The reading requirements necessary for preparation of Professor Frederick Carrick's lecture on the Brain and its environment are taken from Kandel, Schwartz and Jessel, Principles of Neural Science, IIIrd edition.

Pages 2-16

Pages 275-282

Pages 287-293

Pages 296-308

Pages 359-365

Pages 501-510

Pages 533-546

Pages 610-624

 

The following references were utilized By Professor Frederick Carrick

in preparation of his lecture on the Brain and it's environment.

 

1. Adle Biassette, H.; Bourhy, H.; Gisselbrecht, M.; Chretien, F.;

Wingertsmann, L.; Baudrimont, M.; Rotivel, Y.; Godeau, B.; Gray,

F. Rabies encephalitis in a patient with AIDS: a

clinicopathological study. Acta-Neuropathol-Berl. 1996 Oct;

92(4): 415-20; ISSN: 0001-6322.

GERMANY. A 46-year-old man was bitten by a dog in Mali;

anti-rabies vaccination was incomplete. Three months later he

was admitted to hospital with fever and diarrhea. Human

immunodeficiency virus (HIV) serology was positive and CD4

count was 70/mm3. His status worsened rapidly with

confusion hydrophobia and hypersialorrhea. Despite anti-rabies

serotherapy and vaccination, he died suddenly 12 days after

admission. Immunofluorescence on cerebral tissue samples

established rabies encephalitis. Neuropathology showed mild

encephalitis with occasional Babes nodules and rare

perivascular mononuclear cuffs. Intraneuronal Negri inclusion

bodies were remarkably diffuse and abundant. They were

clearly demonstrated by immunocytochemistry and electron

microscopy. Apoptotic neurons were identified in the brain

stem and hippocampus in the vicinity of inflammatory foci. In

contrast, apoptosis could not be demonstrated in non-

inflammatory areas, even where Negri bodies were numerous.

There was no associated HIV encephalitis or opportunistic

infection. The occurrence of rabies encephalitis in AIDS

represents a random association, but is probably not

exceptional as rabies is endemic in many countries and the

AIDS epidemic is spreading worldwide. In this case, although

the incubation duration and clinical presentation were

comparable to those in classical rabies the T-cell-mediated

immunosuppression may account for the weak inflammatory

reaction and unusually abundant viral multiplication. This

observation confirms that all those at risk for rabies,

particularly immunocompromised patients, should receive

complete anti-rabies treatment including vaccines and

specific immunoglobulins, as soon as possible after infection..

0.

2. al Hussain, S.; al Jomard, R. Morphology of neurons in the anterior

hypothalamic area and supraoptic hypothalamic nucleus of the

adult human brain. Ital-J-Neurol-Sci. 1996 Aug; 17(4): 261-6;

ISSN: 0392-0461.

ITALY. Morphological features of neuronal cell types in the

anterior hypothalamic area (AHA) and supraoptic hypothalamic

nucleus (SON) of the adult human brain were analysed in Golgi

impregnated preparations. Four neuronal cell types were

described for the first time in these human nuclei. Type I

neurons were found in both the AHA and SON, while the other

three cell types (types II-IV) were found only in the SON. Type

I neurons had elongated, triangular or multipolar somata,

which emitted 2-5 sparsely branching primary dendrites with

a moderate number of fine spines. Also many of type I neurons

in the AHA had thin dendritic side-branches. Type II neurons

had round or fusiform somata, and two occasionally branching

primary dendrites. Type III neurons were multipolar neurons

with 3-5 densely spined and sparsely branching dendrites.

Their axons had collaterals. Type IV neurons had very small

ovoid somata with one smooth and unbranched primary

dendrite. The neurons in the human AHA and SON were similar

to those observed in the same areas in other mammalian

species, except for the very small neurons in the SON and the

thin dendritic side-branches of type I neurons in the AHA, that

had not been previously described.

3. Alonso, J.; Rovira, A.; Capellades, J.; Ocana, I.; Rio, J.; Wicklow, K.;

Sauter, R.; Gili, J. [Cerebral proton spectroscopy of people

infected with the human immunodeficiency virus].

Espectroscopia de proton en el cerebro de personas infectadas

por el virus de la inmunodeficiencia humana. Med-Clin-Barc.

1996 Sep 28; 107(10): 361-5; ISSN: 0025-7753.

SPAIN. BACKGROUND: This article presents a combined

magnetic resonance imaging and proton spectroscopy protocol

(MRI/1H-MRS) applied to study the brain of human

immunodeficiency virus (HIV) infected patients. The

spectroscopic results were compared with clinical and

radiological parameters. PATIENTS AND METHODS: The proton

spectra of 57 HIV patients and 20 control subjects were

obtained from a volume of interest of 8 cm3 located in the

parietooccipital region of the brain that did not include any

focal lesion. The resonance areas due to N-acetyl aspartate

(NAA), creatine (Cr) and choline (Cho) were obtained. The MRI

exam allowed us to determine the presence of focal or diffuse

lesions and the degree of atrophy. Finally, the clinical

exploration included the performance of a Mini-Mental test.

The NAA/Cr, NAA/Cho and Cho/Cr ratios were correlated with

clinical characteristics, the result of the Mini-Mental test,

the presence of lesions and the degree of atrophy. RESULTS:

There were altered spectral patterns in a volume of the brain

that did not contain any focal lesion. The decrease in the

NAA/Cr or NAA/Cho ratios was significative when considering

the presence of atrophy, the existence of signs of cognitive

deficiencies or the diagnosis of AIDS-dementia complex.

CONCLUSIONS: The spectral changes found in the present study

suggest the existence of neuronal lesions that would be due to

the HIV-infection. A combined MRI/1H-MRS study may provide

a more complete information about the neurological

impairment by HIV and could constitute a marker of AIDS-

dementia complex.

4. Anand, P.; Parrett, A.; Chadwick, L.; Hamlyn, P. Nerve growth

factor concentrations in human cerebral blood vessels [letter].

J-Neurol-Neurosurg-Psychiatry. 1997 Feb; 62(2): 199-200;

ISSN: 0022-3050.

ENGLAND. 0.

5. Annunziato, P. W.; Gershon, A. Herpes simplex virus infections.

Pediatr-Rev. 1996 Dec; 17(12): 415-23; quiz 424; ISSN: 0191-

9601.

UNITED-STATES.

6. Baker, H. F.; Ridley, R. M. What went wrong in BSE? From prion

disease to public disaster. Brain-Res-Bull. 1996; 40(4): 237-

44; ISSN: 0361-9230.

UNITED-STATES. The recent report of 10 cases of a new

variant of Creutzfeldt-Jakob disease (CJD) which could be

related to bovine spongiform encephalopathy (BSE) has

precipitated alarm throughout Europe. The beef trade in the UK

has collapsed and the European beef market has been seriously

damaged. What went wrong? Much of the difficulty of handling

the BSE epidemic arose from the 4-5 year incubation period

which made it difficult to ascertain whether measures taken

to contain the epidemic had been effective. Public

consternation and scientific equivocation arose because these

prion diseases are unlike any other group of infectious

diseases. Rather than being caused by a conventional micro-

organism, the primary pathogenic event consists of the

transformation of a normal protein (the prion protein) into an

abnormal form, which can transmit disease. Prion disease is

endemic in humans and sheep where it is associated with

polymorphisms or mutations within the prion protein gene.

Although the disease in these cases arises spontaneously, it

produces an infectious prion protein. Under certain

circumstances, abnormal prion protein contaminates other

animals or humans resulting in epidemics of acquired prion

disease. This review describes the events of the BSE epidemic

and considers the difficulties in assessing the current risk to

human health.

7. Barton, A. J.; Crook, B. W.; Karran, E. H.; Brown, F.; Dewar, D.; Mann,

D. M.; Pearson, R. C.; Graham, D. I.; Hardy, J.; Hutton, M.; Duff, K.;

Goate, A. M.; Clark, R. F.; Roberts, G. W. Alteration in brain

presenilin 1 mRNA expression in early onset familial

Alzheimer's disease. Neurodegeneration. 1996 Sep; 5(3): 213-

8; ISSN: 1055-8330.

ENGLAND. The expression of the presenilin 1 (PS-1) gene has

been investigated by in situ hybridization in early onset

familial Alzheimer's disease (FAD), late onset Alzheimer's

disease (AD) and normal control brain. Mutations in this gene

are responsible for chromosome 14-linked FAD. We have found

that presenilin 1 mRNA is present throughout the human brain

with a distribution consistent with both a glial and neuronal

localization. The in situ hybridization pattern was similar for

the controls, the early onset FAD cases and the late onset AD

cases. However, one of the two forms of the mRNA for PS-1,

the long form (which contains a sequence encoding a four

amino acid (VRSQ) insert at its 5' end) was significantly

reduced in early onset FAD brain compared with late onset AD.

We suggest that this long transcript may alter the normal

pathway for processing of amyloid precursor protein, the

protein which appears to be central in the pathogenesis of AD..

0; 0; 0.

8. Bauer, L. O. Resting hand tremor in abstinent cocaine-dependent,

alcohol-dependent, and polydrug-dependent patients. Alcohol-

Clin-Exp-Res. 1996 Oct; 20(7): 1196-201; ISSN: 0145-6008.

UNITED-STATES. Laboratory studies of cocaine-exposed

rodents, and positron emission tomographic studies of human

cocaine abusers have suggested that chronic cocaine abuse

downregulates dopaminergic function in the basal ganglia. The

present study sought to provide behavioral evidence for this

phenomenon by demonstrating enhanced levels of resting hand

tremor among patients with previous histories of cocaine

dependence. to determine the specificity of the phenomenon,

patients with previous histories of alcohol dependence,

cocaine/alcohol codependence, and cocaine/opiate

codependence were also evaluated. Patients were assigned to

one of four groups according to DSM-IIIR diagnostic criteria:

(1) cocaine dependent (n = 19); (2) cocaine and alcohol

dependent (n = 12); (3) cocaine and opiate dependent (n = 7); (4)

alcohol dependent (n = 9). All were abstinent from their

primary drug of abuse for a period of 1 to 5 months. The three

patient groups with histories of cocaine dependence exhibited

significantly more resting hand tremor than the alcohol-

dependent and normal control groups. Furthermore, hand tremor

in the former three groups was positively related to the

number of self-reported uses of cocaine and negatively related

to the number of months of cocaine abstinence.. 0; 50-36-2.

9. Berger, D. S.; Bucher, G.; Nowak, J. A.; Gomatos, P. J. Acute primary

human immunodeficiency virus type 1 infection in a patient

with concomitant cytomegalovirus encephalitis. Clin-Infect-

Dis. 1996 Jul; 23(1): 66-70; ISSN: 1058-4838.

UNITED-STATES. We report what we believe is the first case

of primary human immunodeficiency virus type 1 (HIV-1)

infection and simultaneous cytomegalovirus (CMV)

encephalitis, which was confirmed by detection of CMV DNA in

the patient's cerebrospinal fluid with use of the polymerase

chain reaction. This coinfection had an unusual course, and the

patient's clinical status deteriorated despite administration

of combination antiretroviral therapy. The patient responded

clinically only after therapy for CMV infection was added to

his combination antiretroviral regimen. An atypical course and

duration of symptomatic primary HIV-1 infection should

suggest a possible coincident infection with other

opportunistic agents that are normally expected to cause

disease later in the course of HIV-1 infection. Current

recommendations from the Centers for Disease Control and

Prevention list CMV encephalitis as an AIDS-defining event..

0; 0.

10. Berkovic, S. F.; Kuzniecky, R. I.; Andermann, F. Human

epileptogenesis and hypothalamic hamartomas: new lessons

from an experiment of nature [editorial]. Epilepsia. 1997 Jan;

38(1): 1-3; ISSN: 0013-9580.

UNITED-STATES.

11. Berr, C.; Lafont, S.; Debuire, B.; Dartigues, J. F.; Baulieu, E. E.

Relationships of dehydroepiandrosterone sulfate in the elderly

with functional, psychological, and mental status, and short-

term mortality: a French community-based study. Proc-Natl-

Acad-Sci-U-S-A. 1996 Nov 12; 93(23): 13410-5; ISSN: 0027-

8424.

UNITED-STATES. In human beings of both sexes,

dehydroepiandrosterone sulfate (DHEAS) circulating in blood is

mostly an adrenally secreted steroid whose serum

concentration (in the micromolar range and 30-50% higher in

men than in women) decreases with age, toward approximately

20-10% of its value in young adults during the 8th and 9th

decades. The mechanism of action of DHEA and DHEAS is poorly

known and may include partial transformation into sex

steroids, increase of bioavailable insulin-like growth factor 1,

and effects on neurotransmitter receptors. Whether there is a

cause-to-effect relationship between the decreasing levels of

DHEAS with age and physiological and pathological

manifestations of aging is still undecided, but this is of

obvious theoretical and practical interest in view of the easy

restoration by DHEA administration. Here we report on 622

subjects over 65 years of age, studied for the 4 years since

DHEAS baseline values had been obtained, in the frame of the

PAQUID program, analyzing the functional, psychological, and

mental status of a community-based population in the south-

west of France. We confirm the continuing decrease of DHEAS

serum concentration with age, more in men than in women,

even if men retain higher levels. Significantly lower values of

baseline DHEAS were recorded in women in cases of functional

limitation (Instrumental Activities of Daily Living),

confinement, dyspnea, depressive symptomatology, poor

subjective perception of health and life satisfaction, and

usage of various medications. In men, there was a trend for the

same correlations, even though not statistically significant in

most categories. No differences in DHEAS levels were found in

cases of incident dementia in the following 4 years. In men

(but not in women), lower DHEAS was significantly associated

with increased short-term mortality at 2 and 4 years after

baseline measurement. These results, statistically established

by taking into account corrections for age, sex, and health

indicators, suggest the need for further careful trials of the

administration of replacement doses of DHEA in aging humans.

Indeed, the first noted results of such "treatment" are

consistent with correlations observed here between functional

and psychological status and endogenous steroid serum

concentrations.. 651-48-9.

12. Beyreuther, K.; Multhaup, G.; Masters, C. L. Alzheimer's disease:

genesis of amyloid. Ciba-Found-Symp. 1996; 199: 119-27;

discussion 127-31; ISSN: 0300-5208.

NETHERLANDS. Much of the present knowledge on the genes and

genetic processes involved in the genesis of amyloid formation

in Alzheimer's disease (AD) has come directly or indirectly

from the retrospective molecular and genetic analysis of

amyloid beta-protein (A beta or beta A4) deposits and from the

identification of genes involved in inherited susceptibility to

the disease. This analysis shows that the release and

aggregation of the A beta fragment from the amyloid precursor

protein (APP) is involved in APP (AD1), chromosome 14 (AD3),

1 (AD4) and 19(AD2) families as well as in the sporadic forms

of AD, suggesting that AD is a single disease with a common

APP/A beta amyloid pathogenesis. Synthetic A beta protein

readily forms beta sheets, filaments and amyloid at

micromolar concentrations. The principle to inhibit this

process has been worked out by our groups with A beta

variants. The N-terminal and C-terminal A beta sequences,

oxidative radicals, membrane integrity and metal ions also

affect the aggregation of A beta. Amino acid substitutions

within the A beta sequence, as occur in rodents, alter A beta

release and change the degree to which oxidation of the

peptides occurs. Transgenic approaches resulting in

overexpression of human APP have confirmed that A beta

sequence and concentration are critical prerequisites to

amyloid deposition in vivo.. 0; 0; 0.

13. Biesecker, L. G.; Abbott, M.; Allen, J.; Clericuzio, C.; Feuillan, P.;

Graham, JM Jr; Hall, J.; Kang, S.; Olney, A. H.; Lefton, D.; Neri, G.;

Peters, K.; Verloes, A. Report from the workshop on Pallister-

Hall syndrome and related phenotypes. Am-J-Med-Genet. 1996

Oct 2; 65(1): 76-81; ISSN: 0148-7299.

UNITED-STATES.

14. Biesecker, L. G.; Graham, JM Jr. Pallister-Hall syndrome. J-Med-

Genet. 1996 Jul; 33(7): 585-9; ISSN: 0022-2593.

ENGLAND.

15. Binder, J. R.; Frost, J. A.; Hammeke, T. A.; Cox, R. W.; Rao, S. M.;

Prieto, T. Human brain language areas identified by functional

magnetic resonance imaging. J-Neurosci. 1997 Jan 1; 17(1):

353-62; ISSN: 0270-6474.

UNITED-STATES. Functional magnetic resonance imaging

(FMRI) was used to identify candidate language processing

areas in the intact human brain. Language was defined broadly

to include both phonological and lexical-semantic functions

and to exclude sensory, motor, and general executive functions.

The language activation task required phonetic and semantic

analysis of aurally presented words and was compared with a

control task involving perceptual analysis of nonlinguistic

sounds. Functional maps of the entire brain were obtained from

30 right-handed subjects. These maps were averaged in

standard stereotaxic space to produce a robust "average

activation map" that proved reliable in a split-half analysis.

As predicted from classical models of language organization

based on lesion data, cortical activation associated with

language processing was strongly lateralized to the left

cerebral hemisphere and involved a network of regions in the

frontal, temporal, and parietal lobes. Less consistent with

classical models were (1) the existence of left hemisphere

temporoparietal language areas outside the traditional

"Wernicke area," namely, in the middle temporal, inferior

temporal, fusiform, and angular gyri; (2) extensive left

prefrontal language areas outside the classical "Broca area";

and (3) clear participation of these left frontal areas in a task

emphasizing "receptive" language functions. Although partly in

conflict with the classical model of language localization,

these findings are generally compatible with reported lesion

data and provide additional support for ongoing efforts to

refine and extend the classical model.

16. Bingham, P. M.; Spinner, N. B.; Sovinsky, L.; Zackai, E. H.; Chance, P.

F. Infantile spasms associated with proximal duplication of

chromosome 15q. Pediatr-Neurol. 1996 Sep; 15(2): 163-5;

ISSN: 0887-8994.

UNITED-STATES. We describe a case of infantile spasms

associated with a chromosome abnormality (supernumerary

inverted duplication of chromosome 15 [47,XX,+inv dup(15)]).

The patient was nondysmorphic and presented with mild

hypotonia and delay in acquisition of gross motor milestones

before the diagnosis of seizures at age 7 months. Additional

features included unilateral sensorineural deafness and

torticollis. Molecular cytogenetic studies confirmed that the

patient has a large inv dup(15). Inv dup(15) chromosomes are

variable with respect to the size and genetic composition of

the chromosome and in their phenotypic effects. Patients with

small inv dup(15s) may have no phenotypic abnormalities,

whereas patients with large inv dup(15s) may have multiple

abnormalities. ACTH therapy resulted in prompt remission of

seizures and resolution of EEG abnormalities. This is the

second report of a patient with IS and a supernumerary inv

dup(15). Several genes code for neurotransmitter receptor

subunits located in the duplicated region of chromosome 15,

and abnormal dosage of these genes may be involved in the

genesis of seizure activity in carriers of the inv dup(15).

Chromosome analysis may lead to a specific diagnosis in

infants with unexplained infantile spasms.. 9002-60-2.

17. Blok, B. F.; Willemsen, A. T.; Holstege, G. A PET study on brain

control of micturition in humans. Brain. 1997 Jan; 120( Pt 1):

111-21; ISSN: 0006-8950.

ENGLAND. Although the brain plays a crucial role in the control

of micturition, little is known about the structures involved.

Identification of these areas is important, because their

dysfunction is though to cause urge incontinence, a major

problem in the elderly. In the cat, three areas in the brainstem

and diencephalon are specifically implicated in the control of

micturition: the dorsomedial pontine tegmentum, the

periaqueductal grey, and the preoptic area of the

hypothalamus. PET scans were used to test whether these

areas are also involved in human micturition. Seventeen right-

handed male volunteers were scanned during the following four

conditions: (i) 15 min prior to micturition during urine

withholding: (ii) during micturition; (iii) 15 min after

micturition; (iv) 30 min after micturition. Ten of the 17

volunteers were able to micturate during scanning.

micuturition was associated with increased blood flow in the

right dorsomedial pontine tegmentum, the periaqueductal grey,

the hypothalamus and the right inferior frontal gyrus.

Decreased blood flow was found in the right anterior cingulate

gyrus when urine was withheld. The other seven volunteers

were not able to micturate during scanning, although they had

a full bladder and tried vigorously to do so. In this group,

during these unsuccessful attempts to micturate, increased

blood flow was found in the right ventral pontine tegmentum,

which corresponds with the hypothesis, formulated from

results in cats, that this area controls the motor neurons of

the pelvic floor. Increased blood flow was also found in the

right inferior frontal gyrus during unsuccessful attempts at

micturition, and decreased blood flow in the right anterior

cingulate gyrus was found during the withholding of urine. The

results suggest that, as that of the cat, the human brainstem

contains specific nuclei responsible for the control of

micturition, and that the cortical and pontine micturition

sites are predominantly on the right side.

18. Boneh, A. Protein kinase C activity, phosphate uptake and

endogenous substrate phosphorylation are altered in Zellweger

syndrome. J-Inherit-Metab-Dis. 1996; 19(5): 661-6; ISSN:

0141-8955.

NETHERLANDS. Protein kinase C (PKC) is a key enzyme in lipid-

mediated signal transduction. Regulation of PKC activation is

dependent upon the phospholipid constituents of cellular

membranes. PKC is also activated by very long-chain and long-

chain cis-unsaturated fatty acids. The present study was

undertaken as a first step towards elucidating a possible role

for PKC in the pathogenesis of Zellweger syndrome, in which

there are both perturbation of plasma membrane phospholipids

and accumulation of very long-chain fatty acids. PKC activity,

phosphate uptake and endogenous substrate phosphorylation

were examined in intact human skin fibroblasts from

Zellweger patients. PKC catalytic activity was increased in

the membranous fraction of Zellweger cells compared with

control cells, with no apparent translocation of the enzyme

from the cytosolic to the membranous compartment. Phosphate

uptake was increased in both cytosolic and membranous

fractions 2.5-fold and 4.5-fold, respectively. Several proteins

were extensively phosphorylated in Zellweger cells compared

with control cells. These findings indicate that PKC activity is

perturbed in Zellweger cells, but the exact role of PKC in

altered phosphate uptake and protein phosphorylation and its

relevance to the pathogenesis of Zellweger syndrome require

further study.. EC 2.7.1.-; 0.

19. Brandt, M. E.; Bohan, T. P.; Thorstad, K.; McCauley, S. R.; Davidson,

K. C.; Francis, D. J.; Kramer, L. A.; Fletcher, J. M. Reliability of

brain structure morphometry in hydrocephalic children using

MR images. Magn-Reson-Imaging. 1996; 14(6): 649-55; ISSN:

0730-725X.

UNITED-STATES. To assess the ability of human operators to

make decisions about region boundaries in significantly

malformed brains, we performed a study of the reliability of

morphometric measurements of specific brain structures from

MRI in children with hydrocephalus and controls. Cross-

sectional area measures of the corpus callosum, internal

capsules and centrum semiovale, and volumes of the lateral

ventricles were made in 50 children. Independent

measurements were made by two raters on T1 and T2-

weighted MR images. Pearson's correlation coefficients (r) and

intraclass correlation coefficients (ICC) between the two

rater's sets of measures were computed for each structure

across all subjects. ICCs ranged from a low of 0.7502 to a high

of 0.9895. All ICCs were significant at the p < .0001 level and

were generally less than or equal to the corresponding

Pearson's r value in every case. Therefore, the Pearson's r may

overestimate the reliability. The results of this study support

the claim that the ICC should be used rather than the Pearson's

r when assessing interater reliability in situations where

large between-group differences are present. In addition, the

results show that brains malformed by disorders, such as

hydrocephalus, can be reliably assessed using morphometric

measures of MR images.

20. Brazell, V. J. The human side of "mad cow" disease. RN. 1997 Jan;

60(1): 32-4; quiz 35; ISSN: 0033-7021.

UNITED-STATES.

21. Brugere Picoux, J.; Lasmezas, C.; Deslys, J. P.; Adjou, K.; Rerat, A.;

Dormont, D. [Bovine spongiform encephalopathy. Second update

of data collected since the report of February 6, 1996].

Encephalopathie spongiforme bovine. Deuxieme mise au point

des donnees recueillies depuis le voeu du 6 fevrier 1996. Bull-

Acad-Natl-Med. 1996 Jun; 180(6): 1443-9; discussion 1450-4;

ISSN: 0001-4079.

FRANCE. The observation in 1995 and 1996 of 12 cases of a

new variant of Creutzfeldt-Jakob disease (V-CJD) in U.K.

suggested a possible relation between this human cases and

bovine spongiform encephalopathy (BSE). Recent papers about

this topic are reviewed: BSE transmission to macaques,

transmission of scrapie with embryo transfer, incidence of

maternal transmission, PrP protein released by platelets,

diagnostic test by detection of PrP protein in tissues of sheep,

epidemiology of BSE, french regulations, identification of

cattle in U.K.

22. Brugere Picoux, J.; Rerat, A. [Bovine spongiform encephalopathy.

Review of data collected since the statement of February 6,

1996]. Encephalopathie spongiforme bovine. Mise au point des

donnees recueillies depuis le voeu du 6 fevrier 1996. Bull-

Acad-Natl-Med. 1996 May; 180(5): 1007-12; discussion 1012-

5; ISSN: 0001-4079.

FRANCE. The observation in 1995 and 1996 of 10 cases of a

new variant of Creutzfeldt-Jakob disease (V-CJD) in U.K.

suggested a possible relation between this human cases and

bovine spongiform encephalopathy (BSE). Recent papers about

this topic are reviewed : hypothesis of a possible genetic link

between man and cattle, hypothesis of a acquired resistance

against the agent of BSE after a previous infection by a less

virulent agent of ovine origin, importance of polymorphism at

codon 129 according to the hypothesis of a virus-induced

amyloidosis, diagnostic test with cerebrospinal fluid,

epidemiology of BSE and prediction of future BSE spread.

23. Bruning, R.; Wu, R. H.; Deimling, M.; Porn, U.; Haberl, R. L.; Reiser, M.

Diffusion measurements in the ischemic human brain with a

steady-state sequence. Invest-Radiol. 1996 Nov; 31(11): 709-

15; ISSN: 0020-9996.

UNITED-STATES. RATIONALE AND OBJECTIVES: The authors

evaluate the clinical usefulness of a diffusion-weighted

steady-state free-precession (SSFP) sequence to detect acute

and subacute ischemic changes. METHODS: Twenty-four

patients were examined on a 1.5-tesla scanner, using a SSFP-

sequence (repetition time [TR]/ echo time [TE] = 22/3-8

mseconds). The slice thickness was 5 mm, 10 averages, 57

seconds per slice. The diffusion gradient strength was 23

millitesla/m, with b-values from 165 to 598 seconds/mm2.

Diffusion-weighted images (DWI) were compared with T2-

weighted images. RESULTS: The diffusion-weighted SSFP

sequence produced diagnostic quality images in 23 of 24

patients. Diffusion depicted (group 1: 0-12 hours) more acute

lesions (3 of 6) than T2-weighted images (2 of 6); the mean

lesion diameter depicted by diffusion was 10.9 mm (standard

deviation [SD], 12.3) and in T2-weighted images was 4.7 mm

(SD 6.8). A significant correlation (P < 0.017) in subacute

lesions was found when diffusion was compared with turbo

spin echo (mean size difference/T2 = 18.5/17.5 mm, SD

13.2/12.2). CONCLUSIONS: The diffusion-weighted SSFP-

sequence is more sensitive in acute ischemia and delineates

likewise in subacute ischemia, when compared with T2-

weighted imaging.

24. Butterworth, R. J.; Wassif, W. S.; Sherwood, R. A.; Gerges, A.;

Poyser, K. H.; Garthwaite, J.; Peters, T. J.; Bath, P. M. Serum

neuron-specific enolase, carnosinase, and their ratio in acute

stroke. An enzymatic test for predicting outcome? Stroke.

1996 Nov; 27(11): 2064-8; ISSN: 0039-2499.

UNITED-STATES. BACKGROUND AND PURPOSE: Few admission

variables adequately predict neuronal damage and prognosis in

individual patients after stroke. Therefore, there is a need for

a reliable non-invasive surrogate measure of clinical outcome.

METHODS: We have developed a surrogate measure of stroke

outcome using the ratio of serum neuron-specific enolase

(NSE) to human serum carnosinase (HSC) in 124 patients with

acute ischemic or hemorrhagic stroke and 61 matched control

subjects. Serum NSE is known to rise and HSC to fall after

neuronal injury such as cerebral ischemia. RESULTS: Serum NSE

levels were significantly higher and HSC levels lower in the

patient group. The NSE/HSC ratio was elevated in patients

with stroke: median (semiquartile) hemorrhages, 0.072

(0.033); infarcts, 0.039 (0.026); and control subjects, 0.019

(0.014), P = .0001. Patients with a primary intracerebral

hemorrhage had nonsignificantly higher ratios than those with

an infarct (P = .082). The NSE/HSC ratio was significantly

associated with 90-day outcome measured in two out of three

disability and handicap scales: modified Barthel Index (rs = -

.34, P = .001), modified Rankin Scale (rs = .30, P = .002), and

Lindley Score (rs = .19, P = .057). Patients who died or were

institutionalized had higher ratios than those who were

discharged home: 0.069 (0.043) versus 0.038 (0.024), P = .011.

Correlations between the NSE/HSC ratio and outcome were

comparable to those between patient age or consciousness

level on admission and clinical outcome. CONCLUSIONS: We

believe that measurement of NSE, HSC, or their ratio may be

useful in the assessment of patients with acute stroke with

respect to diagnosis and prediction of clinical outcome.. EC

3.4.13.; EC 3.4.13.3; EC 4.2.1.11.

25. Byrum, C. E.; MacFall, J. R.; Charles, H. C.; Chitilla, V. R.; Boyko, O.

B.; Upchurch, L.; Smith, J. S.; Rajagopalan, P.; Passe, T.; Kim, D.;

Xanthakos, S.; Ranga, K.; Krishnan, R. Accuracy and

reproducibility of brain and tissue volumes using a magnetic

resonance segmentation method. Psychiatry-Res. 1996 Oct 7;

67(3): 215-34; ISSN: 0165-1781.

IRELAND. Magnetic resonance (MR) imaging now allows the

qualitative and quantitative assessment of the human brain in

vivo. As MR imaging resolution has improved, precise

measurement of small brain structures has become possible.

Methods of measuring brain regions from MR images include

both manual and semiautomated methods. Despite the

development of numerous volumetric methods, there have been

only limited attempts so far to evaluate the accuracy and

reproducibility of these methods. In this study we used

phantoms to assess the accuracy of the segmentation process.

Our results with simple and complex phantoms indicate an

error of 3-5% using either manual or semiautomated

techniques. We subsequently used manual and semiautomated

volumetric methodologies to study human brain structures in

vivo in five normal subjects. Supervised segmentation is a

semiautomated method that accomplishes the division of MR

images into several tissue types based on differences in signal

intensity. This technique requires the operator to manually

identify points on the MR images that characterize each tissue

type, a process known as seeding. However, the use of

supervised segmentation to assess the volumes of gray and

white matter is subject to pitfalls. Inhomogeneities of the

radiofrequency or magnetic fields can result in

misclassification of tissue points during the tissue seeding

process, limiting the accuracy and reliability of the

segmentation process. We used a structured seeding protocol

that allowed for field inhomogeneity that produced reduced

variation in measured tissue volumes. We used repeated

segmentations to assess intra- and inter-rater reliability, and

were able to measure small and large regions of interest with

a small degree of variation. In addition, we demonstrated that

measurements are reproducible with repeat MR acquisitions,

with minimal interscan variability. Segmentation methods can

accurately and reliably measure subtle morphometric changes,

and will prove a boon to the study of neuropsychiatric

disorders.

26. Chiba, H. [Physiology and pathology of the lipid transport in the

brain]. Rinsho-Byori. 1996 Mar; 44(3): 231-6; ISSN: 0047-1860.

JAPAN. Apolipoprotein E is the major lipid carrier protein in

the brain. This protein is contained in HDL1-like particles in

human and rat cerebrospinal fluid. Cerebrospinal fluid

apolipoprotein metabolism is resistant to the alteration in

plasma lipoprotein metabolism and to age-related changes. In

a patient with multiple sclerosis, immunoreactive

apolipoprotein E was seen in astrocytes and macrophages in

the brain section with a pathological change, indicating that

apolipoprotein E has a significant role in the lipid

redistribution process after demyelination. The epsilon4 allele

is more frequent in Japanese patients with Alzheimer's

disease than in control, as well as Caucasians, suggesting the

significant contribution of the epsilon4 allele to the

pathogenesis of the disease.. 0; 0.

27. Chrzanowska, K. H. [Microcephaly with chromosomal instability

and immunodeficiency--Nijmegen syndrome]. Maloglowie z

niestabilnoscia chromosomowa i zaburzeniami odpornosci--

zespol Nijmegen. Pediatr-Pol. 1996 Mar; 71(3): 223-34; ISSN:

0031-3939.

POLAND. The Nijmegen breakage syndrome (NBS) is a rare

autosomal recessive disease, which belongs to the family of

genetically determined instability syndromes and to the

growing category of ataxia telangiectasia (AT)--related

disorders. The main manifestations include pronounced

microcephaly with mental retardation in most patients, "bird-

like" facies, growth retardation, immunodeficiency,

chromosome instability with multiple chromosome 7 and 14

rearrangements, hypersensitivity to ionizing radiation and

normal AFP level. In light of high predisposition to malignancy,

an accurate diagnosis is very important for the patient.. 0.

28. Cinque, P.; Vago, L.; Dahl, H.; Brytting, M.; Terreni, M. R.; Fornara,

C.; Racca, S.; Castagna, A.; Monforte, A. D.; Wahren, B.; Lazzarin,

A.; Linde, A. Polymerase chain reaction on cerebrospinal fluid

for diagnosis of virus-associated opportunistic diseases of

the central nervous system in HIV-infected patients. AIDS.

1996 Aug; 10(9): 951-8; ISSN: 0269-9370.

UNITED-STATES. OBJECTIVE: To assess the diagnostic

reliability of polymerase chain reaction (PCR) on cerebrospinal

fluid (CSF) for virus-associated opportunistic diseases of the

central nervous system (CNS) in HIV-infected patients. DESIGN:

CSF samples from 500 patients with HIV infection and CNS

symptoms were examined by PCR. In 219 patients the PCR

results were compared with CNS histological findings.

METHODS: Nested PCR for detection of herpes simplex virus

(HSV) type 1 or 2, varicella zoster virus (VZV),

cytomegalovirus (CMV), Epstein-Barr virus (EBV), human

herpesvirus 6 (HHV-6), and JC virus (JCV) DNA.

Histopathological examination of CNS tissue obtained at

autopsy or on brain biopsy. RESULTS: DNA of one or more

viruses was found in CSF in 181 out of 500 patients (36%;

HSV-1 2%, HSV-2 1%, VZV 3%, CMV 16%, EBV 12%, HHV-6 2%,

and JCV 9%). Among the 219 patients with histological CNS

examination, HSV-1 or 2 was detected in CSF in all six

patients (100%) with HSV infection of the CNS, CMV in 37 out

of 45 (82%) with CMV infection of the CNS, EBV in 35 out of 36

(97%) with primary CNS lymphoma, JCV in 28 out of 39 (72%)

with progressive multifocal leukoencephalopathy.

Furthermore, HSV-1 was found in one, VZV in four, CMV in

three, EBV in three, HHV-6 in seven, and JCV in one patient

without histological evidence of the corresponding CNS

disease. CONCLUSIONS: CSF PCR has great relevance for

diagnosis of virus-related opportunistic CNS diseases in HIV-

infected patients as demonstrated by its high sensitivity,

specificity, and the frequency of positive findings.. 0; 0.

29. Colder, B. W.; Wilson, C. L.; Frysinger, R. C.; Chao, L. C.; Harper, R.

M.; Engel, J. Jr. Neuronal synchrony in relation to burst

discharge in epileptic human temporal lobes. J-Neurophysiol.

1996 Jun; 75(6): 2496-508; ISSN: 0022-3077.

UNITED-STATES. 1. Synchronous interactions between neurons

in mesial temporal structures of patients with complex

partial seizures were studied using cross-correlation

analyses. We recorded spontaneous activity from 293 neurons

in 24 patients during the interictal state. Patients had depth

microelectrodes chronically implanted in amygdala,

hippocampal formation, and parahippocampal gyrus to record

epileptic activity. One hundred twenty-five cells were

recorded from the temporal lobe commonly initiating seizures

(ipsilateral temporal lobe), and 168 cells from the

contralateral temporal lobe. Eight hundred forty-three cross-

correlograms were constructed between all pairs of

simultaneously recorded neurons. Cross-correlogram peaks or

troughs that exceeded confidence limits within 200 ms of the

origin were considered evidence of synchronous neuronal

interaction. 2. Synchronous neuronal interactions were

observed in 223 of 843 cross-correlograms. Eighty-six percent

of these 223 cross-correlograms showed significant central

peaks (peak interactions), suggesting excitatory interactions,

whereas the remainder displayed significant central troughs

(trough interactions), suggesting inhibitory interactions. 3.

Cross-correlograms constructed using cells from the

ipsilateral temporal lobe (ipsilateral cross-correlograms)

were more likely to display significant central troughs

(14/262) than cross-correlograms constructed using cells

from the contralateral temporal lobe (6/376; contralateral

cross-correlograms). Similarly, cross-correlograms

constructed using one cell from each hemisphere (11/205;

bilateral cross-correlograms) were also more likely to display

significant central troughs (trough interactions) than

contralateral cross-correlograms. Both ipsilateral (77/262)

and contralateral cross-correlograms (102/376) were more

likely to display significant central peaks (peak interactions)

than bilateral cross-correlograms (13/205). 4. Cells from

different structures in the ipsilateral temporal lobe were

more likely to display significant trough interactions (10/

114) than neurons in different contralateral structures. We

also compared the proportion of significant peak interactions

between cells within the ipsilateral and contralateral sides of

each structure. Neurons in the contralateral entorhinal cortex

were more likely to show peak interactions (21/55) than cells

from the ipsilateral entorhinal cortex (3/31). Also, cells in

the ipsilateral presubiculum showed a higher proportion of

peak interactions (9/16) than their contralateral homologues

(5/30). 5. Neuronal burst discharges were defined as three or

more action potentials (or spikes) separated by interspike

intervals of < or = 30 ms, or two spikes separated by an

interval of < or = 15 ms. The contribution of burst discharge to

synchronous peak interaction was compared between temporal

lobes. Cells used to construct ipsilateral cross-correlograms

displaying significant central peaks (n = 154) were found to

have significantly reduced burst discharge contributions to the

observed synchronous peaks in comparison with their

contralateral homologues (n = 204). When cross-correlograms

were separated by regions, burst discharge contributions to

synchronous peak interactions between cells in the ipsilateral

hippocampus (n = 72) were significantly smaller than the

contributions from cells in the contralateral hippocampus (n =

44). 6. The results suggest that in the interictal state,

synchronous neuronal burst discharge is not a distinguishing

feature of epileptogenic regions of patients with complex

partial seizures, but inhibitory neuronal interactions are

increased in regions of seizure initiation. Increases in the

strength and spread of local inhibition in seizure initiating

regions in these patients may result in a greater proportion of

inhibitory interactions and could also cause increased

synchrony between isolated action potentials.(ABSTRACT

TRUNCATED).

30. Connors, M. H.; Boggan, J. E.; Chong, B.; Kollipara, S. Expansion and

shrinkage of central nervous system tumor coinciding with

human growth hormone therapy: case report. Neurosurgery.

1996 Dec; 39(6): 1243-5; discussion 1245-6; ISSN: 0148-

396X.

UNITED-STATES. OBJECTIVE AND IMPORTANCE: The influence

of human growth hormone (hGH) therapy on the recurrence

rates of childhood central nervous system tumors is

controversial. Because growth hormone has the ability to

increase cell proliferation, it is recommended that hGH

therapy wait until central nervous system lesions are inactive

and antitumor therapy complete, usually 1 to 2 years. CLINICAL

PRESENTATION: We report the enlargement and decrease in size

of a hypothalamic pilocytic astrocytoma in a 12-year-old boy

after two trials of hGH. Partial resection and radiation of the

tumor were performed at 3 years of age, with no change noted

over the next 9 years. His height was less than the 5th centile

with midparental height at the 90th to 95th centiles. Growth

velocity was 3.3 cm/yr. Bone age was normal and there were

no signs of puberty. There was no GH response to clonidine and

L-dopa testing. INTERVENTION: Volume measurements were

performed on gadolinium enhanced tumor images. Growth rate

increased to 11.7 cm and the tumor volume increased 230%

over the 12 months of hGH therapy. Significant tumor

shrinkage (42%) and growth deceleration occurred within the 3

month interval of stopping hGH. Tumor volume again increased

(134%) and decreased (22%) after restarting and then stopping

hGH. No evidence of tumor necrosis or alteration in ventricular

size was found. The patient was asymptomatic. CONCLUSION:

These observations indicate that tumor size change is

associated with the metabolic response to hGH therapy. It is

unclear whether the volume increase represents altered blood-

brain or selective blood-tumor barrier permeability, growth

factor receptors, and/or tumor cell growth.. 12629-01-5.

31. Coria, F.; Cuadrado, N. [Genetic neuroepidemiology].

Neuroepidemiologia genetica. Neurologia. 1995 Dec; 10 Suppl 1:

50-5; ISSN: 0213-4853.

SPAIN. Many human diseases result from the combined effect

of several genetic and non genetic factors. Thanks to the

development of powerful tools for molecular analysis,

researchers in recent years have begun to uncover the genes

behind such multifactorial neurologic and systemic disorders

as Alzheimer's and Parkinson's diseases and diabetes. Likewise

investigators have been able to confirm that the course of

these diseases and their clinical manifestations depend on the

concurrence in the same individual of specific genetic

variations. Because each gene confers a certain degree of

predisposition to a specific disease, they are therefore called

susceptibility genes. The search for and analysis of

susceptibility genes in defined populations is termed genetic

epidemiology. This multidisciplinary science is providing

valuable data that further our understanding and ability to

diagnose of disorders of the nervous system. It also enables us

to predict the clinical course of disease in a given patient

with a high degree of reliability, even when no clinical signs

are yet evident.. 0; 0; 0.

32. Corrigan, F. M.; French, M.; Murray, L. Organochlorine compounds in

human brain. Hum-Exp-Toxicol. 1996 Mar; 15(3): 262-4; ISSN:

0960-3271.

ENGLAND. Having observed polychlorinated biphenyls (PCBs) in

brain tissue obtained post mortem from two men we have

carried out a study of organochlorine compounds in frontal

cortex from patients with Parkinson's disease (PD) and from

controls. No PCBs were found in any of those samples. There

was no difference in the concentration of the DDT metabolite

pp'-DDE in the PD brain samples. Dieldrin (HEOD) was

significantly decreased in PD brain when analysed by lipid

weight. While these findings would not support the hypothesis

that PCBs may contribute to the development of Parkinson's

disease in humans it remains possible that they may cause

damage to the basal ganglia before being displaced from brain

tissue.. 0; 50-29-3; 60-57-1.

33. Costa, A.; Benedetto, C.; Fabris, C.; Giraudi, G. F.; Testori, O.;

Bertino, E.; Marozio, L.; Varvello, G.; Arisio, R.; Ariano, M.;

Emanuel, A. Cortisol in human tissues at different stages of

life. J-Endocrinol-Invest. 1996 Jul; 19(7): 463-71; ISSN:

0391-4097.

ITALY. Aim of the work was to measure the cortisol level in

human tissues at different stages of life, by means of

radioimmunoassay and by chromatography. Viable samples of

13 different tissues were obtained during surgical

intervention from 30 to 70 years old patients of either sex.

Mean tissue cortisol concentration was 78 +/- 35 ng/g,

ranging from 20 +/- 10 ng/g in the thyroid to 124 +/- 76 ng/g

in the kidney. Similar values were measured in the

corresponding tissues from not decayed corpses, so that paired

values could be mediated. However the pancreas, and corrupted

autopsy tissues, gave nil or exceedingly high cortisol

concentration values; in some cases, opposite extreme values

were measured in different organs of the same body. Cortisol

concentration was also measured in 11 sound different tissues

of spontaneously aborted or stillbirth fetuses, between 16 and

36 weeks of gestation. Mean value was 63 +/- 27 ng/g, ranging

from 30 +/- 25 ng/g in the liver to 104 +/- 52 ng/g in the

lungs. Also in fetuses nil or exceedingly high cortisol values

occurred in altered tissues. One hundred and fourteen samples

of limbs and carcasses of 7 to 12 gestational weeks embryos,

obtained from voluntary abortions, were also examined: 20%

gave nil result, in the remaining mean cortisol concentration

was 32 ng/g. In 33 samples of embryos' mixed viscera, RIA and

chromatography gave unreliable exceedingly high values. The

nil and the exceedingly high values measured in the altered

autoptic tissue specimens were inconsistent with the cortisol

blood level measured in the patients, as were those measured

in embryonic tissues with the acknowledged blood and

adrenals cortisol levels at that stage of life. Thus cortisol

may be measured by RIA and by chromatography in sound

tissues, while the values obtained in the pancreas, in

corrupted tissues, and in embryonal viscera do not represent

the hormonal milieu, but are likely artifacts due to

impeachment of the diagnostic system.. 50-23-7.

34. Coull, J. T.; Frith, C. D.; Frackowiak, R. S.; Grasby, P. M. A fronto-

parietal network for rapid visual information processing: a

PET study of sustained attention and working memory.

Neuropsychologia. 1996 Nov; 34(11): 1085-95; ISSN: 0028-

3932.

ENGLAND. The rapid visual information processing (RVIP) task,

a test of sustained attention which also requires working

memory for its successful execution, has been used in a

number of human psychopharmacological studies. Single digits

are presented in quick succession (100 or 200 digits/min) on a

computer screen, and target sequences of numbers must be

detected with a button press. Although previous neuroimaging

studies have implicated the frontal and parietal cortices in

performance of simple sustained attention tasks, the

neuroanatomical substrates of RVIP performance are not yet

known. This information would prove invaluable in the

interpretation of drug effects on this task, possibly

delineating a neuronal network for neurotransmitter action.

Therefore, this study investigated the functional anatomy of

the RVIP task using positron emission tomography (PET)

derived measures of regional cerebral blood flow (rCBF) in

eight healthy volunteers. Subjects were required to perform

variants of the RVIP task which manipulated both the level of

working memory load and the speed of stimulus presentation.

Compared with a rest condition (eyes closed), the RVIP task

increased rCBF bilaterally in the inferior frontal gyri, parietal

cortex and fusiform gyrus, and also in the right frontal

superior gyrus rostrally. In comparison with a simple

sustained attention control condition, the aforementioned

right frontal activations were no longer apparent. We suggest

that these data are consistent with the existence of a right

fronto-parietal network for sustained, and possibly selective,

attention, and a left fronto-parietal network for the

phonological loop component of working memory.

35. Cox, R. W. AFNI: software for analysis and visualization of

functional magnetic resonance neuroimages. Comput-Biomed-

Res. 1996 Jun; 29(3): 162-73; ISSN: 0010-4809.

UNITED-STATES. A package of computer programs for analysis

and visualization of three-dimensional human brain functional

magnetic resonance imaging (FMRI) results is described. The

software can color overlay neural activation maps onto higher

resolution anatomical scans. Slices in each cardinal plane can

be viewed simultaneously. Manual placement of markers on

anatomical landmarks allows transformation of anatomical

and functional scans into stereotaxic (Talairach-Tournoux)

coordinates. The techniques for automatically generating

transformed functional data sets from manually labeled

anatomical data sets are described. Facilities are provided for

several types of statistical analyses of multiple 3D functional

data sets. The programs are written in ANSI C and Motif 1.2 to

run on Unix workstations.

36. De Caro, R.; Parenti, A.; Munari, P. F. Megalodolichobasilaris: the

effect of atherosclerosis on a previously weakened arterial

wall? Clin-Neuropathol. 1996 Jul; 15(4): 187-91; ISSN: 0722-

5091.

GERMANY. The morphological findings of 2 basilar artery giant

fusiform aneurysms are presented. In one case (a 63-year-old

man) the aneurysm was accidentally found at autopsy. Its wall

was mainly formed by fibrous tissue without a smooth muscle

layer and presented fragmented but still recognizable elastic

lamina. In the media there were small well-formed bony

spicules. In the other case (a 59-year-old man) the aneurysm

had broken causing subarachnoid hemorrhage. The wall showed

a marked reduction of smooth muscle cells and thinning and

fragmentation of elastic lamina. A second sacciform aneurysm

was present at the basilar tip. The review of the literature and

the morphological findings of the 2 cases, characterized by

abnormality of the portion of the basilar artery not directly

involved in the aneurysm wall, consisting of a diffuse deficit

of the tunica media and lamina elastica, might suggest that

the fusiform aspect of the aneurysm may be the result of the

degenerative effect of atherosclerosis on a cogenital,

structural or dysmetabolic, or acquired, inflammatory,

weakening of the arterial wall.

37. Dealler, S. A matter for debate: the risk of bovine spongiform

encephalopathy to humans posed by blood transfusion in the UK

[see comments]. Transfus-Med. 1996 Sep; 6(3): 217-22; ISSN:

0958-7578.

Note: Comment in: Transfus Med 1996 Sep;6(3 ):213-5.

ENGLAND. If human infection with bovine spongiform

encephalopathy (BSE) were to occur, donated peripheral blood

from humans that might have become infected from eating

adequate quantities of food containing BSE should, until

evidence is available to the contrary, be assumed to contain

the human form of the disease. The chance of disease transfer

to a blood recipient in 1995, which might in turn cause

clinical disease with an incubation period of 20 years, is

calculated. Transfusion is calculated to be a potential cause of

a maximum of only 0.2% of clinical cases of Creutzfeldt-Jakob

disease (CJD) in the UK population if the BSE epidemic were to

spread to humans. Prospective epidemiological techniques

would be unlikely to demonstrate any such minor contribution

that blood transfusion might make to CJD incidence.. 0.

38. Decruyenaere, M.; Evers Kiebooms, G.; Boogaerts, A.; Cassiman, J.

J.; Cloostermans, T.; Demyttenaere, K.; Dom, R.; Fryns, J. P.;

Van, den Berghe H. Prediction of psychological functioning one

year after the predictive test for Huntington's disease and

impact of the test result on reproductive decision making. J-

Med-Genet. 1996 Sep; 33(9): 737-43; ISSN: 0022-2593.

ENGLAND. For people at risk for Huntington's disease, the

anxiety and uncertainty about the future may be very

burdensome and may be an obstacle to personal decision

making about important life issues, for example, procreation.

For some at risk persons, this situation is the reason for

requesting predictive DNA testing. The aim of this paper is

two-fold. First, we want to evaluate whether knowing one's

carrier status reduces anxiety and uncertainty and whether it

facilitates decision making about procreation. Second, we

endeavour to identify pretest predictors of psychological

adaptation one year after the predictive test (psychometric

evaluation of general anxiety, depression level, and ego

strength). The impact of the predictive test result was

assessed in 53 subjects tested, using pre- and post-test

psychometric measurement and self-report data of follow up

interviews. Mean anxiety and depression levels were

significantly decreased one year after a good test result; there

was no significant change in the case of a bad test result. The

mean personality profile, including ego strength, remained

unchanged one year after the test. The study further shows

that the test result had a definite impact on reproductive

decision making. Stepwise multiple regression analyses were

used to select the best predictors of the subject's post-test

reactions. The results indicate that a careful evaluation of

pretest ego strength, depression level, and coping strategies

may be helpful in predicting post-test reactions,

independently of the carrier status. Test result (carrier/ non-

carrier), gender, and age did not significantly contribute to the

prediction. About one third of the variance of post-test

anxiety and depression level and more than half of the variance

of ego strength was explained, implying that other

psychological or social aspects should also be taken into

account when predicting individual post-test reactions.

39. DeMarchi, J. M.; Caskey, C. T.; Richards, C. S. Population-specific

screening by mutation analysis for diseases frequent in

Ashkenazi Jews. Hum-Mutat. 1996; 8(2): 116-25; ISSN: 1059-

7794.

UNITED-STATES. We describe a partially automated DNA

mutation assay for detecting the most frequent mutations in

the alpha-subunit of beta-hexosaminidase A, the acid beta-

glucosidase and the cystic fibrosis transmembrane

conductance regulator genes for the Ashkenazi Jewish

population. The assay detects carriers for Tay-Sachs disease,

Gaucher disease, and cystic fibrosis with sensitivities of at

least 92%, 96%, and 97%, respectively. Among 1,364 young

adults of Ashkenazic ancestry in the Dor Yeshurim community

who were tested, 52 were Tay-Sachs carriers, 110 were

Gaucher carriers, and 62 were cystic fibrosis carriers. Ten

individuals were carriers for two diseases, and three

unsuspected cases were diagnosed with Gaucher disease based

on mutation test results. In addition to Tay-Sachs mutation

data, results for hexosaminidase A activity were also

available. All of 1,254 samples normal by enzyme quantitation

were also negative for the three alpha-subunit mutations

tested, and all of 43 samples with 'inconclusive' enzyme

results were negative by DNA. Only 52 of 67 samples positive

by enzyme assay were also positive for one of the three

mutations tested for Tay-Sachs disease. The data suggest a

high degree of false positivity inherent in enzyme

identification of carriers. There are no correlative methods to

assess the sensitivity of Gaucher and CF carrier testing. The

results show that population screening can be carried out

efficiently by DNA analysis, with the accrual of carrier

information for three separate diseases conducted as a single

test. Furthermore, the DNA method for Tay-Sachs screening

appears to exceed the specificity of hexosaminidase A enzyme

testing.. EC 3.2.1.52; 0.

40. Deriu, F.; Roatta, S.; Grassi, C.; Urciuoli, R.; Micieli, G.; Passatore,

M. Sympathetically-induced changes in microvascular cerebral

blood flow and in the morphology of its low-frequency waves.

J-Auton-Nerv-Syst. 1996 Jun 10; 59(1-2): 66-74; ISSN: 0165-

1838.

NETHERLANDS. The effect of bilateral cervical sympathetic

nerve stimulation on microvascular cerebral blood flow,

recorded at various depths in the parietal lobe and in ponto-

mesencephalic areas, was investigated by laser-Doppler

flowmetry in normotensive rabbits. These areas were chosen

as representative of the vascular beds supplied by the carotid

and vertebro-basilar systems, which exhibit different degrees

of sympathetic innervation, the former being richer than the

latter. Sympathetic stimulation at 30 imp/s affects cerebral

blood flow in 77% of the parietal lobe and in 43% of the ponto-

mesencephalic tested areas. In both cases the predominant

effect was a reduction in blood flow (14.7 +/- 5.1% and 4.1 +/-

2.4%, respectively). The extent of the reduction in both areas

was less if the stimulation frequency was decreased.

Sometimes mean cerebral blood flow showed a small and

transient increase, mainly in response to low-frequency

stimulation. The morphology was analysed of low-frequency

spontaneous oscillations in cerebral blood flow, attributed to

vasomotion. Present in 41% of the tested areas (frequency 4-

12 cycles/min, peak-to-peak amplitude 10-40% of mean

value), these waves decreased in amplitude and increased in

frequency during sympathetic stimulation, irrespective of

changes in mean flow. The possibility has been proposed that

the sympathetic action on low-frequency spontaneous

oscillations may contribute to the protective influence that

this system is known to exert on the blood-brain barrier in

hypertension.

41. Desgranges, B.; Eustache, F.; Rioux, P.; de, La Sayette V.;

Lechevalier, B. Memory disorders in Alzheimer's disease and

the organization of human memory. Cortex. 1996 Sep; 32(3):

387-412; ISSN: 0010-9452.

ITALY. The Squire and Zola-Morgan parallel organization model

of the memory and the Tulving hierarchical model were

developed mainly through the study of amnesic patients. The

predictions of these two models are different, the first being

more open to double dissociations and less restrictive than the

second. Alzheimer's Disease is characterized by a differential

impairment of the memory systems and by an interindividual

variability which may take the form of dissociations between

preserved and disturbed abilities in some patients. The

objective of this study was to use the memory dysfunctions of

patients with AD to test the validity of the two models.

Analysis of the group data provided an average profile of

memory disturbance consistent both with much of the data

given in AD literature and with the two models. Using a

multiple single-case strategy, we demonstrated several

simple dissociations which are for the greater part compatible

with the two models. Two of the dissociations underline the

limits of the Tulving model, which otherwise accounts for a

lot of results. The study supports the relevance of AD for the

understanding of the cognitive architecture of the human

memory.

42. Domachowske, J. B.; Cunningham, C. K.; Cummings, D. L.; Crosley, C.

J.; Hannan, W. P.; Weiner, L. B. Acute manifestations and

neurologic sequelae of Epstein-Barr virus encephalitis in

children. Pediatr-Infect-Dis-J. 1996 Oct; 15(10): 871-5; ISSN:

0891-3668.

UNITED-STATES. BACKGROUND: Complications of Epstein-Barr

virus (EBV) infection are diverse and include a number of

neurologic manifestations such as meningitis,

meningoencephalitis, cerebellitis, cranial neuritis and others.

In general encephalitis caused by EBV in pediatric patients has

been considered a self-limited illness with few or no

sequelae. METHODS: Charts were reviewed from all patients <

18 years of age admitted to or discharged from the State

University of New York Health Science Center at Syracuse

between 1982 and 1992 with a diagnosis of encephalitis or

meningo-encephalitis. Eleven cases of EBV encephalitis

diagnosed during a 10-year period were reviewed to

characterize the clinical and laboratory findings in the acute

setting and the extent of neurologic sequelae on follow-up.

RESULTS: Acute neurologic manifestations were diverse and

included combative behavior (55%), seizures (36%), headache

(36%) and evidence of focal involvement (27%). Classic

findings of infectious mononucleosis were noted infrequently;

18% each had pharyngitis, adenopathy, positive heterophile

antibody tests or atypical lymphocytosis. Two patients (18%)

had abnormal neuroimaging studies, one in the acute stage and

the other at the time of follow-up. Seven patients (64%) had

abnormal electroencephalograms (EEGs) in the acute setting;

of these three had persistent abnormalities on follow-up.

Forty percent developed persistent neurologic abnormalities

including global impairment, perseverative autistic-like

behavior and persistent left upper extremity paresis.

CONCLUSIONS: Classic signs, symptoms and laboratory findings

in infectious mononucleosis may be absent in Epstein-Barr

virus encephalitis. Neurologic sequelae occur in a substantial

number of patients.

43. Doorduijn, J. K.; van, Lom K.; Lowenberg, B. Eosinophilia and

granulocytic dysplasia terminating in acute myeloid leukaemia

after 24 years. Br-J-Haematol. 1996 Dec 1; 95(3): 531-4; ISSN:

0007-1048.

ENGLAND. Eosinophilia of variable duration, and subsequent

progression to granulocytic sarcoma and acute myeloid

leukaemia, has been infrequently reported in the literature. We

report a patient with eosinophilia and normal cytogenetics

who, after 24 years, showed transformation to a granulocytic

sarcoma of the brain. Haematological counts were normal but

the marrow revealed the cytogenetic abnormality trisomy 8 in

25% of mitoses. Subsequently an AML-M2 developed, showing a

complex karyotype including the trisomy 8 in all metaphases.

FISH analysis combined with cytological examination

identified the trisomy 8 in blasts, eosinophils and dysplastic

granulocytes only. Thus progressive leukaemic transformation

selectively involved the myeloid compartment.

44. Drago, J.; Gerfen, C. R.; Westphal, H.; Steiner, H. D1 dopamine

receptor-deficient mouse: cocaine-induced regulation of

immediate-early gene and substance P expression in the

striatum. Neuroscience. 1996 Oct; 74(3): 813-23; ISSN: 0306-

4522.

UNITED-STATES. Psychomotor stimulants such as cocaine

alter gene expression in neurons of the striatum. Whereas

many of these effects are mediated by D1 dopamine receptors,

the involvement of other dopamine receptor subtypes or

neurotransmitters is likely. To distinguish between these

possibilities, regulation by cocaine of immediate-early genes

and genes encoding neuropeptides was analysed in mice that

lack functional D1 receptors. Gene expression was examined

with in situ hybridization histochemistry. In these animals,

cocaine failed to induce the immediate-early genes c-fos and

zif 268. In contrast, substance P expression was abnormally

increased by this drug. These results demonstrate that some of

the effects of cocaine on gene regulation are mediated via D1

receptor-dependent mechanisms, as evidenced by the absence

of immediate-early gene induction in D1-deficient mice,

whereas others also involve additional, non-D1 receptor

mechanisms, as shown for substance P expression.. 0; 0; 0; 0;

0; 0; 0; 0; 33507-63-0; 50-36-2; 74913-18-1.

45. Durand, P.; Debray, D.; Devictor, D. [Fulminant hepatitis in

children]. Les hepatites fulminantes de l'enfant. Presse-Med.

1996 Oct 19; 25(31): 1501-6; ISSN: 0755-4982.

FRANCE. Viral hepatitis are one of the main causes of

fulminant hepatic failure in children. Other causes of

fulminant hepatic failure include metabolic diseases,

especially in neonates and in infants, toxin-or drug-induced

liver injury, hematologic and immune diseases. The

spontaneous prognosis of fulminant hepatitis is poor with an

overall mortality reaching 80%. The management of children

with fulminant hepatic failure has been significantly

transformed by emergency orthotopic liver transplantation.

With this procedure, 1-year survival rates range from 55 to

80%. However, emergency liver transplantation still

encounters serious problems including patient selection, organ

availability, and the definition of optimum preoperative

management of these patients. Several surgical innovations

have been developed to resolve the shortage in liver grafts

such as liver graft reduction or splitting for transplantation in

two recipients. Because, spontaneous recovery remains

unpredictable, new techniques are proposed such as the

temporary orthotopic transplantation of auxiliary liver grafts,

xenografts with monkey or pigs livers, hepatic assistance and

bioartificial livers. These evolving strategies are currently

under evaluation. Preventive treatment is one of the most

effective approaches to fulminant hepatitis.

46. Eeg Olofsson, O.; Bergstrom, T.; Osterland, C. K.; Andermann, F.;

Olivier, A. Epilepsy etiology with special emphasis on immune

dysfunction and neurovirology. Brain-Dev. 1995; 17 Suppl: 58-

60; ISSN: 0387-7604.

NETHERLANDS. The etiology of epilepsy remains in most cases

an enigma. Based on the finding of a genetically dependent

immune dysfunction in focal epilepsies, brain specimens from

16 patients, ranging in age from 6 to 39 years and operated on

for therapy-resistant focal, mainly temporal lobe epilepsy

were analyzed for the presence of viral DNA. The PCR

technique was used for detection of viral DNA from the herpes

virus group. HSV-1 was found in 44% CMV in 50%, and HHV-6 in

25%. Three patients were positive for more than one of these

viruses. The control material, consisting of only 4 brain tissue

samples, showed DNA from HSV-1 in one case. Until more brain

samples from controls have been examined, caution must be

taken before a viral etiology is applied to focal epilepsy.. 0.

47. Elcuaz, R.; Bordes, A.; Aladro, Y.; Garcia, A.; Perera, A.; Valle, L.;

Canas, F.; Lafarga, B. [Clinical characteristics and

epidemiologic study of a listeriosis outbreak in Grand Canary].

Caracteristicas clinicas y estudio epidemiologico de un brote

de listeriosis en Gran Canaria. Enferm-Infecc-Microbiol-Clin.

1996 Aug; 14(7): 416-21; ISSN: 0213-005X.

SPAIN. BACKGROUND AND METHODS: Human infections caused

by Listeria monocytogenes often present as sporadic cases

without any epidemiological relationship among them; however

they also appear as outbreaks that are usually detected by an

increase in the number of cases diagnosed by hospitals of the

geographic area. Between December 1991 and May 1993,

twenty four cases of listeriosis were detected in three

hospitals of Las Palmas de Gran Canaria; and they were

classified as an outbreak. Our report describes its clinical,

epidemiological and microbiological aspects. RESULTS AND

CONCLUSIONS: Twenty four cases of listeriosis were

diagnosed, 12 occurred in pregnant women or neonates (5 and 7

respectively) and 12 in non pregnant adults. All adult

infections were community-acquired. The incidence rate was,

for the epidemic area, 76.3 cases per million population during

the period considered (18 months). Among non pregnant adults,

9/12 patients had some underlying disease and 9/12 presented

CNS affection (meningitis and/or cerebritis). In the group of

pregnant women, 4 cases occurred in the second trimester and

fetal loss was caused; one case was detected in the third

trimester and four weeks later the patient delivered an

unaffected infant. All cases of neonatal listeriosis presented

as early-onset sepsis. Of the 24 strains of L. monocytogenes,

21 were serotype 4, two were serotype 1 and one was not

typeable. Strains from 12 patients were available for

epidemiological analysis, seven of which corresponded to the

same pattern and there were three more different patterns.

48. Endo, K.; Sasaki, H.; Wakisaka, A.; Tanaka, H.; Saito, M.; Igarashi,

S.; Takiyama, Y.; Sanpei, K.; Iwabuchi, K.; Suzuki, Y.; Onari, K.;

Suzuki, T.; Weissenbach, J.; Weber, J. L.; Nomura, Y.; Segawa, M.;

Nishizawa, M.; Tsuji, S. Strong linkage disequilibrium and

haplotype analysis in Japanese pedigrees with Machado-Joseph

disease. Am-J-Med-Genet. 1996 Sep 20; 67(5): 437-44; ISSN:

0148-7299.

UNITED-STATES. To identify the markers tightly linked to

Machado-Joseph disease (MJD) and to investigate whether a

limited number of ancestral chromosomes are shared by

Japanese MJD pedigrees, a detailed linkage analysis employing

D14S55, D14S48, D14S67, D14S291, D14S280, AFM343vf1,

D14S81, D14S265, D14S62, and D14S65 was performed. The

results of multipoint linkage analysis as well as detection of

critical recombination events indicate that the gene for MJD is

localized in a 4-cM region between D14S280-D14S81. We

found strong linkage disequilibria at AFM343vf1 and D14S81,

and association of a few common haplotypes with MJD. These

results indicate that there is an obvious founder effect in

Japanese MJD and suggest the possibility of the existence of

predisposing haplotypes which are prone to expansions of CAG

repeats.. 0; 0; 0.

49. Ericsson, K.; Hilleras, P.; Holmen, K.; Winblad, B. Human-figure

drawing (HFD) in the screening of cognitive impairment in old

age. J-Med-Screen. 1996; 3(2): 105-9; ISSN: 0969-1413.

ENGLAND. OBJECTIVE: The aim of the study is to test the

hypothesis that freehand human-figure drawing (HFD), can be

used as a complementary screening instrument to

differentiate between demented elderly people and healthy

elderly controls in population based studies. METHOD: HFD was

examined in 668 elderly ( > or = 75 years of age) participants

from an epidemiological study in Stockholm, who were asked

to draw a human figure. The drawings were analysed on the

content of body details and structural characteristics. RESULT:

The results show quite clearly that the body details and the

height decrease with decreasing cognitive function, whereas

the centredness (the distance in cm from the centre of the

figure to the centre of the paper) increases with decreasing

cognitive functioning. Demented people place their figures in

the upper left corner of the sheet, compared with the mostly

well centred figures of non-demented people. Age, on the other

hand, has an influence on the HFD as after 90 years of age most

of the variables show regressive changes. CONCLUSION: The

HFD can help to differentiate between demented and non-

demented subjects as well as between dementia of different

severity. The HFD does not help us, however, to discriminate

between Alzheimer's disease and vascular dementia. Age has

an influence on the HFD in the sense that after 90 years most

of the variables regress to a smaller or more primitive form.

50. Esler, W. P.; Stimson, E. R.; Ghilardi, J. R.; Lu, Y. A.; Felix, A. M.;

Vinters, H. V.; Mantyh, P. W.; Lee, J. P.; Maggio, J. E. Point

substitution in the central hydrophobic cluster of a human

beta-amyloid congener disrupts peptide folding and abolishes

plaque competence. Biochemistry. 1996 Nov 5; 35(44): 13914-

21; ISSN: 0006-2960.

UNITED-STATES. Alzheimer's disease (AD) is pathologically

characterized by the presence of numerous insoluble amyloid

plaques in the brain composed primarily of a 40-43 amino acid

peptide, the human beta-amyloid peptide (A beta). The process

of A beta deposition can be modeled in vitro by deposition of

physiological concentrations of radiolabeled A beta onto

preexisting amyloid in preparations of unfixed AD cerebral

cortex. Using this model system, it has been shown that A beta

deposition is biochemically distinct from A beta aggregation

and occurs readily at physiological A beta concentrations, but

which regions and conformations of A beta are essential to A

beta deposition is poorly understood. We report here that an

active congener, A beta (10-35)-NH2, displays time

dependence, pH-activity profile, and kinetic order of

deposition similar to A beta (1-40), and is sufficiently soluble

for NMR spectroscopy in water under conditions where it

actively deposits. To examine the importance of the central

hydrophobic cluster of A beta (LVFFA, residues 17-21) for in

vitro A beta deposition, an A beta (10-35)-NH2 analog with a

single point substitution (F19T) in this region was synthesized

and examined. Unlike A beta (10-35)-NH2, the F19T analog was

plaque growth incompetent, and NMR analysis indicated that

the mutant peptide was significantly less folded than wild-

type A beta. These results support previous studies suggesting

that the plaque competence of A beta correlates with peptide

folding. Since compounds that alter A beta folding may reduce

amyloid deposition, the central hydrophobic cluster of A beta

will be a tempting target for structure-based drug design

when high-resolution structural information becomes

available.. 0; 0.

51. Exhenry, C.; Nadal, D. Vertical human immunodeficiency virus-1

infection: involvement of the central nervous system and

treatment. Eur-J-Pediatr. 1996 Oct; 155(10): 839-50; ISSN:

0340-6199.

GERMANY. Involvement of the central nervous system (CNS)

contributes substantially to morbidity and mortality of

vertical infection with the human immunodeficiency virus

(HIV)-1. The clinical spectrum ranges from minor

developmental disabilities to severe and progressive

encephalopathy. Progression of the disease varies

considerably. Both direct viral and indirect host-related

pathogenic mechanisms have been proposed. The diagnosis

depends on neurological and neurodevelopmental assessments.

So far, HIV-1-specific antiviral treatment has shown limited

effects on neurological manifestations in symptomatic

children. Thus, efforts are needed to improve prevention and

treatment of CNS involvement. It is still unclear whether early

use of antiretroviral agents is of benefit. CONCLUSION: Since

experience of treatment of HIV-1 infections in adults cannot

easily be translated to children, paediatric clinical trials are

needed to answer questions specific to the unique

characteristics of children.. 0.

52. Feucht, H. H.; Fischer, L.; Sterneck, M.; Broelsch, C. E.; Laufs, R. GB

virus C transmission by blood products [letter]. Lancet. 1997

Feb 8; 349(9049): 435; ISSN: 0140-6736.

ENGLAND.

53. Fleck, L. M. Just caring: the moral and economic costs of APOE

genotyping for Alzheimer's disease. Ann-N-Y-Acad-Sci. 1996

Dec 16; 802: 128-38; ISSN: 0077-8923.

UNITED-STATES. 0; 0.

54. Forss, N.; Merlet, I.; Vanni, S.; Hamalainen, M.; Mauguiere, F.; Hari,

R. Activation of human mesial cortex during somatosensory

target detection task. Brain-Res. 1996 Sep 23; 734(1-2): 229-

35; ISSN: 0006-8993.

NETHERLANDS. We recorded somatosensory evoked fields

(SEFs) from 10 healthy subjects to ulnar and median nerve

stimuli presented at random intervals of 2.4-21.6 s. The

subjects either counted the stimuli or ignored them by reading

a book. The stimuli activated in both conditions the

contralateral SI cortex, the ipsi- and contralateral SII

cortices, and the posterior parietal cortex (PPC), in line with

earlier observations. In addition, a novel response was

observed in nine subjects at 120-160 ms. It was clearly

enhanced by attention and was generated in the mesial cortex

of the paracentral lobule, close to the end of the central

sulcus.

55. Fujita, N.; Suzuki, K.; Vanier, M. T.; Popko, B.; Maeda, N.; Klein, A.;

Henseler, M.; Sandhoff, K.; Nakayasu, H.; Suzuki, K. Targeted

disruption of the mouse sphingolipid activator protein gene: a

complex phenotype, including severe leukodystrophy and wide-

spread storage of multiple sphingolipids. Hum-Mol-Genet. 1996

Jun; 5(6): 711-25; ISSN: 0964-6906.

ENGLAND. The four established or putative sphingolipid

activator proteins derive from a large precursor protein

encoded by a single gene. In addition to generating the four

sphingolipid activator proteins, the precursor protein is

suspected of having functions of its own, as, for example, a

lipid binding/transport protein or a neurotrophic factor. The

gene also appears to encode the Sertoli cell major sulfated

glycoprotein. Sequence similarities have been noted with many

other proteins of diverse functions. One patient and a fetus in

a single family with a complete defect of this gene due to a

mutation in the initiation codon exhibited complex

pathological and biochemical abnormalities. Mutant mice

homozygous for an inactivated gene of the sphingolipid

activator protein precursor exhibit two distinct clinical

phenotypes-neonatally fatal and later-onset. The latter

develop rapidly progressive neurological signs around 20 days

and die by 35-38 days. At 30 days, severe hypomyelination and

periodic acid-Schiff-positive materials throughout the

nervous system and in abnormal cells in the liver and spleen

are the main pathology. Most prominently lactosylceramide,

and additionally ceramide, glucosylceramide,

galactosylceramide, sulfatide, and globotriaosylceramide are

abnormally increased in the brain, liver, kidney, and their

catabolism abnormally slow in cultured fibroblasts. Brain

gangliosides are generally increased, particularly the

monosialogangliosides. The clinical, pathological and

biochemical phenotype closely resembles that of the human

disease. This model not only allows further clarification of

the physiological functions of the four individual sphingolipid

activator proteins but also should be useful to explore

putative functions of the precursor protein.. 0; 0; 0; 0.

56. Fukuyama, H.; Yamauchi, H. [Positron emission tomography and

single photon CT in the diagnosis of cerebrovascular

disorders]. Rinsho-Shinkeigaku. 1995 Dec; 35(12): 1578-80;

ISSN: 0009-918X.

JAPAN. The positron emission tomography (PET) and single

photon emission CT (SPECT) revealed the pathophysiology of

ischemic brain in the transaxial images. There are several

limitations in PET studies by continuous inhalation method

using oxygen-15 labeled gases. There has been a suspicion

about the constancy between the red cell volume and plasma

volume in the ischemic brain. We measured plasma volume by

PET using Cu-62 labeled human serum albumin in cases of

internal carotid artery occlusion. There was no remarkable

plasma volume alterations between the ischemic and non-

ischemic hemisphere. Powers et al demonstrated the

relationships between the intracranial perfusion pressure

changes and vascular or metabolic adaptations. The important

issue in acute stage of stroke is to differentiate the truly

alive and completely destroyed tissue. Cerebral blood flow

change might be affected during acute stage by diaschisis.

Recent examination using benzodiazepine receptor imaging by

SPECT might have a potential to depict the damaged tissues,

because cortical neuronal cell bodies have a lot of the

receptors and an ischemic insult induces the loss of the

receptors. The relationship of crossed cerebellar

hypoperfusion (CCH) and supratentorial circulatory conditions

is attractive, because supratentorial hypometabolism of

oxygen induced more severe CCH in internal carotid artery

occlusion. Therefore, we can predict the supratentorial oxygen

metabolism by CCH in a case of internal artery occlusion. We

have a lots of tools to explore the pathophysiological states of

ischemic brain. These must be dedicated to develop the more

effective therapeutic procedures.. 0.

57. Gatev, P.; Thomas, S.; Lou, J. S.; Lim, M.; Hallett, M. Effects of

diminished and conflicting sensory information on balance in

patients with cerebellar deficits. Mov-Disord. 1996 Nov; 11(6):

654-64; ISSN: 0885-3185.

UNITED-STATES. We studied the effects of altered sensory

information on standing balance in 25 patients with cortical

cerebellar atrophy (CCA), nine patients with olivoponto-

cerebellar atrophy (OPCA), and 10 normal subjects. The total

sway path and its components, the anteroposterior (AP) sway

path and the lateral sway path, were measured under six

conditions: (1) standing on a fixed platform with the eyes open

and visual surroundings fixed, (2) standing on a fixed platform

with the eyes closed, (3) standing on a fixed platform with the

eyes open and visual surroundings AP sway referenced, (4)

standing on an AP sway-referenced platform with the eyes

open and visual surroundings fixed, (5) standing on an AP

sway-referenced platform with the eyes closed, and (6)

standing on an AP sway-referenced platform with the eyes

open and visual surroundings AP sway referenced. Patients

swayed more than normal subjects during normal stance

(condition 1), when the visual information was absent

(condition 2) or distorted (condition 3), and when the

proprioceptive information from the ankles was distorted

(condition 4). Patients swayed much more than normal, and

most fell, when two sensory modalities were affected under

condition 5 (proprioceptive information distorted and visual

information absent) and condition 6 (both proprioceptive

information and visual information distorted). When the

patients' sway was normalized to that of the first condition,

however, only their lateral sway was greater than the sway in

normal subjects. Unlike in normal subjects, the patients'

lateral sway varied with the AP sway to approximately the

same degree in each condition for conditions 1-5. Clinical

ratings of gait and balance were highly correlated with the

sway measures. Quantitative testing of standing balance with

altered sensory information has better sensitivity than normal

stance testing.

58. Gentilini, P.; Laffi, G.; La Villa, G.; Casini Raggi, V.; Romanelli, R.

G.; Buzzelli, G.; Mazzanti, R.; Marra, F.; Pinzani, M.; Zignego, A. L.

[Viral liver cirrhosis: natural course, pathogenesis and clinical

implications of the complications]. La cirrosi epatica virus-

correlata: storia naturale, patogenesi e implicazioni cliniche

delle complicanze. Ann-Ital-Med-Int. 1996 Oct; 11 Suppl 2:

23S-29S; ISSN: 0393-9394.

ITALY. The role of hepatitis B virus (HBV) and hepatitis C

virus (HCV) as a major cause of chronic liver disease is now

accepted worldwide. This study was aimed at evaluating the

natural history of the disease in patients with virus-induced

chronic active hepatitis or cirrhosis, and the influence played

by age, sex and etiology, liver function tests and by the

occurrence of different complications. We retrospectively

examined the clinical records of 506 inpatients: 194 were

affected by chronic active hepatitis (125 males, 69 females,

mean age 45 +/- 11 years, 146 HCV- and 48 HBV-related), and

312 by cirrhosis without clinical evidence of portal

hypertension (178 males, 134 females, mean age 53 +/- 9

years, 249 HCV- and 63 HBV-related). The occurrence of

cirrhosis in the chronic active hepatitis group was then

calculated, together with the occurrence of complications and

the cumulative mortality rate of established cirrhosis. During

follow-up 93 patients with chronic hepatitis developed

cirrhosis. The cumulative probability of developing cirrhosis

in this group was 6.64% at 5 years, 56.1% at 10 years and

86.8% at 15 years. These patients were therefore included in

the cirrhosis group for the final analysis, so that a total of

405 cirrhotic patients were evaluated: these patients had a

cumulative survival rate of 99.1% at 5, 76.8% at 10 and 49.4%

at 15 years. Comparing the age-adjusted death rate of our

patients with the general Italian population, we observed that

in patients with liver cirrhosis it was 3.14 and 2.84 times

higher in men and women, respectively. Bilirubin was an

independent indicator of survival. Several complications, such

as esophageal varices, ascites, jaundice, hemorrhage, hepatic

encephalopathy and hepatocellular carcinoma significantly

reduced the survival rate and were indicated as major

complications, while thrombocytopenia, cholelithiasis and

diabetes did not affect survival and thus were called minor

complications. Incidence of hepatocellular carcinoma was very

high especially in males, without correlation with etiology. In

conclusion, the progression of virus-induced chronic active

hepatitis to cirrhosis is not influenced by sex and etiology.

Similarly, the different etiology does not modify the natural

history of cirrhosis while the occurrence of one or more major

complications significantly shortens survival. The longer

survival rate observed in patients with cirrhosis included in

this study is probably due to the selective inclusion of

patients with early disease and no evidence of portal

hypertension.

59. Gettler, J. F.; Garner, B. F. Long-term survival of an AIDS patient

with a tuberculous cerebral abscess. J-Natl-Med-Assoc. 1996

Sep; 88(9): 605-6; ISSN: 0027-9684.

UNITED-STATES. Unlike other forms of tuberculosis,

tuberculous cerebral abscess is a rare complication of human

immunodeficiency virus (HIV) infection and usually presents at

a late stage of the disease. This article describes a case of

tuberculous cerebral abscess in an HIV-infected patient that

was effectively treated with surgery and chemotherapy. The

patient has survived more than 5 1/2 years since being

diagnosed and remains in good health.. 0.

60. Gilissen, E.; Zilles, K. The calcarine sulcus as an estimate of the

total volume of human striate cortex: a morphometric study of

reliability and intersubject variability. J-Hirnforsch. 1996;

37(1): 57-66; ISSN: 0021-8359.

GERMANY. The human primary visual cortex consists of a

region buried in the calcarine sulcus and a region outside this

sulcus on the free surface of the occipital lobe. Since the

depth of the calcarine sulcus can be easily estimated in

magnetic resonance images of the living human brain, in vivo

morphometry of the human primary visual cortex would be

feasible for studying development, intersubject variability and

interhemispheric asymmetry if the sulcal depth or a

correlated measure such as the intracalcarine surface area

would be a precise and reliable estimate of the total volume of

the human primary visual cortex. The correlations between

total volume of the striate cortex and its intra- and extra-

calcarine surface areas were therefore tested in the present

observations. The total volume of the striate cortex and the

surface areas of its intra-and extracalcarine portions were

measured in Nissl-stained serial sections through 20 adult

human hemispheres. The intra- and extracalcarine portions of

the striate area are not significantly correlated with each

other, but correlated with the total volume of the striate

cortex. The intracalcarine surface area or the depth of the

calcarine sulcus are thus useful parameters for in vivo

estimates of the total size of the striate cortex.

61. Gitelman, D. R.; Alpert, N. M.; Kosslyn, S.; Daffner, K.; Scinto, L.;

Thompson, W.; Mesulam, M. M. Functional imaging of human

right hemispheric activation for exploratory movements. Ann-

Neurol. 1996 Feb; 39(2): 174-9; ISSN: 0364-5134.

UNITED-STATES. We employed positron emission tomography

to examine the functional anatomy of the exploratory-motor

aspect of spatial attention. Subjects moved their right hand

under nonexploratory (control) versus exploratory (active)

conditions. Movement architecture and eye movements were

matched across conditions. Statistical parametric maps of the

exploratory (active) minus nonexploratory (control) tasks in

subjects using their right hand in the right hemispace

demonstrated activation in right cingulate, premotor and

posterior parietal areas. The net activation of multiple right

hemisphere cortical areas ipsilateral to the active limb and

hemispace strongly supports the predicted, distributed

network anatomy and is consistent with possible right

hemispheric dominance for exploratory attentional movements.

62. Gotow, T.; Sakata, M.; Funakoshi, T.; Uchiyama, Y. Preferential

localization of annexin V to the axon terminal. Neuroscience.

1996 Nov; 75(2): 507-21; ISSN: 0306-4522.

UNITED-STATES. To examine the participation of annexin V, a

member of Ca(2+)-dependent phospholipid-binding proteins, in

the process of synaptic vesicle exocytosis, rat central nervous

tissue was analysed using biochemical and morphological

techniques. By both fluorescence and confocal laser scanning

microscopy, immunoreactivity for annexin V was

predominantly localized around neuronal somata and dendrites,

and the reactivity was mostly co-labeled with that for

synaptophysin. The annexin V immunoreactivity was also

detectable, but less intensely, in neuronal perikarya, glial

cells and endothelial cells. Both immunoblot and

immunoelectron microscopic analyses with intact tissues,

synaptosomes and purified synaptic vesicles showed that

annexin V was expressed in neurons, preferentially

concentrated in axon terminals and associated with synaptic

vesicles. Purified synaptic vesicles were relatively

homogeneously distributed in the medium where Ca2+ was

removed and thus the amount of annexin V was reduced

drastically. The vesicles tended to be clustered in the fraction

where endogenous annexin V is maintained, and the clusters

were more conspicuous when purified human annexin V was

added. Synaptic vesicles forming the clusters were not

directly fused with each other but separated by a 10-15 nm

gap that corresponded well with the size of single annexin V

molecules. In axon terminals, globular structures 12-13 nm in

diameter, similar in dimension to annexin V molecules, were

distinctly found to be attached to the cytoplasmic surface of

both vesicle membranes when the two vesicles were close to

each other. These results suggest that annexin V belongs to the

group of synaptic vesicle-associated proteins. Although its

localization and significance in non-neuronal cells were not

analysed here, at least in the axon terminal annexin V may

participate in the cluster formation of synaptic vesicles by

linking with the cytoplasmic surface of the vesicles in a

Ca(2+)-dependent manner.. 0.

63. Griffiths, P. D.; Crossman, A. R. Autoradiography of transferrin

receptors in the human brain. Neurosci-Lett. 1996 Jun 14;

211(1): 53-6; ISSN: 0304-3940.

IRELAND. Transferrin is the major protein concerned with iron

transport in the serum and may provide a route by which iron

enters the brain. This study was designed to show the optimal

binding conditions for in vitro transferrin receptor

autoradiography and to show the regional distribution of

transferrin receptors in the human brain. Optimal binding

conditions were: 120 min incubation with 5.0 nM 125I-

transferrin at 37 degrees C. Transferrin receptors degrade

quickly even with storage at -70 degrees C, therefore binding

studies should be performed within 7 days post mortem.

Transferrin receptors were widely distributed in the human

brain, with high density in the neocortex, moderate densities

in the putamen and caudate nuclei, and very low densities in

the globus pallidus and substantia nigra. Therefore transferrin

receptor density shows a mismatch with the known

distribution of iron in the human brain. The presence and

distribution of transferrin receptors in the human brain are

important because they may provide a route to deliver

lipophobic substances across the blood-brain barrier by

binding them to antibodies raised against transferrin

receptors.. 0; 0; 11096-37-0; 7439-89-6.

64. Grozdanovic, Z.; Gosztonyi, G.; Gossrau, R. Nitric oxide synthase I

(NOS-I) is deficient in the sarcolemma of striated muscle

fibers in patients with Duchenne muscular dystrophy,

suggesting an association with dystrophin. Acta-Histochem.

1996 Jan; 98(1): 61-9; ISSN: 0065-1281.

GERMANY. Previously, we have demonstrated the expression of

the brain-type nitric oxide synthase (NOS-I) in the

sarcolemmal region of somatic and visceral striated muscle

fibers in a variety of mammalian species through the use of

enzyme histochemical and immunochemical techniques. Here

we report that NOS-I protein and its NADPH diaphorase

(NADPHd) activity are co-localized in the sarcolemma of

human skeletal muscles. NOS-I immunolabeling and NADPHd

activity showed no significant variation between type I and II

fibers. In muscle biopsy specimens from patients with

Duchenne muscular dystrophy (DMD), both NOS-I protein and

activity were absent or markedly reduced. We, therefore,

propose that NOS-I is complexed with dystrophin and/or

dystrophin-associated proteins, adding a novel member to the

sarcolemmal dystrophin-glycoprotein complex (DGC). The

nature of the NOS-I-DGC link, and its role in skeletal muscle

physiology and pathophysiology remain to be elucidated.. EC

1.14.13.39; EC 1.6.99.1; 0.

65. Gudovic, R.; Milutinovic, B. Regression changes in inferior olivary

nucleus compared to changes of Purkinje cells during

development in humans. J-Hirnforsch. 1996; 37(1): 67-72;

ISSN: 0021-8359.

GERMANY. Human fetal brains were investigated in order to

establish the regressional process in two directly dependent

structures: in the inferior olivary nucleus and in cerebellar

Purkinje cells. Inferior olivary nucleus is the main origin of

the climbing fibres, that form one of two direct input systems

into the cerebellum. For the purpose 25 fetal human brains at

various stages of development (12.5, 15, 16.5 19.5 24, and 31

postovulatory week), together with one brain of newborn (in

further text: 41. postovulatory week) were used. Numerical

density of nuclei in both regions was determined by

stereological technique. In both structures appeared a

reduction in the number of nuclei, showing specific dynamics

during the intrauterine maturation. The data obtained were

analyzed by graphical and numerical method, that allowed the

formation of the adequate mathematical models for both

structures, that precisely described their regression changes.

The data presented indicate that the reduction is very

pronounced until the 18-19 postovulatory week, and after that

time it is remarkable slowed down. Tha