Irish Neurological association

Irish Neurological Association Proceedings of 28th Annual Meeting, Beaumont Hospital, Dublin, 15tb-16th May, 1992

COMPOUND SKULL BASE FRACTURE: THE CASE FOR SURGICAL REPAIR

D. E. Sakas, G. O'Connor, J. Singh, L. Dins Department of Nonrosurgety, Beaumont Hospital, Dublin 9.

Skull base fractures are potentially compounded, and induce a high risk ofmenlngitis, regardless of the presence of CSF fistula. We present our experience with patients, on whom we performed cranial floor repair, following severe head injury. All patients had skull base fracture with either significant bone displacement involving the air sinuses, or were onmplicatedby meningitis, CSF Fistula, or aerocele. Coronal c ' r Scan with thin cuts demonstrate adequately the extent of the damage, and if it shows significant disruption of the continuity of the skull base, or bone fragments protruding inwards, we conclude that an associated dural tear should exisL the cranial base be ex- plored, the dural defect repaired, and the risk of meningitis eliminated. Radiographic demonstration of an active CSF fistula with positive contrast cistemography, is not part of our investigations. The operative microscope has improved our ability to perform this operation with minimal brain retraction, dissect brain parts that have herniated through the basal dural defect, visualize and seal the defect with a pedicled pericranial graft and lyophilized tkronthin, and preserve the olfactory nerve. Post- operative morbidity and mortal ity were nil. There has been no recurrence of meningitis, or CSF leak.

Conclusion. The hesitation to perform compound skull base fracture repair following severe head injury, because of the risk of inducing further brain damage, increasing the likelihood of post traumatic epilepsy, or causing anosmia, is no longer justi fled. Recent advances in non-invasive imaging and microsorgical techniques allow us to perform this procedure safely. The surgical risk in our hands, we believe, is much smaller than the risk of meningitis from an untreated compound skull bose fracture.

ANTIBIOTIC PROPHYLAXIS AND CSF LEAKAGE: IS IT A FACT OR FICTION?

M.S. Eljamel, Senior Registrar, Department of Neurnsurgery, Beaumont Hospital, Dublin.

The use of prophylactic antibiotic treatment in patients with CSF leakage is still a controversial topic among clinicians. The aim of this study was to determine the possible value of antibiotic prophylaxis on the survival free from meningitis in these patients. The study population comprised of 215 patients, of whom 106 received prophylactic antibiotic treatment and 109 did notreceive arty antibiotics. All patients had definite CSF leakage, The antibiotic regimen consisted of 500 mgs of penicillin and 500 rags of sulphonamide, both were given 6 hourly within 3 days of the onset of CSF leak and wele continued for at least 7 days after the CSF leak has stopped. The two groups of patients were closely matched for age, sex, type and duration of CSF leakage and the presence or absence of skull fractures. However, there were more patients with pneumocephalus and facial fractures among those who recoived antibiotics, Uinvariante analysis of these two particular factors showed that they were not significant adverse prognostic factors. The Kaplan-Meyer product limit technique was used to estimate the probability of survival free from miningitis in the two groups. The survival data was analysed using the Logrank test. The survival free from menigitis in the two groups was not significandy different in the twogroups. Further-

more, the survival curves of survival free from meldngids in the two groups were almost identical in the first four weeks during which antibiotics were given. Thermmber of gram negative meningitis and partially treated cases (negative cultures) was significantly higher among those who received antibiotics.

Therefore, there is no scientific evidence to support the routine use of antibiotic prophylaxis in CSF leaks and it is ethically justifi- able to with-hold such treatment and ins tituto a vigil ant and constant observation of these patients to detect early signs of meningitis, instituting appropriate therapy should meningitis occur.

CHANGING TRENDS IN THE MANAGEMENT OF PINEAL TUMOURS - A 3 DECADE EXPERIENCE.

B. Mat,hew, B. Clements, A. Irvine, W.L Gray and D.P. Byrncs. Northern Ireland Regional Neurosurgical Unit, Royal Victoria

Hospital, Belfast.

Historically, direct surgical excision of pineal tumours has been associated with significantly high morbidity and mortality. As a result standard treatment consituted shunting of secmadary hycko- cephalus and irradiation of the primary tumour. Twenty to twenty- five percent of primary pineal tumours are either benign, encapsu- lated or radioresistant, making this approach suboptimal. Refine- ments in diagnostic imaging, cytopathology, clinical chemistry, stereotactic and microsurgery should allow us to make a definitive histological diagnosis, and design a more accurate treatment regimen.

We report our experience of primary pineal tumours in 14patients from 1959-1992. The series included 12 males and 2 females, ranging in age from 6-54 years (mean 24 years) with duration of symptoms prior to diagnosis of 7 days to 1 year (mean 10 weeks). Eight of fourteen patients had pretreatment CT scans - Histological subtype was correctly predicted in 33% of cases. Ten of fourteen has CS F cytology performed - this yielded a diagnosis in 20% of patients. Eight patients had turnout marker status recorded. All patients had a shunt procedure performed and seven underwent craniotomy and excision biopsy. There were no perioperative deatks mid minhunl morbidity, Ten of fourteen had adjuvant radiotherapy, two also had chemotherapy, All in all only nine of fourteen patients had a definitive histological diagnosis.

Whilst tumour marker status, CSF cytology and CT scan may be helpful collectively we feel ti~at an accurate tissue diagnosis. obtained directly or stereotaxy is vitalin rationalising the approach to management of these rare neoplasms,

DURAL ARTERIOVENOUS MALFORMATIONS.

I. C. Bailey, Northern Ireland Regional Neurosurgical Unit, Royal Victoria

Hospital, Belfast.

Dural arterinvenous malformations usually involve the venous sinuses along the base of the skull, and especially the transverse and sigmoid sinuses. It is estimated that they make up 10% of all intracranial A.V.M.'s. They are now being diagnosed more commonly, as selective angiography has become more sophisticated. The pathophysiology of this condition is still not clearly understood,

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but there are strong suggestions that it may be an acquired lesion, whereas cerebral arterial malformations are congenital in origin.

We have reviewed the clinical presentation and the radiological findings of four cases seen during the last four years. The presenting features were varied but included tinnitus and headache, with art audible bruit in three instmaces and massive intracerebral haernor- rhage in the fourth. A constant radiological finding was partial or complete occlusion of the involved dural sinus.

Two of the eases which involved the transverse sinus were treated by isolation and excision of the sinus, one case resolved spontane- ously, and the other was treated conservatively. The results to date have been excellent.

THE VALUE OF REPEAT ANGIOGRAPHY IN SUBARACH- NOID HAEMORRHAGE.

N. Pathirana, S. Refsum*, K.E. Bell, C.S. McKinstry. Department of Neurorediology and *Neurosurgery, Royal

Victoria Hospital, Belfast.

It is ganerally agreed that cerebral angiography should be re- peated in patieats with acute subarachnoid haemorthage (SAH) if the first study shows spasm or is incomplete. If the first study is normal or equivocal the role of a repeat angiogram is less clear. The aim of this study w as to assess the usefulness of repeat angiography in SAH.

Of 216 consecutive cases of SAH, 30 had repeat angiography. Seven aneurysms were found in 15 cases where the first study was equivocal or showed only spasm. Three aneurysms were found in 15 cases where the first study had been considered normal. No patient suffered a reblecd between the first and second studies.

In 14 further cases initial angiography was normal and no further study was planned: one patient died from a rebleed several weeks later. Finally in one case initial angiography showed art aneurysm: surgery was delayed and two repeat studies were normal. Conclusion:

Repeat angiugraphy is justified in the presence of spasm or where the initial findings are equivocal. It should also be carried out, possibly limited to a specific vessel, even if the first study is considered normal.

EPILEPSY AND EMPLOYMENT, MARTIAL, EDUCATION AND SOCIAL STATUS.

N. Callaghan, M. Crowley, T. Goggin. Department of Neurology, Cork Regional Hospital, Department of

Statistics, University College Cork.

Patients with epilepsy have difficulty achieving certain goals during life, due to emotional and economic disadvantages, which may be associated with the disease. The study was carried out to determine employment, marital, education and social status of epileptic patients attending a Seizure Clinic. This involved die overall assessment of 343 patients. Employment, educational, marital and social status were evaluated.

The social group, employment and marital status did not compare favourahly with the overallpopulation. Forty per cent in social group 5 and 6 compared with 14% of the population. Thirty three per cent of male patients aged 20 years or more were married compared with 65% in the community. The corresponding statistics for females were 46% and 73% respectively. The male unemployment rate of 34% compared poorly with the unemployment rate of 13% fur the period during which the study data was collected. A significant

475

number of the study group had not progressed to secondary or third level education, The relationships between social variables and characteristics of the patients' illness were investigated and it was found that patients with lower educational achievements were significantly more likely to have poor seizure control (P<0.00I) and to require treatment with polypharmacy (P<0.001). The results were similar in relation to employment status. Seizure conu'ol achieved by patients decreased with social grouping. The percentages with excellent control for social groups 1 to 6 were as follows- 50%, 37%, 42%, 27%, 19% and 10% respectively (P<0.001). Low marital rates may be related to social and psychological factors, together with the effects of anti-epileptic medication on hormones supporting sexual behavionr.

TEMPORAL LOBE EPILEPSY AND MENTAL CHANGE

N. MacDermott, K.J. O'Driscoll, D. Neary, N.A. Pearson, LS. Snowden, S. Brown, M. Vaughan.

Manchester Royal Inf'trmary, Oxford Road, Manchester.

Fifteen patients with temporal lobe epilepsy since childhond who had achieved normal intelligence and seconda~ education, underwent mental decline in youth, sufficient to require admission to a Centre for Epilepsy. Recurrent head trauma and status epilepsy had not occured at the time of their decline. The patients now aged 17 - 48 were investigated to determine the nature of their mental decline. Ne urological ev aluatinn assessed historic al and physical evidence of recurrant head trauma. SPET scanning determined functional/ structural cerebral change. Nenropsychology defined the characteristics of mental decline. Assessments were blind to each other.

Two major syndromes emerged. Five patients exhibited a sub- cortical dementia with neurological signs of white matter disorder and bilateral ~terior cerebral changes on SPET. There was ahistory and signs of currant head trauma. Six patients exhibited a temporal lobe syndrome or circumlocution, concrete thinking and paranoia. Neuxological signs and other evidence of head trauma were absent and SPET was normal.

Four patients presented features of both syndromes and one adoles- cent without head trauma and SPET showed bilateral anterior abnormality. Thus the mental decline in temporal lobe epilepsy that occurs in early adulthood may be a result of ftmctional/structural changes inexplicable by recurrent trauma.

CORPUS CALLOSOTOMY: LONG-TERM SURGICAL AND PSYCHOLOGICAL OUTCOME IN 16 PATIENTS.

McMackin D., Phillips J., Burke T., Murphy S., Staunton H. Richmond Institute of Neurology, Beaumont Hospital, Dublin.

Since the 1980's there has been a revival of interest in corpus callosotomy as a treatment modality for severe intractable epilepsy. In this study restdts are presented of long-term outcome (1-8) years, in 16 patients who have undergone partial or complete corpus calinsum section at the Richmond Institute. Our results indicate improvement in toxic/clonic and akinetic seizures in a significant proportion of patients (greater than 75%), with no significant im- provemertt in partialor focal seizure. Three patients withgeneralised seizures and no history of akinetic seizures were seizure free at longterm follow up. All five patients with akinetic seizures were signillcantly improved following surgery, with one patient experi- encing complete cessation of all seizure types.

Detailed neuropsyehological evaluation revealed evidence of

476 Irish Neurological Association

dis-ennaeetion syndrome in one patient only. In addition those with higher IQ did aignificenfly better than those in the moderate/severe mentally handicapped range. Despite significant medical gains, psycho-social improvements were evidenced in only three patients. These findings suggest that corpus callosotomy is a useful surgical procedure but only in a carefully selected population.

I J.MS November, 1993

We conclude that the varied clinical types of HD in N.I., and a prevalence rate comparable to v alues estimated in the rest of the U.K. and Europe, provide support for the hypothesis that HD has origi- nated from more than one original mutation, as one single mutation would have to be extremely ancient to account for the uniformity throughout Europe.

SUDDEN UNEXPLAINED DEATH IN EPILEPTIC PATIENTS (SUDEP) - A REGIONAL HOSPITAL PERSPECTIVE.

R.L. Cristea, M. Houfihane, M.A. Farrall. Richmond Institute of Neurology and Neurosurgery, Beaumont

Hospital, Dublin.

Sudden unexplained death in patients with epilepsy (SUDEP) is well recognised. Previous studies have attempted to identify high risk groups, such as black males with structural brain lesions. Absent or sub-therapeutic levels of anti-epileptic drugs are a common finding post-mortam. We retrospectively reviewed autopsy cases of sudden death in epileptics between November 1985 and January 1992 at Beaumont Hospital, Dublin, Ireland. Fourteen cases (8 males. 6 females), of unexplained, sudden death were identified in a general autopsy population of 1875. All were white, age ranged 9 - 69 years, median 30.5. The most common post-mortem findings were pulmonary oedema (9) and aspiration of gastric contents (4). Cenoral nervous system gross examination revealed transtentorial herniation (3), r septum pallucidum (2), and evidence for an old cerebral injury (1), Six patients had normal gross neuropathologic examinations. Microscopic findings revealedno abnormalities (8), neuronal migration defects (3), hypoxic ischaemic changes (2), mesial temporal sclerosis (2), and low grade astrocytoma (1). In seven patients tested thepost-murmm anti-epileptic drug levels were absent in five and sub-therapentie in two.

We believe on the basis of these findings that the most likely explanation for SUDEP is neurogenic puimonary oedema or a fatal cardiac arrhythmla, in probability phase linked to an ictal discharge.

PREVALENCE AND AGE ANALYSIS OF ONSET, DURATION, DIAGNOSIS AND DEATH IN HUNTINGTON'S DISEASE PATIENTS IN NORTHERN IRELAND - 1920 - 1992

P.L Morrison, N.C. Nevin. Department of Medical Genetics, The Queen's University of

Belfast, Northern Ireland.

Since the delineation of Huntington's Disease (HD) in 1872, Ireland has contributed to the global dissemination of HD to several p~ts of the world. No detailed studies on the epldemiology and genetics of HD in Irdaud have ever been undertaken. We have carried out an exhaustive study using multiple ascertainment sources, which provides a virtually complete ascertainment of HD in North- em Ireland (NI).

Over a 72 year period, 85 HD families with 95 living affected patients were identified. The minimum prevalence rate calculated was 6.1/100,000. Of the living cohort, 5% had late onset HD, 4% juvenile onset HD and 2% very early (childhood) onset HD. The range of age of onset was 3 -72 years. The average age of onset was 40.9 years, age at diagnosis was 44.4 years2 Age at death for patients born between 1920 and 1992 was 56.2 years with an average duration of illness of 16.4 years.

REFLECTIVE AND FRONTAL LOBE GUIDED SACCADES IN THE DIAGNOSIS OF HUNTINGDON'S DISEASE.

P.L Morrisen, A.D. Collins, M. Gibson. Department of Medical Genetics and Neurology, Royal Victoria

Hospital, Belfast.

Clinically obvious abnormalities of eye movement and gaze are well described in Huntington's disease. Patients may show head thrusts, unstable fixation, and slowed saccades. Other subclinical abnormalities may be present, but the importance of these in early diagnosis is unknown.

We have performed a clinical and laboratory study o f eye move- merits in our population. Following ascertainment 85 families, which included 95 living affected patients were identified. Of these 25 (26 %) had early signs of HD. 16 of the se 25 have been studied to date using a video protocol, and quantified analysis of 5 saccadic paradigms.

Results: Three lab recordings were abandoned due to movement artifact. Of the remainder all showed subclinical abnormalities, especially of frontally guided (anti) saeeades. Less than 50% had saccadic slowing.

Conclusion. Quantative oculomotor studies may produce early clues to the diagnosis of Huntington's disease.

DIFFERENT EFFECTS OF PERSISTENT ENTEROVIRUS INFECTIONS IN POLYMYOSITIS AND POSTVIRAL

FATIGUE SYNDROME.

W.M.H. Behan 1, K.S. Simpson 2, H.M. Cavanagh 2, J.W. Gow 2, 13. Gillesple 2, and P.O. Behan z

University of Glasgow Departments of Pathology I and Neurol- ogy 2, Glasgow.

Polymyositis (PM) is an inflammatory myopathy in which the aetiology is unknown, but in which the accumulating data strongly suggest au autoimmtm e pathogenesis. The histological picture is o f muscle fibre necrosis and inflammation, whilst in postviral fatigue syndrome (PFS), a disorder characterised by severe fatigue with myalgia an dpsychiatric symptoms, the histological picture of muscle is essentially normal. Enteroviruses have been implicated on epidemiologieal and serological studies in both. We have used the polymarase chain reaction (PCR) and an enteroviral specific probe and found persistent enteroviral genomic material in both PM and PFS muscle biopsies. Furthermore, we used a radiolabeUed full length eDNA probe derived from co xsackie E 1 in an in situ technique to look for viral DNA in PCR positive cases. Coxsackie genome was clearly identifiable in the muscle biopsy specimens of patients with PM but negative in PCR positive cases of PFS. The virus excites au inflammatory reaction only in PM. A murine animal model for PFS developed in our laberatory showed positive mucle PCR using enterovims probes and conspicuous increase in interleukin IL6 within the brain. These results provide major clues in the search for the v.etiology of these two puzzling disorders.

Vol. 162 No. II

DIRECT EFFECTS OF CYCLOSPORIN A AND CYCLO- PHOSPHAMIDE ON DIFFERENTIATION OF NORMAL

HUMAN MYOBLASTS IN CULTURE.

O. Hardiman, R.M. Skier, R.H. Brown Jr. Massachusetts General Hospital, Boston MA and University

College, Dublin, Ireland.

The direct effects of the commonly used immunosuppresaive agents cyulosporin A, eyclophospbamide and azathioprine were examined in cultures of elonally derived anenral human myablasts. When applied to cultures in doses reflecting the theraputir dose in vivo, both cycinsporln A and eyclosphosphamide had dose-related repredueible effents on myobl~t f~asion. Fesiort was enhanced by cyelophosphernide, and inhibited by eyelosporin A. These findings indicated that immunosnppressive agents may have effects on muscle that are independent of their ability to regulate the immune system.

AN ALTERED EXPRESSION OF NEURONAL CELL SUR- FACE GLYCOPROTEINS IN ALZHEIMER'S DISEASE - A

POTENTIAL DIAGNOSTIC TOOL?

A.M. Gillian, *D. O'Mabony, & K.C. Breen. Department of Pharmacology, University College, Bdfidd, Dublin 4 and *Mercer's Institute for Research on Ageing, St.

James's Hospital, Dublin 8.

Alzbeimer's disease (AD) is an insidious progressive dementia which is chasacterised by its pathology ofneurofibrillary tangles and senile plaques. The latter contain 42 amino acid BA4 pelypepfide derived by proteolysis from a larger membrane bound glyeoprotein, the amyloid precursor protein (APP), which has been demonstrated to play a role in the mediation of neural cell adhesion.

As an alteration in APP processing in AD may upset the neural cell adhesion, it was of interest, therefore, to examine the resulting expression of the nerve cell adhesion molecule (NCAM) in AD.

Levels of the soluble form of NCAM were significantly raised in AD serum, and were also raised in CSF samples from AD patients. There was no change, however, in the serum levels of another cell surface glycoprotein, CNS 130. Furthermore, there was no detectable change in totalbrainlevels of the proteins. Western blot ana]ysls of AD brain samples using the anti-NCAM antibody demonstrated the appearance uf bands corresponding to extra proteulytic fragments of the glyeopsoteins, probably as a result of neural cell degeneration. These results indicate that an elevation in serum levels of cell surface glycoproteins is selective and may therefore prove interesting in the quest for a peripheral early marker for CNS neural cell degeneration. This work was supported by the Health Research Board and the Sandoz Foundation for Gerontological Research . K.B. is a H.J. Heinz Newman Scholar.

CHRONIC PAININ MULTIPLE SCLEROSIS : NEUROPSY- CHOLOGICAL AND CLINICAL CORRELATES.

J. Hutchinson, T. Burke, M. Hutchinson. University College and St. Vincents Hospital, Dublin.

Chronic pain in multiple sclerosis (MS) is a frequent but under- researched complaint. Twenty-six pairs of MS subjects with and without chronic pain matched for age, sex, education level, socio- economle status and for disability and duration of MS and 25 healthy control subjects were evaluated on self-report of physical and psy-

477

cho-social limitation and on a range of cognitive tests. MS pain subjects were more deficieaat cognitively than either the non-pain MS subjects or the controls, The MS pain group experienced more psycho-social limitation than did the non-pain MS group, but this did not appear to be caused by the cognitive deficit, nor could mood entirdy explain the cognitive dysfunction. Pain was experienced as a distinct entity, not related to either cognitive deficit or effective factors. Verbal memory emerged as a specific area of deficit in the MS pain group alone. In particular, patients with neurogenic pain tended to have significant verbal memory impairment whereas those with musculo-skeletai pain had relatively intact verbal memory. It is hypotheslsed that the subjects with neurngonic pain were preferen- tially affected by lesions of cerebral structurs subserving memory, affect and pare sensation.

DISTRIBUTION OF YoT-CELLS AND THEIR SUBPOPULA- TIONS IN BLOOD AND CEREBROSPINAL FLUID FROM

MULTIPLE SCLEROSIS PATIENTS.

A.G. Droogan t, A.D. Crockard 2' S.A. Hawkins ~, T.A. McNeill 2. Northern Ireland Neurology Service ~ and Regional Immunology

Laboratory ~, Royal Victoria Hospital, Belfast.

Yo T-lymphoeytes may initiate or amplify autoimmune disease and show distinct patterns of migration in a range of pathological states. Using monoulunal antibodies and flow eytnmetry, T- lymphocytes bearing the aB T-cell receptor (TCR), yo TCR, V~ TCR and V02 TCR were measured in paired blued and cerebrospinal fluid (CSF) samples from 25 patients with active Multiple Sclerosis (M S), 7 patients with inflammatory neurological disease, 12 patients with non-inflammatory neurological disease (NIND) and peripheral blood from 25 normal subjects. In peripheral blood no significant differences were nbsetwed in the percentage of yo T-cells and their subpopulatio~ between MS patients and the control groups. How- ever,in the CSF of MS patients cun'lpared to NIND patients there was a significant decrease in the percentage of total YoT-cells (median [range]: MS, 2.9% [0.5-11.4]; NIND 5.9% [2.1-15.7];P<0.01) Vol T-cells (M S, 0.7% [0.2-5.91; NIND 3.2% [ 1.0 -8.2]; P < 0,01 ) and V02 T-cells (MS. 1.8% [0.1-6.2]; NIND 2.9% [1.8-9.7]: P < 0.01) but not aB T-cells (Ms 97A% [794-120.01; NIND 97.0% [83.1-102.7]; P--4).80). This may be due to sequestration of yo T-cells within the brain in MS.

A CONTROLLED STUDY OF OXYGEN FREE RADICALS IN GUILLA1N BARRE, MULTIPLE SCLEROSIS AND

ASEPTIC MENINGITIS.

N.J. Gutowski 1, S. Chirico ~' J.M. Pinkham ~, C. Smith 2, D. A.kanu 2. H. Kaur ~, R.C. Strange t, B. Halliwell 2, R.P. Murphy ~

~North Staffordshire Royal Infirmary, Stoke-on-Trent, 2King's College, London.

This controlled study wag designed to ascertain if oxidative damage caused by free radicals could be detected in the sera or CSF of Guillain B arre (GB), definite Multiple Sclerosis or aseptic manin- girls patients. An ele ration of free radical activity produce might lead to newer therapeutic options. The control group consisted of patients with either migraine, cl-~onic headache, benign intracranial hypertension or functional disorders. Routine haematological, biochemical and bacteriological analysis were perforuted on a/1 sampled.

Increased end products of lipid peroxidation in particular have


been implicated in free radical associated human disease. Hence lipid peroxidation was assayed; and in addition aseorbate, dehydroascorbate, protein carbonyls and belomyein detectable iron were measured. Serum lipid peroxidatinn was significantly elevated in pre-treatment GB patients compared to post-treatment (plasmapheresis or steroids) GB patients or controls. Serum asonrbate levels seem depressed in both GB and meningitis patients. Only in the meningitis group were seaxun protein carbonyls increased.

YIELD OF SYSTEMIC STAGING IN PATIENTS PRESENT- ING WITH PARENCHYMAL BRAIN LYMPHOMA.

R.L. Cristea, L. Rogers, J. Weick, M. Estes. The Cleveland Clinic, Cleveland, Ohio, U.S.A.

Because parenchymal brain metastasis is rare in systemic lymphoma, we sought to determine the degree of systemic staging necessary in patients presenting with parenchymal brain lymphoma. We restrospoctively reviewed 20 patients with histologic proof of brain lymphoma since 1981 (isolated leptomealingeal lymphoma was excluded); age ranged 36 to 85 years, median 59. Seventeen patients had no history of systemic lymphoma (study group). Three others had non-Hodgkin's lymphoma (2) or Hodgkin's disease (1) diagnosed 5-6.5 years prior and were excluded from analysis. Stag- ing of the study group including physical examination (17), bone marrow aspiration and biopsy (14), CT scan of the abdomen (1%), pelvLs (8), and chest (7), and chest x-ray (15) revealed no evidence of lymphoma. No clinical evidence of systemic lymphoma developed at a median follow-up of 60 weeks, range 2-.?.08. CSF examination was diagnostic or su~tfieinns of ]eptomeningeal lympboma in 8/12 and 4/6 who underwent ophthalmological examination had uveal lymphoma, CSF and eye examination should be performed in patients presenling with brain lymphoma; our data suggest, however, that the risk of concurrent systemic lympboma is low and systemic staging may be unnecessary.

SPECIFICITY OF ANTI-PURKINJE CELL ANTIBODIES IN PATIENTS WITH SMALL CELL LUNG CANCER.

G.M. Elringtun, C.S. Morris. Departments of Clinical Neurology and Neuropathology,

Radcliffe Infirmary, Oxford OX2 6HE.

Neurological paraneoplastic syndromes are caused by autoantibodies against neural tissue. Anti-Purkinje cell antibodies (APCA) have been detected in small call lung cancer (SCLC) patients with paranaoplastic syndromes other than eerebellar degeneration, particularly subacute sensory neuropathy (SSN). Patients with the lambert-eaton myasthenic syndrome (LEMS) hay e antibodies to voltage gated calcium channels, which are present in Purkinje ceils.

We sought APCA in serum from patients with LEMS & SCLS (n=10), LEMS and no detectable cancer (n=5), SCLS and other paraneoplastic syndromes (n=3), uncomplicated SCLC (n=21), and other neurological diseases (n=6). APCA were detected by a two layer immunoporoxidase technique at six dilutions from 1:400 to 1:12800. Preliminary data had shown that healthy control serum was consistently negative for APCA at these dilutions.

Cytoplasmic and nuclear Purkinje cell, and molecular layer cell, staining was detected at dilutions ofgreator than 1:1600 in 4 subjects' serum SCLS/SSN (n=2); LEMS/SSN (n=l); SCLS/braimtem encephalopathy (n=l). Among sera from uncomplicated SCLC

].JM.S. November, 1993

patients, one gave cytoplasmic staining up to 1:6400 but there was neither nuclear nor molecular cell staining; of the remaining 19 SCLC sera, cytoplasmic staining was present at 1:400 in four and at 1:800 in one. No other sera stained Purkinje cells at >1:400.

Anti-nuclear APCA > 1:1600 appear to be specific for non-LEMS paraneoplastic syndromes complicating SCLC. Negative results in LEM S patients suggest that the Porkinje cell VGCC populatiun may be below the resolution of this technique. The development of paraneoplastic syndromes in SCLC patients may depend upon (1) autoantibody specificity and (2) access to target tissue.

OBSERVING OPTIC NEURITIS RECOVERY USING ACH- ROMATIC AND CHROMATIC CONTRAST SENSITIVITY

TECHNIQUES.

J.P. Reffin ~, S.J. Tregear ~, L.J. Ripley 2, J.E. Rees 3. Sussex Eye Hospital, Brighton1; University of Sussex, Brighton2;

Hurstwood Park Neurological Centre, Haywards Heath s.

Achromatic mad chromatic contrast sensitivity techniques were used to analyse the recovery of visual function following an optic neuritis attack in 20 patients. Results were analysed by developing a numerical model of the recovery process using two variables for each condition and each patient. The first variable describes the size of the initial deficit and the second variable describes the recovery rate. Analysis of individual data suggested a division into three subgroups being, those who recover all faculties at the same rate, those who recover low frequency anhromatie contrast sensitivity more rapidly, and those who show a di~sneiation between recovery to red/green and tritan contrast sensitivity. It has been found that total recovery time varied widely between patients with their predicted recovery time varying between 34 days and 8 years. It has also been shown that a comparison with clinical features at initial presentation and their result~mt contrast sensitivities showed a tendancy for the patients with normal optic discs to have poorer initial achromatic and chromatic contrast sensitivities than those patients with swollen discs indicating that a more posterior lesion has a greater initial effect on central vision.

MALIGNANT FIBROSARCOMA OF THE BRACHIAL PLEXUS - A NEUROSURGICAL DILEMMA.

A.D. Irvine, B. Clements & W.J. Gray. Northern Ireland Regional Neurosurgical Unit, Royal Victoria

Hospital, Belfast.

Since Courvoisier described the fi~t mmour of the brachial plexus in 1886, reports have been sporadic with very few large series. Malignant tumours constitute less than 5% making their incidence extremely rare. As with turnouts more commonly associated with peripheral nerves management of those involving the brachial plexus requires a precise understanding of pathological variation.

We report the case of a 6 year old girl who presented with a 6 month history o f diminished seasation and power in her right arm. A discrete mass was detected in theipsilatcral supraclavicular fossa end a CT scan demonstrated a soft tissue mass confluent with the trunks of the brachial plexus. Surgicalbiopsy revealed this to be amalignant fibrosarcoma. Combination chemotherapy was administered and the turnout responded significantly with a 50% reduction in size. Definitive surgical excision was attempted subsequently however despite tumour shrinkage the trunks of the plexus were intimately

Vol. 162 No. it

involved with the tumour necessitating sacrifice of the plexus in achieving satisfactory local excision. At present there are no signs of turnour dissemination and further adjuvant chemotherapy has been plarmed.

This is only the third such case reported in the world literature and is the earliest age of presentation. It presents a major management dilonma in both surgical and psychological terms. Forequarter amputation although mnltilating probably offers the best hope of cure, however the patient is free of disease presently and has been shown to have a chemosertsitive tomour. In the light of minimal elinicopathological data for reference mad significant parent reluc- tance for radical surgery we do not propose an aggressive surgical policy presently.

BROWN-SEQUARD SYNDROME SECONDARY TO A SPINAL ARTERIOVENOUS MALFORMATION (AVM) : A

CASE REPORT.

D.P. O'Bfien, J. Singh, P.S. Dias. Department of Neurosurgery, Beaumont Hospital, Dublin 9.

A seven year old dextromanual boy presented with a two day history of right sided weakness of sudden onset. He also complained of neck discomfort. His speech was preserved. He had no other complaints. Clinical examinations revealed that he was afebfile, fully alert and orientated. He had marked neck stiffness with head tilt to the left side. There were no cranial nerve abnormalities or cerebellar signs. He had Grade 3/5 weakness of his right upper limb and 4/5 weakness of the right lower limb. Power was normal on the left. He had decreased pain and temperature sensation on the left side. He had bilateral extensor plantar responses. He had normal two point discrimination and joint sense. A MRI scan of the cervical-dorsal spinal cord revealed an extensive cervical spinal arteriovenous malformation with an intramedullary haematoma at the level of C6. Spinal Arteriography confirmed the above depicting file feeding vessel coming from the right second intercostal artery off the aorta. The patient underwent a cervionMorsal (C2-T3) laminotomy for total resection of this lesion which turned out to be an intradural paramedullary arteriuvenons malformation (AVM). This was confirmed histologically. Post-operative MRI scan and Spinal Artoriogrsphy showed no evidence of residual AVM. Climcally, the bey became neurologically asymptomatic. He required a cervical halo brace for rigid fixation of his cervical spine due to the development of lordosis.

A case of Brewn-Sequard Syndrome is presented and spinal arterinvenous malformation reviewed.

THE ANTERIOR SPINAL ARTERY SYNDROME - RECENT AETIOLOGY INSIGHTS

N. Herity, S. Hawkins. Department of Neurology, Royal Victoria Hospital, Belfast.

The anterior spinal artery syndrome can be diagnosed clinically when there is a sudden onset o f paraplegia with a s ensory level to pain and temperature but preservation of the sensory modalitie s conveyed in the posterior colunms.

Consideration will be made of two middle aged women with the syndrome who recovered to be able to walk and live independently. The pathological phenomenon of fibrocartilaginous embelism of the anterior spinal artery has been well described in autopsy eases. Only once ever, by chance, has this pathological process been established

479

during life. It is probably underrecognised. The details of the clinical presentations allow speculation that fibrocartilaginous em- bolism was the pathological process operating in the two cases described.

CHOREA DUE TO MOYA MOYA DISEASE : RESPONSE TO STEROIDS.

J.B. McMenamin. Our Lady's Hospital for Sick Children, Crumlin, Dublin 12.

A 10 year oM girl presented with a 6 month history of progressive chorea with prominent facial grimacing mad intermittent tongue protrusion. There had been no change in intellectual function. On examination she had pulmonary branch stenosis with generalised chorea but no other abnormal findings. Investigations including Serum Auto-antibodies. ASO titre, Copper and Ceruloplasmin, Urinary Organic Acids and Thyroid Function tests were normal. In addition CSF. EEG, Evoked Responses and peripheral nerve studies and CT brain sean were normal. A tentative diagnosis of Sydenhams Chorea was made and she was started on Prednlsolone. Within one month her chorea had resolved. An MRI scan of the brain subsequently confirmed marked abnormalities in the white matter. Lysosomal enzymes werenormal. Fourmontbs after her initial presentation she developed an acute onset of global aphasia wifla right sided apsaxia. She also had horizontal nystagmus and became Incontinent. Metabolic tests were normal. A repeat Ei~G confirmed a moderate generalised dysrhythmia and Visual Evoked Responses were abnormal. A repeat MRI brain scan confirmed extensive changes in the white matter but also abnormalities extending into grey matter. She was treated with Prednisolone and over the following 2 months her clinical condition improved. Bilateral Carotid Angiography subsequently cortfirmed occlusion of both internal carotid arteries with marked collateral anastomosis charac- teristic of Moya Moya disease. Currently the patient has a mild residual dysphasia and memory disturbance and is off steroid treatment. Her response to steroids suggest an inflammatory basis for her vasculopathy and that treatment with steroids may be valuable in sonm patients with Moya Moya disease.

SIMULTANEOUS OCCURRENCE OF DIABETES MELL1TUS, DIABETES ISIPIDUS AND OPTIC ATROPHY (DIDMOAD

SYNDROME) IN TWO FAMILIES - CLINICAL AND NEUROPATHOLOGICAL FEATURES.

M. Mirakhur, I.V. Allen, S. Hawkins. Department of Neuropathology and Neurology, Royal Victoria

Hospital, Belfast.

We report two members o f two families who died at the age of 39 years and 22 years respectively. Both had optic atrophy, diabetes inalpidus and diabetes mellitos (DIDMOAD Syndrome) with onset occurring in early childhood. One of them had a sister who died of the same disease but necropsy was not performed. There are five reports on full necropsy findings in this syndrome and these are the first ones to be reported from Ulster. They included the expected atiophy of hypothalmic nuclei, degeneration of the optic nerve, chiasm and tract and degeneration of brain stem and cerebellum. lmmunocytocbemistry has also been preformed to study the cytoskeletal abnormalities in specific sites and the findings shall be reported.


AD1ES PUPILS AND FACIAL NUMBNESS.

M. Hutchinson, B. Bresnihan. Adelaide & St. Vincent's Hospitals, Dublin.

Three patients are described who presented with art unusual clincal picture of trigeminal sensory loss in association with myotonic pupils. There was an associated unusual sensory nenronopathy in two patients with a marked sensory ataxia which affected the arms more than the legs. One patient had sclerodetma and another had an ill-defined connective tissue disorder. All three patients had evidence of the sicca syndrome which was not sumptomatic and not a presenting feature.

This disorder may be classified as a sensory neuronopathy or gangliortitis. It seems to be benign, relatively non-progressive and due to lymphocytic infiltration of the ciliary, gassedan and dorsal root ganglia.

[J MS. November, 1993

when she had a second subarachnoid hsemorrhage. It was doe to rupture of a right internal carotid artery aneurysm. This was an unruptured right middle cerebral artery aneurysm were successfully clipped.

The wrapped left middle cerebral aneurysm enntinued.to increase in size. It caused complex partial seizures, followed by dysphasia and a right hemiparesis, Hydrocephalus occurred in 1991, because the mass occluded the foramen o fMonro, and a ventrieulo -peritoneal shunt was inserted. This led to worsening ofber herniparesis, as it led to an increase in brain shift, and eventually the mass was excised, although it was necessary to sacrifice the internal carotid artary in order to do so. This l)raennian measure was undertaken beeanse her health was rapidly deteriorating, and she had devdoped total aphasia and hemiplegia. Her progress since operation has been satisfactory, although she remains hemiplegic.

WHEN BLINDNESS IS FIq'TING.

M. Fitzsimmons, B. Sawhney, V. Patterson. Department of Nemoingy, Royal Victoria Hospital, BelfasL

Sudden deterioration in vision is a frightening experience for both patient and doctor especially if it occurs in a partially sighted individual. This case had a reversible and treatable cause of blindness. A 14 year old boy developed Lebers optic atrophy. Over the next 18 months there was slight improvement in vision. At the age of 16 he presented with a fight humonymons hemianopla and a CT scan showing a left occipital infarct. Muscle biopsy showed abnormal mitochondria. Again his vision improved somewhat. Following a tonic clonic seizure a year later he was started on oral carbamazepine. His third episode of visual deterioration occurred at the age of lg with a 3 day history of headache, flashing lights and sickness. He was disorierttated with poor attention. Visual acuity was reduced to finger comating on the right and light perception on the left. EEG-was slow with a run of spikes from the fight posterior quadrant lasting 50 seconds. Oral earbamazepine was increased, his mental state and vision improved over the next few days end his EEG reverted to normal. This last episode of visual deterioration was due to partial epileptic status affecting the right occipital lobe, a reminder that blindness can be fitting.

A GROWING GIANT INTRACRANIAL ANEURYSM.

1. C. Bailey. Department of Neurological Surgery, Royal Victoria Hospital,

Belfast.

Giant aneurysms are defined as those greater than 2.5cms in diameter. The natural history of these large aneurysms is still debatable. Many are found to have laminated clot within their lumen, which may protect them from rupture. There are reports of such aneurysms remaining static, decreasing in size or even disappearing, but in general it must be assumed that most giant aneurysms generally increase in size, mad some may rupture.

This 32 year old lady presented with subaracimoid haemorrhage in 1977. Angiography disclosed multiple anenrysms but the cause of the bleeding was a broad-based aneurysm on the left middle cerebral artery. This was treated by wrapping with muslin, as the neck was incompletely seen at surgery, end therefore not deemed suitable for clipping. This was followed by common carotid ligation, from ~hich there were no sequalae, and she did very well until 10 years later,

LONG TERM TREATMENT OF SEVERE SPASTIC1TY WITH INTRATHECAL BACLOFEN.

V. Patterson, D. Byrnes, M. Watt, Kui-Chung Lee. Departments of Neurology and Neurnsurgery, Royal Victoria

Hospital. Belfast.

Intrathecal baclofan abolishes spasticity in many patients with neurological diseases but there are few studies on its longterm effectiveness. We have used a manually-operated pump to deliver baclofen in 21 patients with spasticity, the majority of whom had multiple sclerosis. All were chair-bound. All but one patient achieved a working system. Symptoms were completely controlled in 13 patients, partially controlled in 3. and the system was ineffec- tive in 5 patients. Mean duration of implantation was 26 months (0- 70). Complications requiting pump removal occurred 9 times and there were 3 significant baclofen overdoes giving 1 serious compli- cation every 50 pump-months.

Intrathecal baclofen therapy is an effective longterm management in most patients with severe spasticity.

SENSORY TESTING VERSUS NERVE CONDUCTION VELOCITY 1N DIABETIC POLYNEUROPATHY.

J. M.T. Redmond, M. J. McKenna, M. Feingold, B. K. Abroad. Neurophysiology Laboratory; Department of Neurology;

Department of Medicine; and Division of Binstatistics, Research Epidemiology and Computing; Henry Ford Hospital, Detroit,

Michigan.

We ~ught to evaluate the utility of quantitative sensory testing (QST) and nerve conduction velocity (NCV) studies as measures of distal symmetric polyneuropathy (DSP). We studied 36 diabetic patients divided into four clinical categories of increasing severity. QSTincluded thermal testing and vibration thresholds. NCV studies included mdian, peroneal and sural nerves. Results of QST mad NCV were compared among clinical groups using survival methodology. Tile log rank statistic showed significant differences among the groups; the direction of the differences was consonant with clinical severity. For each diabetic patient the result of each measurement was classified as normal or abnormal; more diabetic patients had abnormal NCV than either vibration tests or thermal tests. In conclusion, findings of QST and NCV~m'e in keeping with clinical categorization of patients, QST and NCV are complementary tests, and the sura] sensory study is the best single predictor of DSP.

Vol. 162 NO. It

SERUM NEURONE SPECIFIC ENOLASE AS A MARKER OF CEREBRAL INFARCT VOLUME

M. Watt t, R.T. Curmingham 3, I. Winder ~, C. S. McKinstry ~, J, A. Lawson *, C. F, Johnston 3, S. A. Hawkin#, & K. D. Buchanan 3. Department of Neurology t, Department of Neuroradilology 2, Royal Victoria Hospital, Beffast. Department of Medicine 3,

Queen's University, BelfasL Department of Radiology ~, Belfast City Hospital.

Stroke is the third leading cause of death in the U.K. At present there is no treatment, other than supportive care, fur the acute stroke victim. A variety of agents are currently beIng examined as putential treatments. These studies are made more difficult by the fact that outcome, the usually employed endpoint depends on both site and volume of infarction, whereas therapy can only hope to influence volume. Infamt volenae can be measured by imaging techniques such as CT scanning, with which only mound 60% of infarcts will be seen, and MRI which is not widely available. Therefore a biochemical m~ker of neuronal damage could be of great value. The aim of this study was to examine serum neurone specific enolase (nse) as a petential marker of nenronal damage in acnte stroke. A previously established radinimmunoassay has been used to measure nse concen- trations in serum samples taken from 65 patients admitted within 24 hours of their first stroke, brsinstom strokes were excluded. Samples were taken on admission and after 24, 48, 72 and 96 hours. CT acmxs were performed after an average of 5 days and infarct volume measured by a semi-automated method. A statistically significant correlation between cerebral infarct volume and masimom serum nse concentration over the 96 hour period was observed. Serum nse concentration may prove to be a useful marker of neuronal damage in the study of stroke.

'THE EPIDEMIOL(X]Y OF MOTOR NEURONE DISEASE : A MAJOR CLUE TO ITS AETIOLOGY"

G. Dean, M. Elian. P.O. Box 1851, Ballsbridge, Dublin 4.

In the past Motor Neurone Disease (MND) was thought to have a uniform prevalence in all parts of the world except for Guam and the Kii Peninsula. There is good evidence that this is not correct. The mortality for MND is increasing repidly in a large number of countries including the Republic of Ireland and the United Kingdom whereas there is no increase but rather some fall, in the mortality fro m Multiple Sclerosis (MS). MND has a higher mortality in Australia and New Zealand than in Englmad and Wales but in South Africa the mortality is low, less thanhalfof that in England and Wales. Among immigrants to Britain from the Indian Subcontinent - Indians, Paki- stanis and Bangladeshi - males have half and the females one-fifth of the expected morality from MND that occurs in the general population. The reasons for these differences in MND mortality will be discussed.

REPORT OF A NEW PERSONAL COMPUTER BASED SYSTEM FOR CAPTURING .~ID TRANSMITHNG COM-

PUTED TOMOGRAPHIC IMAGES.

W. P. Gray, D. Q. Ryder, T. F. Buekley. Department of Neurological Surgery, Cork Regional Hospital,

Ireland.

The value of interhospital transmission systems for computed tomographic (CT) images is well reengnised (1). They improve the clinical management of emergency neurosurgical referrals and void urmecessary hiterhospital transfers. However, these systems are

481

expensive since they require a direct hardware connection to the CT scanner.

We report the development of a new system which obviates the need for any connection to the CT scanner. This is achieved using a handheld optical scanning device which reeds the image from the printed hard copy of the scan as it is drawn across it. The device transfers the image to a personal computer which automatically sends it to the receiving computer over a normal telephone line. Typical transfer times are 40- 90 seconds at 9600 baud for a 256 grey acale image.

This system has been successfully tested over long distances. It reliably delivers high quality images at a low cost.

Reference ( 1 ) Lee T. Effect of a new computed tomogral~ic image transfer sysmm on managememt of referrals to a regional neurosurgical service. Lancet 1990, 336: 101-3.

DEGENERATION AFTER NERVE SECTION. THE CONTRO- VERSY THAT WASN'r.

K. Breathnach, Department of Anatomy, University College, Dublin, Earlsfon

Terrace. In their classic study of "sensory disturbances from cerebral

lesions" (1911) Gordon Holmes, as outlined in a previous communi- cation, was apparently persuaded by Henry Head to agree that "the thalamus is the centre of consciousness for certain dements of sensation". Head convInced himself that the thalamic syndrome was best explained in terms of release of "protopathic sensation" from the control of cortical "epierit!c: sensation", the terms he and W.H.R. Rivers introduced in their interpretation of "a human experi- ment in nerve division" (1908). The left superficial cutanoo us branch of the radial nerve was divided in Heads arm, and Rivers assiduously mapped the recovery of sensation over the next three years. "Protopathic sensibility" intertse, diffuse, unpleasant and intolerably disagreeable, recovered first, and gradually faded as precise, dis- crimmative, localised "epicritic sensibility" gradually suppressed it, until the normal dominance of epicotic sensation was fully restored.

At the outbreak of World War 1 Rivers served as a Neuro- psychiatriat and from his experiences of war nurses and psychother- apy, he believed he could explain "Instinct and the Unconscious" (1920) in terms similar to those introduced by himself end Head. The individual response to cataclysmic stress was protopathic in nature and the protective group instinct of the herd was epicdtic. Like Holmes and Head he was beguiledby the distinction. The curious feature is that epicritic and protopathic sensations lived on until mid-century, in spite of the fact that Trotter and Davies (1909,1913) and E.G. Boring (1916) failed to find them in extensive studies. That no controversey developed after the failure of confir- marion is today incomprehensible, and epiuritic and protopathic held unassailed away in textbooks and in teaching until World War 2.

CEREBELLAR'ATAXIA RELATED TO A BENIGN OVAR- IAN DERMOID CYST : A CASE REPORT.

A. Ooi, M. Farrell, C. Doherty, M. Hutchinson. St. Vincent's Hospital, Dublin.

A 43 year old woman presented with an unsteady gait of two months duration in December 1991.

She had been attending the Gynaecology Service because of a strong family history of ovarian carcinoma. A routine ultrasound of pelvis revealed a cyst suggestive of benign dermoid neoplasia. Examination showed a moderate gait ataxia with a f-me nystagmus on right and left lateral gaze.

482 1fish Neurological Association

After routine neurological investigations including MRI brain, CT posterior fessa, and CSF studies revealed no primary corebellar pathology. Serum for anti-Purkinje cell antibodies was drawn and the cyst was surgically excised.

Although the serum studies were negative, the cyst did contain mature neural tissue.

Within two days of an uneventful post-op recovery it became clear that the symptoms of cerobellar ataxia had dramatically resolved.

Previous studies of parancoplastie cerebellas degeneration in relation to ovarian neoplasms have focussed on malignancy; here we present the first suspected case with benign ovarian neoplasm to be described.

Paranoophstic cerebellar degeneration has been shown to occur in both seropositive and seronegative patients and, as others have suggested, may be due to an as yet unidentified antineuronal autoimmune mechanism.

THORACIC POLYRADICULOPATHY: ABDOMINAL WALL SWELLING AND SENSORY SYMPTOMS 1N DIABETES

MELLITUS.

F. J. Hayes, J. L T. Redmond, M. M. McKerma. Departments of Endocrinology & Metabolism and Neurology,

St. Vineent's Hospital, Dublin 4.

Neuropathy is increasingly being implicated as a source of significant morbidity in diabetes mellitus. While the clinical mani- festarions of symmetric distal sensorimotor polyneuropathy (DSP) and autonomic neuropathy, tend to be readily recogrlised, the entity of thoracic polyradiculopathy goes largely undetected. We describe two patients with type 2 diabetes who presented with abdomin al pain secondary to thoracic polyradiculopathy. Both patients had associated syml~nmatic DSP, In the first patient, abdominal pain occurred in asaneiafion wi~h m~ked abdominal distension, and extensive negative gastrointesthml kavestigations were performed b~fore the correct diagnosis was made by EMG showing thoracic paraspinal muscle denervarion. In the second case, trancal sensory symptoms alone were evident at the time of diagnosis of diabetes mellitus. While muscle laxity was absent, extensive paraspinal muscle denervation was detected. Toi~estut, in,aldese reductase inhibitor, was associated with good clinical response of symptoms due to DSP and thoracic polyradlenlopathy.

The pathogeneals of thor aeic polyradiculopathy is uncertain but is likely to be the result of multiple infarcts along the course of thoracic spinal neawes accounting for an array of clinical presentations.

PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY ASSOCIATED WITH PRIMARY BILIARY CIRRHOSIS: A

CASE REPORT.

J. M. T. Redanond, M, Farrell, M. Hutchinson. Department of Neurology, St. Vincent's Hospital, and Department

of Neuropathology, Beaumont Hospital, Dublin.

Progressive multifocal leukoencephalopathy (PML), a demyelinating disorder associated with infection by papovavirus, is characteriscd by widespread demyelinating lesions. We report the case of a 61 year old man with a past history of primary biliasy cirrhosis who was admitted for investigation of a slowly progressive right hemiparesis. The clinical condition gradually deteriorated with myoclonic jerks and dysphasia` Initial brain MRI was normal. A second MRI four weeks after admission showed abnormalities in TI weightedimages in theleft thalamus, putamen, and in the left trig one.

IJM.S November, 1993

A couple of weeks later, CSF analysis revealed araised antibody titrc to JC virus that was also present in the serum. A transient clinical response was obtained to intravenous and intrathecal cytosine arabinoside. However, the patient deteriorated clinically and devel- opod hepatic encephalopathy and died four months after admission. Histoiogic evaluation of the cerebral cortex and subcorrical white matter showed microglial nodule formation involving the neocortex with proliferation of astrocytos. Demyelination was demonstrated in the centrum semiovale with large bizarre astrocytes. Asla'ecytic hyperplasia was also seen in the left thalmus and medulla.

The unusual features of this ease included the value of CSF analysis in making the diagnosis, the transient response to antiviral medication, and finally the uncommon autopsy findings. PML can occur in the absence of underlying immunosuppresaion.

CEREBELLAR ENCEPHALITIS DUE TO EPSTEIN-BARR VIRUS INFECTION.

M. Watt, J. M. Gibson, V. H, Patterson. Department of Neurology. Royal Victoria Hospital. Belfast.

Neurological complications have been reported to occur in up to 1% of cases of primary Epstein-Burr virus infection, and include meningoencephaliris, aseptic meningitis, optic neuritis, facial palsy, transverse myelitis and polyneuritis. We report the cases of 2 teenage boys, admitted within 10 days of each other, with a cerebellar disturbance of sub-acute onset; they appeared otherwise normal apart from elevated titres of anti-EBV IgM. consistent with a recent Epstein-Ban" virus infection. They have since made a full recovery. This would be in keeping with previous reports in which neurological involvement has been the only, or major, manifes teflon of infectious mononucleosis. The pathogenesis remains tmclear but has been suggested as being either an antibody mediated post-infectious meningoencephalitis or a primary viral infection of the nervous system. Wewereenabletoprovidesupport foreither; 15mlsclotred blood, spun, labelled with flourescent dye and tested against normal cerebellar tissue did not show any specific staining of Purkinje cells; likewise, virus particles were not isolated from their CSF. EBV infection should be considered in the neurological differential diagnosis, even in the absence of obvious systemic illness.

SMOKING IMPAIRS SENSATION IN HEALTHY INDIVIDUALS

I. M. T. Redmond, M. J. McKerma, M. Feingoid, B. K. Ahmed. Neuropbysiology Laboratory; Department of Neurology;

Department of Medicine; and Division of Biostatisrics, Research Epidemiology and Computing; Hemy Ford Hosptial, Detroit,

Michigan.

The aim of this s mdy was to obtain normative data on thermal and vibratory sensation in adults and to examine the effect of side of measurement, handedness, age, sex, height, body size, akin temperature, smoking habits ~ d alcohol comsumption. There was no effect of side or handedness, but most of measurements wexe influenced by one or more of the other variables. Smoking was the leading predictor variable affecting over 55% of measurements, and was followed in rank order by body mass index, sex, height, age, skin temperature, and alcohol consumption. It is recommended that each laboratory establish its own reference values for all medaliries tested, and that predictor variables be taken into account. For research purposes it is necessary to take the average of two sets of results m establish baseline status, and groups for comparison should be matched for predictor variables.

Vol. 162 No. 11

RAPIDLY PROGRESSIVE FATAL NEUROMYOPATHY OF CHILDHOOD.

M. A. Farrell, M. Moran, M. Burke, M. D. King. Richmond Institute of Neurology & Netm0sttrgery, Beaumont

Hospital, Dublin 9.

Pathologically proven non-paraneopiastie neuromyopathy is ex- ceptiounlly rare. A 17 yeax old boy developed rapidly progressive symmetrical biceps weakness and had ganeralised areflexia but no fasciculations and normal sensation. Creatino kinase [14201u (10- 30)] and CSF protein [2070 mgnffL (<460)] were elevated. Electromyography showed reduced recruitment and low amplitude polyph asic units. Abnormal large units were present in the quadricops. Conduction velocity in the left peroneal nerve w as 45 m/See. Biopsy of quadrieeps and biceps muscles demonstrated widespread degeneration withregener ation, fibre atrophy, granular staining on Gomori's tricbrome and increased numbers of subsareolemmal mitocbondria with concentrically arranged tubular cristae. The sural nerve showed a profound leduction of large myelinated fibres with thinning of myelin sheaths, maerophages containing myelin debris and rare onion bulbs. The patient has not responded to immunosuppressive therapy. One of four siblings died 4 years after onset of an identical illness at 13 years. Review of the muscle biopsy showed similar appearances to those seen in the current patient. The presence of smtetural mitoehondrial alterations in this neuromyopathie condition combined with farnilial necurence suggests an underlying genetic defect.

INTRASELLAR GANGLIOCYTOMA ASSOCIATED WITH FUNCTIONAL PITUITARY GLAND M1CROADENOMA.

M. Hourihane, M. Morun, M. Burke and M. A. Farrell Richmond Institute of Neurology & Neurosurgary, Beaumont Hospital,

Dublin 9.

The association of hypothalmic and intrasellas gangliocytemas with development of pituitary microadanomas and syndromes of pituitary hypersecredon is rare. We report our observations in two such tumours. A 46 year old male with classical aeromegaly and HGH hypeseeretion and a 56 year old female with biochemically proven pituitary dependant Cushing's disease were found to harbour small intrasellar tumours without any attachment of tumour to hypothalmus. Both tumours were composed of large irregularly arranged ganglion cells having well formed neurofilament positive neuritic processes. Clusters of small cells which stained positively for HGH and ACTH respectively were intermixed with the neuritic processes in each tumour. GFAP positivity was not present. In addition the ACTH secreting tumonr contained well formed epithelial lined, cytokeratin positive tubules. Both patients have shown clinical and biochemical improvement and are now turnout free. It is postu- lated that uncontrolled release of HGH-releasing factor (RF) and Corticotrophm-RF by ganglion cells led to the adenomatous trans- formation of pituitary cells.

THE EXPRESSION OF MYOSIN HEAVY CHAIN ISOFORMS BY HUMAN MYOBLAST

O. Hardiman ~, R. H. Brown Jr), R. M. SklarL 1Massachusetts General Hospital, Boston, MA; and 2Dept.

Physiology, University College Dublin.

At least 4 different mysoin heavy chain (MHC) isoforms can be detected in nonelonal cultured human muscle, reflecting the separa- tion of fibre types/n vivo. The developmental pattern of MHC isoform expression has not been fully established in human muscle~

483

it is unclear whether the expression of the different MHC isoforms is determined by the clonal origin of myohlasts, by the duradon of time in culture, or both. The expression of fast, slow and embyronic myosin heavy chain isforms were analysed by immunobhit in 11 cultures of clonally derived myotubes fi-om four different biopsies. The fast MHC isoform was present in all cultures, and the majority of cultures also expressed the slow isoform. Two of the clonally derived cultures expressed the fast but not the slow or embryonic iso forms. Fous clones were analysed by immtmoblot at differant time points following differentiation. In these clones, the expression of the fast isoform preceded that of the slow and embryonic. The addition of methylprednisolone to cultures enhanced the expression of the slow and embryonic isoforms, with little effect on the fast. These findings indicate the following: 1. Adult human satellite cells maintain a nerv e-independent capacity to express beth fast and slow MHC isofom~; 2. The expression of particular isoforms can be modulated by extrinsic nonneural agents; 3. Sequential isoform expression in mixed cultures is a function of genetic switching in individual clones.

CLONAL HETEROGENEITY OF GLUCOCORTICOID RESPONSE IN PRIMARY HUMAN MYOBLASTS.

O. Hardiman 1~, R. M. Sklar t, R. H. Brown Jr). 1Massachusetts General Hospital, Boston MA; 2Newman Scholar,

Dept. Physiology, University College Dublin.

Methylprednisolone (mepd) sdmulated myoblast differentiation of primary mixed cultures from normal muscle, as assessed by the fusion index. In some mixed cultures from Ducherme and Becket Muscular Dystrophy (DMD, BMD), glucocor ticoids inhibit myobi~ t fusion 1. Analysis of clones derived from individual myoblasts from normal DMD heterozygote and dystrophic muscle has revealed 2 typos of cell, those that are fusion-enhanced by mepd, and those that are fusioninhibited. The overall response of mixed cultures to mepd w&~ reflected in the proportion of fusion-enhanced and fusion- inhibited clones that could be derived from the f'trst passage. Non- clonal cultures that were fusion-enhanced yielded a mixture of fusion-enhanced and fusion-inhibited clones. By contrast, fusion-inhibited mixed cultures yielded only fusion-inlfibited clones. Subeloning of fusion-enhancod clonesyielded both fusion-enhanced and fusion-inhibited clones; attempts to subclone fusion-inhibited clones were unsuccessful. The magnitude of the glucocorticoid response correlated inversely with the age of the patient, and the number of passages of myoblasts. These findings suggest that the effect o f steroids on myoblast fusion be a marker of the proliferative age of myoblast clones. This is of importance to myoblast transfer protocols in which myoblast clones with a maximum capacity for proliferation are required for injection. Reference

1. l la rdim an, O., 13 rown, R. tl..rr., Beggs, A. H., Specht, L., Sklar, R. M. Differential glucocordcoid effects on the fusion of Duchenne]Becker and control muscle cultures: ph amaacological detection of accelerated ageing in dystrophic muscle.

MITOCHONDRIAL NEUROPATHIES.

V. Reid, J. O. Dowd, R. Mmphy. Department of Neurology, Adelaide Hospital, Dublin 8.

We report 4 cases of histupathologieally proven mituehondrial myopathies which presented in the last 6 months and compare their clinical presentations, with a review of the recent literature on the investigation and diagnosis of mitochondrial myopathies.


Case 1 - wrongly diagnosed as myesthenia gravis aged 34 presented to us aged 62 with ptosis, ophthalmoplegia, difficulty with speech and weakness in his arms. Biopsy was positive for mitochondrial myopathy.

Case 2 - investigated for the past 4 years for ataxia with no conclusive diagno sis made. Presented aged 74 with increasing ataxia and frequent falls. Examination revealed slow eye movantents with impaired upward gaze, inereaseA tone with normal power in all four limbs, brisk reflexes and upgoing plantars. He was grossly ataxic and investigation revealed a raised CPK which was am'ibuted to his fall. Muscle biopsy revealed mitochondrial myopathy. Family history was positive for motor neurone disease.

Case 3 - an 18 year old boy presented with a 1 year history of fatique and pain in both of his shoulders, worse after exercise and he no riced he was intermittently weak on his left aide when trying to pick up heavy objects. Examination revealed mild ptosis, but his father also had this so it may just have been a familial likeness. Muscle biopsy revealed mitochondrial myopathy.

Case 4 - 55 year old man previously diagnosed as dystrophia tnyotonic, with two brothers who died of the same cotnpalint and two brothers alive with "drooping eyelids". Presented with progressive ptosis and external opthalmoplegia. Investigations included a raised CPK and muscle biopsy was positive for mitochondrial myopathy.

ANEURYSMAL BONE CYST OF THE FRONTAL BONE: CASE REPORT

E. Rashad, D. P. O'Brien, M. A. Farrell, J. 'roland, L P. Phillips. Departments of Neurosurgery, Neuropathology mad

Net~uradiology, Beaumont Hospital, Dublin.

A sixteen year old right handed male presented with a painful lump in the frontal bone area of the scalp. Six weeks prior to presentation, he complained of frontal headaches, worse in the morning and exacerbated by coughing and sneezing. These headaches progressively w ~ and became associated with nausea and vomiting. Two weeks prior to admission, the headaches subsided and then he noticed an enl urging, painful ]urn p on his forehead. There was no history of seizures or trauma. Clinically, this was a four centimetre bony lesion in the right frontal region without focal neurological signs. Radiologica] investigations confirmed a lyric bone lesion. This lesion was totally excised and histology confirmed this to be an aneurystnal bone cyst.

Aneurysmal Bone Cysts (ABD) are non-nenplastic osseous lesions of unknown aetinlogy. They are generally ftnmd in long bones and rarely found in the skull. They usually occur in children and yonog adults. ABCs usually present as a palpable scalp mass with or without signs of raised intracranial pressure (ICP). Symptoms of raised ICP occur in one-third of parients. The growth of the cyst is symmetrical, involving both the inner and outer tables of the skull. Complete excision of these skull lesions is the treatment of eheiee, with or without eranioplasty. We present the ease of a young male presenting with this unusual, rare tumour of the frontal bone. The clinical, radiological and pathological features are outlined and the literature reviewed.

NEOCORTICAL FIELD POTENTIAL IN FOCAL EPILEPSY.

H. Staunton, Peng Shu-Ming, A. Coughlin. J. P, Phillips. Richmond Institute of Neurology & Neurosurgery, Beaumont

Hospital, Dublin.

Seizures of a focal origin have a prevalenea of 4 per thousand. At

IJ.M S. November, 1993

most 60% are drug controlled. Of these about 50% prove to have foci which meet the criteria for surgery. Thus, there are approximately 2,500 such patients in the Irish Republic who are candidates for such surgery.

A number of procedures can be performed for intractable temporal lobe epilepsy. Despite the fact that the amygdal a and hippoc ampu s have a well known propensity for seizure discharge, one can see a curative response in certain cases to neocorricoctomy alone. Further, the spike potential (paroxysmal depolarization shift - PDS) aeen hi temporal lobe epilepsy cannot be explained on the basis of passive volume conduction fxom a potential generated in deeper structures.

We have performed laminar field potentials in an examination of the surface spike. We have established, in four cases, a sink-source relationship in such fields, indicating a capacity for the superficial cortical structurs to create epileptic discharges. It i s possible that in temporal lobe epilepsy there are:

(1) those cases in whom there is a main generator in the hippocampus and/or amygdala with"driven satellite foci" which are nevertheless capable of independent epileptogenic activity in the neocortex and (2.) those cases in which the primary generator is in the nencortex.

MODERATE HYPOTHERMIA PROTECTS AGAINST SPINAL CORD ISCHAEMIA.

D. M. McCoy, A. Qureshi,* M. A. Black, P. A. Grace, D. Bouchier-Hayes*, R. Dwyer.

Departments of Anaesthesia and Surgery*, Beaumont Hospital, Dublin 9.

Paraplegia has a 40% incidence after thoracic aortic cross- clamping, due to spinal cord ischaemia ~. Lowering brain temperature by only a few degrees during isehaemia is known to confer a marked protective effect 2. We investigated whether moderato hypothermia in fl ueneed neurological outcome after spinal cord ischaemia. Twelve dogs breathing N20 66% in 02 received 1.3% end-tidal isoflurane. After thoracototny, the descending thoracic aorta w as cross-clamped for 30 minutes. Mean arterial pressure (MAP) was monitored proximal and distal to the cross-clamp and pulmonary artery (PA) pres- sures and cardiac output (CO) measured, Cerebro spinal fluid (CSF) pressure was measuredvia a needle inserted in the cistema magna. Sodiumnitroprusside (SNP) (2-10 ug/kg/tnin) maintained the proximal MAP in the range 100-20(} mmHg. In group I (n-7) (randomly assigned) body temperature (measured by pulmonary artery thermistor probe) was reduced by packing the thorax with ice prior to cross clamp, and maintained at this level with cooled intravenous normal saline infusion during the ischaemic insult. In group 11 (n=5) temperature was maintained at pre clamp levels using an overhead lamp and heating blanket. 24 hours later neurological status was assessed by scoring motor function in the hind legs (Tarlov scale: O=-fully patalysed. 4=no neurological deficit).

Proximal and distal MAP, CSFP and spinal cord perfusinn pressure did not differ between bn-oups. However neurological out- comes were significantly different: Tm'lov Score 0 (isoBurane). versus 2 (isoflurane and hypotherrnia) (medians) (13<0.05 Mann Whitney U). We conclude that moderate hypothermia protects against spinal cord ischemia due to decreased perfusion pressure during thoracic aortic crossclamp. References: 1. McCullogh, J. L, l lollier H. L, Nu gnat M. Paraplegia after thoracic aortic occlusion. J. Vase, Surg. 7:153-60.I988 2. Busto. R., Dalton Dietrich, W. Globus M. Y. T., Valdes, L, Seheinberg. P., Ginsberg, M. D. Small differences in intraischemie brain temperature critically determine the extent of ischemie neurona/injury. J. Cereb. Blood Flow Metabol. Vol. 7: No. 6.1 987

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