. 1998 Jan; 44(1): 189-92;

ISSN: 0022-5282.

UNITED-STATES. In a 4-year period, 17 consecutive patients

with posttraumatic chronic regional pain syndrome were

treated with a new technique, Movelat manipulation therapy.

At average follow-up of 8 months, satisfactory results were

achieved in 15 patients (88%), but 2 patients, 1 with digital

nerve injury and 1 with ulnar nerve injury, did not respond to

the therapy. Factors associated with good clinical response

include chronic regional pain syndrome type I, i.e., dystrophy

produced by a trauma to the hand but not involving a specific

nerve injury, early-stage disease (within 3 months after

trauma), and involvement of the upper limbs. Complications

were rare and mild (pain over the tourniquet site in 3%,

temporary dizziness in 1%). This therapy is simple and safe

and recommended for early treatment of chronic regional pain

syndrome.

72. Nath, R. K.; Mackinnon, S. E.; Stelnicki, E. Reflex sympathetic

dystrophy. The controversy continues. Clin-Plast-Surg. 1996

Jul; 23(3): 435-46; ISSN: 0094-1298.

UNITED-STATES. The chronic pain syndrome encompassed by

the term RSD is poorly understood. The confusion is caused in

large part by frequent misdiagnosis and excessive use of

sympatholytic procedures in inappropriate circumstances.

Recently, pain specialists have redefined the specific criteria

for regional pain syndromes having sympathetic maintaining

factors, emphasizing application of placebo testing in

diagnosis and attention to anatomic principles in

pharmacologic and surgical treatment. The authors believe

that three-phase bone scanning is a valuable adjunct to

clinical judgment in making the proper diagnosis. Current

thinking suggests that sympathetic maintained pain exists but

that it may comprise only approximately 10% of regional pain

cases. Once the appropriate diagnosis is made, classically

described sympatholytic procedures are reasonably used.

Alternative techniques, such as spinal cord stimulation, may

have an important role in refractory cases of sympathetically

maintained pain.

73. Nelson, D. V.; Novy, D. M. Psychological characteristics of reflex

sympathetic dystrophy versus myofascial pain syndromes.

Reg-Anesth. 1996 May; 21(3): 202-8; ISSN: 0146-521X.

UNITED-STATES. BACKGROUND AND OBJECTIVES. Reflex

sympathetic dystrophy (RSD) has sometimes been hypothesized

to derive from a unique psychological predisposition because

of its enigmatic features, as well as the profound behavioral

and emotional characteristics manifested by some patients.

This study compares the psychological characteristics of RSD

and myofascial pain syndrome (MPS) patients to discern the

extent of any aspects unique to RSD. METHODS. The patients

included 58 with RSD and 214 with MPS, all of whom

completed the Minnesota Multiphasic Personality Inventory

(MMPI) as well as a pain questionnaire. Additional pertinent

demographic and clinical characteristics were ascertained.

RESULTS. The only significant demographic group differences

revealed a higher proportion of RSD patients not working (P <

.05) and a higher proportion of RSD patients receiving Workers'

Compensation payments (P < .001). The RSD patients had

shorter duration of pain (P < .01) and were taking fewer pain

medications (P < .01) than the MPS group, but the two groups

had comparable numbers of pain-related surgeries, pain

intensity ratings, perceived ability to cope, and ongoing extent

of involvement in social or recreational activities. A wide

range of functioning was in evidence for both groups on the

MMPI clinical scales, but with duration as a covariate, the RSD

group had significantly (P < .05) lower scores on the

hypochondriasis, depression, hysteria, and psychasthenia

scales and higher scores on the hypomania scale. The duration

covariate was significant (P < .05) only for the infrequency

(rare responses) and depression scales. Duration and certain

scale scores were inversely correlated. CONCLUSIONS. With

only a few exceptions, RSD and MPS patients appear

comparable with respect to a wide range of demographic,

clinical, and psychological functioning indices. A specific

psychological profile, uniquely neurotic or otherwise, has yet

to be demonstrated in terms of any etiologic or maintenance

factors in RSD.

74. Okada, S.; Ishii, K.; Hamada, H.; Tanokuchi, S.; Ichiki, K.; Ota, Z.;

Shimizu, M.; Hiraki, Y. Relationship between cardiac autonomic

neuropathy and diabetic microangiopathies and

macroangiopathy in patients with non-insulin-dependent

diabetes mellitus. J-Int-Med-Res. 1996 Jan; 24(1): 92-8; ISSN:

0300-0605.

ENGLAND. The relationship between cardiac autonomic

neuropathy and diabetic microangiopathies and

macroangiopathy was investigated in 103 patients with non-

insulin-dependent diabetes mellitus. Cardiac autonomic nerve

function was assessed by determining the uptake of

[123I]metaiodobenzyl-guanidine into the myocardium.

Cardioparasympathetic nerve function was assessed by

comparing electrocardiographically the expiratory and

inspiratory respiratory rate (RR) interval ratios, during a

period of deep breathing, and the coefficients of variation of

the RR intervals. Nerve conduction velocity measurements

were used to assess diabetic somatic neuropathy, and

measurement of pulse-wave velocity provided an indication of

the extent of aortic sclerosis. The only correlations between

the parameters of cardiac autonomic neuropathy and

parameters of diabetic microangiopathies and

macroangiopathy were between the expiratory to inspiratory

RR interval ratio and both the conduction velocity of the tibial

nerve and pulse-wave velocity, and between the heart to lung

ratio (cardiac autonomic nerve function) and nephropathy.

These correlations may have occurred by chance; alternatively

they may indicate a difference in the onset mechanisms of

cardiac parasympathetic and sympathetic neuropathies in

diabetics.

75. Okada, S.; Tanokuchi, S.; Ishii, K.; Hamada, H.; Ichiki, K.; Ota, Z.;

Shimizu, M.; Hiraki, Y.; Nagashima, H. Diversity of the

neuropathies in patients with non-insulin-dependent diabetes

mellitus. J-Int-Med-Res. 1996 Jan; 24(1): 122-31; ISSN: 0300-

0605.

ENGLAND. The relationships between cardiac autonomic

neuropathies, diabetic somatic neuropathy, metabolic

parameters, general parameters (such as age and duration of

illness) and diabetic microangiopathy and macroangiopathy

were investigated in 103 patients with non-insulin-dependent

diabetes mellitus (NIDDM). Spearman's correlation coefficients

were calculated for the comparisons of all the parameters of

the neuropathies with all the other parameters. Variables

were selected using a stepwise procedure and multiple

regression analysis was carried out using these variables. The

results of the regression analysis show that diabetic

neuropathy is correlated with vascular parameters including

blood pressure and pulse-wave velocity, as well as with

parameters of sugar and lipid metabolism. The results confirm

the diversity of the clinical characteristics of the

neuropathies in patients with NIDDM and confirm that these

neuropathies do not always occur in parallel.. 0.

76. Okazaki, A.; Iida, T.; Muramatsu, T.; Shirai, T.; Murata, K.;

Takayanagi, T. Bullous pemphigoid associated with Shy-Drager

syndrome. J-Dermatol. 1998 Jul; 25(7): 465-8; ISSN: 0385-

2407.

JAPAN. We report a patient with Shy-Drager syndrome who

developed multiple tense blisters mainly on the extremities.

Circulating anti-basement membrane zone autoantibodies were

detected by the indirect immunofluorescence method.

Immunoblot analysis using normal human epidermal extracts

demonstrated that this patient's serum reacted only with 230

kD bullous pemphigoid antigen (BPAG1). Concerning the

pathoetiology of the association of bullous pemphigoid and

Shy-Drager syndrome, we discuss a sequence similarity

between BPAG1 and dystonin, a candidate gene for dystonia

musculorum.. 0; 0; 0; 0; 0; 0; 0.

77. Oosterhuis, H. J. Acquired blepharoptosis. Clin-Neurol-Neurosurg.

1996 Feb; 98(1): 1-7; ISSN: 0303-8467.

NETHERLANDS. A review is given of the aetiology and possible

treatment of acquired (non-congenital), blepharoptosis, which

is a common but not specific sign of neurological disease. The

diagnostic categories of upper eyelid drooping are scheduled

as (a) pseudo-ptosis due to a local process or overactivity of

eye closure, including blepharospasm, and (b) true ptosis due

to a paresis of the eyelid levators (m. tarsalis superior or m.

levator palpebrae) or to a disinsertion of the m. levator

palpebrae (aponeurotic ptosis). A paresis of the m. tarsalis is

due to a lesion in the central, intermediate or peripheral

neuron of the sympathetic chain and constitutes one of the

components of Horner's syndrome. A paresis of the m. levator

palpebrae may be due to a failure in central innervation, in

oculomotor (n.III) function, in neuromuscular transmission or

to a lesion in the muscle itself.

78. O'Suilleabhain, P.; Low, P. A.; Lennon, V. A. Autonomic dysfunction

in the Lambert-Eaton myasthenic syndrome: serologic and

clinical correlates. Neurology. 1998 Jan; 50(1): 88-93; ISSN:

0028-3878.

UNITED-STATES. Autonomic dysfunction is a recognized

feature of the Lambert-Eaton myasthenic syndrome (LES).

However, the characteristic pattern of dysautonomia has not

been clearly documented and its pathophysiologic basis is not

known. We therefore abstracted autonomic symptomatology

and results of quantitative tests for salivation, and

vasomotor, cardiovagal, and sudomotor reflexes from records

of 30 LES patients. Dry mouth (77%) and impotence (45% of

men) were the most common symptoms. Composite Autonomic

Scoring Scale results were abnormal in 93% of patients, and

autonomic failure was severe in 20%. The frequency of

specific test abnormalities were the following: sudomotor

function, 83%; cardiovagal reflexes, 75%; salivation, 44%; and

adrenergic function, 37%. Although voltage-gated N-type

calcium (Ca2+) channels are implicated in autonomic

transmission, the low frequency of serum antibodies to N-type

Ca2+ channels found in the patients of this study (31%

positive) argues against a pathogenic role in mediating LES-

related dysautonomia. In contrast, 93% of the patients were

seropositive for P/Q-type Ca2+ channel antibodies. A subset of

these antibodies is thought to impair neuromuscular

transmission. Autoantibodies of thyrogastric or glutamic acid

decarboxylase specificity (markers of predisposition to type 1

diabetes mellitus) were found in 45% of patients, and type 1

antineuronal nuclear antibody (or anti-Hu, a marker of

autoimmune neuropathy associated with small-cell lung

carcinoma) was found in 3%. No autoantibody correlated with

autonomic dysfunction severity. Sensorimotor neuropathy was

documented in five patients, and was not significantly

associated with autonomic neuropathy. Autonomic failure was

most severe in older subjects with cancer (p = 0.02, age by

cancer interaction).. 0.

79. Papay, F. A.; Verghese, A.; Stanton Hicks, M.; Zins, J. Complex

regional pain syndrome of the breast in a patient after breast

reduction. Ann-Plast-Surg. 1997 Oct; 39(4): 347-52; ISSN:

0148-7043.

UNITED-STATES. Complex regional pain syndrome (CRPS) is

characterized by devastating pain, swelling, and cutaneous

discoloration that result from vasomotor dysfunction caused

by an abnormally accelerating sympathetic loop reflex after

trauma or surgery. Although in the extremities CRPS is well

documented as reflex sympathetic dystrophy, it only has been

reported anecdotally in the breast after modified radical

mastectomy and never reported after breast reduction. We

report CRPS in the right breast of a 27-year-old woman after

revision breast reduction surgery. The patient had signs of

CRPS and symptoms of pain, swelling, epidermal scaling, and

cutaneous temperature changes lasting more than 1 year.

Liquid crystal thermographic scanning revealed a persistent,

clinically significant hypothermic region in the affected

breast. Intravenous phentolamine temporarily relieved the

symptoms. Subsequent sympathetic blockade of the stellate

ganglion alleviated chronic CRPS symptoms. Surgeons should

be alert that CRPS may need to be considered in the

differential diagnosis of chronic disproportionate pain after

breast surgery. Early identification and treatment will help

alleviate persistent CRPS symptoms and avoid soft-tissue

changes.. 0; 2180-92-9.

80. Parrillo, S. J. Reflex sympathetic dystrophy in children. Pediatr-

Emerg-Care. 1998 Jun; 14(3): 217-20; ISSN: 0749-5161.

UNITED-STATES. Reflex sympathetic dystrophy is becoming

increasingly recognized in the pediatric population, yet there

is very little about the disease in standard pediatric texts and

nothing in the emergency medicine literature. Failure to

diagnose Reflex sympathetic dystrophy in a timely fashion

greatly decreases the likelihood of recovery. The diagnosis is

primarily clinical. This report is intended to increase

emergency physicians' awareness of this painful, disabling

problem.

81. Pieron, M.; Scheen, A. J.; Corhay, J. L.; Radermecker, M. F.;

Lefebvre, P. J. [Bronchial reactivity in diabetic patients].

Reactivite bronchique chez les patients diabetiques. Rev-Mal-

Respir. 1997 Nov; 14(5): 379-85; ISSN: 0761-8425.

FRANCE. The data of the literature concerning bronchial

reactivity in diabetic patients are controversial. Therefore,

we studied the influence of the presence of a diabetic cardiac

autonomic neuropathy (CAN) on the ventilatory parameters

measured during a methacholine-induced bronchoconstriction

test. Ten insulin-dependent diabetic patients without CAN, ten

insulin-dependent diabetic patients with CAN and ten healthy

volunteers, all non-smokers and free of respiratory symptoms,

have undergone a functional respiratory check-up before the

methacholine test. The presence of CAN was classically

studied by the decrease in heart rate changes during three

standardized tests (deep breathing at 6 cycles/min, Valsalva

manoeuver, orthostatism) which all mainly explore the

parasympathetic function. The bronchial response to

methacholine was similar in the healthy subjects and in the

diabetic patients without CAN. However, the fall in forced

expiratory volume in 1 second induced by the highest dose of

methacholine was significantly less marked in the diabetic

subjects with CAN than in the two other groups. These results

suggest that the diabetic autonomic neuropathy also involves

the vagal innervation of the respiratory tract.. 55-92-5.

82. Pilo, L.; Ring, H.; Quinn, N.; Trimble, M. Depression in multiple

system atrophy and in idiopathic Parkinson's disease: a pilot

comparative study. Biol-Psychiatry. 1996 May 1; 39(9): 803-7;

ISSN: 0006-3223.

UNITED-STATES. Multiple system atrophy (MSA) is a disease

causing parkinsonism in which response to levodopa is

classically absent, poor, or transient. Idiopathic Parkinson's

disease (IPD) itself, which responds favorably to levodopa, has

been associated with the development of disease-related

depression. Over and above the clinical and pathological

characteristics of IPD, MSA causes additional, more

widespread, clinical and pathological deficits. We have

compared motor disability and mood in 12 patients with MSA

and 12 with IPD. There was more severe motor disability, but

no clinical evidence of depression among the MSA patients

studied, and their Beck Depression Inventory scores did not

differ significantly from the group with IPD. We conclude that

depression does not appear to be more common in MSA than in

IPD.

83. Pozzessere, G.; Rossi, P.; Valle, E.; Froio, C. P.; Petrucci, A. F.;

Morocutti, C. Autonomic involvement in multiple sclerosis: a

pupillometric study. Clin-Auton-Res. 1997 Dec; 7(6): 315-9;

ISSN: 0959-9851.

ENGLAND. To study pupillary autonomic function in multiple

sclerosis (MS), we examined 36 subjects with low disability,

preserved visual acuity and no recent history (2 years) of optic

neuritis or actual visual complaints. Compared to controls, MS

patients showed a greater dilatator reaction with darkness

and, for the light reflex, a lower amplitude and contraction

rate and a greater recovery of pupillary diameter 5 s after the

stimulus. Within the MS group, no difference was found

comparing patients with or without the following

characteristics: nuclear magnetic resonance imaging evidence

of midbrain lesions; increased visual evoked potential P100

latency; and a previous history of optic neuritis. No correlation

was found between P100 latency, duration of disease and

pupillometric parameters. Our results indicate that in MS

patients there is autonomic dysfunction with a reduction of

parasympathetic tone and a relative increase in sympathetic

dilatator tone to the pupils. We suggest that pupillary

abnormalities could be due to non-specific impairment of the

central pathways subserving pupil functions.

84. Puvi Rajasingham, S.; Smith, G. D.; Akinola, A.; Mathias, C. J.

Abnormal regional blood flow responses during and after

exercise in human sympathetic denervation [see comments]. J-

Physiol-Lond. 1997 Dec 15; 505( Pt 3): 841-9; ISSN: 0022-

3751.

Note: Comment in: J Physiol (Lond) 1997 Dec 15;505 ( Pt

3):549.

ENGLAND. 1. Blood pressure, superior mesenteric artery (SMA)

and skeletal muscle blood flow, cardiac index (CI) and

systemic vascular resistance responses to supine leg exercise

were measured in six age-matched normal subjects (controls)

and in eleven subjects with sympathetic denervation due to

primary autonomic failure (AF). 2. During exercise, blood

pressure rose in controls but fell markedly in AF. After

exercise, blood pressure rapidly returned to baseline in

controls but remained low in AF. During exercise, systemic

vascular resistance fell in controls and AF but tended to fall

further in AF and remained low post exercise. CI increased

similarly in controls and AF. 3. During exercise, SMA blood

flow fell similarly in controls and AF, but the fall initially

was slower in AF; recovery was more rapid post exercise in

controls. SMA vascular resistance tended to rise less and more

slowly in AF and remained elevated post exercise. 4. Forearm

muscle (FM) blood flow and FM vascular resistance did not

change from resting values in controls or AF post exercise.

After exercise, leg muscle (LM) blood flow rose and LM

vascular resistance fell equally in both groups although LM

blood flow remained elevated, 10 min post exercise in AF. 5. In

sympathetically denervated humans, increased blood flow (due

to excessive vasodilatation, lack of sympathetic restraint, or

both) in leg muscle during and after exercise in combination

with impaired splanchnic vasoconstriction in the early stages

of exercise may have contributed to exercise-induced

hypotension.

85. Smith, G. D.; Mathias, C. J. Differences in cardiovascular

responses to supine exercise and to standing after exercise in

two clinical subgroups of Shy-Drager syndrome (multiple

system atrophy). J-Neurol-Neurosurg-Psychiatry. 1996 Sep;

61(3): 297-303; ISSN: 0022-3050.

ENGLAND. BACKGROUND: In chronic autonomic failure of

varying aetiologies, there are differences in the

cardiovascular responses to supine leg exercise and to

standing after exercise. Whether this occurs between the

different subgroups with Shy-Drager syndrome (SDS) is

unknown. METHODS: Fourteen patients with the cerebellar form

(SDS-C) and 11 patients with parkinsonian features (SDS-P)

were studied. RESULTS: Both groups had a similar degree of

autonomic failure and postural hypotension. Their responses

were compared with nine patients with idiopathic Parkinson's

disease (IPD) and 15 normal subjects (controls), all with

normal autonomic function. With supine exercise, blood

pressure and heart rate rose similarly in controls and patients

with IPD and there was no fall in blood pressure on standing

after exercise. In both SDS groups there were abnormal

responses to exercise: blood pressure fell in SDS-C, but did not

fall or rise in SDS-P. Heart rate increased similarly in both

SDS groups, calculated systemic vascular resistance fell

similarly, but cardiac index rose more in SDS-P than SDS-C.

Resting plasma noradrenaline concentrations were subnormal

in both forms of SDS, and did not increase with exercise.

Postural hypotension was enhanced after exercise to the same

extent in SDS-C and SDS-P. CONCLUSIONS: The greater

cardiovascular abnormalities in response to exercise in SDS-C

suggests that cerebellar or brain stem autonomic pathways

are impaired to a greater extent in SDS-C than in SDS-P.

Pooling SDS subgroups, therefore, may obscure

pathophysiological differences to certain stimuli. Clinically

when postural hypotension is being assessed, separation of the

subgroups may not be essential, as they responded similarly.

86. Stotz Potter, E.; Benarroch, E. Removal of GABAergic inhibition in

the mediodorsal nucleus of the rat thalamus leads to increases

in heart rate and blood pressure. Neurosci-Lett. 1998 May 15;

247(2-3): 127-30; ISSN: 0304-3940.

IRELAND. The mediodorsal nucleus of the thalamus (MD) has

connections with central autonomic centers involved in

cardiovascular control and undergoes severe degeneration in

fatal familial insomnia, a human disease characterized by

progressive dysautonomia. Microinjections of the GABAA

antagonist bicuculline methiodide (BMI) into the medial and

central portion of the MD lead to significant, dose-dependent

increases in both heart rate and blood pressure. Similar

injections into surrounding regions elicited little to no change.

The data suggest that the medial and central portion of the MD

plays a role in central cardiovascular regulation. Neurons of

the MD may be under tonic GABAergic inhibition, and disruption

of circuits at this level may underlie dysautonomia in many

neurological diseases.. 0; 0; 40709-69-1; 485-49-4; 56-12-2.

87. Straub, R. H.; Antoniou, E.; Zeuner, M.; Gross, V.; Scholmerich, J.;

Andus, T. Association of autonomic nervous hyperreflexia and

systemic inflammation in patients with Crohn's disease and

ulcerative colitis. J-Neuroimmunol. 1997 Dec; 80(1-2): 149-

57; ISSN: 0165-5728.

NETHERLANDS. The autonomic nervous system modulates

gastrointestinal motility, secretion and mucosal immunity. Its

dysfunction may be of pathogenetic importance in

inflammatory bowel disease (IBD). This study aimed at

investigating the autonomic nervous function in patients with

IBD. Forty-seven patients with IBD, 28 with Crohn's disease

(CD) and 19 with ulcerative colitis (UC), were investigated by

means of 5 cardiovascular and 2 pupillary standardized

autonomic nervous function tests. In CD and UC, cardiovascular

autonomic neuropathy was very rare (0%, 5%), whereas

pupillary autonomic neuropathy was more prevalent (21%,

21%). In contrast to autonomic neuropathy, overall

cardiovascular (CD: 29%, UC: 26%) and pupillary autonomic

hyperreflexia (46%, 37%) were found more often. Patients with

CD and UC demonstrated elevated percentiles in the

respiratory sinus arrhythmia test as compared to controls

(RSA: 82.3 +/- 3.9%, 80.0 +/- 5.9%, controls: 50.0% +/- 1.5%, p

< 0.0001). CD patients with, as compared to patients without,

RSA hyperreflexia had significantly higher CDAIs (p < 0.001),

increased erythrocyte sedimentation rates (p < 0.005) and

more often extraintestinal disease manifestations (p < 0.001).

UC patients with, as compared to patients without, pupillary

latency time hyperreflexia had lower hemoglobin (p < 0.05),

lower albumin (p < 0.01) and increased erythrocyte

sedimentation rates (p < 0.05). Autonomic hyperreflexia was

significantly associated with more severe inflammation and

systemic disease in IBD. Hyperreflexia may be a response to

inflammation or a pathogenetic element that drives mucosal

inflammation.

88. Streeten, D. H.; Scullard, T. F. Excessive gravitational blood

pooling caused by impaired venous tone is the predominant

non-cardiac mechanism of orthostatic intolerance. Clin-Sci-

Colch. 1996 Apr; 90(4): 277-85; ISSN: 0143-5221.

ENGLAND. 1. In a group of 40 patients with orthostatic

intolerance due to hypotension and/or tachycardia, we have

compared the pathogenetic roles of impaired contractility of

the arterioles and the veins by measuring contractile

responsiveness of the arterioles, reflected by increases in

diastolic blood pressure and of the veins reflected by

measurements of reduction in venous diameter during

intravenous noradrenaline infusions. 2. Compared with 27

healthy subjects, patients with diffuse autonomic

insufficiency showed striking supersensitivity in diastolic

blood pressure (six out of eight) and venous constrictive

responses (seven out of eight patients) to noradrenaline,

consistent with impaired arteriolar and venous innervation. 3.

In contrast, the patients with hyperadrenergic orthostatic

hypotension (n = 16) and orthostatic tachycardia (n = 16)

showed diastolic blood pressure responses to noradrenaline

that were almost invariably within the 95% confidence limits

of the changes in normal subjects but supersensitive

constrictive responses of foot veins in 22 of 32 subjects and

subnormal venous responses in two individuals. The rate of

noradrenaline infusion calculated to cause 50% of maximal

venous constriction (the ED50) was significantly lower in the

patients [mean (SEM) 6.8 (1.9) ng/min] than in the normal

subjects [mean (SEM) 23.2 (3.0) ng/min, P < 0.025]. 4. The

finding of significantly supersensitive foot vein constrictive

responses to noradrenaline infusion in the patients of all three

groups and supersensitive blood pressure responses

exclusively in the patients with diffuse autonomic

insufficiency indicates that venous pooling in the legs was the

predominant pathogenetic mechanism of orthostatic

intolerance in all three types of patients studied. 5. Correction

of the orthostatic hypotension and/or tachycardia by external

compression in virtually all patients confirmed this

conclusion.. 51-41-2.

89. Stutts, J. T.; Kasdan, M. L. Psychosocial aspects of hand injuries

and diseases. Occup-Med. 1998 Jul; 13(3): 513-9; ISSN: 0885-

114X.

UNITED-STATES. More than 50% of patients with a diagnosable

psychiatric disorder initially present with somatic symptoms,

which often effectively distract the physician from the true

illness. Moreover, the occupational setting is ripe for the

emergence of psychosocial issues.

90. Tamura, K.; Tsuji, H.; Nishiue, T.; Tokunaga, S.; Yajima, I.; Higashi,

T.; Iwasaka, T. Determinants of ventricular arrhythmias in

hemodialysis patients. Evaluation of the effect of

arrhythmogenic substrate and autonomic imbalance. Am-J-

Nephrol. 1998; 18(4): 280-4; ISSN: 0250-8095.

SWITZERLAND. BACKGROUND/AIMS: In chronic hemodialysis

patients, we evaluated determinants of repetitive ventricular

tachyarrhythmias which included late potentials and heart

rate variability. METHODS: We compared the presence of late

potentials and heart rate variability obtained by ambulatory

electrocardiogram (ECG), findings of echocardiography, and

laboratory data between patients with and those without

ventricular arrhythmias of Lown class 4A or 4B. Ambulatory

ECG was recorded for 24 h from the beginning of hemodialysis.

Heart rate variability was evaluated by the standard deviation

of the normal RR interval (SDNN). RESULTS: Thirty patients

(17%) had ventricular arrhythmias of Lown class 4A or 4B.

They were older than patients without such arrhythmias

(p=0.0021). Left-ventricular wall motion score (2.0+/-3.9 and

0.3+/-1.2, respectively, p < 0.0001) and left-ventricular mass

index (167 +/-59 and 140+/-44 g/m2, respectively, p=0.0053)

were larger in patients with ventricular arrhythmias of Lown

class 4A or 4B than in those without. Stepwise logistic

regression analysis was performed to select variables related

to ventricular arrhythmias of Lown class 4A or 4B from the

following 8 candidate variables; age, sex, presence of

ischemic heart disease, diabetic nephropathy as the primary

renal disease, presence of late potentials, SDNN, left-

ventricular wall motion score and left-ventricular mass index.

Higher left-ventricular wall motion score (p < 0.0001), older

age (p=0.0022) and male sex (p=0.0235) were the variables

associated with ventricular arrhythmias of Lown class 4A or

4B. CONCLUSION: In patients receiving hemodialysis,

predominantly with chronic glomerulonephritis, ventricular

arrhythmias of Lown class 4A or 4B were not associated with

arrhythmogenic substrate revealed by late potentials or

autonomic dysfunction assessed by heart rate variability.

Left-ventricular wall motion abnormalities, age and sex were

significant factors.

91. Tentolouris, N.; Katsilambros, N.; Papazachos, G.; Papadogiannis,

D.; Linos, A.; Stamboulis, E.; Papageorgiou, K. Corrected QT

interval in relation to the severity of diabetic autonomic

neuropathy. Eur-J-Clin-Invest. 1997 Dec; 27(12): 1049-54;

ISSN: 0014-2972.

ENGLAND. The aim of this study was to investigate to what

extent the existence of objective signs of diabetic autonomic

neuropathy affects the corrected QT interval (QTc) in diabetic

subjects. A total of 105 diabetic subjects (type 1, n = 53; type

2, n = 52) as well as 40 matched (by age and sex) control

subjects were studied. All subjects underwent the battery of

five Ewing tests. Autonomic neuropathy was diagnosed if two

of the five tests were abnormal. In addition, the result of each

test was considered as normal (grade = 0), borderline (grade =

1) or abnormal (grade = 2), and on the basis of the sum of the

scores we calculated a total score for autonomic neuropathy.

The QTc interval was measured at rest, and a value > 440 ms

was considered abnormal. The QTc interval was significantly

more prolonged in diabetic persons with autonomic neuropathy

than in those without neutopathy and in control subjects:

408.4 +/- 24.2 ms vs. 394.6 +/- 27.9 ms and 393.6 +/- 25.5 ms

respectively (P = 0.001). Furthermore, multivariate analysis

controlling for age, sex, systolic and diastolic blood pressure,

body mass index (BMI), waist-hip ratio (WHR), smoking, type

and duration of diabetes, type of treatment, HBA1c and total

score of autonomic neuropathy eliminated the role of all these

factors as potential confounders except for the total score of

autonomic neuropathy, which was found to affect QTc interval

independently and significantly (P = 0.012). In summary, the

present study confirmed the well-known relation between

autonomic neuropathy and QTc interval; in addition, it showed

that QTc prolongation is associated with major degrees of

autonomic neuropathy.

92. Testa, D.; Filippini, G.; Farinotti, M.; Palazzini, E.; Caraceni, T.

Survival in multiple system atrophy: a study of prognostic

factors in 59 cases. J-Neurol. 1996 May; 243(5): 401-4; ISSN:

0340-5354.

GERMANY. The various clinical features of multiple system

atrophy (MSA) make the diagnosis of the disease difficult,

especially in its early stages, when signs of differentiated

neuroanatomical system involvement have not yet appeared.

Mortality studies may be affected by the variability of the

diagnostic criteria and selection bias. We used strict clinical

and MRI criteria to diagnose MSA in 59 patients. Patients with

parkinsonian and cerebellar onset were compared. Median

survival time from the onset of the first motor symptom was

7.5 years. Our results indicated a trend (P = 0.09) for the

Northwestern University Disability Scale score to correlate

with mortality, but we failed to find other characteristics

identifying subgroups or predictors for survival.

93. Thimineur, M. A.; Saberski, L. Complex regional pain syndrome

type I (RSD) or peripheral mononeuropathy? A discussion of

three cases. Clin-J-Pain. 1996 Jun; 12(2): 145-50; ISSN: 0749-

8047.

UNITED-STATES. OBJECTIVE: Peripheral nerve pathology

commonly results in symptoms that suggest a diagnosis of

complex regional pain syndrome (CRPS) type I (RSD). We briefly

review common symptoms of peripheral nerve pathology

(referred pain, hyperpathia, and autonomic changes) and

present three illustrative cases of peripheral nerve injury

misdiagnosed and treated as RSD. The nonspecificity of current

taxonomy regarding CRPS as it relates to the three cases is

emphasized. DESIGN: The study is case series. SETTING: All

three of the cases were diagnosed and treated for their painful

symptoms at a university hospital clinic that provides

multispecialty evaluations for painful conditions. PATIENTS:

The three patients all had work-related injuries resulting in

pain, hyperpathia, and autonomic changes in one of their upper

extremities. Their injuries were representative of common

peripheral nerve lesions, one being a neuroma, one an irritative

lesion, and one an entrapment. RESULTS AND CONCLUSIONS: The

clinical entity of CRPS quite apparently encompasses

symptomatology caused by peripheral nerve entrapment,

irritative lesions, and neuroma. As such, its use as a

diagnostic end point may overlook these treatable conditions.

As illustrated in these cases, peripheral nerve pathology may

prove a diagnostic challenge and alternative techniques of

investigation other than electrophysiologic studies are often

helpful.

94. Tiller, W. A.; McCraty, R.; Atkinson, M. Cardiac coherence: a new,

noninvasive measure of autonomic nervous system order.

Altern-Ther-Health-Med. 1996 Jan; 2(1): 52-65; ISSN: 1078-

6791.

UNITED-STATES. Although cardiac sympathovagal regulation

has been studied during stress using power spectral density

analysis of heart rate variability, little is known about its

regulation during emotional states. Using heart rate

variability measurements, we studied autonomic balance in 20

subjects trained in a mental and emotional self-management

technique called Freeze-Frame. The study was conducted in

two environments: under controlled laboratory conditions, and

under real-life stressful conditions in the workplace. Power

spectral density plots of R-R intervals obtained from

electrocardiogram recordings were divided into three regions:

low frequency (predominantly sympathetic activity),

midfrequency, and high frequency (parasympathetic activity).

Measurements were taken for a 5-minute baseline period,

followed by a 5-minute period of positive emotional

expression. Three unique conditions of autonomic nervous

system order can be clearly discriminated in the data: (1)

normal heart function mode, (2) entrainment mode, and (3)

internal coherence mode. The internal coherence mode is new

to the electrophysiology literature. We provide supporting data

for modes 2 and 3 and show that a group of 20 subjects trained

in this technique can enter and maintain these states at will.

We found that, when one is in the entrainment mode, other

physiological systems lock to the entrainment frequency,

which is approximately 0.1 Hz. The results suggest that

emotional experiences play a role in determining

sympathovagal balance independent of heart rate and

respiration and further suggest that positive emotions lead to

alterations in heart rate variability that may be beneficial in

the treatment of hypertension and reduce the likelihood of

sudden death in patients with congestive heart failure and

coronary artery disease.

95. Toyry, J. P.; Niskanen, L. K.; Lansimies, E. A.; Partanen, K. P.;

Uusitupa, M. I. Autonomic neuropathy predicts the development

of stroke in patients with non-insulin-dependent diabetes

mellitus. Stroke. 1996 Aug; 27(8): 1316-8; ISSN: 0039-2499.

UNITED-STATES. BACKGROUND AND PURPOSE: Our aim was to

determine the predictive factors for stroke in patients with

non-insulin-dependent diabetes mellitus (NIDDM). METHODS: We

studied 133 patients with NIDDM at the time of diagnosis and

5 and 10 years later. RESULTS: The number of new fatal or

nonfatal strokes was 19 (14.7%; 14 after 5-year examination).

High initial fasting blood glucose (odds ratio [OR], 1.2; 95%

confidence interval [CI], 1.04 to 1.4) and the use of beta-

blocking agents (OR, 6.7; 95% CI, 2.1 to 21.5) at baseline and

the presence of parasympathetic neuropathy (OR, 6.7; 95% CI,

1.5 to 29.9), or sympathetic autonomic nervous dysfunction

(OR, 1.1; 95% CI, 1.01 to 1.2), hypertriglyceridemia (OR, 5.7;

95% CI, 1.1 to 31.0), or use of beta-blocking agents (OR, 6.4;

95% CI, 1.3 to 31.2), and high fasting plasma glucose (OR, 1.2;

95% CI, 1.0 to 1.5) determined at 5-year examination predicted

the development of stroke. CONCLUSIONS: Autonomic

neuropathy is an independent risk factor for stroke in NIDDM..

0; 0; 57-88-5.

96. Toyry, J. P.; Niskanen, L. K.; Mantysaari, M. J.; Lansimies, E. A.;

Uusitupa, M. I. Occurrence, predictors, and clinical significance

of autonomic neuropathy in NIDDM. Ten-year follow-up from

the diagnosis. Diabetes. 1996 Mar; 45(3): 308-15; ISSN: 0012-

1797.

UNITED-STATES. Little is known about the occurrence and

predictive factors of autonomic neuropathy and its

relationship to cardiovascular mortality in NIDDM patients,

and no long-term follow-up studies including nondiabetic

control subjects are available. A total of 133 patients with

newly diagnosed NIDDM (70 men) and 144 control subjects (62

men) were examined at baseline and after 5 and 10 years of

follow-up. Deep-breathing tests (baseline, 5-year, and 10-

year) and active orthostatic tests (5- and 10-year) were

performed. Criteria for autonomic neuropathy were

parasympathetic (expiration-to-inspiration ratio </- 1.10),

sympathetic (systolic blood pressure decrease >/- 30 mmHg in

the orthostatic test), and combined autonomic neuropathy

(parasympathetic with sympathetic neuropathy). The frequency

of parasympathetic neuropathy (NIDDM patients versus control

subjects) was 4.9 vs. 2.2% (P = 0.224) at baseline, 19.6 vs.

8.5% (P = 0.017) at 5 years, and 65.0 vs. 28.0% (P < 0.001) at

10 years of follow-up. The frequency of sympathetic

neuropathy was 6.8 vs. 5.6% (P = 0.709) at 5 years and 24.4 vs.

9.0% (P = 0.003) at 10 years of follow- up. These figures for

combined autonomic neuropathy were 2.1 vs. 1.8% (P = 0.869)

at 5 years and 15.2 vs. 4.2% (P = 0.007) at 10 years of follow-

up. NIDDM patients with parasympathetic neuropathy at the

10-year examination showed worse glycemic control and

higher insulin values than those without parasympathetic

neuropathy. Furthermore, in our subjects, women were more

prone to have parasympathetic neuropathy than men.

Parasympathetic neuropathy at baseline was more frequent in

those who died from a cardiovascular cause than those who did

not (13 vs. 3%, P = 0.045). Similarly, sympathetic autonomic

nervous dysfunction at the 5-year examination predicted the

10-year cardiovascular mortality. In conclusion, the frequency

of autonomic neuropathy in NIDDM patients increases sharply

with time. The development of autonomic neuropathy is

connected with poor glycemic control. Interestingly, a high

insulin level seems to have a predictive role in the

development of parasympathetic autonomic neuropathy

irrespective of obesity and glycemia.. 0; 11061-68-0.

97. Toyry, J. P.; Partanen, J. V.; Niskanen, L. K.; Lansimies, E. A.;

Uusitupa, M. I. Divergent development of autonomic and

peripheral somatic neuropathies in NIDDM. Diabetologia. 1997

Aug; 40(8): 953-8; ISSN: 0012-186X.

GERMANY. There is no information on the mutual occurrence

and the development of autonomic and peripheral somatic

neuropathies based on long-term follow-up of patients with

non-insulin-dependent diabetes mellitus (NIDDM). We

investigated the relation between the changes in autonomic

function values and electrodiagnostic values, and the relation

between the occurrence of autonomic neuropathy and

peripheral somatic polyneuropathy in a group of patients with

newly diagnosed NIDDM (n = 133, aged 45-65 years) at baseline

and 5 and 10 years later. Parasympathetic autonomic

neuropathy was diagnosed on the basis of heart rate

variability during deep-breathing and sympathetic autonomic

neuropathy on the basis of fall in systolic blood pressure

while changing from supine to standing. Polyneuropathy was

diagnosed on the basis of both clinical criteria and

electrodiagnostic studies (nerve conduction velocity and

response-amplitude values). In 10 years 36 patients died,

mainly from cardiovascular causes. Altogether 78 patients

completed the study. At 10 years, parasympathetic autonomic

neuropathy was diagnosed in 61.3% of those with

polyneuropathy and 66.7% of those without. Likewise, the

frequency of sympathetic autonomic neuropathy was similar in

those with polyneuropathy (21.9%) and those without (26.5%).

The respective figures for combined (both parasympathetic and

sympathetic) autonomic neuropathy were 10.0% and 18.8%. The

worsening of parasympathetic and sympathetic autonomic

function values was not related to the worsening in

electrodiagnostic results with time. In conclusion, the

development of autonomic and peripheral somatic neuropathies

was divergent in patients with NIDDM suggesting different

pathophysiological processes for these neuropathies.

98. van, der Laan L.; Goris, R. J. Reflex sympathetic dystrophy after a

burn injury. Burns. 1996 Jun; 22(4): 303-6; ISSN: 0305-4179.

ENGLAND. Reflex sympathetic dystrophy (RSD) is a disease

that can appear after minor trauma or operation to an

extremity. The injury may vary from a simple contusion to a

fracture. The prevalence of burns as a cause of RSD, within a

population of 829 patients with RSD, was studied

retrospectively. Prospectively, we documented the medical

history, signs and symptoms of all patients with RSD, seen by

our department during the period from January 1984 to 31

December 1994. Four patients had developed RSD after a burn

injury, resulting in a prevalence of 0.5 per cent. Though the

clinical signs of early RSD are similar to those of a (thermal)

burn, alertness to recognize inflammatory signs, in

combination with the increase in complaints after exercise, is

necessary for early diagnosis and treatment of the

complicating RSD.

99. van, der Laan L.; Kapitein, P.; Verhofstad, A.; Hendriks, T.; Goris, R.

J. Clinical signs and symptoms of acute reflex sympathetic

dystrophy in one hindlimb of the rat, induced by infusion of a

free-radical donor. Acta-Orthop-Belg. 1998 Jun; 64(2): 210-7;

ISSN: 0001-6462.

BELGIUM. The acute phase of reflex sympathetic dystrophy

(RSD) is characterized by the classical signs and symptoms of

inflammation (rubor, calor, dolor, tumor and impaired

function). As free radicals are involved in acute inflammation,

we studied the effects of free radicals in an animal model,

especially as to signs and symptoms found in acute RSD.

Awake rats were given continuous intra-arterial infusion (1

ml/h) in the left hindlimb, with saline (n = 6) or the free-

radical donor tert-butylhydroperoxide (tert-BuOOH, 25 mM, n =

6). During a 24-h infusion period the skin temperature, volume,

skin color, function and pain reactions of the paws were

observed. After 24 h the rats were killed and both

gastrocnemius muscles were histologically analyzed. Infusion

with tert-BuOOH induced in the left paw an increased skin

temperature, increased volume, redness of the plantar skin,

impaired function and increased pain sensation, while these

acute RSD signs and symptoms were absent in the saline

infused animals. The alterations in pain sensation

(spontaneous, mechanical and thermal pain) were similar to

findings in the neuropathic animal model. The gastrocnemius

muscles of the saline infused rats and the contralateral

gastrocnemius muscle of the tert-BuOOH infused rats showed

no histological tissue damage. In the left gastrocnemius

muscle free-radical-related damage was visible. Induction of

free-radical formation in one hindlimb of awake rats mimics

the acute signs and symptoms of acute RSD, with alterations

in pain sensation as found in the classical neuropathic animal

model of RSD, as well as in acute RSD patients.. 0; 0; 0; 75-

91-2; 7647-14-5.

100. Verrotti, A.; Chiarelli, F.; Morgese, G. Autonomic dysfunction in

newly diagnosed insulin-dependent diabetes mellitus children.

Pediatr-Neurol. 1996 Jan; 14(1): 49-52; ISSN: 0887-8994.

UNITED-STATES. In order to evaluate the presence of

electrophysiologic signs of autonomic dysfunction (AD) in

newly diagnosed diabetic children, cardiovascular reflex tests

were performed in 55 (30 female, 25 male) newly diagnosed

insulin-dependent diabetes mellitus (IDDM) patients aged

10.3-20.7 years (mean +/- S.D.: 15.2 +/- 5.6). Ten (18.2%)

diabetic children had cardiovascular AD, defined as abnormal

results in 2 of 5 tests. Autonomic function tests were

assessed at entry and after 12, 24, and 36 months of the study.

All diabetic children received human insulin and followed an

intensive insulin treatment (3 or 4 injections per day),

associated with a teaching program of self-management of the

disease. In the 3 years of follow-up, all children improved the

quality of metabolic control (glycosylated hemoglobin, HbA1c:

10.3 +/- 1.1% versus 7.7 +/- 0.9; P < .01) and manifested no

significant difference between baseline and follow-up values

of autonomic function tests which remained unchanged in spite

of this improvement. Cardiovascular autonomic dysfunction

can be present in newly diagnosed IDDM children and it seems

to be stable in children who follow an intensive insulin

injection therapy.. 0; 11061-68-0.

101. Vetrugno, R.; Liguori, R.; Cevoli, S.; Salvi, F.; Montagna, P. Adie's

tonic pupil as a manifestation of Sjogren's syndrome. Ital-J-

Neurol-Sci. 1997 Oct; 18(5): 293-5; ISSN: 0392-0461.

ITALY. We here report two cases of Adie's tonic pupil,

associated with clinical sensory polyneuropathy and Sjogren's

syndrome, in one of whom it actually heralded the onset of the

syndrome. Electrophysiology studies indicated absent H

reflexes but normal peripheral nerve conductions, thus

suggesting an involvement of the dorsal roots or spinal

ganglion that would be in line with previously published

reports of dorsal ganglionitis as the primary neuropathological

lesion in Sjogren's syndrome. We suggest that all cases of

tonic pupils should be screened for polyneuropathy and

Sjogren's syndrome.. 0.

102. Wakabayashi, K.; Takahashi, H. Neuropathology of autonomic

nervous system in Parkinson's disease. Eur-Neurol. 1997; 38

Suppl 2: 2-7; ISSN: 0014-3022.

SWITZERLAND. Lewy body formation has been considered to be

a marker for neuronal degeneration, because postmortem

studies of Parkinson's disease (PD) patients have shown loss

of neurons in the predilection sites for Lewy bodies. We

systemically studied the autonomic nervous system in

patients with PD. Lewy bodies were widely distributed in the

hypothalamus, sympathetic system (intermediolateral nucleus

of the thoracic cord and sympathetic ganglia) and

parasympathetic system (dorsal vagal and sacral

parasympathetic nuclei). The number of neurons in the

intermediolateral nucleus was significantly reduced.

Furthermore, Lewy bodies were also found in the enteric

nervous system of the alimentary tract, cardiac plexus, pelvic

plexus and adrenal medulla. These findings indicate that both

central and peripheral autonomic nervous systems are involved

in the disease process in PD.

103. Weck, M.; Tank, J.; Baevski, R. M.; Molle, A.; Matthies, K.; Ploewka,

K. Impaired activation of the baroreflex loop as early sign of

sympathetic damage in diabetics with normal heart rate

variability at rest. Acta-Med-Austriaca. 1997; 24(5): 175-9;

ISSN: 0303-8173.

AUSTRIA. The objective of the study was to define the

impairment of sympathovagal balance in patients with

diabetes mellitus (DM) and coronary heart disease (CHD)

compared to healthy controls (HC) showing similar heart rate

variability (HRV) at supine rest. 88 DM (41 m, 47 f; age 62 +/-

1 years; BMI 27.1 +/- 1.5 kg/m2; HbA1c 7.9 +/- 0.4%), 49 CHD

(27 m, 22 f; age 62 +/- 1 years; BMI 27.1 +/- 1.6 kg/m2; HbA1c

5.2 +/- 0.1%) and 16 HC (8 m, 8 f; age 59 +/- 1 years; BMI 26.4

+/- 0.5 kg/m2; HbA1c 5.0 +/- 0.1%) were investigated. Time

series of heart period duration (HPD) were obtained during 2

min deep breathing (6/min), 5 min of supine rest and for 5 min

at upright position using a RR memory device (BHL 6000,

Baumann-Haldi Switzerland, modified ECG lead, 1 kHz). Mean

HPD, coefficient of variation (CV), total power (TP) and

integral power in the HF (0.15 to 0.5 Hz), MF (0.05 to 0.15 Hz)

and LF (0.015 to 0.05 Hz) frequency bands as well as (MF-

HF)/(MF + HF) as spectral index were calculated. As to be

expected we found significantly lower values of CV, TP and

HPD in DM compared to HC. The CV of HRV did not differ

significantly between DM and CHD but TP and HPD of CHD

patients were significantly higher in comparison to DM.

Therefore, the deterioration of HRV was most pronounced in

the DM group. For further analysis we calculated data of

subjects with CV's in the upper quartile (> or = 3.52) of the CV

at supine rest. The aim of this procedure was to compare

subjects with similar high HRV at supine rest. With this

method we obtained from all subjects 12 HC, 11 DM and 12

CHD. These DM had a significant decrease of CV, TP and the

integral power at the HF frequency band during active

orthostasis compared to HC and CHD. The spectral index

increased significantly during standing in HC and CHD but was

unchanged in DM. These changes were accompanied by a nearly

similar increase of HRV during deep breathing. In conclusion,

DM with normal reaction to deep breathing did not activate the

sympathetic baroreflex loop during active orthostasis. This

could be an early sign of sympathetic dysfunction in DM with

normal HRV at supine rest.

104. Yang, T. F.; Chan, R. C.; Liao, S. F.; Chuang, T. Y.; Liu, T. J.

Electrophysiologic evaluation of autonomic function in

cerebral palsy. Am-J-Phys-Med-Rehabil. 1997 Nov; 76(6): 458-

61; ISSN: 0894-9115.

UNITED-STATES. The presence of clinical autonomic

dysfunction in patients with neurologic diseases, such as

multiple sclerosis, Parkinson's disease, and cerebrovascular

accident, has become increasingly recognized in the past

decade. Very few autonomic tests have been done on pediatric

patients thus far. The purpose of this study was to investigate

the autonomic function in patients with cerebral palsy using

two noninvasive tests: sympathetic skin response (SSR) and R-

R interval variation (RRIV). Twenty-four patients with

cerebral palsy and 24 control subjects between the ages of 4

and 12 yr were enrolled in this study. There was no significant

difference of mean latency, amplitude, or amplitude ratio of

SSR between the two groups under electric stimulus, startling

stimulus, and deep breathing conditions. No significant

difference in frequency of absent response and asymmetric

response was also noted. Mean heart rate under relaxed sitting

condition was significantly higher in the study group.

Significant negative correlation between heart rate and age

was noted in the control group but was not present in the

study group. Also, there was no statistical difference of mean

RRIV between the two groups. No objective evidence of

autonomic disturbance in patients with cerebral palsy was

found in this study.

105. Zuurmond, W. W.; Langendijk, P. N.; Bezemer, P. D.; Brink, H. E.; de

Lange, J. J.; van loenen, A. C. Treatment of acute reflex

sympathetic dystrophy with DMSO 50% in a fatty cream. Acta-

Anaesthesiol-Scand. 1996 Mar; 40(3): 364-7; ISSN: 0001-5172.

DENMARK. Acute Reflex Sympathetic Dystrophy (acute RSD)

was defined using a reproducible classification. Elevated

temperature of the affected extremity ("calor"), measured by

the dorsal side of the observer's hand and mentioned by the

patient, pain ("dolor") measured by the Visual Analogue Scale

(VAS), redness ("rubor"), edema ("tumor") and limited active

range of motion ("functio laesa"), all contributed to the

classification system. Patients scoring 4 to 5 positive

symptoms were considered to have acute RSD. A prospective,

randomized and double blind study was performed in 32

patients, all suffering from acute RSD. In all of these patients

the primary injury was the result of a previous accident. One

patient was taken out of the study because of his surgery. The

study involved treatment with a fatty cream with 50%

dimethyl sulfoxide (DMSO, group A), or without DMSO (placebo,

group B), both for 2 months. All patients received

physiotherapy applied within pain limits. Application of the

creams resulted in both groups in an improvement of RSD-

scores and VAS-scores after 2 months. However, the

improvement of the RSD score in patients of group A (DMSO-

group) was significantly (P < 0.01) better compared to group B.

The results suggest a certain activity of DMSO 50% cream in

patients suffering from RSD and is, therefore, recommendable..

0; 0; 0; 0; 67-68-5.