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