proceedings association british neurologists society ... · neurovascular compression in cra-nial...

200 JYournal ofNeurology, Neurosurgery, and Psychiatry 1995;59:200-218

Proceedings of the Association of British Neurologists and theSociety of British Neurological Surgeons, University ofLiverpool, 5-7 April 1995

Platform Presentations

LOWER CRANIAL NERVE COMPRESSIONSYNDROMESJB Miles, PR Eldridge, L Dunn,I Mackenzie, J Meaney. Walton Centre forNeurology and Neurosurgery, Liverpool, UK

Objectives-To validate the causal signifi-cance of compression and the effectivenessand safety of microvascular decompression(MVD) in syndromes of the V, VII, VIII, IXand Xth cranial nerves.Design-Clinical, neurological, and imagingassessment, including magnetic resonancetomo-angiography (MRTA),2 followed byMVD during continuous brainstem (audi-tory) monitoring.Patients-110 patients with trigeminalneuralgia (TGN); 25 patients withhemifacial spasm (HFS); two patients withtinnitus; six patients with IX or X neuralgia;six patients with systemic hypertension(HBP).Outcome-The quantitative effectiveness ofMVD including morbidity data, has beenestablished by questionnaire and interviewover the 15 year study.Results-6% of TGN recurred; 20% HFSwas unrelieved or recurred; the two patientswith tinnitus were satisfactorily relieved; fiveof the six patients with IX/X neuralgia werecured, and three of the six patients withHBP seemed improved.Conclusions-Microvascular decompressionis more effective than other existingmethods in relieving symptoms and signs oflower cranial nerve compression syndromeswith acceptable safety using diagnosticimaging and interoperative monitoring.Outcome measurement supports thecompression as being significantly involvedin achieving relief rather than this beingsimply due to nerve injury.[SBNS]

1 Janetta PJ. Neurovascular compression in cra-nial nerve and systemic diseases. Ann Surg1980;192:518-25.

2 Meaney JFM, Miles JB, Nixon TE, et al.Vascular contact with the fifth cranial nerveat the pons in patients with trigeminal neural-gia: detection with 3D FISP imaging. A7RAm_JRoentgenology 1994;163:1447-62.

RISK OF ACCIDENTS IN DRIVERS WITHEPILEPSYD Chadwick. University of Liverpool,Liverpool, UK

Between June 1992 and May 1993 a surveywas undertaken of driving and accidentexperience in people with a history ofepilepsy communicating with the DVLA.24 000 questionnaires were issued as part ofthe routine relicensing of drivers at threeyear intervals, of which just over 17 000were returned (response rate of 71 %). Inaddition, 468 questionnaires were issued todrivers reporting recent seizures at theDVLA of which 291 were returned(response rate of 62%). The questionnaireused identical methods to a cohort of non-

epileptic drivers carried out between 1990and 1991 by the Transport and RoadResearch Laboratory (8888 drivers). Bothsurveys were retrospective and collecteddata for three years before the survey.The relative risk for involvement in any

accident during this period was 0 95 (95%CI 0-88-1-02). However, the adjustedrelative risk for involvement in an injuryproducing accident was somewhat raised inthe epilepsy group 1 1 (0-91-1-3). Theadjusted odds ratio for serious physicalinjury was 1-37 (1-02-1-84). In the epilepsygroup there were 12 drivers involved in anaccident resulting in a fatality, but none inthe TRL group.The factors that influenced the risk of

accident rates were not dissimilar in the twogroups, age, driving experience, andmileage being the most important. Theabsence of any seizure within the three yearperiod of the survey halved the risk ofinvolvement in an accident producing someform of serious physical injury or fatality.No other clinical factors in the epilepsygroup seemed to influence accident risks.[ABN]

NEURAL TRANSPLANTATION BY ERHITCHCOCK: TREATING PARKINSON'SDISEASE BY GRAFTS OF FOETALMESENCEPHALON AND STRIATUMCHA Meyer. Midlands Centre forNeurology and Neurosurgery, Birmingham,UK

Objectives Experimental neurosurgicaltreatment of Parkinson's disease.Design-Since 1988, 55 patients with severeParkinson's disease (Hoehn and Yahr stageIV, V) have been treated at the MidlandsCentre for Neurology and Neurosurgery bystereotactic implantation of brain tissuefrom spontaneously aborted humanfoetuses. Each implantation used a singlefoetus of second trimester (11-20 weekspost-gestational); grafts dissected 5-12hours after foetal expulsion, disaggregatedmechanically, and implanted as clumps (notas cell suspensions); there was no immuno-suppressive therapy.

Consecutive patients formed five seriesdiffering in site of implantation (caudate orputamen, unilateral or bilateral) and innature of graft (foetal mesencephalon aloneor with striatum).

In the final series (described here) graftsof foetal mesencephalon and striatum wereimplanted bilaterally into the head of thecaudate nucleus.Patients-Seven patients (Hoehn and Yahrstage IV, V) aged 46-62 years.Results-All seven patients improved. Therewere very good group improvements,statistically significant (Wilcoxon), in a widerange of activities of daily living measures,clinical neurology, and timed motor taskssustained throughout 18 months followup-with substantial reduction in need fordopaminergic medication.

Conclusions-Results were strikingly betterthan those of all four previous series, whichused foetal mesencephalon alone. Thisexperimental technique shows promise fortreating Parkinson's disease.[SBNS]

EN BLOC TEMPORAL LOBECTOMY VSELECTIVE AMYGDAIOHIPPOCAMPECTOMY ASTREATMENTS FOR INTRACTABLE EPILEPSYDUE TO HIPPOCAMPAL SCLEROSISJM Oxbury, CBT Adams, SM Oxbury, KNCarpenter, SA Renowden. RadcliffeInfirmary, Oxford, UK

Various surgical treatments for intractableepilepsy due to hippocampal sclerosis arerecommended. It is uncertain which is mosteffective. Sixty nine patients with thiscondition were treated-50 by Falconertype en bloc temporal lobectomy (TL), 19by selective amygdalohippocampectomy(AH). The two methods are compared. Ofparticular concern was seizure outcome andverbal memory loss after left operations.Eleven AH were transcortical via the middletemporal gyrus, and eight were trans-sylvian.Two years after operation 54% had not

had any seizure since surgery; 23% hadexperienced only very occasional seizures(77% in Engel grades I+II). Thirty of 41cases (73%) were seizure free five yearsafter operation. Neither seizure outcomenor serious unwanted effects differedsignificantly according to type of surgery.

Neuropsychological assessment two yearsafter operation showed less verbal memorydecline after left AH than after left TL. LeftAH, but not left TL, gave significantimprovements in verbal IQ and non-verbalmemory. Performance IQ improved in bothgroups. Postoperative MRI showed lesstemporal lobe white matter disruption aftertrans-sylvian AH than after the transcorticalapproach.

It is concluded that trans-sylvian AH ispreferable to TL as a treatment for mostcases of intractable epilepsy due to lefthippocampal sclerosis and probably alsothat due to right hippocampal sclerosis.[ABN]

VOLUMETRIC MAGNETIC RESONANCEIMAGING (MRI) IN CHRONIC EPILEPSYSM Sisodiya, SL Free, DR Fish, SDShorvon. Institute ofNeurology, London, UK

Volumetric MRI enables detailed examina-tion of cortical anatomy. An investigationinto chronic epilepsy using MRI and post-processing techniques is being carried out.

Scanning patients with primary gener-alised epilepsy showed underlying structuralabnormalities in six cases. Three had sub-ependymal heterotopia and three hippo-campal asymmetry: one of these improvedafter temporal lobectomy. Reconstruction of

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a three dimensional image of the cerebralhemispheres and comparison of the gyralpattems demonstrated with control subjectsshowed gyral abnormalities in 16 of 30patients with normal MRI on inspection ofroutine two dimensional images, and addi-tional gyral abnormalities in three of 18patients with cortical dysgenesis observed on

routine images. Analysis of the distributionof cerebral grey/white matter, by division ofreconstructed objects into coronal 1 Oths,allows measurement of cerebral structure.The existence of structural abnormalitybeyond the visualised lesion was shown in 15of 18 patients with cortical dysgenesis on

routine imaging and in nine of 14 patientswith lesions demonstrated only on recon-

structed images. This supports the hypothe-sis that cortical dysgenesis is an extensivedisorder. Block analysis has also shownstructural disruption in patients with mentalretardation and epilepsy with normal imag-ing and reconstruction.

Volumetric MRI is important in theinvestigation of epilepsy. Visual inspectionof data alone is not adequate in cases whereno lesion is found.[ABN]

IMPROVEMENT OF EPILEPSY AFTER

STEREOTACTIC RADIOSURGERY FORARTERIOVENOUS MALFORMATIONS

AA Kemeny, M Foroughi, L Walton, DMCForster. National Centre for StereotacticRadiosurgery, Sheffield, UK

Objectives-Stereotactic radiosurgery (STRS)is primarily aimed at achieving thrombo-obliteration of arteriovenous malformations(AVMs). This study was carried out to eval-uate the degree of amelioration in seizureactivity observed clinically as an additionalbenefit after STRS.Design-Telephone interview with patientsand analysis of the prospectively maintaineddepartmental database.Patients-From over 1500 patients 269 hadepilepsy; 149 were included in the analysiswho fulfilled the following criteria: activeepilepsy before STRS; follow up longerthan two years; angiographic control.Outcome measures-The patients classifiedthe severity and frequency of their seizuressubjectively into improved/no change/worse.A category of "no fits" was assigned tothose with no seizures for a year.Results-Fifty four per cent of patientsbecame fit free and 29% had subjectiveimprovement. The size of the AVM nidusand the radicality of the treatment planaffected the change in epilepsy. More than50% of those whose AVMs were not obliter-ated reported worthwhile improvement intheir epilepsy.Conclusion-Improvement after STRS inepilepsy is a realistic expectation in mostpatients with AVM.[SBNS]

"C-FLUMAZENIL PET AND VOLUMETRIC MRIIN MESrAL TEMPORAL LOBE EPILEPSYMJ Koepp, MP Richardson, DJ Brooks,DR Fish, JS Duncan. Institute ofNeurology, London, UK

"C-Flumazenil PET has been used to

compare extent of loss of benzodiazepinereceptor density with volumetric MRI inpatients with epilepsy due to mesialtemporal sclerosis (MTS). "IC-Flumazenilbinding has been shown to be reduced inMTS. To date, no studies have formallycompared extent of sclerosis with volu-metric MRI and 3D "IC-flumazenil PET-changes. It is hypothesised that thereduction of hippocampal BZ/GABA-Areceptor density in patients with MTS isover and above the loss of hippocampalvolume.Ten patients with MTS and pronounced

unilateral hippocampal sclerosis werestudied. "IC-Flumazenil PET Vd imageswere produced. These scans were co-registered with high resolution MRI. Thehippocampus and neighbouring gyri weredelineated on coronal MRI sections.Asymmetry indices were compared with 10age matched normal controls. In all patientsreduction of "C-flumazenil Vd wascorrelated with the side of hippocampalsclerosis. On average 'IC-flumazenil Vd wasreduced in the smaller hippocampus by20%. Changes of BZ/GABA-A receptordensity in the other hippocampus lay withinnormal limits.

In conclusion, there is a reduction ofBZ/GABA-A receptor density in thehippocampus of patients with mesialtemporal lobe epilepsy over and above lossof hippocampal volume. This may be ofimportance in planning the extent ofresection in patients with MTLE.[ABN]

SEIZURE FUNCTIONAL OUTCOME ONE YEARAFTER HEMISPHERECTOMYGF Tuite, JH Cross, BG Neville, WFJHarkness. Great Ormond Street Hospitalfor Sick Children, London, UK

One year outcome data after hemispherec-tomy in 20 paediatric patients with severemedically intractable seizures is presented.Their average age at the time of surgerywas 4-7 years (range 0-5 to 16) with anequal number of boys and girls. All patientshad a preoperative hemiparesis but theyvaried widely in their neurological disability.Two patients presented with epilepsia par-tialis continua. Preoperative electro-encephalography and MRI localised theabnormality to a single hemisphere in allcases (9L, 1 1R).A functional hemispherectomy was the

preferred operation, but anatomical hemi-spherectomy was performed in three cases.Most had hemimegancephaly (five) orcortical dysplasia (six); the rest had aporencephalic cyst (four), Rasmussen'sencephalitis (two), Sturge-Weber syndrome(two), and a large extradural cyst (one). Allpatients tolerated their operation well, withno complications in the immediate peri-operative period.

All of the patients have had at least a 75%reduction in seizure frequency and 16 areseizure free (follow up one to 3-5 years).Three patients (15%) have required theplacement of a ventriculoperitoneal shunt.There was worsening of motor function intwo patients. The striking feature in mostcases was the developmental improvementthat occurred after surgery.[SBNS]

SUBARACHNOID HAEMORRHAGE AND THE

INFLAMMATORY CYTOKINE RESPONSERH Lye, WR Lamb, PEC Brenchley.University of Manchester, Manchester, UK

Introduction-There is evidence that theimmune system is activated afteraneurysmal subarachnoid haemorrhage(SAH). The proinflammatory cytokinesIL-if?, IL-6, and TNFa were measured in10 patients who had had SAH.Method-The in vivo cytokine response wasmonitored in eight patients by dailymeasurements of systemic concentrations inserum during their hospital stay. A wholeblood endotoxin stimulation assay was alsoused for in vitro assay of cytokine in thesame blood samples. This gave a measure ofeach patient's total capacity to producecytokines in response to a stimulus.Results-All patients underwent craniotomywith clipping of the aneurysm. No systemiccytokine release was seen among this group,but the in vitro cytokine pattem during thepostoperative period showed an upwardtrend in two patients, a downward trend inthree, and violent oscillation of cytokine overseveral days in the remaining three patients.Interestingly, all patients showed a signifi-cant decrease in their ability to secrete TNFaafter operation (P < 0 05) with a highly sig-nificant corresponding increase in the twotypes of soluble TNF receptors TNFp55 andTNFp75 (P < 0-001). Similar findings werenot seen for either IL-la or IL-6.Conclusions-These preliminary findingshighlight an alteration of the physiologicalproduction of TNFa and its receptors p55and p75 after SAH.[SBNS]

THE EFFECT OF CSF PROTEINCONCENTRATION ON SUBSEQUENT SHUNTCOMPLICATIONS: A PROSPECTIVE STUDYHL Brydon, RD Hayward, WFJ Harkness, RBayston, EJ Thompson, G Keir. Hospital forSick Children, Institute of Child Health, andInstitute ofNeurology, London, UK

Objective-To assess the effect of the CSFprotein concentration on subsequent shuntcomplications.Design-A prospective clinical studycomparing complications arising within twomonths of surgery to the peroperative CSFprotein concentration.Subjects-Ninety five patients undergoingshunt surgery (new insertion or revision)during a 15 month period. In total, 132definitive shunt operations were performed.Outcome measures-Shunt infection, obstruc-tion and overdrainage occurring within eightweeks of surgery. Statistical analysis was per-formed with the Mann-Whitney U test andthe X 2 test.Results-There were 12 instances of shuntobstruction, 10 shunt infections and threepatients with significant overdrainage. Thetotal protein content of the infection (0 -1 >P > 0 05) and obstruction groups (0 5 > P >0-1) was not significantly different fromthose without complications. The over-drainage group had significantly less CSFprotein (0-5 > P > 0-02), but this group onlyconsisted of three CSF specimens, all ofwhich had a normal protein content.Conclusions-A raised CSF protein contentdoes not increase the risk of shunt

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complications; there is no reason whyshunting should be delayed in patients witha high CSF protein content.[SBNS]

NUMB-CLUMSY HAND IN HIGH CERVICALCORD LESIONSV Kunanandam, K Ajay Kumar. Hull RoyalInfirmary, Hull, UK

Objective-To determine the pathogenicmechanism for numb-clumsy hand.Design-Correlation of clinical features,radiological findings (MRI), and pathologyin patients with numb-clumsy hand.Subjects-30 patients with numb-clumsyhands were identified in a series of 53patients operated for high cervical cordlesions (above C3/4) during the period1993 to 1994 (September).Assessment-Evidence of posterior columninvolvement, cord signals in MRI and/orsevere compression in CT myelogram, andnature of pathological lesions.Results-Numb-clumsy hand was associatedwith no objective posterior column involve-ment (clinical or EMG/NC study), withsevere cord compression on CT myelo-gram/MRI and cord signal on MRI and withpathological lesions: C3/4 spondylosis (19),C1/2 rheumatoid subluxation (five),tumours (two), and others (four).Conclusion-Numb-clumsy hand was associ-ated with high cervical (C 1-C4) lesions caus-ing severe anterior central compression ofcord and intrinsic cord signal on MRI, butminimal objective neurological signs.Pathogenic mechanisms are probably relatedto compression and disconnection of spino-cerebellar tracts. 1 2

1 Chang MH, Liao KK, Cheung SC, et al."Numb clumsy hands" and tactile agnosiasecondary to high cervical spondyloticmyelopathy: a clinical and physiological cor-relation. Acta Neurol Scand 1992; 86:622-5.

2 Voskuhl RR, Hinton RC. Sensory impairmentin the hands secondary to spondylotic com-pression of the cervical spinal cord. ArchNeurol 1990;47:309-1 1.

[SBNS]

FAMILIAL NON-SPECIFIC DEMENTIA MAPS TOCHROMOSOME 3JM Brown, T Ashworth, S Gydesen, MRossor, J Collinge. St Mary's HospitalMedical School, London, UK

A substantial minority of patients with prese-nile dementias lack distinctive inclusionbodies, plaques or tangles on pathologicalexamination. Such dementias typically pre-sent with evidence of frontal lobe dysfunc-tion and are probably the commonest causeof frontal lobe dementia. These dementiashave been termed non-specific dementias(NSD). Half the reported cases ofNSD havea positive family history of dementia. Clinicaland molecular genetic studies on the largestpublished family with NSD have been con-ducted. There are 20 affected patients inthree generations, the disease is inherited inan autosomal dominant fashion. After run-ning over 170 microsatellite probes andexcluding 75% of the genome the diseaselocus has now been assigned to the peri-centromeric region of chromosome 3.Haplotype analysis shows a common regionshared between all affected patients. The

maximum pairwise lod score obtained was+4-2, multipoint analyses increased this to+ 5-8. The clinical studies show that pater-nally inherited cases develop the disease at asignificantly lower age than their affectedparent. This anticipation of the disease onsetwith paternal transmission suggests that thedisease is produced by a trinucleotide repeatexpansion mutation. Some other neuroge-netic diseases, including Huntington's dis-ease, have now been related to suchmutations.[ABN]

NEUROPSYCHOLOGY AND SPECT IN

DIAGNOSIS AND STAGING OF ALZHEIMER'SDISEASEJDW Greene, JR Hodges. University ofCambridge, Cambridge, UK

Given the advent of drug treatments fordementia of the Alzheimer type (DAT), itis important to develop more accurate meth-ods for diagnosis and staging. The role ofneuropsychology and SPECT imaging indiagnosis and staging DAT in 33 patientswith mild disease and 30 matched controlswere studied. Working, episodic, remote,and semantic memory were assessed. Tc-HMPAO SPECT was analysed quantita-tively to obtain measures of regional cerebralblood flow.

For diagnosis, the memory tests werehighly discriminatory, with a canonical cor-relation coefficient of 0 975. The most usefultest was delayed verbal recall; SPECT datawere, by contrast, of less use in predictinggroup membership; 33% of patients had nor-mal SPECT scans.

For staging, stepwise regression analysisusing all memory tests could predict 79% ofthe variance in mini mental state examina-tion scores. The measures most useful forstaging were immediate, recognition, seman-tic, and autobiographical memory. SPECTdata were again of less use in modellingseverity of disease; only 38% of the variancein MMSE scores could be predicted fromSPECT data.

Neuropsychology has been shown to besuperior to SPECT both in diagnosis andstaging DAT. Different memory tests areuseful for diagnosis and staging: delayed ver-bal recall is the best discriminator, whereasimmediate, recognition, autobiographical,and semantic memory, are of most use instaging severity.[ABN]

MITOCHONDRIAL ENCEPHALOMYOPATHYWITH MULTIPLE MITOCHONDRIAL DNA

DELETIONS: A REPORT OF TWO FAMILIES ANDTWO SPORADIC CASES WITH UNUSUAL

CLINICAL AND NEUROPATHOLOGICALFEATURESRM Chalmers, M Brockington, RS Howard,BRF Lecky, AE Harding. National Hospitalfor Neurology and Neurosurgery, London,UK

Most diseases associated with abnormalitiesof human mitochondrial DNA (mtDNA)occur either sporadically or show maternalinheritance. Recently, a mitochondrialmyopathy associated with multiple deletionsof mtDNA has been identified in pedigreesthat show an autosomal dominant mode of

inheritance. In this syndrome the mtDNAdefect is presumed secondary to a nucleargene mutation.The first two British kindreds with this

disorder and two sporadic cases arereported. All patients had adult onsetprogressive external ophthalmoplegia andmuscle weakness, with muscle biopsyappearances of a mitochondrial myopathy.Southern blot analysis of total muscle DNAdigested with appropriate restrictionenzymes shows a pattern consistent withmultiple populations of mtDNA.The cases exhibit, in addition, some

unusual clinical features. These include pig-mentary retinopathy and tremor; the last waslevodopa responsive and associated withrigidity and micrographia in one family. Onefamily and both sporadic cases have a neu-ropathy and nerve biopsy confirms the indi-cation of an axonal neuropathy suggestedfrom nerve conduction studies. Postmortemexamination of one patient showed pro-nounced nigral degeneration, which is ofinterest in relation to the parkinsonism seenand the evidence of mitochondrial chain dys-function in Parkinson's disease.[ABN]

OCULOMOTOR FUNCTION IN MOTOR NEURONDISEASES Shaunak, E O'Sullivan, R Orrell, R Lane,C Kennard. Charing Cross and WestminsterMedical School, London, UK

Although it is generally held that the oculo-motor system is spared in motor neuron dis-ease (MND), several recent oculographicstudies of reflexive saccades and smooth pur-suit in MND have yielded conflicting results.Oculomotor function was therefore studiedin a range of paradigms, some of which areconsidered to involve the frontal lobes intheir execution.

Eye movements in 17 patients with MNDand 11 age matched normal controls wererecorded with the scleral search coil tech-nique. Smooth pursuit, fixation, andreflexive, remembered, and antisaccadeparadigms were studied. Results were anal-ysed for saccadic latency, peak velocity, gain,final eye position (FEP), antisaccade/remembered saccade error rates, pursuitpeak velocity gain, and square wave jerk fre-quency. Reflexive saccades were unimpairedin patients with MND compared with con-trols. Error rates for both antisaccades andremembered saccades were elevated in theMND group (antisaccades: MND 31-9(24-9)%, control 10-3 (9 6)%, P = 0 004;remembered saccades: MND 30-8 (24 6)%,control 12-4 (14-4)%, P = 0 02). Anti-saccade and remembered saccade latencieswere also prolonged (antisaccades: MND376-9 (87 9) ms, control 303 5 (53 9) ms,P= 0-01; remembered saccades: MND 318- 1(74-9) ms, control 258-3 (43 76) ms, P =

0-02). Square wave jerk frequency wasincreased in patients with MND (MND 32-9(19-6) min, control 8-7 (4 7) min, P =

0-0007), but smooth pursuit showed noimpairment.

These findings of prolonged volitionalsaccade latency and high distractability sug-gest prefrontal impairment in MND, and areconsistent with the results of recent neuro-psychological, pathological, and PET studies.[ABN]



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A STUDY OF THE CAUSES OF LATE

FUNCTIONAL DETERIORATION AFTER

PREVIOUS POLIOMYELITISD Kidd, RS Howard, E Chroni, FWHeatley, A Williams, GT Spencer. StThomas' Hospital, London, UK

Many patients with previous poliomyelitisdevelop "postpolio syndromes" (PPS) inwhich late functional deterioration follows a

period of relative stability. The frequencywith which PPS can be attributed to clearlydefined causes remains uncertain. A reviewof newly referred patients with previouspoliomyelitis seen consecutively over a threeyear period was undertaken. There were

156 patients (51 men, 105 women, mean

age 52 (range 17-80) years) who haddeveloped acute paralytic poliomyelitis at a

mean age of 6 (0-5-42) years. Eighteenpatients had been ventilated, and 124 hadhad severe weakness during the acute

illness. After recovery, 22 were wheelchairbound and 31 mobile with severe disability.140 patients (90%) developed symptoms offunctional deterioration at a mean timeinterval of 42 (17-76) years after theparalytic illness. Functional deteriorationwas associated with orthopaedic disordersin 125 cases, neurological disorders in 16,respiratory disorders in 12, and others in13. Treatment of bone and jointabnormalities including surgery (26 cases)and provision of orthotic appliances (70cases) with physiotherapy, was associatedwith functional improvement in 90% ofthose followed up. Other treatmentsincluded weight loss, ventilatory support,and pain relief. In this series progressivepostpolio muscular atrophy was not noted.Functional deterioration was common andassociated with orthopaedic, neurological,respiratory, and general medical factorswhich are potentially treatable.[ABN]

EFFECT OF SURGICAL REPAIR ON PAIN RELIEF

AFTER BRACHIAL PLEXUS INJURY IN ADULTS

JS Berman, M Taggart, P Anand, R Birch.Royal London Hospital Medical Collegeand Royal National Orthopaedic Hospital,London, UK

The characteristic pain after root avulsionbegins within weeks of brachial plexusinjury in adults, and persists for many years,

still being severe in 25% of patients afterfour years. Surgical treatment of avulsioninjuries led to preliminary observations thatthe surgery accelerated resolution of pain.This study examined the effect of surgery-nerve grafting and transfer-on the durationand severity of root avulsion pain. 487patients were studied prospectively over 10years with preganglionic root avulsionsalone or in combination with postganglionicruptures or lesions in continuity (LIC).They were compared with pure ruptures

and pure LIC. In the avulsion group, painwas reduced substantially at an average timeof 175 days after operation, in comparisonwith 68 days for the pure rupture group and58 days for the pure LIC group. Of the 39pure avulsion patients, only three reportedno improvement in their pain 20 monthsafter surgery, the median time forimprovement being 120 days. Thesefindings support the view that nerve repair

accelerates pain amelioration after brachialplexus injury. Pain resolution usually

preceded recovery of motor or sensory

function.[ABN]

A COMPREHENSIVECLINICONEUROPHYSIOLOGICAL STUDY OF

IDIOPATHIC GENERALISED EPILEPSIES WITH

ABSENCES IN ADULTS

CP Panayiotopoulos, S Giannakodimos. StThomas' Hospital, London, UK

Eighty five patients over 16 years of age withtypical absences (TAs) were studied. All hadEEG confirmation, and 47% had additionalvideo-EEG documentation of TAs.Prevalence of TAs was 22% among 410adults with epilepsies and 63% among idio-pathic generalised epilepsies (IGE).The clinico-EEG manifestations of

absences were syndrome related. 30 patientshad juvenile myoclonic epilepsy; all had mildabsences and myoclonic jerks on awakening,and all but two had generalised tonic-clonicseizures (GTCS). 10 patients had juvenileabsence epilepsy; all had severe absences andall but one GTCS. 11 patients had eyelidmyoclonia with absences, a syndrome char-acterised by eyelid myoclonia with absences,and photosensitivity. Seven patients hadperioral myoclonia with absences, a syn-

drome characterised by TAs associated withictal rhythmic perioral myoclonia. Fourpatients had predominantly photo or patterninduced TAs. Twelve of the remainingpatients, showed remarkably similar clinico-EEG features, which were not fortuitous. Anew syndrome characterised by "phantomabsences" and GTCS, is proposed. Thename "phantom absences" has been coinedto denote TAs that are so mild that they are

inconspicuous to the patient and invisible to

the observer. Absences in adults constitute a

diagnostic challenge to physicians and haveimportant implications in the syndromicclassification and management of patients.[ABN]

DIAGNOSIS AND MANAGEMENT OF EPILEPSY

AND "FUNNY TURNS" IN A SPECIALIST CLINIC

D Smith, B Dafalla, DW Chadwick. WaltonCentre for Neurology and Neurosurgery,Liverpool, UK

Patients with "funny turns" are oftenreferred to neurologists with a previous or

possible diagnosis of epilepsy. This studywas designed to assess the frequency of fac-tors contributing to and the consequences ofan erroneous diagnosis of epilepsy, the out-

come of patients referred with "refractoryseizures", and the relative roles of clinicalacumen and investigations in establishingthe cause of "funny turns".A retrospective analysis ofthe case records

of 324 patients was conducted. The samplewas divided into those exposed to antiepilep-tic drugs (AEDs) (n = 184), of whom 92were said to have refractory seizures, andthose who had not received treatment (n =140).The overall misdiagnosis rate was 26-1%

(46/184) with inadequate history taking andmisinterpretation of EEG equally respon-

sible. 19/40 reported side effects from AEDswhile unnecessary driving restrictions and

employment difficulties were encounteredby 12/33 and 5/33 respectively. Of thoselabelled "refractory epilepsy", 12 did nothave epilepsy and 41 were rendered seizurefree or significantly improved by optimal useof AEDs or surgery. Diagnosis of "funnyturns" was usually achieved on clinicalgrounds alone except in patients withpseudoseizures on AEDs 58% of whomrequired investigation to refute the diagnosisof epilepsy.

These data show suboptimal diagnosticand management services for patients with"funny turns" and epilepsy respectively andprovides a strong case for expansion of spe-cialist services and open access to multidisci-plinary epilepsy clinics.[ABN]

REGIONAL CEREBRAL BLOOD FLOW DURING

POSTERIOR SEIZURES: AN HMPAO SPECT

STUDYR Duncan, J Patterson, R Roberts, DHadley. Universities of Glasgow andDundee, UK

Ictal HMPAO SPECT was used to study fivepatients whose clinical and surface videoEEG data indicated seizure origin in the areaof the temporoparieto-occipital junction(TPOJ). Three patients had structurallesions in the area of the TPOJ. HMPAOinjection was carried out from 30-120 sec-onds from clinical seizure onset. Acquisitionwas carried out with a Strichmann 810 dedi-cated head imager.

Interictal SPECT showed perfusiondeficits in keeping with anatomical lesions inthree patients, hemispheric hypoperfusionon the side of electrical seizure origin in afourth, and was normal in a fifth. Ictalimages showed hyperperfusion in the area ofthe TPOJ in all five patients. This hyper-perfusion extended anteriorly into the ipsi-lateral lateral temporal cortex and pole, withhyperperfusion of a lesser degree in the ipsi-lateral mesial temporal cortex, and ipsilateralhyperperfusion of the basal ganglia in twopatients. In two patients there was ictalhypoperfusion of the rest of the ipsilateralhemisphere. Measured as a change in asym-metry index with respect to the interictalimage, ictal hyperperfusion was most pro-nounced at the TPOJ in all five cases.

It is concluded that ictal SPECT showsdistinct patterns of perfusion in TPOJseizures, and can distinguish them frommesial temporal seizures.[ABN]

EYELID MYOCLONIA WITH ABSENCES: A

CLINICAL AND VIDEO-EEG STUDY IN ADULTS

S Giannakodimos, CP Panayiotopoulos. StThomas' Hospital, London, UK

Eyelid myoclonia with absences (EMA) is anidiopathic generalised epilepsy (IGE) syn-drome, manifested by eyelid myocloniaassociated with brief absences and photosen-sitivity. The ictal EEG shows generalised3-6 Hz spike/polyspike-slow wave activity,mainly on eye closure.

This is the first clinical and video-EEGstudy of adults with EMA. Their prevalencein the adult epilepsy clinic was 2-7% among410 epileptic patients, 8-1% among patientswith IGE, and 12-9% among patients with


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IGE with absences. There were 11 patientswith EMA, all female, with a mean age of30 9 years. Reported onset of absences wasat a mean age of 7-8 years. Eyelid myocloniawith absences was confirmed with video-EEG in 10 patients. The seizures consistedof eyelid myoclonia alone or preceding brief(3-6s) absences. Ictal clinical and EEGmanifestations occurred mainly after eyeclosure and were inhibited by total darkness.Only five patients showed photoparoxysmalresponses as adults, although all had a pre-vious clinical and/or EEG documentation ofphotosensitivity. None of the patients hadself induced seizures. Infrequent generalisedtonic clonic seizures occurred in 10 patients,and were precipitated mainly by lights, sleepdeprivation, fatigue, and menstruation. Mildmyoclonic jerks of the upper limbs occurredin six patients.

Eyelid monoclonia with absences is oftenresistant to medication and persists intoadult life.[ABN]

MEDULLOBLASTOMA: HAS THERE BEEN ANYIMPROVEMENT IN FIVE YEAR SURVIVAL OVERTHE PAST 20 YEARS?KM David, ATH Casey, RD Hayward, WFJHarkness. Great Ormond Street Hospital forSick Children, London, UK

The large number of cases of medulloblas-toma seen at Great Ormond Street allows aunique opportunity to view longitudinallythe effect that modem methods of investiga-tion and treatment have on the prognosis. Acohort of cases treated over a 10 year periodfor whom a five year follow up was available(1980-9) have been analysed. Eighty chil-dren were admitted during this time, ofwhom 75 went on to receive radiotherapyand/or chemotherapy (operative mortality5%). Forty patients were alive and 38 diseasefree at five years after diagnosis-90% ofthose with favourable prognostic features (noperioperative spinal metastases, radicalsurgery) and 10% of those without.Comparison with an earlier cohort of casesfrom this institution (1965-74) gives the fol-lowing information.'Modem methods of investigation and

treatment have clearly improved the outlookfor patients with medulloblastoma-but thegreatest advance remains the changes toradiotherapy regimes introduced in the1 960s. It is suggested that there is unlikely tobe any further dramatic improvement untilradical changes can be made in either thechemotherapy regimes (for example, highdose chemotherapy with bone marrow res-cue) or in immunological or gene mediatedtherapies.

1 McIntosh N. Medulloblastoma-a changingprognosis? Arch Dis Child 1979;54:200-3.

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MORBIDITY OF SURGERY FORINTRAVENTRICULAR CRANIOPHARYNGIOMASMJ Davies, TT King. Royal LondonHospital, London, UK

Craniopharyngiomas situated wholly withinthe third ventricle are rare and only 31 cases

(including the cases to be presented here) arereported in the literature.' Five cases treatedsurgically by one of the authors (TTK) arereviewed. Three of these have previouslybeen reported. Two new cases are presentedand long term follow up on the earlier casesis reported. Intraoperatively these tumoursoften have only a small area of attachment tothe floor of the third ventricle and it is tempt-ing to attempt to remove the tumour com-pletely. In all cases the surgery wasuneventful, yet postoperatively three of thefive cases had significant morbidity, includ-ing memory deficits, diabetes insipidus, andabnormalities of central temperature control.The place of radiotherapy in the treatmentof these tumours is discussed.2 It is con-cluded that attempts at total surgical excisionshould be approached with caution as signif-icant long term morbidity can follow suchsurgery and that perhaps the better alter-native is subtotal removal followed by radio-therapy.

1 Iwasaki K, Kondo A, Takahashi JB, YamanobeK. Intraventricular craniopharyngioma: reportof two cases and review of the literature. SurgNeurol 1992;38:294-301.

2 Fischer EG, Welch K, Shillito J, et al.Craniopharyngiomas in children. Long termeffects of conservative surgical procedurescombined with radiation therapy. J Neurosurg1990;73:534-40.

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BLOOD TRANSFUSION AND SURVIVAL FROMINTRINSIC SUPRATENTORIAL HIGH GRADEGLIOMASJD Palmer, RM Pickering, DW Ellison, DASchilder, F Iannotti. Wessex NeurologicalCentre, Southampton, UK

Objectives-To measure the influence ofblood transfusion on survival in patients withintracranial gliomas.Design-Prospective data were examinedfrom 175 patients who had entered EORTCglioma studies. Transfusion data were col-lected retrospectively from case records.Patients-All patients were over 16 years old,steroids had been stopped by the 10th post-operative day, survival was expected formore than eight weeks, and all had similarcourses of radiotherapy.Outcome measures-Survival was establishedby telephone interview of the next of kin.Results-Hazard ratios of factors affectingsurvival were calculated by a series of Coxregression models. Variables significantlyaffecting survival were age (P = 0-0016),pathology (P = 0 0075), and tumour loca-tion (P = 0 0075). After adjustment,Karnovsky index, tumour resection, oradjunctive treatment had no significanteffect on survival. Blood transfusion was asignificant protective factor for glioblastomawith a hazard ratio of 0-38 (95% confidenceintervals 0-22-0-67), had no effect foranaplastic astrocytoma 1-70 (0-58-4-94),and was a risk factor for other pathologies4-56 (0-97-21-35). The different riskbetween these groups is highly statisticallysignificant (P = 0 0003).Conclusions-In this group of patients trans-fusion improved survival from glioblastoma.Suppression of cell mediated immunity isknown to occur in patients with glioblastomasimilar to that seen in AIDS; further modula-tion by transfusion may play a part inimproving survival.[SBNS]

AN ECONOMIC ARGUMENT IN FAVOUR OFENDOSCOPIC THIRD VENTRICULOSTOMY AS ATREATMENT FOR OBSTRUCTIVEHYDROCEPHALUSHS Ching, P Barlow. Southern GeneralHospital, Glasgow, UK

Objectives-To examine the health resourceimplications of performing endoscopic thirdventriculostomy as an alternative to CSFshunting in appropriate patients.Design-A retrospective study of caserecords and radiographs of patients shuntedde novo at the Institute of NeurologicalSciences, Glasgow for the two year period1990-1.Outcome measures-The number of repeatoperations and extra days in hospitalrequired due to shunt complications com-pared with the predicted extra operationsand days in hospital for patients suitable forendoscopic third ventriculostomy.Results-One hundred and fifty new shuntswere inserted in the two years. Of these, 23patients (15%) were judged suitable forendoscopic third ventriculostomy as an alter-native to CSF shunting. Eight out of 23patients required a total of 29 repeat opera-tions and an extra 230 days in hospital dueto shunt complications. Assuming an 80%success (shunt free) rate for endoscopic thirdventriculostomy, nine operations and 74 beddays per year could be saved by using thistechnique.Conclusions-In units undertaking a largenumber of CSF shunt insertions, investmentin neuroendoscopic equipment has thepotential to release significant resources forother uses.

[SBNS]

VINCRISTINE AND CARBOPLATIN TREATMENTFOR PROGRESSIVE LOW GRADEASTROCYTOMA OF BRAINMS Ashraf, P May, H McDowell. RoyalLiverpool Children's Hospital, Liverpool,UK

Five children (age range 4 months to 8 yearsand 4 months), with progressive low gradeastrocytoma (grades I and II) were treatedwith weekly vincristine (1-5 mg/M2) and car-boplatin (500 mg/M2), every three weeks.Response in terms of either reduction intumour size or progression of disease, wasachieved in all cases. The follow up period is20 to 10 months (mean seven months).Initial response suggests combinationchemotherapy with vincristine and carbo-platin is effective in holding the disease anddelaying the use of radiotherapy particularlyin younger children.[SBNS]

EVALUATION OF MICROENVIRONMENTALHYPOXIA OF BRAIN TUMOURS: MODIFYINGFACTORSGS Cruickshank, R Duckworth. Institute ofNeurological Sciences, Glasgow, UK

Objectives-To assess the impact of PaCO2and blood pressure on oxygenation ofhuman brain tumours at operation measuredby dynamic stepping micropolarography andmicrolaser Doppler.Design-The stratification of patients basedon the oxygen profile of their intracranial



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tumours may allow assessment of the impactof chemical modifiers on radiation sensi-tivity.' Characterisation of factors that influ-ence intracranial tumour hypoxia, is thereforeimportant. The question is whether the effectof altered arterial PaCO2 and blood pressureon cerebral blood flow and brain oxygena-tion devalue the significant level of hypoxiarecorded in these tumours at operation.Subjects-Twelve anaesthetised patientswere ventilated at an FiO2 of 0-25-0-3, andend tidal CO2 and PaO2 and PaCO2 weremonitored. The Eppendorf PO2 histographprobe was used to measure PO2 profiles intumours in these patients. Concurrently,microlaser Doppler flow measurements weremade from brain, brain around tumour andtumour.Outcome measures and results-Alteration inPO2 indices and variations in erythrocyte fluxwere assessed in response to a fixed changein pCO2 or mean arterial blood pressure; forreproducibility and reversibility of effect,altering the PaCO2 from 37 mm Hg to 31mm Hg and back resulted in a reversible fallin median tumour PO2 of 4-2 (0-4) mm Hg,and in PO2 levels of less than 2-5 mm Hgfrom 26-3% to 46-5%. Changes in micro-regional blood flow (erythrocyte flux)recorded in the tumour, peritumoural area,and distant brain illustrate a pronounced im-pairment ofreactivity in the peritumoural area.Conclusions-Altering PaCO2 has a repro-ducible impact on tumour PO2 that may bedue to direct or shunting effects on cerebralcirculation. The tumour ApO2/APaCO2 seenis less than that for normal brain and doesnot overlap with tumour P02 data fromextracranial tumours, suggesting thathypoxia in intracranial tumours may be animportant factor in their resistance to treat-ment. Multichannel microlaser Dopplertechniques used in conjunction with tissueoxygen measurement allows the factors con-trolling tumour hypoxia to be evaluated andpossibly manipulated therapeutically.

1 Rampling R, Cruikshank GS, Lewis AP, et al.Direct measurement of PO2 distribution andbioreductive enzymnes in human malignantbrain tumours. J Radiat Oncol Biol Phys1994;29:427-3 1.

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SAFE NEUROSURGERY? AN AUDIT OFACOUSTIC NEUROMA SURGERY IN A SMALLNEUROSURGICAL UNITPJ Kane, B Gendeh, M Hawthorne, FPNath. Middlesbrough General Hospital andNorth Riding Infirmary, Middlesbrough, UK

Aims-To audit the results of acoustic neu-roma surgery in a neurosurgical unit withsmall catchment population and comparewith published standards of outcome.Design-Retrospective study of 64 patientswith acoustic neuroma operated on by asingle neurosurgeon and neurootologist inthe period 1986-94. All patients were oper-ated on by the suboccipital retromastoidapproach. Peroperative facial nerve monitor-ing became available in 1992. Specific auditpoints included referral pattern; mortality;postoperative CSF leak; postoperative facialnerve function at six months; and hearingpreservation.Results-Sixty nine per cent of patients werereferred by otologists, the remainder fromneurologists, general physicians, or generalpractitioners. Two patients (3%) died aftersurgery. Both had large tumours (>5-5 cm).

Five patients (8%) developed CSF leak thatwas treated operatively in four and by spinaldrainage in one. All patients with smalltumours (<10 mm) had good recovery offacial nerve function (those house grade IM);27% of patients with medium sized tumours(11-25 mm), and 33% of patients with largetumours (>26 mm) had a facial palsy. Tenpatients had some preserved hearing on theaffected side at the time oftheir presentation.Postoperatively four were deaf, three hadretained non-useful hearing, and three hadretained useful hearing.Conclusion-The results compare favourablywith published data. Acoustic neuromasurgery can be performed safely in a smallneurosurgical unit.[SBNS]

COMPLICATIONS OF SKULL BASE GLOMUSSURGERY: 10 YEARS OF OTONEUROSURGICALEXPERIENCEPC Whitfield, DA Moffat, DG Hardy.Addenbrooke's Hospital, Cambridge, UK

Objectives-To review and evaluate the man-agement and outcome in patients under-going surgery for skull base glomus tumours.Design-Retrospective analysis of all patientswith large skull base glomus tumours treatedby combined otoneurosurgery during a 10year period.Subjects-Fifteen patients with glomus jugu-lare tumours, three patients with glomusvagale tumours, and one patient with a glo-mus hypoglossus.Outcome measures-(a) Performance of addi-tional procedures to treat post-operativecomplications. (b) Functional outcome on adisability scale at long term follow up.Results-An infra/transtemporal approach(n = 15) for Fisch grade C and D tumours,or a tympanomastoid approach (n = 4) forFisch grade B tumours was performed. Thefacial nerve was transposed in 12 patients(63%). Cranial nerves were necessarily tran-sected in eight cases (42%); in six of thesepreoperative signs of cranial nerve involve-ment were evident. Ten patients (53%)underwent surgery to treat complications;teflon injection of vocal cord (n = 5), tarsor-rhaphy (n = 3), wound re-exploration (n =3), surgery for facial palsy (n = 2), thyro-plasty (n = 2), ventriculoperitoneal shunt(n = 1), and squint surgery (n = 1). At longterm follow up (mean 3-24 years) 68% ofpatients had an excellent functional outcome,11% a good outcome, and 21% a fair outcome.Conclusions-Most patients with a pre-existing lower cranial nerve palsy adapt tointraoperative cranial nerve transectionextremely well. A multidisciplinary team is re-quired to achieve optimal results in patientswith these complex skull base tumours.[SBNS]

RADIOSURGERY/STEREOTACTICRADIOTHERAPY FOR SOLITARY BRAINMETASTASES: A NON-INVASIVE ALTERNATIVETO SURGERYM Brada, RE Wurm, AP Warrington, RWLaing, S Sardell, F Hines, JD Graham. RoyalMarsden Hospital and Institute of CancerResearch, London, UK

Fifty two patients with 57 solitary brainmetastases (one to two lesions) were treatedwith stereotactic radiotherapy/radiosurgery(SRT) with or without whole brain irradia-

tion (WBRT). Twenty nine patients receivedSRT as primary treatment. Twenty threepatients were treated for recurrence aftersurgery and WBRT and after WBRT alone.The SRT dose was 20 Gy in two fractions inmost patients prescribed to the 90% isodose.The target volume encompassed the enhanc-ing tumour and a 2 mm margin.

Local progression free survival (PFS) was61% at one year (27 months median PFS)and the median survival was seven months.Most patients had a functional improvementas measured by the Barthel index. Neuro-logical deterioration was noted in twopatients within one month of SRT.The results of SRT in patients with soli-

tary brain metastases in terms of local con-trol and survival seem equivalent toneurosurgical excision; SRT is a useful pal-liative treatment-it is well tolerated, non-invasive, and outpatient based and isparticularly of value in patients whose sur-vival is determined by the systemic course ofdisease. The role ofWBRT in combinationwith SRT remains to be defined.[SBNS]

INCIDENCE OF BRAIN METASTASES ANDRELATION TO SYSTEMIC TUMOUR SITESR Grant, D Collie. Westem GeneralHospital, Edinburgh, UK

There are no good epidemiological studies ofbrain metastases in the United Kingdom.The incidence of brain metastases in theLothian region, the frequency of singlemetastasis and metastases of unknownprimary, and the clinical relations withprimary site were examined.

Incident cases of intracranial tumour(1989 + 1990) in the Lothian region wereidentified by examining all CT reports fromthe three scanners that cover SE Scotland,supplemented by multiple cross referencedcancer registration and neurological data-bases. Case records were retrieved andpatient data analysed.One hundred and eighty four people with

brain metastases were identified (incidence:12-6/100 000 pop/year). Brain metastasesaccounted for 46-5% of all intracranialtumours; 37% of brain metastases weresingle; 34% of patients had no history ofsystemic malignancy at neurological presen-tation and no systemic site was ever identi-fied in 15% (27). Lung (104), breast (22),melanoma (14), GI tract (seven) and renal(four) were the most commonly identifiedprimary sites. All patients with brain meta-stases from breast and GI tumours and 13with melanoma, had a history of malignancybefore neurological presentation. This pro-vides useful clinical information for investi-gating patients with brain metastases.[ABN]

EFFECTS OF ARTERIOVENOUSMALFORMATIONS ON CORTICALORGANISATION: IMPLICATIONS FOR SAFETYOF RESECTIONGM McKhann II, JM Eskridge, DLSilbergeld, AB Harris, GA Ojemann, HRWinn. University of Washington, Seattle,USA

Objectives-Preoperative and intraoperativefunctional mapping can be used to identifythe Rolandic and language cortex in patients



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with arteriovenous malformations (AVMs)near essential cortex. Using these techniquesto facilitate safe excision of AVMs, theimpact that these lesions have on corticalorganisation has been studied.Design-Utilising preoperative super-selective amobarbital (WADA) testing andintraoperative stimulation mapping, the

topographical organisation of functional cor-tex in patients with AVMs was investigated.Patients-Thirty eight consecutive patientswith AVMs near essential functional cortexwere evaluated.Results-Individual variability exists in the

topographical location of sensory and motorcortex and of sites essential for languagefunction in patients with AVMs. Most

patients with AVMs reorganise functionalcortex into atypical topographical configura-tions by displacing Rolandic and/or languagecortex. Two patients were found to have

motor cortex located within AVMs.Conclusions-There is variability and plastic-ity ofhuman cortical organisation in patientswith AVMs. Most AVMs displace functionalcortex and can potentially be safely resected.Preoperative superselective WADA testingand/or intraoperative stimulation mappingare recommended for lesions near eloquentcortex to determine those that may be unre-sectable.[SBNS]

"DYNAMICS" OF INTRACRANIAL ANEURYSMS

DEMONSTRATED BY COLOUR TRANSCRANIALDOPPLER ULTRASOUNDJM Wardlaw, J Cannon. University of

Edinburgh, Edinburgh, UK

It is possible to demonstrate intracranialaneurysms and AVMs using directionallysensitive conventional colour transcranialDoppler ultrasound (CDI), which has been

available for several years. A recent develop-ment of colour Doppler technology "colourDoppler energy" (CDE) or "powerDoppler" is considerably more sensitive to

flowing blood. This technique has been

applied to the detection of intracranialaneurysms in patients presenting with sub-arachnoid haemorrhage (SAH).

As many patients as possible presenting to

our regional neurosurgical service withrecent SAH (either CT or LP proved) were

assessed by colour transcranial Doppler,using the temporal bone windows, blind to

the result of angiography although not to the

distribution of blood on CT.Thirty nine patients have been examined

so far (mean age 50), the first nine with CDIand the last 30 patients with CDE.Aneurysms were recognised as an area of

colour where no normal artery was expected,and on CDE by apparently greater "pulsatil-ity" than normal arteries during the cardiaccycle. CDE reduced the proportion of

patients with a poor bone window (10% with

CDE and 33% with CDI). CDE detected 26

of 29 aneurysms demonstrated angiograpil-cally (90%), whereas CDI only detected55%. In five patients who underwenaneurysm coiling it was possible to see the

coil being inserted into the aneurysm and tc

measure the residual neck with CDE. Th(

mean change in aneurysm cross sectionaarea between systole and diastole was 49OX(± 28%) and in the cross sectional area of ar

adjacent normal artery was 18% (± 20%)mean of difference -30-8%, 99% CI -6


to - 2%. CDE is an appreciable improve- ID

ment in colour TCD technology. Further G

evaluation is required to see whether c

aneurysm "pulsatility" could be used as an f

indicator of impending rupture. S

[ABN] C

SERUM LIPOPROTEIN (A) CONCENTRATIONSIN FAMILIAL CEREBROVASCULAR ANEURYSM t

C Bolger, JP Phillips. Beaumont Hospital, t

Dublin, Eire and Walton Hospital,Liverpool, UK

Objective-To investigate serum lipoprotein(a) (Lp(a)) concentrations in patients withfamilial cerebrovascular saccular aneurysms.Design-Case identification from family his-tory of patients admitted with proved sub-

arachnoid haemorrhage secondary to

saccular cerebrovascular aneurysm. Patientswith two or more first degree relatives withproved aneurysmal subarachnoid haemor-rhage were recruited. They were screenedwith cerebral angiography and fasting serumLp(a) was measured.Patients-To date 15 patients (siblings) from

two families have been identified (eightmales and seven females, mean age 37 9

(10-2) years).Outcome measures-Serum Lp(a) concentra-tions in relation to the presence or absence of

saccular aneurysm.Results-Four patients died from subarach-noid haemorrhage before enrolment into the

study; one patient refused investigation. Of

the remaining 10 patients: seven had asymp-tomatic aneurysms identified on cerebralangiogram. Six of these patients had serum

Lp(a) concentrations above normal (> 30

mg/dl; mean (SD) 54-7 (11-8) mg/dl). Two

patients had normal cerebral angiograms andthe Lp(a) in both patients was normal (10and 25 mg/dl). In the remaining patient(with an Lp(a) concentration of 64 mg/dl)angiography was incomplete.Conclusions-There is evidence of an associa-tion between raised serum concentration of

Lp(a) and cerebral aneurysms.[SBNS]

A MAGNETIC RESONANCE SPECTROSCOPICSTUDY OF PARKINSONISM RELATED TO

BOXINGAJ Lees, CA Davie, Z Pirtosek, GJ Barker,DP Kingsley, DA Miller. Institute of

Neurology, London, UK

Proton magnetic resonance spectroscopy,localised to the lentiform nucleus, was car-

ried out in three exprofessional boxers who

developed a parkinsonian syndrome, six

patients with idiopathic Parkinson's disease

f and in six age matched controls. The three

boxers all showed a pronounced reduction in

the absolute concentration ofN-acetyl aspar-tate (NAA), compared with the patients with

i Parkinson's disease and the control group.l~~~~~~~~~~~~~~

t This reduction is likely to reflect neuronale loss occurring in the putamen and globusD pallidus, and supports the hypothesis that

e the extra pyramidal syndrome, which occurs

in exboxers, is distinct from idiopathic10 Parkinson's disease. These findings will be

n related to the existing literature on post trau-

matic Parkinson's syndrome.0 [ABN]

MPLEMENTATION OF HEAD INJURY

3UIDELINES: WHERE ARE WE AFTER TWO

-YCLES OF A REGIONAL AUDIT?D Pickard, S Jackson, J Addison, P

Smielewski, HK Richards. University ofCambridge, Cambridge, UK

Guidelines for the management of headinjuries have been widely adopted but maynot be followed in practice.' A criterionbased audit was conducted in the eight dis-trict general hospitals of East Anglia to assessthe quality of documentation, investigation,and care of patients with head injury.First cycle (1 October 1992-31 March1993)-4789 patients' notes (1458 admit-ted) were extracted and those that did not

meet the guidelines were peer reviewedanonymously in each hospital. Documen-tation was poor (no record ofGCS 52%, BP37%, or skull radiograph 71%). Failure at

peer review for skull radiograph, admissionand/or CT criteria was 12% (range 8-15%between hospitals): under investigation10%, over investigation 2-4%.Second cycle (1 December 1993-28 February1994)-a structured head injury proformawas introduced in October 1993.2 A total of2718 notes (786 admissions) were auditedand peer reviewed. Documentationimproved by 37% to 89% (P < 0-001).Overall failure for skull radiograph, admis-sion, and/or CT criteria was not significantlyreduced (13%; range 10%-19%). Over-investigation was reduced from 2-4% to

11% (P < 0-001) but underinvestigationincreased from 10% to 12% (P < 0-001).Conclusion-Improvement in documentationand provision of a "structured" proformahave not yet improved the implementation of

guidelines agreed by regional consensus.

1 Suggestions from a group of neurosurgeons.Guidelines for initial management after headinjury. BMJ 1984;288:983-5.

2 Wallace SA, Bennett J, Perez-Avila CA, GullanRW, et al. Head injuries in the accident andemergency department: are we usingresources effectively? Journal of Accident andEmergency Medicine 1994;11:25-31.

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ASSESSMENT OF THE GtIASGOW HEAD INJURY

OUTCOME PREDICTION PROGRAM

JJ Nissen, PA Jones, DF Signorini, JDMiller. Westem General Hospital,Edinburgh, UK

Objectives-To compare the predicted v

actual outcome after head injury using the

Glasgow head injury prediction program,'and to determine how often predictions wereavailable.Design-Retrospective data (1989-94) from

325 patients with severe head injury,haematoma requiring evacuation and/orcoma >six hours, with Glasgow outcomescale (GOS) scores at six to 24 months fol-

low up. The program calculates the proba-bilities of death/vegetative state (GOS 1 or

2), severe disability (GOS 3), or moderatedisability/good recovery (GOS 4 or 5) on

admission after severe injury, before evacua-

tion of acute haematoma, or at 24 hours,three days, or seven days for those in coma.Results-Predictions were possible in 77%,96%, 19%, 34%, and 53% of "suitable"patients at admission, operation, 24 hours,three days, and seven days respectively.Paralysis/ventilation meant that predictionswere often unavailable, ranging from 15% of

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hours. Seventy four per cent of predictionswere correct (highest probability), 16%pessimistic, and 10% optimistic. Of thosepatients whose eventual outcome was eitherGOS 4/5 or GOS 1, 84% were correct; forthose GOS 3, only 12% were correctly pre-dicted. Of 64 pessimistic predictions, 48%were predicted to die or be vegetative, butactually achieved a moderate or good outcome.Conclusion-The Glasgow head injury pre-diction program can be a useful adjunct inclinical decision making in some headinjured patients.

1 Murray LS, Teasdale GM, Murray GD, et al.Does prediction of outcome alter patientmanagement? Lancet 1993;341:1487-91.

[SBNS]

OUTCOME IN CHILDREN WITH SEVERE HEADINJURY: IMPORTANCE OF CEREBRALPERFUSION PRESSUREPJ Kane, IR Chambers, A Davidson, LTreadwell, NV Todd, A Jenkins, PJCrawford, RP Sengupta, AD Mendelow.Newcastle General Hospital, Newcastle-upon-Tyne, UK

Objectives-To determine what factors maybe important in predicting outcome aftersevere head injury in children.Design-Retrospective study of 50 children(age < 16) with a severe head injury(Glasgow coma score on admission to theneurosurgical unit < 8) who requiredintracranial pressure (ICP) monitoring as aroutine part of their management. Meanarterial blood pressure, ICP, and cerebralperfusion pressure (CPP) were measuredcontinuously. Outcome was determined atsix months with the Glasgow outcome scale.Independent outcome is defined as thosechildren who made a good recovery or weremoderately disabled.Results-Nine patients died. In the remain-der the outcome scores at six months afterinjury were: seven severely disabled; 12moderately disabled; 22 good recoveries.Glasgow coma score on admission, injuryseverity score, and average maximum ICPwere not reliable predictors of outcome. Aswith a previous study,' higher values ofaverage minimum CPP were associated withbetter outcome: good recovery 58-5 (3 9)mm Hg; moderately disabled 58-4 (2 5) mmHg; severely disabled 44-5 (4-3) mm Hg;dead 45-3 (16-2) mm Hg. Average mini-mum CPP values >50 mm Hg were associ-ated with a significantly greater chance ofindependent outcome (X2 P < 0.05).Conclusion-This study points to the impor-tance of CPP as a determinant of outcomeafter severe head injury in children.

1 Mendelow AD, Allcut DA, Chambers IR, et al.In: Avezaat CJJ, et al, eds. ICV VII. Berlin:Springer Verlag 1993:544-8.

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LABORATORY TESTING OF THREEINTRACRANIAL PRESSUREMICROTRANSDUCERSM Czosnyka, Z Czosnyka, JD Pickard.University of Cambridge, Cambridge, UK

Three comparably priced ICP microtrans-ducers have become available on the UnitedKingdom market, each characterised by theirmanufacturer as having very low zero drift

over long periods, an excellent frequencyresponse, and a low measurement error. Thethree microtransducers, coded A, B, and C,were examined in the pressure flow test rigdesigned for assessment of hydrocephalusshunts. All three microtransducers compliedwith their manufacturer's specifications,giving high quality readings under test condi-tions. Some differences were noted, how-ever, 24 hour zero drifts were lower than 0-8mm Hg and C had the lowest. The tempera-ture drift was very low in B and C but A hada drift of 0-27 mm Hg/°C; A had a staticerror of less than 0-3 mm Hg, B of less than2 mm Hg, and C of less than 8 mm Hg. Thelimits of the frequency response were notdetectable in A and B (band width widerthan 50 Hz), whereas C had a band widthlimited to 18 Hz.Conclusion-Transducer B scored the bestoverall but all three behave satisfactorily onbench testing. The use of each transducer ineveryday clinical practice requires evaluation.[SBNS]

A PROSPECTIVE COMPARISON OF SUBDURALFIBREOPTIC AND FLUID FILLED SINGLELUMEN BOLT PRESSURE TRANSDUCERS INVENTILATED PATIENTSS Bavetta, JC Sutcliffe, P Chumas, OCESparrow, PJ Hamlyn. The Royal LondonHospital and St Bartholomew's Hospital,London, UK

A clinical comparison was made betweensubdural fibreoptic and fluid filled subduralbolt pressure transducers in ventilatedpatients with head injury. Twelve patientshad both devices inserted in neighbouringsites. In two patients there were obvioustechnical problems with the fibreoptic sys-tem and the results were therefore excludedfrom further analysis. In the remaining 10patients 2167 pairs of simultaneous record-ings were made for up to nine days and thedegree of correspondence studied. A quarterof paired readings differed by more than5 mm Hg. Neither device showed a predis-position to reading either higher or lowerthan the other, but in individual patientsthere was often a consistent bias. The pro-portion of closely corresponding readingstended to diminish after four days, and wheneither device registered a very high or lowvalue. Clinically relevant episodes, definedas a reading of over 20 mm Hg by only oneofthe two monitors, occurred 221 times withboth systems equally likely to produce higherreadings. Differences in treatment would belikelier if such values occurred on conse-cutive occasions, which happened, on aver-age, about every 30 hours. The relativeaccuracy of the two devices remains uncer-tain, but there is evidence suggesting thatboth are prone to errors.[SBNS]

EVIDENCE OF SMALL VESSEL CHANGESCAUSING ISCHAEMIA AFTER SUBARACHNOIDHAEMORRHAGEJG Rowe, N Soper, R Ouwerkerk, RSC Kerr,B Rajagopalan. MRC Spectroscopy Unit andthe Radcliffe Infirmary, Oxford, UK

Objective-To follow cerebral blood flow(CBF) with single photon emission com-puted tomography (SPECT), metabolism

with phosphorus magnetic resonance spec-troscopy, and vasospasm with angiographyand transcranial Doppler (TCD), in a 62year old woman with subarachnoid haemor-rhage from a right middle cerebral artery(MCA) aneurysm.Results-On day 3, she was well. Spectro-scopy on the right showed acidosis (pH6-35) suggesting anaerobic metabolism, butwas normal contralaterally (pH 7-01, controlmean 7-02 (SD 0-0 1). On day 6, she becamedrowsy and confused. Spectroscopy showeddecreased high energy phosphates, suggest-ing cell death. SPECT showed pronouncedhypoperfusion of the right frontal lobe (CBFrelative to cerebellum 0-58 (SD 0-03) right,0-87 (SD 0 02) left). There was no TCDevidence of vasospasm (right MCA velocity43 (SD 1) cm/s, left 35 (SD 2, controls 58(SD 2). Angiography (day 7) did not showvasospasm.

Aneurysm clipping was complicated byrebleeding, and postoperatively by vaso-spasm (MCA velocity >120 cm/s). A recov-ery SPECT (day 25) showed hyperaemia(0-98 (SD 0-03) in the right frontal lobe.Conclusions-Angiography and TCD onlyexamine large and medium sized arteries.The SPECT finding of hypoperfusion, laterreversing, without angiographic vasospasm,suggests that there may be changes in smallvessels beyond angiographic resolution. Theexistence of such changes could explaindiscrepancies between ischaemia and angio-graphic vasospasm complicating subarach-noid haemorrhage.[SBNS]

FAMILIAL HEMIPLEGIC MIGRAINE AND THEAGE RELATED SPECTRUM OF CEREBRALAUTOSOMAL DOMINANT ARTERIOPATHYLINKED TO CHROMOSOME 19M Hutchinson, J O'Riordan, M Javed, EQuin, T Willcox, N Parfrey, D Macerlaine,TG Nagy, E Toumier-Lasserve. St Vincent'sHospital, Dublin, Eire, and Inserm U25,Paris, France

Cerebral autosomal dominant arteriopathywith subcortical infarcts and leucoence-phalopathy (CADASIL) is a recentlydescribed familial cerebrovascular disorderwhich has been shown to map to chromo-some 19ql2. Familial hemiplegic migrainehas also been shown to be linked to chromo-some 19 close to the CADASIL locus. TheCADASIL phenotype is defined, as a mini-mum, by brain MRI abnormalities in an atrisk family member. By this criterion 10 of15 fully investigated members of a large Irishfamily had CADASIL. Brain MRI showed aleucoencephalopathy of varying severity.The spectrum of presenting featuresincluded familial hemiplegic migraine, com-mon and classical migraine, transient cere-bral ischaemic attacks, recurrent strokes, andspinal cord infarction. One family memberwas asymptomatic; two members developeda pseudobulbar palsy and subcorticaldementia. In five familial hemiplegicmigraine started in childhood and four ofthese have subsequently developed the fea-tures of CADASIL. Familial hemiplegicmigraine has not been reported in associa-tion with CADASIL. Linkage analysisshowed significant lodscores (3-91 withD19S226) with markers close to the locusfor the CADASIL gene. We postulate thatfamilial hemiplegic migraine is, in this



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family, a manifestation of the CADASILgene inheritance, which may produce vari-able, age related clinical manifestations.[ABN]

WHICH METHOD OF MEASUREMENT SHOULDBE USED TO ASSESS CAROTID ANGIOGRAMS?GR Young, PRD Humphrey, TE Nixon,ETS Smith. Walton Centre for Neurologyand Neurosurgery, Liverpool, UK

In future, non-invasive techniques will obvi-ate the need for digital subtraction angio-graphy (DSA) in the assessment ofextracranial internal carotid artery stenosis.There is currently much debate concerningwhich measuring technique to adopt whenassessing the degree of carotid stenosis fromangiograms. Using both DSAs and magneticresonance angiograms (MRAs), fourdifferent methods-the "ECST", the"NASCET", the "common carotid artery asdenominator" and the visual impression("eyeballing") methods-were assessed andtheir reproducibility determined. One hun-dred and ten DSAs and 74 MRAs wereassessed using all four methods by two inde-pendent observers on two separate occa-sions. Measurements were made usingvernier scale calipers. The mean (SD) of thedifferences were calculated for each method(the smaller the SD the better the agree-ment).

For DSA, the mean (SD) of the differ-ences between observers were: ECST, 6-4(8-9)%, NASCET, 9-6 (21-3)%; commoncarotid, 6-5 (13-8)%, eyeballing, 0-1 (10-7)%respectively. The consistency improved byeach technique as the degree of stenosisincreased. Similar results were obtained forMRA and intraobserver agreement.

Using calipers, the ECST and the com-mon carotid techniques show least variationin reporting. The NASCET technique seemsthe least reproducible. Interestingly, eye-balling is a highly reproducible technique.[ABN]

ACE GENE POLYMORPHISM,CEREBROVASCULAR DISEASE, AND CAROTIDATHEROMAH Markus, J Barley, S Jeffery, R Lunt, JMBland, N Carter, MM Brown. King'sCollege School of Medicine and Dentistryand St George's Hospital Medical School,London, UK

The molecular basis of the genetic predispo-sition to stroke is largely unknown. A dele-tion polymorphism in intron 16 of the ACEgene has been reported as an independentrisk factor for myocardial infarction althoughthe mechanism of this effect remainsunknown.With the polymerase chain reaction the

insertion-deletion polymorphism was deter-mined in 101 white patients (mean age 64 8)and 137 age matched controls (mean age63 9). All patients presented with a carotidterritory acute ischaemic event and carotidduplex was performed to assess degree ofinternal carotid artery stenosis and commoncarotid artery intima-media (IM) thickness.Plasma ACE concentrations were measured.The DD genotype was commoner in

patients with cerebrovascular disease than incontrols (36/101 v 30/137, P = 0 02). D:I

allele frequency was 0 59:0 41 in cases and0-48:0-52 in controls (P = 0-01). The DDgenotype conferred a relative risk of cere-brovascular disease of 2-4 (95% CI1-14-5-05; P = 0 02). The relation was inde-pendent of age, sex, smoking history, dia-betes, or cholesterol but seemed to bemediated at least in part through hyper-tension. Neither the DD genotype nor theD allele was positively associated withcarotid stenosis or intima-media thickness.The DD genotype is a new risk factor for

cerebrovascular disease. It seems to exert itsaction at least partly through hypertension inthis age group, although not via extracraniallarge vessel disease. In view of the lack ofassociation with carotid artery wall thicknessand stenosis it may act via small vessel dis-ease, altered functional vessel responses, or

by predisposing to cardiac disease.[ABN]

CONTINUED ISCHAEMIC DAMAGE AFTERCEREBRAL INFARCTION IN HUMANSDE Saunders, FA Howe, A van denBoogaart, MA McLean, JR Griffiths, MMBrown. St George's Hospital MedicalSchool, London, UK

The aim was to study the ischaemic penum-bra in humans, by measuring metabolicchanges after middle cerebral artery territory(MCA) infarction using proton magneticresonance spectroscopy ('H-MRS; 1-5T sys-tem). Localised IH-MRS was carried outwithin the area of infarction and contralat-eral hemisphere. Peak areas were obtainedby variable projection time domain fittinganalysis. The unsuppressed water signal wasused as internal concentration standard. Tenpatients with acute MCA territory infarctionwere studied within 28 hours of stroke andfor three months. Significant changes wereseen in the initial infarct spectra comparedwith the contralateral spectra (table 1).Lactate, a marker of anaerobic metabolism,was detected within the infarct but not in thecontralateral hemisphere. N-acetyl aspartate(NAA), a neuronal marker, and total crea-tine (Cr/PCr) were significantly reduced.Further reductions in NAA and Cr/PCroccurred within the first week (table 2). A fallin lactate was seen within the infarct coreover the first seven to 10 days.

In conclusion, neuronal loss is detectednon-invasively by 'H-MRS within 28 hoursof stroke onset. The continuing loss of neu-rons may represent continued ischaemicdamage after MCA infarction.[ABN]

STATUS DYSTONICUS ET RIGIDITUS: AREPORT OF 10 CASESH Manji, RS Howard, NP Hirsch, DHMiller, L Carr, NP Quinn, CD Marsden.National Hospital for Neurology andNeurosurgery and Great Ormond StreetHospital for Sick Children, London, UK

Patients with primary and secondary dys-tonic syndromes may develop increasinglyfrequent and severe episodes of generaliseddystonia and rigidity, which may be refrac-tory to conventional drugs and, in severecases, result in bulbar and ventilatory com-

promise.Ten cases (mean age 21-4 (range 6-39)

years) are presented. The diagnoses wereathetoid cerebral palsy (three), post-traumatic dystonia (three), postencephalitichemidystonia (one), relapsing/remittingdystonia-parkinsonism (one), infantile stri-atal necrosis (one), and myoclonic dystonia(one). Possible precipitating factors includedinfection (one) and starting clonazepam(two). Eight patients were ventilated for amean period of 61 (range 7-300) days. Theindications for ventilation were bulbar(four), respiratory (five), exhaustion (eight),and metabolic disturbance as a result ofrhabdomyolysis (one).Drug treatment used included benzhexol,

tetrabenazine, pimozide, baclofen (oral andintrathecal), chlorpromazine, and carba-mazepine. One patient improved with ben-zhexol and another with tetrabenazine. Twopatients underwent thalamotomy-bilateral(no improvement); unilateral (improved).Two patients died, four returned to theirprestatus condition, two improved partially,and two eventually recovered.Thus patients with status dystonicus et

rigiditus should be managed in intensive careunits; standard drug treatment may be bene-ficial; paralysis and ventilation may be ofbenefit and the prognosis is guarded, partlydepending on the underlying condition.[ABN]

ABNORMAL MOTOR UNIT SYNCHRONISATIONBETWEEN ANTAGONIST MUSCLES INDYSTONIASF Farmer, G Sheean, JC Rothwell, CDMarsden. Institute of Neurology, London,UK

Activity in networks of neurons that providecommon presynaptic input to motoneuronsproduces synchronisation of motor unit dis-charges. Cross correlation of EMGs frompairs of synergistic muscles in humans may

Table 1

Initial data (day 1) Contralateral (n=7) Infarct (n=10) P value (t test)NAA (mmol/l) 14-7 (1-6) 88 (4 3) < 0 001Cr/PCr (mmol/l) 11-6(1*0) 9-4(2 9) < 005Cho (mmol/l) 2-1 (0-3) 1-9 (0-5) NSLactate (mmol/1) 0 19-2 (0-5) < 0-001

Table 2

Infarct Value day 1 (n=7) Value 7 days (n=7) P value (t test)NAA (mmol/l) 10-6 (3 4) 6-1 (4-1) < 0-05Cr/PCr (mmol/l) 10-5 (2 6) 4-4 (2-8) < 0 01Cho(mmol/l) 1-8 (0-6) 1-7 (0-7) NSLactate (mmol/l) 16-9 (9 3) 11-4 (7-2) NS

Values for tables 1 and 2 are means (SD).



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show motor unit synchronisation and thuscommon presynaptic input. By contrast,antagonist motoneurons-for example, flexorand extensor carpi radialis (FCR/ECR)-donot produce synchronised discharges even

when a subject deliberately cocontracts themuscles.

Dystonia is characterised by involuntarycocontraction of antagonist muscles. Onehypothesis concerning abnormal muscle co-

contraction in dystonia is that it is producedby abnormal common presynaptic input to

motoneurons of antagonist muscles. Fivepatients with dystonia were studied (twoidiopathic, three symptomatic, four women,ages 17-60). Simultaneous multiunit EMGswere recorded using concentric needle elec-trodes inserted into FCR and ECR, whilepatients cocontracted the muscles. For com-parison, identical recordings were obtainedfrom four healthy subjects (three men, ages

28-33). The times of motor unit occurrence

from FCR and ECR were cross correlated.Cross correlograms of the healthy subjectswere flat. Those of dystonic subjects con-

tained a peak at time zero (median size k =

1-6; median duration = 38 ms), indicatingabnormal motor unit synchronisation.

It is concluded that in dystonia antagonistmotoneurons share abnormal common pre-

synaptic inputs. Activity of these inputs mayproduce abnormal muscle cocontraction.[ABN]

EMG GUIDED TREATMENT OF TASK SPECIFICDYSTONIA WITH BOTULINUM TOXIN A

GL Sheean, NMF Murray, AJ Lees, CDMarsden. National Hospital for Neurologyand Neurosurgery, London, UK

The role ofEMG in selection and placementof injections of botulinum toxin (BTX-A) inthe treatment of limb dystonia is ill defined.EMG guided injections ofDysport) BTX-Awere used to treat 37 patients with task spe-

cific dystonia, 30 with writer's cramp (14simple, 16 dystonic), five with primary writ-ing tremor and two with musician's dystonia.After clinical inspection during the relevanttask, likely active muscles were sampled witha teflon coated, 23G monopolar needlewhich allowed simultaneous EMG recordingand injection. Usually two or three, but up tosix, muscles were treated in each session.Commonly injected muscles included thewrist flexors/extensors, thumb and deep fin-ger flexors, and index finger extensors. Initialdoses ranged from 20-80 MU and follow upwas from 1-60 months. Initial evaluationand treatment generally took 45-60 minutesand up to 30 minutes at follow up.

Subjective improvement occurred in 86% ofpatients (good to excellent in 76%). Fourshowed no benefit. Significant weaknessdeveloped in nine and involved an adjacent,non-injected muscle in one case. EMG isessential for accurate identification of targetmuscles (both before and after BTX-A) andis particularly important for muscle selectionin tremor.[ABN]

PRIMARY WRITING TREMORPG Bain, LJ Findley, TC Britton, MAGresty, JC Rothwell, PD Thompson, CDMarsden. MRC Human Movement andBalance Unit, London, UK

The clinical and neurophysiological features

of 21 patients with primary writing tremor(PWT) are described. This was first des-cribed by Rothwell, et al (1979). Subse-quently there has been controversy as towhether PWT is a forme fruste of essentialtremor or a variant of writer's cramp.Twenty one patients with PWT were

studied using surface polymyography andaccelerometry. Seven had a family history ofPWT. In 13 patients the forearm reciprocalinhibition curve of the median nerve H reflexwas also examined. The results of reciprocalinhibition were compared with those of 10normal controls and to those obtained frompatients with writer's cramp.The median frequency of PWT was 5-5

Hz (range 4-1-7-3 Hz). Segregated EMGactivity was typically alternating in forearmflexor/extensor pairs but co-contraction,extensor activation alone and varying EMGpatterns were also recorded. Forearm recip-rocal inhibition was normal in PWT. Thepatients were subclassified on clinicalgrounds into type A (task induced) and typeB (positionally sensitive) PWT. The only dif-ferences between these two groups were thatcocontracting tremor and tremor induced bytendon taps to the volar aspect of the wristoccurred in type B but not type A cases.Dominant hand writing speed was signifi-cantly reduced in PWT compared with agematched controls (PWT 73-1 letters/min;controls 138-8 letters/nin (P < 0 0001).

It is concluded that patients with PWTcan be distinguished from writer's cramp asboth the disynaptic and presynaptic phasesof reciprocal inhibition are normal in PWTwhereas the amount of presynaptic inhibi-tion is decreased in writer's cramp; PWT canalso be differentiated from hereditary essen-tial tremor on clinical criteria.[ABN]

ADHESION MOLECULES IN AXO-GLIALINTERACTIONSCE Shaw, R Milner, C ffrench-Constant,DAS Compston. University of Cambridge,Cambridge, UK

With recent advances in immunotherapy formultiple sclerosis (MS) the possibility existsfor promoting remyelination by endogenousor transplanted oligodendrocytes. Themolecular mechanisms regulating axo-glialinteraction in developmental myelinationand repair are largely unknown. To study therole of the adhesion molecules, myelin asso-ciated glycoprotein (MAG), and integrins inmediating this interaction an in vitro modelof myelination co-culturing rodent sensoryneurons and glia has been used. The regionalexpression of MAG by oligodendrocytes isunder axonal influence and MAG proteinbinds to axons confirming a specific ligandinteraction. MAG expression occurs afterthe initial recognition and ensheathmenttakes place and blocking antibodies do notinhibit myelin formation, suggesting thatMAG plays only a minor part in myelination.Integrins are heterodimeric adhesionmolecules, composed of a and ,B subunitsthat recognise specific molecules in theextracellular matrix. With immunoprecipita-tion of cross species co-cultures a6Af61 andavfl5 were greatly upregulated during myeli-nation, suggesting that they may have a rolein mediating this event. With a better under-standing of the molecular mechanisms thatcontrol myelination it is hoped that treat-

ments can be developed which enhanceendogenous or transplanted oligodendrocyterepair.[ABN]

IS THE VARIATION IN PREVALENCE OF

MULTIPLE SCLEROSIS WITHIN THE UNITEDKINGDOM DUE TO MIGRATION, LATITUDE,OR GENES?PM Rothwell. Western General Hospital,Edinburgh, UK

There is debate about the extent to which theworldwide latitudinal gradient in the preva-lence of multiple sclerosis is due to genetic orenvironmental factors. Northern Scotlandhas the highest prevalence in the world.Recent findings of high rates in southernEngland, however, have cast doubt on theassumption of a latitudinal gradient withinthe United Kingdom. To assess this a preva-lence study was performed in south eastScotland. In addition, as susceptibility tomultiple sclerosis seems to be either inher-ited or determined in childhood, mortalityfrom multiple sclerosis within England andWales was analysed by place of birth as wellas place of death to detect any confoundingdue to migration.

Between 1969 and 1974 all deaths regis-tered in England and Wales were linked withbirth records. Place of birth and normal resi-dence before death were obtained for the3151 deaths in which multiple sclerosis was acause and analysed within the nine standardregions. There was no latitudinal gradient byplace of death, but the proportion of patientsborn outside the region in which they diedwas higher in southern regions (41%-63%)than northern regions (8%-18%). Re-analysis by place of birth, however, still failedto show a latitudinal gradient withinEngland. There were fewer than expecteddeaths of people born in Wales (observed/expected = 0-64, 95% CI = 0 54-0-75). Thepreliminary prevalence rate of multiple scle-rosis in the Lothian and Border regions ofScotland (population 850 000) was in excessof 200/100 000. This is similar to rates innorthern Scotland and significantly higherthan the rates in southern England. Thesedata suggest that there is no latitudinal gradi-ent within either England or Scotland, butthat there are differences in the prevalence ofmultiple sclerosis between England,Scotland, and Wales. This most probablyreflects the genetic susceptibility of therespective populations.[ABN]

SERIAL BRAIN AND SPINAL CORD MRI INMULTIPLE SCLEROSISJW Thorpe, D Kidd, IF Moseley, BEKendall, WI McDonald, DH Miller.Institute ofNeurology, London, UK

The dynamics of multiple sclerosis havebeen extensively studied by serial MRI of thebrain; less is known about the spinal cord.Monthly gadolinium-enhanced brain andspinal cord MRI over a period of one yearwere carried out in 10 patients with relaps-ing-remitting multiple sclerosis. Six patientshad a total of 11 clinical relapses of whicheight implicated the spinal cord. One hun-dred and sixty seven active (enhancing ornew non-enhancing) lesions were detected in



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the brain, of which only one was symp-tomatic; conversely six of the 19 active spinalcord lesions found were symptomatic. Allbut seven of the active lesions were in the sixpatients who relapsed. Both cord and brainactivity were more common around the timeof relapse. There was a strong associationbetween the cord and brain MRI activity.No progressive cord atrophy was detectedfrom measurements of cord cross sectionalarea.

In relapsing-remitting multiple sclerosis,therefore, imaging the brain alone will detect90% of active lesions. The lack ofprogressivecord atrophy, suggesting that significantaxonal loss has not occurred, is in keepingwith their good recovery after relapse: thiscontrasts with our previous findings in pro-gressive multiple sclerosis in which increas-ing cord atrophy developed whereas activelesions were rare in the absence of superim-posed relapses. The concurrence of brainand spinal cord lesions implies a systemictrigger for disease activity.[ABN]

DAILY URINARY NEOPTERIN EXCRETION ASAN IMMUNOLOGICAL MARKER OF DISEASEACTIVITY IN MULTIPLE SCLEROSISG Giovannoni, M Lai, DH Miller, JWThorpe, D Kidd, AJ Thompson, G Keir,EJ Thompson. Institute of Neurology,London, UK

The excretion of neopterin, a marker ofmacrophage activity in patients with multiplesclerosis was investigated. Daily urinaryneopterin:creatinine ratios (UNCR) weremeasured by high performance liquid chro-matography for a 12 week period in eightcontrols, eight patients with non-relapsingprogressive, and nine patients with relapsingmultiple sclerosis. Infection, relapses, day today fluctuations in neurological function andGd enhanced MRI were monitored.The mean UNCR was significantly higher

in patients with non-relapsing progressive(191 (86) umol/mol) and relapsing disease(558 (652) ,umol/mol) compared with con-trols (119 (28) ,umol/mol, P = 0-001). Oneasymptomatic peak in the serial UNCRs wasfound in one control subject compared withan average 2-2 (2 3) peaks/patient/month innon-relapsing progressive and 3-2 (1-3)peaks/patient/month in patients with relaps-ing multiple sclerosis (P = 0 002); two peakswere temporally associated with a clinicalrelapse and clearly defined peaks and eleva-tions in the baseline were noted around 21days after an upper respiratory tract infectionin three patients. Patients with day to dayfluctuations in neurological function hadhigher mean UNCR than patients without(526 (624) v 182 (69) pmol/mol, P = 0 05).Mean UNCRs tended to be higher inpatients with MRI activity than patientswithout activity (512 (631) v 203 (84) ,umol/mol, NS).

Neopterin seems to be a promisingimmunological marker that can detect sub-clinical inflammation in patients with mul-tiple sclerosis.[ABN]

MULTIPLE SCLEROSIS: RECURRENCE RISKSFOR RELATIVESN Robertson, J Deans, M Fraser, DAS

Compston. University of Cambridge,Cambridge, UK

Familial recurrence risks in multiple sclerosisare becoming of increasing interest to geneti-cists in search of disease susceptibility genesand to clinicians who counsel patients. Littlecontemporary information is available forthe United Kingdom, the population ofwhich continues to exhibit an overall trend ofincreasing prevalence and incidence; ratesderived from genetically and geographicallydistant populations may not be relevant. Inaddition crude risks, which have previouslybeen used, may be misleading in youngerpopulations because of the widely dis-tributed age at onset profile of multiple scle-rosis. The personal details and disease statushave been systematically recorded for morethan 11 000 relatives of 650 probands withprobable or definite disease listed on theCambridgeshire Multiple Sclerosis Registerand prevalent on 1 July 1993 to providecrude and age adjusted recurrence risks forfirst, second, and third degree relatives.Maximal crude risks were found for sisters offemale probands (5%) and age adjusted risksfor female offspring of male probands (7%).Risk was found to fall proportionately withgenetic distance from the proband but wasstill significantly raised in first cousins(1-0%) compared with background preva-lence (132 x 105) suggesting that the pheno-menon of familial aggregation in multiplesclerosis is likely to depend on geneticfactors.[ABN]

DO CELLS FROM THE ADULT HUMAN BRAINHAVE REMYELINATING POTENTIAL?DAS Compston, NJ Scolding, J Sussman,CE Shaw. University of Cambridge,Cambridge, UK

Limited remyelination ofsome multiple scle-rosis lesions is known to occur but recurrentlesions lead to permanent demyelination anddisability. Advances in immunotherapy mayarrest the inflammatory process and the realpossibility exists for repairing areas ofdemyelination in an immunologically stableenvironment. Possible strategies for repairinclude promoting endogenous remyelina-tion or transplantation of cells with myelinat-ing potential. To determine whicholigodendrocytes derived from adult humanbrain have remyelinating potential, theirinteraction with neuronal cultures was stud-ied in vitro. Embryonic rat dorsal root gan-glion neurons were purified in culture andcells dissociated from human temporal lobewhite matter were added. Co-culture withaxons improved oligodendrocyte survival butdid not lead to proliferation. Within oneweek oligodendrocyte precursors wereextending processes along axonal bundlesand by four weeks there was extensiveensheathment of axons by membranes posi-tive for myelin basic protein. After six weeks,there were loose wraps of myelin aroundaxons by transmission electron microscopybut compact myelin was not detected. Thisconfirms that isolated adult human oligo-dendrocytes can correctly identify exposedaxons as the target for ensheathment butspecies barriers may exist in this model thatprevent compact myelin formation. Thisattempt at remyelination by human oligo-

dendrocytes in vitro is encouraging for theprospects of repair in multiple sclerosis.[ABN]

CREATION OF COMPOSITE DATA SETS FORUSE WITH THE ISG IMAGE DIRECTEDSURGICAL SYSTEMPR Eldridge, JN Roberts, RHT Edwards.Walton Centre for Neurology and Neuro-surgery and the University of Liverpool,Liverpool, UK

Computed tomography and MRI provideinformation conceming anatomy, pathology,and function of the brain. Many data sets arecreated for a single patient. Image directedsystems enable accurate surgical localisationfrom these data sets. Unfortunately the ISGimage directed system only utilises a singledata set at a time. It would be desirable tomake use of information from more than onedata set in surgery; such as different MRsequences, or combinations ofMRI and CT.A method combining data sets for the ISG

system is illustrated, using the image analysisprogram "analyze". Raw CT and MRI dataare transferred to "analyze" using XDimage.Features of importance are segmented ineach data set. The data sets are combinedand retumed to the ISG system for surgery.This process requires close involvement ofthe operating surgeon.The technique has wide application.

Examples shown include intrinsic tumours,acoustic neuroma; pituitary tumours,caroticocavemous fistulas (utilising MRangiography). Bony detail, best obtainedfrom CT, can be combined with data con-ceming tumours best seen on MRI (acoustic,pituitary, cavemoma, base of skull tumours).The method compensates for the geometricdistortion ofMRI data sets, which can resultin inaccuracy twice as great as when usingCT.[SBNS]

EMBOLISATION OF INOPERABLEINTRACRANIAL ANEURYSMS BYENDOSACCULAR PACKING WITH PLATINUMCOILSJ Byme, A Molyneux, R Kerr, P Brennan, AHope, S Renowden. Radcliffe Infirmary,Oxford, UK

Over a 30 month period 154 patients werereferred for endovascular treatment of inop-erable intracranial aneurysms. Patients werejudged unsuitable for surgical clipping, beingunfit for craniotomy because of theaneurysm's size, location, and wall thickness.Six patients were not treated by the endovas-cular route because of unsuitable anatomyand 18 patients were treated for additionalcoincidental aneurysms. A total of 164aneurysms were therefore treated in 173 pro-cedures using Guglielmi detachable coils(Target Therapeutics). Most patients pre-sented with subarachnoid haemorrhage(74%) and over 70% were treated withinthree weeks of the ictus. Forty five per cent ofaneurysms were on vertebrobasilar arteries.The aneurysms were packed with multiple

electrolytically detached coils to form stableintraluminal thrombus and all patients havebeen offered follow up angiography at sixmonths. Procedural complications haveincluded aneurysm rupture and coil fractures



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but the most common adverse event hasbeen the formation of thromboemboli. As aresult anticoagulants are now given duringand for up to 48 hours after treatment.The overall morbidity (permanent deficit)

was 5% and mortality 4%. Complicationsoccurred with similar frequency in the ante-rior and posterior circulations.[SBNS]

POSTERIOR CIRCULATION ANEURYSMS: TOCOIL OR CLIP?KW Lindsay, E Teasdale, P Mathew.Institute of Neurological Sciences, Glasgow,UK

Objective-To determine the optimal man-agement of patients with posterior circula-tion aneurysms.Design-All patients with posterior circula-tion aneurysms undergoing treatment byeither direct clipping or by coil embolisationwere reviewed over a five year period from1989 to 1994.Patients-Of 76 patients included in thestudy, 66 underwent direct clipping and 10underwent coil embolisation.Outcome measures-Aneurysm features (size,direction of fundus, neck height, and width).Outcome as assessed by the Glasgow out-come scale. Complications during coilingand occlusion rate.Results-Of 66 patients undergoing operativeclipping, 60 (90%) made a favourable out-come; three patients died. In five of the sixpatients making an unfavourable outcome(severe disability or death), the aneurysm layat the basilar bifurcation. Aneurysm featuresdid not seem to relate to outcome.

In 10 patients, 32 coil embolisation proce-dures were performed. Two patients re-bledduring coiling, of whom one died. In-advertent placement occurred in fourpatients, but only one developed a neurolog-ical deficit. Complete occlusion wasachieved in two patients and > 90% occlu-sion in four. Revascularisation occurred onthree occasions.Conclusions-Coil embolisation is not per-formed without some risk. The long termeffects of incomplete occlusion remainunknown. Use should be restricted to techni-cally difficult aneurysms, particularly thoseat the basilar bifurcation.[SBNS]

NEUROPEPTIDE CONCENTRATIONS IN HUMANCEREBRAL ARTERIESL Chadwick, P Milner, G Burnstock, PJHamlyn. St Bartholomew's Hospital, andUniversity College, London, UK

Objectives-To document the concentrationsofneuropeptide Y (NPY), vasoactive intesti-nal polypeptide (VIP), substance P (SP), andcalcitonin gene related peptide (CGRP) inseveral arteries of the Circle of Willis inhumans and to determine whether there isany change in the concentrations with post-mortem delay.Design-Documentation and comparisonbetween neuropeptide concentrations in themiddle and posterior cerebral arteries andthe middle cerebral trifurcation.Subjects-Twenty nine male and 18 femalecontrol cadavers (mean age 73-8 (1-71) yearsand mean postmortem delay 27-91 (1-41))hours.

Outcome measures-Concentrations of NPY,VIP, SP, and CGRP measured by enzymelinked immunosorbent assay and expressedas pmol/g tissue.Results-Neuropeptide concentrations weresignificantly higher in the middle cerebralartery compared with the posterior cerebralartery (NPY 13-53 (2-85) v 7-18 (1-39),P < 0 05; VIP 13-72 (2-93) v 3-34 (0 77),P <0-01; SP 8-83 (1-43) v 1-23 (0-46),P < 0-001; CGRP 26-93 (3 02) v 2-12(0 32), P < 0-001). There was no differencebetween the concentrations in the middlecerebral artery compared with its trifurca-tion. There was no correlation betweenneuropeptide concentrations and either ageor postmortem delay.Conclusion-Neuropeptide concentrationsare consistently higher in the middle cerebralartery than in the posterior cerebral artery inhumans. The concentrations are stable andindependent of postmortem delay. Post-mortem cerebral vessels are thus suitable forcomparative studies ofneuropeptide concen-trations in disease.[SBNS]

PEROPERATIVE MEASUREMENT OF JUGULARVENOUS OXIMETRY DURING CAROTIDENDARTERECTOMYAJ Davidson, AD Mendelow, IR Chambers.Newcastle General Hospital, Newcastle-upon-Tyne, UK

Objective-To measure jugular bulb oxygensaturation during carotid endarterectomy.Design-Prospective observational study.This was performed by means of a 40 cm 4FG Opticath fibreoptic catheter connectedto an Oximetric 3 computer and recordedcontinuously on a paper hard copy and aPsion computer. The fibreoptic catheter waspositioned by the surgeon under direct visionvia a facial vein tributary into the jugularbulb on the operative side and removed atthe end of the procedure. Other monitoringincluded ECG, invasive blood pressure, cen-tral venous pressure, pulse oximetry, andend tidal capnography.Patients-Twenty patients undergoingcarotid endarterectomy under either local orgeneral anaesthesia depending on the prefer-ence of the patient and surgeon.Outcome-Assessed correlation of peropera-tive monitoring with neurological outcome.Results-The system provided a reliable,continuous, real time monitor of jugularbulb saturation during surgery. Observationof changes in jugular bulb saturation andcorrelation with neurological deficits in thosepatients under local anaesthesia alone greatlyaided in blood pressure manipulation and inthe decision as to the need to insert tempo-rary bypass shunts. Typical patterns seenincluded falls in jugular saturation associatedwith hypotension and hence inadequatecerebral perfusion and profound falls in satu-ration on carotid cross clamping relieved byinsertion oftemporary shunts. Equally usefulwere stable readings obtained during generalanaesthesia or cross clamping, thus avoidingthe potentially deleterious side effects thatmight occur with shunt insertion, fluid load-ing, or inotropic drug treatment given in anattempt to raise blood pressure.Conclusions-Jugular venous oximetry is avaluable monitor to aid in the peroperativecare of patients undergoing carotid endarter-ectomy.[SBNS]

EFFECT OF ANGIOGRAPHY, ANAESTHESIA,AND OPERATION ON INTRACRANIAL PRESSUREIN PATIENTS WITH ANEURYSMALSUBARACHNOID HAEMORRHAGESS Praharaj, IR Chambers, A Clark, PJCrawford, A Jenkins, NV Todd, RPSengupta, A Gholkar, AD Mendelow.University of Newcastle-upon-Tyne andRegional Medical Physics Department,Newcastle-upon-Tyne, UK

Design-A total of 18 patients with suspectedaneurysmal subarachnoid haemorrhage(SAH) had intracranial pressure (ICP) mon-itoring measured during transfemoral digitalsubtraction angiography under local anaes-thesia. Five ofthese also had their ICP moni-tored during anaesthesia and operation. ICPwas measured with a Camino pressure trans-ducer. Simultaneously, blood pressure wasmeasured either with a cuff or arterial line.Results-Angiography raised the ICP in allpatients. Preangiographic ICP was 11-59(1-54) mm Hg, whereas perangiographicpeak ICP was 23-61 (4 68) mm Hg.Intubation during anaesthesia was the singlemost important variable affecting ICP,which rose from between 3 and 87 mm Hg(average rise 24-5 (15-9) mm Hg). A corres-ponding rise in MAP, however, ensured arelatively stable CPP.Conclusion-These findings suggest that thegreatest haemodynamic disturbance topatients with subarachnoid haemorrhageoccurs during intubation. This strengthensthe case for performing angiography underlocal anaesthesia.[SBNS]

EXPERIENCES WITH AN ARTIFICIAL CERVICALJOINTBH Cummins. Frenchay Hospital, Bristol, UK

Most surgical procedures on the cervicalspine include fusion by bone, metal, or both.Over the years such fusion at one or morespaces may be associated with osteophyticdegeneration at the adjacent levels, causingradiculopathy or myelopathy.

In 10 "last ditch" spaces of such disorderaccompanying multiple levels of congenitalor surgical fusion, an artificial joint designedby the author and made by the engineeringdepartment ofthe hospital has been inserted toprovide some degree offlexibility at that level.The joint is of stainless steel in two pieces,

the upper with a downward facing domeslightly smaller in radius than the shallowersaucer of the upper face of the lower piece.This allows rotation, limited flexion, andextension and glide. Locking screws main-tain the joint in position.

Technically this has proved satisfactoryand nine of the 10 joints have provided reso-lution ofsymptoms in these difficult cases.[SBNS]

BIOCOMPATIBLE OSTEOCONDUCTIVEPOLYMER V ILIAC GRAFT IN ANTERIORCERVICAL DISCECTOMY: A PROSPECTIVECOMPARATIVE STUDY ON 110 PATIENTSOPERATED ON FOR CERVICAL DISC DISEASEAA Madawi, M Powell, HA Crockard. TheNational Hospital for Neurology andNeurosurgery, London, UK

Donor site morbidity is a significant problemin anterior cervical fusion. A biocompatible



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osteoconductive polymer (BOP) has beenused in many centres to circumvent theseproblems. A prospective study was per-formed to evaluate its use and long termeffectiveness.Materials and methods-One hundred andten patients, 50 for iliac graft (IG) and 60 forBOP were recruited into this study. 72patients were radiculopathic, 20 myelo-pathic, and 18 were radiculomyelopathic.The mean duration of symptoms was 20-3months. The Smith Robinson technique wasused in 70, and the Cloward technique in 40patients; 80 patients had one level, 30 hadtwo level surgery. Patients were followed uproutinely at three to six month intervals(range 3-24, mean 11 months) at which timeclinical and radiological assessment weremade, including CT and MRI.Results-The mean hospital stay was 5 9days for those with IG, and 5-1 days forBOP. Complications were recorded as twoneck haematomas required evacuation, oneiliac wound haematoma was managed con-servatively, two graft non-unions withkyphus (IG) required revision and plating,two complete graft extrusions (one IG, oneBOP) required revision. Partial graft extru-sion occurred in 18 IG (2-5 mm, mean 3mm) and seven BOP (2-4 mm, mean 2-6mm). Kyphus was found in 13 IG (3-30,mean 12-3 degrees) and in four BOP (mean3-15, 10-3 degrees). The BOP sank into theadjacent endplates and the space collapsedafter Smith Robinson procedures. Also scle-rosis was found around BOP within one totwo months, but even by 18 months therewas no incorporation or degradation.Osseous union was seen to begin within oneto two months and had finished by one yearin 48 cases after IG.Conclusion-BOP can act as a good spacerafter anterior cervical fusion, with evidenceof incorporation or degradation through theperiod of follow up.[SBNS]

EXTENDED ANTERIOR CERVICAL DISCECTOMYWITHOUT FUSION: A SIMPLE AND SUFFICIENTOPERATION FOR MOST CASES OF CERVICALDEGENERATIVE DISEASERS Maurice-Williams, N Dorward. RoyalFree Hospital and School of Medicine,London, UK

Of 291 operations performed for cervicaldegenerative disease causing cord or rootinvolvement over a 12 year period 187 havebeen treated by extended anterior discec-tomy without fusion, removing bone oneither side of the posterior disc space so as togive a wide exposure of the anterior spinaland root dura. This technique has been usedfor 73% of the cases operated on in the pastfour years. Nine patients (4-8%) requiredadditional posterior decompression for co-existing spinal or root canal stenosis. By thefirst postoperative follow up at two to fourmonths 94 5% of patients showed clear neu-rological or functional improvement, 3%were unchanged, 1% were worse, and 1* 5%had died (the deaths were in elderly patientswith severe myelopathy and intercurrentdisease). Minor treatable complicationsoccurred in 3-2%. Only two patients (1%)complained of postoperative neck pain.Patients were mobilised immediately aftersurgery without a collar and most left hospi-tal within one to four days. A single level

decompression was sufficient in 92% ofpatients and only one patient required morethan two levels to be decompressed. In 79%of cases soft disc protrusions contributed tothe compression whereas in 21 % osteo-phytes alone caused the compression. Webelieve that this simple technique is a suffi-cient surgical treatment for most cases of cer-vical degenerative disease. It does not requirea fusion and avoids the specific problems andcomplications associated with Cloward typeoperations. A long term follow up study ofthese patients is presently being carried outand to date no late problems have becomeapparent.[SBNS]

A RANDOMISED CONTROLLED TRIAL

COMPARING AUTOMATED PERCUTANEOUSLUMBAR DISCECTOMY AND LUMBAR

MICRODISCECTOMYG Findlay, S Chatterjee, P Foy. WaltonCentre for Neurology and Neurosurgery,Liverpool, UK

Much interest has been created by theadvent of automated percutaneous lumbardiscectomy (APLD) in the management ofcontained lumbar disc hemiation. Severalpapers have reported encouraging results butto date attempts to perform controlledstudies of APLD have been hampered bynumbers too small to be statistically signifi-cant. We designed a randomised study with aseparate blind assessor to compare theresults of APLD with conventional lumbarmicrodiscectomy. It was planned to recruit180 patients to the study and to complete atwo year follow up in all cases. During thestudy it became apparent to the non-blindparticipants that the results of APLD weremuch worse than surgery and it was decidedto analyse the results at that stage.

This produced 70 patients who had smallcontained disc hemiation proved by MRI (allother types were excluded from the study).In the APLD group only eight of 30 (26 7%)achieved excellent or good results, whereasthe corresponding figure for the micro-discectomy group was 32 of 40 (80%).Moreover for patients in the APLD groupwho had unsatisfactory results and opted forsubsequent microdiscectomy (20 of 22patients), the success rate was only 13 of 20(65%). Thus the eventual success rate ofthose treated by APLD alone or APLD andmicrodiscectomy was 21 of 30 (70%).A recent controlled study comparing

APLD to chemonucleolysis also showedpoor results for APLD. Our study shows thatin the chosen group of patients APLD is in-effective. Our success rate for microsurgeryfor all types of disc hemiations was found tobe 91% and better than the 80% we foundfor the selected group in our study.

It is considered that a trial is needed tocompare the results ofAPLD with conservativetreatment and that a third control group ofpatients who had neither surgery nor APLDwould have been valuable in this study.[SBNS]

SPINAL MENINGIOMAS: A 20 YEAR REVIEW

AT King, B Doshi, RW Gullan, MM Sharr,JR Bartlett. Brook General Hospital,London, UK

This is a retrospective study of 78 patients

with spinal meningiomas operated on over20 years at the Brook Neurosurgical Unit.The age range was 22-91 (mean 61-5) years,with 12 men (15%) and 66 women (85%),followed up for a mean of 10-5 years. Theclinical history was of less than one year in55 patients and less than six months in 26patients. There was poor correlationbetween presenting complaint and relationto the spinal cord: 15 lay anteriorly, 15anterolaterally, 23 laterally, 17 posterolater-ally, and eight posteriorly. Plain radiologywas normal in the entire intradural group (74patients) with only one example of tumourcalcification. Three out of the four extra-dural group showed abnormalities on plainradiology. Two tumours were purely extra-dural and a further two both intradural andextradural. The dural attachment was ex-cised in 20 cases. Full recovery offunction wasachieved in 57 patients (73%), and a further17 (22%) were improved to independentmobility. There was one recurrence, at 14 yearsafter operation. By contrast with previousseries, histology showed no striking predomi-nance ofpsammomatous meningiomas.[SBNS]

TRANSDURAL MICRONEUROSURGICALEXCISION OF PROLAPSED DORSAL DISCSJM Rice Edwards, D Peterson. CharingCross Hospital, London, UK

A tenet of neurosurgical practice is that alaminectomy should not be done for theremoval of dorsal discs. This belief is basedon historical evidence of operations per-formed with rongeurs and of impossibleattempts to remove anterior lesions extra-durally.An operation is described which involves

careful removal of laminae with a drill and atransdural microapproach to the dorsal disc.

After removing the lamina the spinal cordis not usually displaced against the dorsaldura and a very adequate view of the anteriordisc can be obtained laterally without signifi-cant retraction of the spinal cord. It has beenpossible to completely remove the hard discprolapse in the 10 cases where this approachhas been used.The technique has the following advan-

tages: the approach is familiar to neuro-surgeons whereas the anterolateral andtransthoracic approaches can be difficultunless practised often, the spinal cord can beinspected for adhesions to the dura (whichoccurred in two cases with associated arach-noiditis), it is safe providing that the discprotwsion is not of an extreme size. This canbe judged from MRI.[SBNS]

Poster Presentations

ASYMPTOMATIC CNS ABNORMALITIES IN

BEHCET'S DISEASE: A MAGNETIC RESONANCESTUDYF Ahmed, JM Bamford, KS Blanshard, BANoble. St James's University Hospital, andthe General Infirmary, Leeds, UK

Involvement of the CNS occurs in up to onethird of patients with Behcet's disease, desig-nated as neuro-Behcet's disease. Clinicalfeatures are either due to cerebral venousthrombosis or parenchymal pathology.



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Prognosis of neuro-Behcet's disease ispoor and early intervention may modify thecourse of the disease. Early diagnosis is,therefore, desired before the development ofdisabling neurological sequelae. Asympto-matic abnormalities on MRI have beenreported but their prevalence is unknown.The results of the initial assessment of acohort of patients with MRI and angiogramwho will be followed prospectively arereported.

Eighteen patients fulfilling the Inter-national Study Group (ISG) criteria for thediagnosis were assessed. None had symp-toms and signs on routine examination tosuggest current or previous involvement ofthe nervous system. Their disease activitywas assessed according to ISG recommenda-tions.

Magnetic resonance angiograms werenormal in all cases. Thirteen patients hadnormal MRI. Other scans showed high sig-nal intensity in T2 weighted images, either assingle lesions >10 mm or multiple lesions(5-10 mm) in the cortex and periventricularregion.

It is concluded that asymptomatic MRabnormalities in patients with Behqet's dis-ease are uncommon and are not related toduration of the disease or its activity inother systems. Whether these abnormalitiespredict future neuro-Beh9et's disease isuncertain.[ABN]

A SURVEY OF THE USE OF LAMOTRIGINE ANDVIGABATRIN IN CLINICAL PRACTICED Chadwick, G Schapel. University ofLiverpool, Liverpool, UK

A survey was undertaken betweenSeptember 1993 and February 1994 thatidentified 356 patients who had been pre-scribed either vigabatrin or lamotrigine sincethe licensing of these compounds from acomprehensive database of just over 2200patients with epilepsy attending the MerseyRegional Epilepsy Clinic since 1989. Thepopulation had a chronic refractory epilepsy;90% were having more than one seizure perweek and two thirds were receiving one drug.Two hundred and twenty three patients

were exposed to lamotrigine for up to 56months and 217 patients to vigabatrin for upto 72 months. The median maximum doseof lamotrigine achieved was 400 mg per dayand that for vigabatrin was 2 g per day.Kaplan-Meier survival curves were plottedfor time to withdrawal of the new drug. Theprobability of continuing on lamotrigine at40 months was 57% compared with 43% forvigabatrin. The commonest adverse eventcausing withdrawal of lamotrigine was skinrash (5% of patients exposed), whereasadverse psychiatric consequences were thecommonest reason for withdrawal of vigaba-trin (8% of cases). Ten per cent of patientsexposed to lamotrigine became seizure freecompared with 6% exposed to vigabatrin.Most of the patients becoming seizure freeon lamotrigine were those with generalisedepilepsies and there was little differencebetween the outcomes for the new drugs inpartial epilepsies.

This survey indicates that there is little dif-ference in the efficacy of these two noveldrugs in the partial epilepsies. The side effectprofile of the drugs seems radically differentas does the range of activity, lamotrigine

exhibiting a broad range of activity; and par-ticular effectiveness in more resistant formsof idiopathic generalised epilepsy.[ABN]

MAGNETIC RESONANCE SPECTROSCOPY INIDIOPATHIC PARKINSON'S DISEASE WITHMOTOR OSCILLATIONSK Ray-Chaudhuri, G Lemmens, C Ellis,J Dawson, JD Parkes, S Williams, PN Leigh.King's College School of Medicine andDentistry, London, UK

The mechanism of motor fluctuations inidiopathic Parkinson's disease (IPD) is notclear. The striatal neuronal and biochemicalalterations that have been reported inParkinson's disease are based mainly onpostmortem, not in vivo studies. MRS wasused with regions of interest in the putamenand cerebellum to investigate eight patientswith probable idiopathic Parkinson's disease(mean age 54 (9) years) on levodopa treat-ment with motor fluctuations. Results werecompared with results with six age matchedhealthy controls (mean age 52 (10) years).Spectra were collected using a STEAMsequence at an echo time of 136 ms. Scanswere obtained in the "off" rather than "on"condition.

There was a significant reduction in puta-men NAA/creatine ratios in patients withidiopathic Parkinson's disease (mean 1-104(SD 0-17)) compared with controls (mean1-62 (SD 0-21), P < 0-001). NAA/cholineratios were also lower in the putamen ofpatients with idiopathic Parkinson's disease(1-106 (SD 0-16)) compared with controls(1-52 (SD 0 36), P < 0 03). There were nosignificant differences between values forthese ratios in the cerebellum in idiopathicParkinson's disease compared with controls.These findings suggest the presence ofaxonal and/or neuronal damage in the puta-men in late idiopathic Parkinson's disease.This may be related either to the diseaseitself or to the effects of long term levodopatreatment.[ABN]

A COMPREHENSIVE ELECTROPHYSIOLOGICALEVALUATION OF PHRENIC NERVE INJURYRELATED TO OPEN HEART SURGERYE Chroni, RL Patel, N Taub, GE Venn, RSHoward, CP Panayiotopoulos. St Thomas'Hospital, London, UK

Phrenic nerve damage is a widely recognisedcomplication of open heart surgery. Aprospective electrophysiological study ofphrenic nerve function was conducted in 59patients undergoing cardiopuimonarybypass. The right and left phrenic nerveswere stimulated percutaneously at the neckand the diaphragmatic responses wererecorded with surface electrodes placed overthe 8th intercostal space. The latency, ampli-tude, duration, and area of the evokedresponses were measured before and afterthe operation. Postoperatively no responseswere elicited in two patients bilaterally, infive from the left, and in two from the rightphrenic nerve. Comparison of the postopera-tive with the preoperative group values in theremaining patients showed that the ampli-tude and area of the left phrenic nerve werelower in the postoperative study, indicating

that some of the nerve fibres were notconducting. There were no statistically sig-nificant differences between pre and post-operative values of latency or duration on theleft or any of the parameters on the right.Follow up studies showed that in eight ofnine nerves with unobtainable responsespostoperatively, the conduction returned infour to seven weeks. The findings suggestedthat the amplitude and area of the diaphrag-matic response are more sensitive thanlatency in detecting phrenic nerve paresisassociated with open heart surgery.[ABN]

SPONTANEOUS INTRACRANIAL HYPOTENSIONFROM A CSF LEAK IN A PATIENT WITHMARFAN'S SYNDROMERJ Davenport, SJS Chataway, CP Warlow.University of Edinburgh, Western GeneralHospital, Edinburgh, UK

A 21 year old girl with known Marfan's syn-drome first presented in 1992 with a shorthistory of severe postural headaches reminis-cent of a postlumbar puncture (LP) head-ache. Other than the features ofher Marfan'ssyndrome the examination was normal. ACT brain scan was normal but at LP theopening pressure was unrecordable. Sherecovered spontaneously but two years laterpresented with an identical headache ofsuch severity that she required admission tohospital.

Radioisotope cistemography showed anabnormal accumulation of isotope at the TIlevel suggestive of a CSF leak; CT myelo-graphy confirmed this leak and multiplearachnoid diverticulae around the lumbro-sacral nerve roots were also identified.A saline epidural infusion led to an imme-

diate and dramatic improvement in hersymptoms. This improvement has beenmaintained after two thoracic epidural auto-logous blood patches and a subsequentradioisotope cistemogram has not shownany leak.

It is proposed that the patient's headachewas caused by a low CSF pressure secondaryto a CSF leak from the spinal subarachnoidspace, after rupture of one of the manydemonstrated arachnoid diverticulae, whichare associated with Marfan's syndrome. Therupture may have been either spontaneous or

following minor unrecognised trauma.Treatment with site specific epidural bloodpatching has proved remarkably successful.[ABN]

AN INVESTIGATION OF DIFFUSE AXONALINJURY OCCURRING AFTER CLOSED HEADTRAUMACA Davie, R Greenwood, IF Moseley, WIMcDonald, DH Miller. Institute ofNeurology, London, UK

Pathological studies in patients with severehead injury often show changes of diffuseaxonal shearing. The abnormalities detectedby MRI are often insufficient to explain thedeficits of memory or changes in personalitythat can occur. Proton magnetic resonancespectroscopy (MRS) may be useful inassessing the presence of axonal loss or

dysfunction by observing changes inN-acetylaspartate (NAA)-a neuronalmarker. MRI and MRS have been carried



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out in six patients with a history of severehead trauma and in eight healthy controls.MR images of the brain were collected fol-

lowed by long echo proton MRS from alocalised volume of normal appearing whitematter (NAWM) in the left frontal lobe. Infive patients there was mild cerebral atrophywith ventricular dilatation and in onepatient, more severe cerebral atrophy. Therewas a significant reduction in the concentra-tion of NAA (median 8-22 mM, range6-5-8-8 mM) from frontal NAWM in thepatient group compared with healthy con-trols (median 10-44 mM, range 8-76-10-81mM, P < 0 006). There was no significantdifference in the concentrations of creatineand choline containing compounds betweenthe two groups. Proton MRS may be usefulin measuring the extent and degree of axonalloss and/or dysfunction occurring afterclosed head injury.[ABN]

AUTOSOMAL DOMINANT DESMINOPATHYWITH A DISTAL MYOPATHY AND HEART

BLOCKDJ Dick, JR Anderson. Norfolk and NorwichHospital, Norwich, and Addenbrooke'sHospital, Cambridge, UK

Three members of one family over two gen-erations were found to have heart block anda distal myopathy. Neurological examinationof the index case showed a distal pattern ofmuscle weakness with preserved reflexes andnormal sensation. Electromyography, ECG,serum creatine kinase, and an ischaemic lac-tic test were normal. A muscle biopsyshowed selective type 2 atrophy and mainlysubsarcolemmal eosinophilic plaques ofcytoplasm. Immunohistochemical stainingshowed that these deposits stained positivelyfor desmin and to a lesser extent for ubiqui-tin, actin, and glycogen. Electromicroscopyof the deposits showed the presence of glyco-gen granules and electron dense granularmaterial, which was often closely associatedwith a smeared Z band.

These are the muscle biopsy features of adesminopathy, which in this family is inher-ited as an autosomal dominant trait. This is arare disorder in which there is accumulationof desmin in skeletal muscle and presumablythere are similar abnormalities to be found incardiac muscle or the conducting system.Desmin is one of the intermediate neurofila-ments and is a structural component of thecytoskeleton of mature muscle. It anchorsmyofibrils in the transverse plane and to thesarcolemma. The mechanism by whichdesmin accumulates in this condition is notclear.

Previously reported cases have beenmainly sporadic and if cardiac involvementoccurred, it took the form of cardiomyo-pathy.[ABN]

A METHOD OF CHARACTERISING CEREBRALATROPHYM Doran, N Roberts, RHT Edwards.University of Liverpool, Liverpool, UK

The aim was to evaluate the patterns andmorphological characteristics of cerebralatrophy using volumetric MRI.

Volumetric MRI was obtained from

patients with focal aphasic syndromes. Theimages were analysed with stereological gridsto determine the cross sectional area (A) andcortical boundary length (B) on each image.Profile plots of the cross sectional areas (A)and the complexity parameter B/ A of indi-vidual lobes of each hemisphere were obtained.The data for patients with focal aphasic

syndromes show distinct anterior or poste-rior patterns of morphological change in thetemporal lobes. Interval scans show that sig-nificant quantitative changes can on occa-sion be precisely determined within a periodof one year. The pattern of the changes hasbeen related to the evolving pattern of neuro-psychological deficit.

It is concluded that stereology represents arapid and efficient method of determiningmorphological data to characterise the pat-tern of atrophic change in cortical disease.[ABN]

INCREASED SOLUBLE VCAM-1CONCENTRATIONS IN CSF OF PATIENTS WITHMULTIPLE SCLEROSISAG Droogan, SA McMillan, JP Douglas, SAHawkins. Royal Victoria Hospital, Belfastand Belfast City Hospital, Belfast, UK

Factors determining the composition andtemporal evolution of the inflammatory infil-trate in multiple sclerosis are unknown, butthe profile of adhesion molecule expressionat the site of the lesion may play a part.Activated cerebral vascular endothelial cellsexpress leucocyte, vascular cell, and intracel-lular adhesion molecules (E-selectin,VCAM-1, and ICAM-1), which facilitateleucocyte adhesion to endothelium andmigration into inflammatory lesions. Serumand CSF concentrations of soluble (s) E-selectin, sVCAM-1, sICAM-1 were deter-mined by enzyme linked immunosorbentassay in 51 patients with clinically definitemultiple sclerosis, 28 patients with inflam-matory neurological disease (IND), and 39patients with non-inflammatory neurologicaldisease (NIND). Concentrations ofsVCAM-1 in CSF were significantlyincreased in multiple sclerosis (mean (SD),18-3 (9 9) ng/ml) compared with IND (11-3(7 3) ng/ml, P= 0-001) and NIND (10-6(5-3) ng/ml, P = 0-0001). Serum and CSFconcentrations of sICAM-1 were normal ineach group. Serum sE-selectin was de-creased in multiple sclerosis compared withNIND (45 5 (23-9) ng/ml v 55 0 (19 9)ng/ml, P = 0 03). In multiple sclerosis, highCSF concentrations of sVCAM- 1 andsICAM- 1 correlated with the presence ofCSF oligoclonal bands (P = 0 004 and P =

0-001 respectively). Measurement of solubleVCAM-1 in CSF therefore seems to provide amarker of disease activity in multiple sclerosis.[ABN]

PERIPHERAL NERVE BLOCKADE WITH PHENOL

IN SPASTICITY: A MYOMETRIC AND

FUNCTIONAL ASSESSMENTS Duckworth, N Jordan. Hunters MoorRegional Rehabilitation Centre, Newcastle-upon-Tyne, UK

Spasticity remains a significant cause ofneurological disability. Oral pharmacologicaltreatment is associated with unwanted sideeffects. Selective tone management is of

value. Peripheral nerve phenol injectionshave been shown to produce tone changes.This effect may be of functional value in themanagement of spasticity. A myometricparadigm was employed alongside standardmeasures of disability to assess the effect oflocal phenol injection into different sites in23 patients with spasticity. The amount offorce required to move the selected jointthrough a predetermined range was madebefore and after injection and at one monthafter injection. Results showed significanttone reduction in ankle plantarflexion (n =14, P < 0-001), knee flexion (n = 14, P <0-01), leg adduction (n = 9, P < 0-01),elbow flexion (n = 9, P < 0 05), and fingerflexion (n = 2, P < 0-001). The effect wasmaintained at one month after injection atthe ankle (n = 7, P < 0-001), knee(n = 14, P < 0 05), leg adduction (n = 9, P< 0-01), and fingers (n = 2, P < 0-05) butnot at the elbow (n = 7, P = 0-51). Overallactivities of daily living scores improved sig-nificantly after injection (n = 23, P < 0-01).This study confirms the usefulness of periph-eral nerve blockade with phenol in the man-agement of spasticity using a myometricparadigm and standard measures of overallfunction.[ABN]

INTEROBSERVER VARIATION IN CLASSIFYINGMULTIPLE SCLEROSISHL Ford, MH Johnson, AS Rigby. StJames's University Hospital, Leeds, UK

With the advent of new treatments formultiple sclerosis, clinicians must be able touse diagnostic criteria reliably and to definethe course of multiple sclerosis.The aim wasto determine if the Poser diagnostic criteriacould be applied consistently in practice andthe course of multiple sclerosis defined.The case records of 85 consecutive out-

patients with multiple sclerosis were retro-spectively analysed by two independentobservers. The cases were classified: clini-cally definite multiple sclerosis, laboratorysupported definite multiple sclerosis, clini-cally probable multiple sclerosis, laboratorysupported probable multiple sclerosis, sus-pected multiple sclerosis, and unable toclassify; the course was defined as earlyrelapsing-remitting, benign, secondary pro-gressive, primary progressive, and unable toclassify.The statistical agreement between the two

observers was measured by calculating theK statistic. There was substantial agreementin classifying the cases (K = 0-65, 95% CI =

0 52-0-78). The main disagreement lay indefining attacks of multiple sclerosis (K =0 30, 95% CI = 0-03-0 57). There wasmoderate agreement for determining thecourse of multiple sclerosis (K = 0-57, 95%CI = 0-43-0-71). The observers differed intheir definition of primary progressive andsecondary progressive cases. This may bedue to the poor documentation of relapsesand remissions early in the disease course.The efficacy of new treatments is being

judged on relapse rate. Consensus definitionof an attack of multiple sclerosis is there-fore essential. Each attack should bewell documented, as should subsequent clin-ical improvement. The definition must berigorously adhered to to allow consistentapplication of diagnostic criteria in practice.[ABN]



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P-roceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons

ANTI-PURKINJE CELL CYTOPLASMIC

ANTIBODIES. ARE THEY NON-SPECIFICMARKERS OF CEREBELLAR CELL DAMAGE?

A Gibson, M Hadjivassiliou, A Milford-Ward, GAB Davies-Jones. Royal Hallamshireand Northern General Hospitals, Sheffield,UK

Anti-Purkinje cell antibodies that recognisediscrete antigenic components withinPurkinje cell cytoplasm have been desig-nated "anti-Yo" and shown by some authorsto be strongly associated with paraneoplasticcerebellar degeneration. The association isnot universally accepted and may merelyreflect Purkinje cell damage.We have used an immunofluorescent

method, with monkey cerebellar slices as

substrate, to detect the presence of a

Purkinje cell cytoplasmic antibody inpatients with and without cerebellar signs as

well as a group of age and sex matchedhealthy controls.A total of 60 ataxic patients with mean age

49 years and a male to female ratio of unitywere compared with 28 neurological controlswithout cerebellar signs and 50 healthy con-

trols with mean age 50 years and the samemale to female ratio. The proportions ofcytoplasmic antibody positives in the threegroups were 40/60, 1/28, and 4/50 respec-tively. Using analysis of proportions showsthat these differences are significant at the0-001 level or less.

It remains to be seen if the antibodydescribed here is the same as that describedelsewhere but further characterisation of theantigenic determinant with western blotanalysis should resolve this issue.[ABN]

EVALUATION OF A COMPUTER ASSISTEDQUANTIFICATION OF MULTIPLE SCLEROSISLESIONS IN CRANIAL MRIJ Grimaud, M Lai, JW Thorpe, P Adeleine,D Plunmmer, G Barker, PS Tofts, WIMcDonald, DH Miller. Institute ofNeurology, London, UK

Several computer assisted techniques formeasuring multiple sclerosis lesion volumeon MRI have been developed to provide an

objective, quantitative, and sensitive means

for monitoring disease activity, particularlyin the context of treatment trials. We haveevaluated three techniques: manual tracing(similar to that of the North American,Binterferon trial), automated lesion contour-ing (local lesion based threshold) and inten-sity based thresholding for the whole brain.Contiguous 5 mm thick axial proton densityimages ofthe brain were obtained on a 1-5 TMR imager in eight patients with definitemultiple sclerosis. Analysis of the scans were

performned twice, independently by threeoperators, using the three different tech-niques.The table shows the results. The auto-

mated lesion contouring technique was themost reproducible and should therefore beconsidered for use in treatment trials.Further studies are needed to assess sensitiv-ity to changes in lesion load over time.[ABN]

LNK, A NEW CLASS OF PROTEIN KINASE: HIGHEXPRESSION LEVELS IN GLIOMAS AND DURINGHUMAN NERVOUS SYSTEM DEVELOPMENTNJ Gutowski, R Ludwig, C Proschel, M-JBlouin, M Noble. Ludwig Institute forCancer Research, London, UK

Protein kinases that include growth factorreceptors are central in mediating divisionand differentiation in the nervous systemand may also be important as cell lineagemarkers.From the cDNA library of a human

glioblastoma multi forme cell line of theoligodendrocyte type 2 astrocyte (0-2A) lin-eage a new protein kinase LNK was cloned.LNK represents a new class of proteinkinase. Although it contains all conservedmotifs in the kinase domain it has low overallamino acid homology to other kinases in thisdomain. It also has two LIM domains in theamino terminal region possibly indicating atranscriptional role. By northern analysis ontotal RNA, the LNK 3.3 kb band has beenfound to be upregulated in many glioblas-toma primary cell populations. In the mousethe highest expression of murine LNK isfound in the nervous system. Expression ofLNK has been found in the developinghuman nervous system by in situ hybridisa-tion.

These findings may be consistent withLNK having a regulatory role in humanCNS development, in gliomas, and also as acell lineage marker.[ABN]

UPTAKE OF SERVICES FOR EPILEPSY AND THECOSTS OF CAREA Jacoby, G Baker, D Chadwick.Universities of Newcastle-upon-Tyne andLiverpool, UK

Epilepsy is a common neurological conditionand it is estimated that in the UnitedKingdom there are presently around 300000people with active epilepsy. The resourceimplications of caring for these patients arelikely, therefore, to be significant. A recentcommunity study in one health region in theUnited Kingdom has examined the uptake ofmedical services among people with epilepsyand calculated the direct and indirect costsof their care. Patients included in the studywere identified through the medical recordsof a random sample of general practitionersin the region. The pattern of care was verydifferent for adults and children, reflecting inpart the natural history of epilepsy: thus 86%

Intraobserver agreement Interobserver agreement

Techniques Min Max Mean (SD) Min Max Mean (SD)

Manual 0-4 18-5 9 0 (5-2) 4 9 21-7 11-0 (5-8)Contouring 0-1 7-7 2-48 (2-1) 1-8 6-6 4-5 (1-6)Thresholding 0-2 22 7-5 (6 9) 2-8 19-2 11-4 (4-9)

Coefficients ofvariation of the measurement techniques (0 0 = perfect reproducibility).

of children had a hospital outpatient atten-dance in the past year compared with only31% of adults; children were also signifi-cantly more likely to have had diagnosticinvestigations.The largest single cost to the health service

was the drug cost, representing around 40%of the total; inpatient costs also accountedfor a sizeable proportion of costs. Uptake ofmedical services was significantly higheramong patients with frequent seizures-forexample, mean number of inpatient admis-sions was 1-6 among adults with frequentseizures, compared with 0-01 among thoseseizure free. People with frequent seizuresalso had a much higher uptake of state bene-fits. Improvements in the management ofepilepsy may improve seizure control, whichis the key to reducing the direct and indirectcosts of this condition.[ABN]

WEEKLY V MONTHLY BRAIN MRI FORDETECTION OF DISEASE ACTIVITY INMULTIPLE SCLEROSISHM Lai, T Hodgson, DG MacManus,S Webb, WI McDonald, DH Miller.Institute ofNeurology, London, UK

In therapeutic trials, MRI at monthly inter-vals is often used to evaluate efficacy. At suchan interval, 80% of new lesions on T2weighted images enhance, and conversely,about 50% of new gadolinium enhancinglesions are not associated with clear changeson the T2 weighted image. Weekly T2 andgadolinium enhanced brain MRI were per-formed for three months in three patientswith clinically active relapsing progressivemultiple sclerosis.On weekly MRI 38 new enhancing lesions

were seen. On monthly scans there were 33new enhancing lesions but only 13 new T2lesions. Twenty five lesions were followed upfrom when they first enhanced until they hadstopped enhancing. Of these 11 (44%)enhanced for less than four weeks (one tothree weekly scans). All new T2 lesionsshowed initial enhancement.

Monthly gadolinium enhanced MRI isonly slightly less sensitive than weekly scan-ning in detecting disease activity, and is stillthe preferred frequency for monitoring treat-ment as it is easier to perform. It seems fromweekly studies that virtually all new T2lesions undergo an initial phase of enhance-ment-as this may occasionally be as short asone to two weeks, it is sometimes missed onmonthly studies.[ABN]

X LINKED DOMINANT HEREDITARY MOTORAND SENSORY NEUROPATHY WTH CX32MUTATIONBRF Lecky, PK Thomas, S Malcolm.Walton Centre for Neurology andNeurosurgery, Liverpool, Royal FreeHospital School of Medicine, and Instituteof Child Health, London, UK

Three generations of a family are describedwith hereditary motor and sensory neuro-pathy (HMSN). Inheritance is consistentwith X linked dominant transmission, whichis also suggested by the more severe clinicalinvolvement of affected males. The onset ofsymptoms is in early adolescence with distal



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wasting and weakness. A prominent symp-tom, not characteristic of HMSN types I orII, is of painful cramping of the extremitiesboth on exercising affected muscles andspontaneously at night. Disability in affectedfemales is not severe whereas affected maleshave considerable difficulty with both man-ual function and gait.

Nerve conduction studies show reducedor absent sensory action potentials with mildto moderate slowing of motor conductionvelocities, although not to the extent usuallyfound in HMSN type I.The gene for the gap junction protein con-

nexin 32 is located on the long arm of the Xchromosome. This group of proteins isknown to connect the cytoplasmic compart-ments of cells. Their precise role is notknown in neural tissue but Cx32 occurs inmammalian peripheral nerve at the nodes ofRanvier and in Schmidt-Lantermanincisures. Several different point mutationsof Cx32 have been described. The presentfamily shows a methionine for valine sub-stitution at position 139.[ABN]

NEUROPHYSIOLOGICAL EXAMINATION OFRESPIRATORY MUSCLE FUNCTION INMYOTONIC DISORDERSVP Misra, RS Howard, BD Youl. NationalHospital for Neurology and Neurosurgery,London, UK

Ventilatory failure in myotonic dystrophy isassociated with weakness of the respiratoryand bulbar muscles and a defect in centralcontrol. Previous reports have suggested thepresence of myotonia in the primary andaccessory muscles of respiration but this hasnot been studied in detail. Four patients withmyotonia (three with myotonia dystrophicaand one with myotonia congenita) werestudied.A concentric needle EMG examination

was carried out by a standard technique. Theobliquus extemus abdominis, eighth inter-costal, and diaphragm muscles yieldedmyotonic discharges in all patients.Additionally, all patients with myotonia dys-trophica showed abnormally polyphasicmotor units of short duration and low ampli-tude at all three loci.The findings confirm that myotonia may

affect respiratory muscles, including thediaphragm. In addition, myopathic changesare present in the diaphragmatic muscles ofpatients with myotonic dystrophy.[ABN]

TREATMENT OF PAINFUL FEET AND MOVINGTOES WITH BOTULINUM TOXIN A

AP Moore, J Pilling. Walton Centre forNeurology and Neurosurgery, Liverpool andNorfolk and Norwich Hospital, Norwich,UK

Treatment of an underlying cause may helpin the syndrome of painful feet and movingtoes, but conventional treatment is oftenineffective in idiopathic cases. Treatmentwith botulinum toxin A (BTx-A) has notbeen previously reported. We describe threewomen with painful feet and moving toesunresponsive to conventional treatment, andtreated with local injections of BTx-A.

Patient 1, aged 47 had 18 months painand wiggling of the right little toe. Injectionof 150-250 MU Dysport three monthly intoabductor digiti quinti and the fourthinterosseous muscle has greatly improvedinvoluntary movements and pain since 1989.

Patient 2 aged 50 had five years of wig-gling movements of all toes and severe painin the left foot and lower leg. Infiltration of500 MU Dysport through the plantar fasciacontrolled movements for six months. Paincleared for one month, and for a further yearwas reduced with an additional transcuta-neous nerve stimulator. Further BTx-Ainjections controlled the movements but notthe pain.

Patient 3, aged 37 had 18 years of painand movements of all toes and both feet.Dysport (250 MU) infiltrated into each footas for patient 2 suppressed the movements,but gave only two weeks of pain relief, andadjunctive measures were unsuccessful.Booster doses of 300 MU/foot added nobenefit.More studies are needed, but BTx-A may

control the movements and sometimes thepain in patients with this syndrome.[ABN]

HEADACHES IN THE ACCIDENT ANDEMERGENCY DEPARTMENT: ARE WEOVERINVESTIGATING?S Moreea, S Goodacre, MH Johnson.St James's University Hospital, Leeds, UK

A prospective study was conducted on allpatients presenting to the accident and emer-gency department with a primary complaintof headache. They were followed up afterthree months to reach a final diagnosis.

In three months, there were 93 cases outof 21 187 attendances (0 44%). Seriouspathology was found in 10 patients: threesubarachnoid haemorrhages (3 2%), oneintracerebral haemorrhage, three meningitis,and three cerebral neoplasms. 48 patients(52%) were seen acutely by a neurologist, 31being admitted. This included all cases ofserious pathology except for one neoplasm.39 patients (42%) described a headache ofsudden onset, the remaining having aheadache of gradual onset. Of the first, sixhad serious pathology: the three subarach-noid haemorrhages (7*7%), the intracerebralhaemorrhage, one neoplasm, and onemeningitis. They were among the 18 (46%)patients admitted from this group. Forty fivepatients (48%) were referred by their generalpractitioner. Serious pathology was found intwo of the 18 describing a sudden headache(one neoplasm and one subarachnoid haem-orrhage) and two of the 27 with a headacheof gradual onset (both neoplasms). In all, 30(32%) had a head CT, eight in the acutephase. There were seven other subarachnoidhaemorrhages presenting differently duringthe study period.The results highlight suboptimal use of

resources. Is there a case for headache cardssimilar to head injury cards used in the acci-dent and emergency department?[ABN]

DETECTION OF ENTEROVIRUS RNA IN THE

CNS OF PATIENTS WITH PREVIOUS PARALYTIC

POLIOMYELITISP Muir, F Nicholson, J Ajetunmobi, M Li,GT Spencer, RS Howard, JE Banatvala.

United Medical and Dental Schools and StThomas' Hospital, London, UK

It has been suggested that the postpolio syn-drome may be due to persistence or reactiva-tion of poliovirus infection in the CNS. Inview of this we decided to search forpoliovirus and other enteroviruses in theCNS of patients with previous poliomyelitisto determine whether or not these virusespersist in the CNS in humans. Cerebrospinalfluid and peripheral blood was studied usinga nested polymerase chain reaction for thepresence of enterovirus RNA. Thirtypatients have been studied. EnterovirusRNA was detected in CSF from one patient,in CSF and lymphocytes from a secondpatient, and in serum only from a thirdpatient. By contrast enterovirus RNA wasnot detected in 30 CSF samples from controlpatients without evidence of infectious dis-ease. Four patients with previous poliomyeli-tis died from unrelated causes during thestudy. Fresh postmortem samples were avail-able from three and enterovirus RNA wasdetected in spinal cord tissue from twopatients. Nucleotide sequencing studies areunderway to identify the enterovirusserotypes involved. It is unlikely that allpatients in whom enterovirus was detectedhad acute infections. If this point is con-firmed by further study, these results suggestthat enteroviruses do persist in the CNS ofpatients with previous poliomyelitis, asrecently demonstrated in patients withmotor neuron disease. Further study is nowrequired to understand the biological andclinical relevance of these findings.[ABN]

AN ASSESSMENT OF DIETARY AND SERUM ow)-3POLYUNSATURATED FATTY ACIDS ANDANTIOXIDANTS IN NEWLY DIAGNOSEDPATIENTS WITH MULTIPLE SCLEROSISHES Mulholland, SA Hawkins, JJ Strain, ISYoung, MBE Livingstone. Royal VictoriaHospital, Belfast, Queen's University,Belfast, and University of Ulster, NorthernIreland

There has been a longstanding interest in therelation of polyunsaturated fatty acids to thepathogenesis and treatment of multiplesclerosis. As soon as patients are given thediagnosis of multiple sclerosis most makechanges to their eating habits. Many studiesin the past, performed on patients withestablished multiple sclerosis, have shownlow serum linoleic acid. There is little infor-mation on serum fatty acids in newly diag-nosed patients. The lipid profile andantioxidant status in 30 newly diagnosedpatients in the Neurology Department inBelfast were examined and compared withcontrols matched for age, sex, race, socio-economic group, and smoking habits.Dietary intake was assessed using a seven daydietary history and a self administered foodfrequency questionnaire. Patients and con-trols ate similar diets. Serum and red cellmembrane fatty acids were determined bygas liquid chromatography. Vitamins A, E,and carotenoids were measured by high per-formance liquid chromatography (HPLC).Lipid peroxidation was measured using theTBA reaction. No major differences in theblood concentrations of the variables exam-ined were found.[ABN]



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Proceedings ofthe Association ofBritsh Neurologists and the Society ofBritish Neurological Surgeons

Hhal POLYMORPHISM IN THE CYP2D6 GENE ISNOT ASSOCIATED WITH PARKINSON'S DISEASEIN CAUCASIANSDJ Nicholl, AC Williams, P Bennett, SL Ho,DB Ramsden. University of Birmingham,Birmingham, UK

A genetic association between mutant allelesof the cytochrome P-450 (CYP2D6) geneand Parkinson's disease has been reported.Polymorphisms in this gene, situated onchromosome 22, give rise to the autosomalrecessive, poor debrisoquine metaboliserphenotype, and to a more than twofoldincreased risk of developing Parkinson's dis-ease in Caucasian populations. Recently, anovel mutation, an Hhal polymorphism inCYP2D6 exon 6, was shown to confer a five-fold increased risk of Parkinson's disease inJapanese; we have studied this further in aCaucasian population where Parkinson'sdisease is more common.DNA was analysed by the polymerase

chain reaction (PCR) in 96 patients withsporadic Parkinson's disease and 60 con-trols, the products digested with Hhalrestriction endonuclease, and the digest pat-tems studied after gel electrophoresis.Although the overall prevalence of thismutant allele was significantly higher in thispopulation than in Japanese (36% v 12%,X2= 63-2, P < 0 001), there was no statisti-cally significant difference in the distributionof the alleles between the patients withParkinson's disease and controls(Parkinson's disease: 42 wild type (wt), 41heterozygotes (he), 13 mutant homozygotes(mu); v controls:23 wt, 30 he, 7 mu) (X 2(3 x 2 table) = 0-79; P = 0 68). Therefore, itseems that the Hhal polymorphism is not, byitself, ofmajor significance to the aetiology ofParkinson's disease in Caucasians.[ABN]

CLINICAL, CSF, AND MRI FINDINGS INDEVIC'S NEUROMYELITIS OPTICAJI O'Riordan, H Gallagher, DPE Kingsley,EJ Thompson, WI McDonald, DH Miller.Institute ofNeurology, London, UK

Eleven patients with Devic's neuromyelitisoptica are described. The mean age of pre-sentation was 36-3 years and 10 patientswere female. Severe deficits in ambulationand vision were the rule rather than theexception with vision reduced to countingfingers or worse in nine patients and sixremaining wheelchair bound. There wasnegative local synthesis of CSF oligoclonalbands in nine. Two patients had evidence ofa connective tissue disorder and six had non-specific increases of various autoantibodies.Ten patients underwent MRI of brain andspinal cord. In nine there were diffuse abnor-malities involving both cervical and thoraciccords with extensive cord swelling. Theremaining patient had linear signal change inthe upper cervical and thoracic cords. Abrain MRI was normal in six; in four therewere multiple deep white matter lesions.Two patients had signal change in the opticnerves; one with associated swelling. Devic'sneuromyelitis optica remains a disorder ofunknown aetiology with poor outcome.Thus MRI is a valuable tool in diagnosis,aiding the prognostic evaluation by thephysician.[ABN]

FUNCTIONAL INTEGRITY OF CORTICALDYSPLASIAMP Richardson, MJ Koepp, DJ Brooks, DRFish, JS Duncan. Institute of Neurology andMRC Cyclotron Unit, London, UK

The extent of altered benzodiazepine recep-tor density in focal cortical dysgenesis was

determined. It was hypothesised that thearea of functional abnormality in corticaldysgenesis exceeds the structural abnormal-ity seen on MRI.

"IC-Flumazenil PET was performed todetermine volume of distribution (IIC-FMZ-Vd) in four patients. These scans were

co-registered with high resolution MRI pixelby pixel. The extent of dysplasia was definedon the MRI and regions of interest placed onthe abnormality, surrounding cortex, distantcortex, and in corresponding areas in theopposite hemisphere. The same set ofregions of interest was applied to the PETand '1C-FMZ-Vd determined. The resultswere compared with 10 normal controlsand expressed as asymmetry indices.Additionally, each of these same fourpatients' 'IC-FMZ-Vd scans were analysedby Statistical Parametric Mapping (SPM),via automated volumetric normalisation,analysis of covariance (ANCOVA), andpixel-by-pixel t test in comparison with 24normal controls.The regions of interest based approach

showed significant differences betweenasymmetry indices in patients and controlsfor the lesion region (P < 0-01) and for thesurrounding cortex (P < 0 02) but not fordistant cortex. These findings were con-

firmed at the P < 0-00 1 level in SPM, whichadditionally showed the anatomical extent ofchanges in detail. It was concluded that theexistence of more widespread functionalcompared with structural abnormality mayin part explain the poor outcome of focalresections in patients with focal dysplasia.[ABN]

NON-FATAL INJURIES IN AN UNSELECTEDPOPULATION OF PATIENTS WITH EPILEPSYD Smith, GA Baker, A Jacoby, DWChadwick. Walton Centre for Neurologyand Neurosurgery, Liverpool, UK

The excess mortality associated withepilepsy, and the factors responsible for thisare well documented. Epidemiological dataindicate that fatal accidents are over-represented as a cause of death in epilepsy.Non-fatal accidents are much more commonbut their incidence, and associated factors,are not known with any certainty.A postal questionnaire has been sent to a

heterogenous, community based population(n > 1000) ofpatients with epilepsy. In addi-tion to clinical and demographic details andpreviously validated scales of psychosocialwellbeing, this contains a series of questionsabout injuries; burns, head and dentaltrauma, fractures, and submersion accidentsincurred in the previous 12 months.

Data are available on 346 adults. Burnsand scalds, head injury, dental injury, frac-ture, and submersion accidents are reportedin 14%, 18%, 8%, 5%, and 13% respec-tively.

Multivariate analysis, designed to identifyfactors associated with these injuries, are

being conducted. These data should permitestimates of individual risk, which facilitates

sensible patient counselling, includingadvice about preventative measures, withoutengendering undue alarm.[ABN]

FELBAMATE INHIBITS VERATRIDINE INDUCEDGLUTAMATE RELEASE FROM MOUSE CORTEXJ Srinivasan, A Richens, JA Davies.University of Wales College of Medicine,Cardiff, UK

Felbamate, a new anticonvulsant drug, hasan affinity for the strychnine insensitiveglycine site on the NMDA receptor. Thisstudy compared the effects of felbamate and5,7 dichlorokynurenic acid (5,7 DCKA), apotent glycine site antagonist, on glutamaterelease.

Glutamate was released from coronalslices of mouse cortex by potassium (60mM) or veratridine (20 ,uM). Perfusate wasassayed for glutamate by high performanceliquid chromatography. Basal release ofglutamate was 3-5 pmol/mg tissue/2 min.The first pulse of potassium or veratridineinduced a pronounced (eight to ninefold)release of glutamate over basal and the sec-ond pulse produced an average release of 90(8%) and 67 (6%) respectively of the firstpulse. Felbamate (100pM to 800 pM) hadno effect on basal or potassium stimulatedrelease of glutamate. Veratridine stimulatedrelease was significantly inhibited by felba-mate in concentrations of 400 pM (39-2(6%), n = 12, P < 0-01) and 800pM (37 9(5%), n = 8, P < 0-01). 5,7 DCKA (50 to200 pM) had no effect on glutamate release.The inhibitory effect of felbamate on glu-

tamate release is probably unrelated to itsaffinity for the glycine site on the NMDAreceptor and may be due to inactivation ofsodium channels.[ABN]

EUROPEAN COMMUNITY STROKE PROJECT:RESOURCE USE, COSTS, AND OUTCOME OFDIFFERENT CARE PACKAGES FOR STROKEJ Stewart, R Howard, R Ross Russell, ARudd, C Wolfe. St Thomas' Hospital,London, UK

Stroke imposes a major burden on healthresources worldwide; in the UnitedKingdom acute stroke care alone costs 4-6%of the NHS budget. There is a 1 0-fold varia-tion in age standardised mortality for strokein people under 65 years across Europe,which is not reflected in the incidence ratesand therefore may be related to different pat-terns of care for stroke patients.The EC stroke project has been estab-

lished to explore the relation betweenresource use, costs, and outcome of stroke.The study involving 24 hospitals from nineEuropean countries, commenced on1 January 1993. Methodology has beendeveloped to enable standardised data col-lection to describe stroke care in terms oftype and severity of stroke, hospital admis-sion rates, bed type, duration of stay, use oftests, amount of rehabilitation, and outcomeat one year.

Initial resource data on 2438 strokepatients are reported for the first year. In twoSpanish centres, 81% of patients wereadmitted to neurology beds compared with0% in two British centres. In one German



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centre, 94% of patients received brain imag-ing, 80% carotid Dopplers, and 36% cerebralangiography, compared with 30%, 0%, and1% respectively in one British centre. It willbe interesting to correlate this wide variationin resource use with outcome at one year.[ABN]

SPINAL MULTIPLE SCLEROSIS: A CLINICALAND MRI STUDYJW Thorpe, D Kidd, IF Moseley, DASCompston, WI McDonald, DH Miller.Institute of Neurology, London andUniversity of Cambridge, Cambridge, UK

Although MRI detects the white matterlesions of multiple sclerosis within the brainwith high sensitivity, a minority of patientshave normal brain imaging. Nineteenpatients are described, selected from over170 who had undergone brain and spinalcord imaging, in whom minimal (n = 9) orno (n = 10) brain abnormalities were found.Eleven had clinically definite or laboratorysupported definite multiple sclerosis accord-ing to the Poser criteria; four had clinicallyprobable disease, and four had progressive

isolated myelopathy. All had presented withsymptoms and signs referable to the spinalcord or optic nerves. Eleven had a primaryprogressive course, seven relapsing-remittingand only one secondary progressive.Moderate or severe disability was the rule inthe primary progressive patients whereas therelapsing remitting patients were minimallydisabled. All had at least one lesion visible inthe spinal cord. In patients in whom thediagnosis of multiple sclerosis is not sup-ported by abnormalities on brain MRI,imaging of the spinal cord can be of consid-erable value.[ABN]

"PURE" AUTOSOMAL DOMINANT SPASTICPARAPLEGIA WITH LATE ONSET DEMENTIA: A

FAMILY STUDYS Webb, J Hutchinson, M Hutchinson. StVincent's Hospital, Dublin, Eire

The phenotype of "pure" forms of hereditaryspastic paraplegia (HSP) is highly variable.Familial dementia has not been reported ineither the pure or complicated forms ofHSP.A family with autosomal dominant HSP is

presented. Thirty eight of the 54 livingmembers of the family were examined; therewere 11 affected persons of the 26 membersover the age of 30. Both the severity of theparaplegia and the age of onset (20-50 years)varied considerably, with one asymptomaticperson at the age of 62 years.One member died of a four year dement-

ing illness at the age of 62. Four othermembers of this sibship aged 62-70 hadneuropsychological examination; all showedevidence of a dementia. Vocabulary, reading,and computational abilities were relativelypreserved by contrast with a pronounceddeficit in perceptual and constructionalability. Verbal learning and memory werebelow expectation but not as dramaticallyimpoverished as visual recall. All four pro-files were remarkably similar, worsened withage, and all four tended to use overt compen-satory verbal analysis in visuoconstructivetasks. Magnetic resonance imaging showed ageneralised atrophy. This dementia is diffi-cult to classify; it differs from Alzheimer'sdisease in having intact reasoning and rela-tively preserved verbal skills and is charac-terised by a progressive predominantlyparietal cognitive loss in the seventh decade.[ABN]



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