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

Proceedings ofthe Association ofBritish Neurologists and the J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from Society of British Neurological Surgeons, University of Liverpool, 5-7 April 1995

Platform Presentations epileptic drivers carried out between 1990 Conclusions-Results were strikingly better and 1991 by the Transport and Road than those of all four previous series, which LOWER CRANIAL NERVE COMPRESSION Research Laboratory (8888 drivers). Both used foetal mesencephalon alone. This SYNDROMES surveys were retrospective and collected experimental technique shows promise for JB Miles, PR Eldridge, L Dunn, data for three years before the survey. treating Parkinson's disease. I Mackenzie, J Meaney. Walton Centre for The relative risk for involvement in any [SBNS] Neurology and Neurosurgery, Liverpool, UK accident during this period was 0 95 (95% CI 0-88-1-02). However, the adjusted Objectives-To validate the causal signifi- relative risk for involvement in an injury cance of compression and the effectiveness producing accident was somewhat raised in EN BLOC TEMPORAL LOBECTOMY V and safety of microvascular decompression the group 1 1 (0-91-1-3). The SELECTIVE AMYGDAIOHIPPOCAMPECTOMY AS (MVD) in syndromes of the V, VII, VIII, IX adjusted odds ratio for serious physical TREATMENTS FOR INTRACTABLE EPILEPSY and Xth cranial nerves. injury was 1-37 (1-02-1-84). In the epilepsy DUE TO HIPPOCAMPAL SCLEROSIS Design-Clinical, neurological, and imaging group there were 12 drivers involved in an JM Oxbury, CBT Adams, SM Oxbury, KN assessment, including magnetic resonance accident resulting in a fatality, but none in Carpenter, SA Renowden. Radcliffe tomo-angiography (MRTA),2 followed by the TRL group. Infirmary, Oxford, UK MVD during continuous brainstem (audi- The factors that influenced the risk of tory) monitoring. accident rates were not dissimilar in the two Various surgical treatments for intractable Patients-110 patients with trigeminal groups, age, driving experience, and epilepsy due to hippocampal sclerosis are neuralgia (TGN); 25 patients with mileage being the most important. The recommended. It is uncertain which is most hemifacial spasm (HFS); two patients with absence of any seizure within the three year effective. Sixty nine patients with this tinnitus; six patients with IX or X neuralgia; period of the survey halved the risk of condition were treated-50 Falconer six patients by with systemic hypertension involvement in an accident producing some type en bloc temporal lobectomy (TL), 19 (HBP). form of serious physical injury or fatality. by selective amygdalohippocampectomy Outcome-The quantitative effectiveness of No other clinical factors in the epilepsy (AH). The two methods are compared. Of MVD including morbidity data, has been group seemed to influence accident risks. particular concern was seizure outcome and established by questionnaire and interview [ABN] verbal memory loss after left operations. over the 15 year study. Eleven AH were transcortical via the middle Results-6% of TGN recurred; 20% HFS temporal gyrus, and were was eight trans-sylvian. unrelieved or recurred; the two patients Two years after operation 54% had not with tinnitus were satisfactorily relieved; five NEURAL TRANSPLANTATION BY ER had any seizure since surgery; 23% had of the six patients with IX/X neuralgia were HITCHCOCK: TREATING PARKINSON'S experienced only very occasional seizures cured, and three of the six patients with DISEASE BY GRAFTS OF FOETAL (77% in Engel grades I+II). of 41 MESENCEPHALON Thirty HBP seemed improved. AND STRIATUM cases were seizure free five CHA (73%) years Conclusions-Microvascular decompression Meyer. Midlands Centre for after operation. Neither seizure outcome is more effective than other existing Neurology and Neurosurgery, Birmingham, nor serious unwanted effects differed UK methods in relieving symptoms and signs of significantly according to type of surgery. lower cranial nerve compression syndromes Objectives Neuropsychological assessment two years with acceptable safety using diagnostic Experimental neurosurgical after operation showed less verbal memory imaging and interoperative treatment of Parkinson's disease. monitoring. decline after left AH than after left TL. Left http://jnnp.bmj.com/ Design-Since 55 Outcome measurement supports the 1988, patients with severe AH, but not left TL, gave significant compression as being significantly involved Parkinson's disease (Hoehn and Yahr stage improvements in verbal IQ and non-verbal in achieving relief rather than this being IV, V) have been treated at the Midlands memory. Performance IQ improved in both simply due to nerve injury. Centre for Neurology and Neurosurgery by MRI showed less stereotactic groups. Postoperative [SBNS] implantation of tissue temporal lobe white matter disruption after 1 Janetta PJ. Neurovascular compression in cra- from spontaneously aborted human nial nerve and systemic diseases. Ann Surg trans-sylvian AH than after the transcortical 1980;192:518-25. foetuses. Each implantation used a single approach. 2 Meaney JFM, Miles JB, Nixon TE, et al. foetus of second trimester (11-20 weeks It is concluded that trans-sylvian AH is

Vascular contact with the fifth on September 29, 2021 by guest. Protected copyright. cranial nerve post-gestational); grafts dissected 5-12 preferable to TL as a treatment for most at the pons in patients with trigeminal neural- hours after foetal expulsion, disaggregated gia: detection with 3D FISP imaging. A7R cases of intractable epilepsy due to left Am_JRoentgenology 1994;163:1447-62. mechanically, and implanted as clumps (not hippocampal sclerosis and probably also as cell suspensions); there was no immuno- that due to right hippocampal sclerosis. suppressive therapy. [ABN] Consecutive patients formed five series RISK OF ACCIDENTS IN DRIVERS WITH differing in site of implantation (caudate or EPILEPSY putamen, unilateral or bilateral) and in D Chadwick. University of Liverpool, nature of graft (foetal mesencephalon alone Liverpool, UK or with striatum). VOLUMETRIC MAGNETIC RESONANCE In the final series (described here) grafts IMAGING (MRI) IN CHRONIC EPILEPSY Between June 1992 and May 1993 a survey of foetal mesencephalon and striatum were SM Sisodiya, SL Free, DR Fish, SD was undertaken of driving and accident implanted bilaterally into the head of the Shorvon. Institute ofNeurology, London, UK experience in people with a history of caudate nucleus. epilepsy communicating with the DVLA. Patients-Seven patients (Hoehn and Yahr Volumetric MRI enables detailed examina- 24 000 questionnaires were issued as part of stage IV, V) aged 46-62 years. tion of cortical anatomy. An investigation the routine relicensing of drivers at three Results-All seven patients improved. There into chronic epilepsy using MRI and post- year intervals, of which just over 17 000 were very good group improvements, processing techniques is being carried out. were returned (response rate of 71 %). In statistically significant (Wilcoxon), in a wide Scanning patients with primary gener- addition, 468 questionnaires were issued to range of activities of daily living measures, alised epilepsy showed underlying structural drivers reporting recent seizures at the clinical neurology, and timed motor tasks abnormalities in six cases. Three had sub- DVLA of which 291 were returned sustained throughout 18 months follow ependymal heterotopia and three hippo- (response rate of 62%). The questionnaire up-with substantial reduction in need for campal asymmetry: one of these improved used identical methods to a cohort of non- dopaminergic medication. after temporal lobectomy. Reconstruction of Proceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons 201

loss of SUBARACHNOID HAEMORRHAGE AND THE a three dimensional image of the cerebral compare extent of benzodiazepine J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from hemispheres and comparison of the gyral receptor density with volumetric MRI in INFLAMMATORY CYTOKINE RESPONSE pattems demonstrated with control subjects patients with epilepsy due to mesial RH Lye, WR Lamb, PEC Brenchley. showed gyral abnormalities in 16 of 30 temporal sclerosis (MTS). "IC-Flumazenil University of Manchester, Manchester, UK patients with normal MRI on inspection of binding has been shown to be reduced in routine two dimensional images, and addi- MTS. To date, no studies have formally Introduction-There is evidence that the tional gyral abnormalities in three of 18 compared extent of sclerosis with volu- immune system is activated after patients with cortical dysgenesis observed on metric MRI and 3D "IC-flumazenil PET- aneurysmal subarachnoid haemorrhage routine images. Analysis of the distribution changes. It is hypothesised that the (SAH). The proinflammatory cytokines of cerebral grey/white matter, by division of reduction of hippocampal BZ/GABA-A IL-if?, IL-6, and TNFa were measured in reconstructed objects into coronal 1 Oths, receptor density in patients with MTS is 10 patients who had had SAH. allows measurement of cerebral structure. over and above the loss of hippocampal Method-The in vivo cytokine response was The existence of structural abnormality volume. monitored in eight patients by daily beyond the visualised lesion was shown in 15 Ten patients with MTS and pronounced measurements of systemic concentrations in of 18 patients with cortical dysgenesis on unilateral hippocampal sclerosis were serum during their hospital stay. A whole routine imaging and in nine of 14 patients studied. "IC-Flumazenil PET Vd images blood endotoxin stimulation assay was also with lesions demonstrated only on recon- were produced. These scans were co- used for in vitro assay of cytokine in the structed images. This supports the hypothe- registered with high resolution MRI. The same blood samples. This gave a measure of sis that cortical dysgenesis is an extensive and neighbouring gyri were each patient's total capacity to produce disorder. Block analysis has also shown delineated on coronal MRI sections. cytokines in response to a stimulus. structural disruption in patients with mental Asymmetry indices were compared with 10 Results-All patients underwent retardation and epilepsy with normal imag- age matched normal controls. In all patients with clipping of the aneurysm. No systemic ing and reconstruction. reduction of "C-flumazenil Vd was cytokine release was seen among this group, Volumetric MRI is important in the correlated with the side of hippocampal but the in vitro cytokine pattem during the investigation of epilepsy. Visual inspection sclerosis. On average 'IC-flumazenil Vd was postoperative period showed an upward of data alone is not adequate in cases where reduced in the smaller hippocampus by trend in two patients, a downward trend in no lesion is found. 20%. Changes of BZ/GABA-A receptor three, and violent oscillation of cytokine over [ABN] density in the other hippocampus lay within several days in the remaining three patients. normal limits. Interestingly, all patients showed a signifi- In conclusion, there is a reduction of cant decrease in their ability to secrete TNFa BZ/GABA-A receptor density in the after operation (P < 0 05) with a highly sig- hippocampus of patients with mesial nificant corresponding increase in the two IMPROVEMENT OF EPILEPSY AFTER over and above loss types ofsoluble TNF receptors TNFp55 and STEREOTACTIC RADIOSURGERY FOR of hippocampal volume. This may be of TNFp75 (P < 0-001). Similar findings were ARTERIOVENOUS MALFORMATIONS importance in planning the extent of not seen for either IL-la or IL-6. AA Kemeny, M Foroughi, L Walton, DMC resection in patients with MTLE. Conclusions-These preliminary findings Forster. National Centre for Stereotactic [ABN] highlight an alteration of the physiological Radiosurgery, Sheffield, UK production of TNFa and its receptors p55 and p75 after SAH. Objectives-Stereotactic radiosurgery (STRS) [SBNS] is primarily aimed at achieving thrombo- obliteration of arteriovenous malformations (AVMs). This study was carried out to eval- SEIZURE FUNCTIONAL OUTCOME ONE YEAR uate the degree of amelioration in seizure AFTER activity observed clinically as an additional GF Tuite, JH Cross, BG Neville, WFJ benefit after STRS. Harkness. Great Ormond Street Hospital THE EFFECT OF CSF PROTEIN Design-Telephone interview with patients for Sick Children, London, UK CONCENTRATION ON SUBSEQUENT SHUNT and analysis of the prospectively maintained COMPLICATIONS: A PROSPECTIVE STUDY departmental database. One year outcome data after hemispherec- HL Brydon, RD Hayward, WFJ Harkness, R Patients-From over 1500 patients 269 had tomy in 20 paediatric patients with severe Bayston, EJ Thompson, G Keir. Hospital for http://jnnp.bmj.com/ epilepsy; 149 were included in the analysis medically intractable seizures is presented. Sick Children, Institute of Child Health, and who fulfilled the following criteria: active Their average age at the time of surgery Institute ofNeurology, London, UK epilepsy before STRS; follow up longer was 4-7 years (range 0-5 to 16) with an than two years; angiographic control. equal number of boys and girls. All patients Objective-To assess the effect of the CSF Outcome measures-The patients classified had a preoperative hemiparesis but they protein concentration on subsequent shunt the severity and frequency of their seizures varied widely in their neurological disability. complications. subjectively into improved/no change/worse. Two patients presented with epilepsia par- Design-A prospective clinical study A category of "no fits" was assigned to tialis continua. Preoperative electro- comparing complications arising within two

those with no seizures for a year. encephalography and MRI localised the months of surgery to the peroperative CSF on September 29, 2021 by guest. Protected copyright. Results-Fifty four per cent of patients abnormality to a single hemisphere in all protein concentration. became fit free and 29% had subjective cases (9L, 1 1R). Subjects-Ninety five patients undergoing improvement. The size of the AVM nidus A functional hemispherectomy was the shunt surgery (new insertion or revision) and the radicality of the treatment plan preferred operation, but anatomical hemi- during a 15 month period. In total, 132 affected the change in epilepsy. More than spherectomy was performed in three cases. definitive shunt operations were performed. 50% of those whose AVMs were not obliter- Most had hemimegancephaly (five) or Outcome measures-Shunt infection, obstruc- ated reported worthwhile improvement in cortical dysplasia (six); the rest had a tion and overdrainage occurring within eight their epilepsy. porencephalic cyst (four), Rasmussen's weeks of surgery. Statistical analysis was per- Conclusion-Improvement after STRS in encephalitis (two), Sturge-Weber syndrome formed with the Mann-Whitney U test and epilepsy is a realistic expectation in most (two), and a large extradural cyst (one). All the X 2 test. patients with AVM. patients tolerated their operation well, with Results-There were 12 instances of shunt [SBNS] no complications in the immediate peri- obstruction, 10 shunt infections and three operative period. patients with significant overdrainage. The All of the patients have had at least a 75% total protein content of the infection (0-1 > reduction in seizure frequency and 16 are P > 0 05) and obstruction groups (0 5 > P > seizure free (follow up one to 3-5 years). 0-1) was not significantly different from "C-FLUMAZENIL PET AND VOLUMETRIC MRI Three patients (15%) have required the those without complications. The over- IN MESrAL TEMPORAL LOBE EPILEPSY placement of a ventriculoperitoneal shunt. drainage group had significantly less CSF MJ Koepp, MP Richardson, DJ Brooks, There was worsening of motor function in protein (0-5 > P > 0-02), but this group only DR Fish, JS Duncan. Institute of two patients. The striking feature in most consisted of three CSF specimens, all of Neurology, London, UK cases was the developmental improvement which had a normal protein content. that occurred after surgery. Conclusions-A raised CSF protein content "C-Flumazenil PET has been used to [SBNS] does not increase the risk of shunt 20222Proceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons

complications; there is no reason why maximum pairwise lod score obtained was inheritance. In this syndrome the mtDNA shunting should be delayed in patients with +4-2, multipoint analyses increased this to defect is presumed secondary to a nuclear J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from a high CSF protein content. + 5-8. The clinical studies show that pater- gene mutation. [SBNS] nally inherited cases develop the disease at a The first two British kindreds with this significantly lower age than their affected disorder and two sporadic cases are parent. This anticipation of the disease onset reported. All patients had adult onset with paternal transmission suggests that the progressive external ophthalmoplegia and NUMB-CLUMSY HAND IN HIGH CERVICAL disease is produced by a trinucleotide repeat muscle weakness, with muscle biopsy CORD LESIONS expansion mutation. Some other neuroge- appearances of a mitochondrial myopathy. V Kunanandam, K Ajay Kumar. Hull Royal netic diseases, including Huntington's dis- Southern blot analysis of total muscle DNA Infirmary, Hull, UK ease, have now been related to such digested with appropriate restriction mutations. enzymes shows a pattern consistent with Objective-To determine the pathogenic [ABN] multiple populations of mtDNA. mechanism for numb-clumsy hand. The cases exhibit, in addition, some Design-Correlation of clinical features, unusual clinical features. These include pig- radiological findings (MRI), and pathology mentary retinopathy and tremor; the last was in patients with numb-clumsy hand. NEUROPSYCHOLOGY AND SPECT IN levodopa responsive and associated with Subjects-30 patients with numb-clumsy DIAGNOSIS AND STAGING OF ALZHEIMER'S rigidity and micrographia in one family. One hands were identified in a series of 53 DISEASE family and both sporadic cases have a neu- patients operated for high cervical cord JDW Greene, JR Hodges. University of ropathy and nerve biopsy confirms the indi- lesions (above C3/4) during the period Cambridge, Cambridge, UK cation of an axonal neuropathy suggested 1993 to 1994 (September). from nerve conduction studies. Postmortem Assessment-Evidence of posterior column Given the advent of drug treatments for examination of one patient showed pro- involvement, cord signals in MRI and/or dementia of the Alzheimer type (DAT), it nounced nigral degeneration, which is of severe compression in CT myelogram, and is important to develop more accurate meth- interest in relation to the parkinsonism seen nature of pathological lesions. ods for diagnosis and staging. The role of and the evidence of mitochondrial chain dys- Results-Numb-clumsy hand was associated neuropsychology and SPECT imaging in function in Parkinson's disease. with no objective posterior column involve- diagnosis and staging DAT in 33 patients [ABN] ment (clinical or EMG/NC study), with with mild disease and 30 matched controls severe cord compression on CT myelo- were studied. Working, episodic, remote, gram/MRI and cord signal on MRI and with and semantic memory were assessed. Tc- pathological lesions: C3/4 spondylosis (19), HMPAO SPECT was analysed quantita- C1/2 rheumatoid subluxation (five), tively to obtain measures of regional cerebral OCULOMOTOR FUNCTION IN MOTOR NEURON tumours (two), and others (four). blood flow. DISEASE Conclusion-Numb-clumsy hand was associ- For diagnosis, the memory tests were S Shaunak, E O'Sullivan, R Orrell, R Lane, ated with high cervical (C1-C4) lesions caus- highly discriminatory, with a canonical cor- C Kennard. Charing Cross and Westminster ing severe anterior central compression of relation coefficient of 0 975. The most useful Medical School, London, UK cord and intrinsic cord signal on MRI, but test was delayed verbal recall; SPECT data minimal objective neurological signs. were, by contrast, of less use in predicting Although it is generally held that the oculo- Pathogenic mechanisms are probably related group membership; 33% ofpatients had nor- motor system is spared in motor neuron dis- to compression and disconnection of spino- mal SPECT scans. ease (MND), several recent oculographic cerebellar tracts. 1 2 For staging, stepwise regression analysis studies of reflexive saccades and smooth pur- using all memory tests could predict 79% of suit in MND have yielded conflicting results. 1 Chang MH, Liao KK, Cheung SC, et al. the variance in mini mental state examina- "Numb clumsy hands" and tactile agnosia Oculomotor function was therefore studied secondary to high cervical spondylotic tion scores. The measures most useful for in a range of paradigms, some of which are myelopathy: a clinical and physiological cor- staging were immediate, recognition, seman- considered to involve the frontal lobes in relation. Acta Neurol Scand 1992; 86:622-5. tic, and autobiographical memory. SPECT 2 Voskuhl RR, Hinton RC. Sensory impairment their execution. in the hands secondary to spondylotic com- data were again of less use in modelling Eye movements in 17 patients with MND pression of the cervical . Arch severity of disease; only 38% of the variance and 11 age matched normal controls were Neurol 1990;47:309-1 1. in MMSE scores could be predicted from recorded with the scleral search coil tech- http://jnnp.bmj.com/ [SBNS] SPECT data. nique. Smooth pursuit, fixation, and Neuropsychology has been shown to be reflexive, remembered, and antisaccade superior to SPECT both in diagnosis and paradigms were studied. Results were anal- staging DAT. Different memory tests are ysed for saccadic latency, peak velocity, gain, useful for diagnosis and staging: delayed ver- final eye position (FEP), antisaccade/ FAMILIAL NON-SPECIFIC DEMENTIA MAPS TO bal recall is the best discriminator, whereas CHROMOSOME 3 remembered saccade error rates, pursuit M immediate, recognition, autobiographical, peak velocity gain, and square wave jerk fre- JM Brown, T Ashworth, S Gydesen, are of most use in J St and semantic memory, quency. Reflexive saccades were unimpaired Rossor, Collinge. Mary's Hospital on September 29, 2021 by guest. Protected copyright. Medical School, London, UK staging severity. in patients with MND compared with con- [ABN] trols. Error rates for both antisaccades and A substantial minority ofpatients with prese- remembered saccades were elevated in the nile dementias lack distinctive inclusion MND group (antisaccades: MND 31-9 bodies, plaques or tangles on pathological (24-9)%, control 10-3 (9 6)%, P = 0 004; examination. Such dementias typically pre- MITOCHONDRIAL ENCEPHALOMYOPATHY remembered saccades: MND 30-8 (24 6)%, sent with evidence of frontal lobe dysfunc- WITH MULTIPLE MITOCHONDRIAL DNA control 12-4 (14-4)%, P = 0 02). Anti- tion and are probably the commonest cause DELETIONS: A REPORT OF TWO FAMILIES AND saccade and remembered saccade latencies of frontal lobe dementia. These dementias TWO SPORADIC CASES WITH UNUSUAL were also prolonged (antisaccades: MND have been termed non-specific dementias CLINICAL AND NEUROPATHOLOGICAL 376-9 (87 9) ms, control 303 5 (53 9) ms, (NSD). Half the reported cases ofNSD have FEATURES P= 0-01; remembered saccades: MND 318- 1 a positive family history ofdementia. Clinical RM Chalmers, M Brockington, RS Howard, (74-9) ms, control 258-3 (43 76) ms, P = and molecular genetic studies on the largest BRF Lecky, AE Harding. National Hospital 0-02). Square wave jerk frequency was published family with NSD have been con- for Neurology and Neurosurgery, London, increased in patients with MND (MND 32-9 ducted. There are 20 affected patients in UK (19-6) min, control 8-7 (4 7) min, P = three generations, the disease is inherited in 0-0007), but smooth pursuit showed no an autosomal dominant fashion. After run- Most diseases associated with abnormalities impairment. ning over 170 microsatellite probes and of human mitochondrial DNA (mtDNA) These findings of prolonged volitional excluding 75% of the genome the disease occur either sporadically or show maternal saccade latency and high distractability sug- locus has now been assigned to the peri- inheritance. Recently, a mitochondrial gest prefrontal impairment in MND, and are centromeric region of chromosome 3. myopathy associated with multiple deletions consistent with the results of recent neuro- Haplotype analysis shows a common region of mtDNA has been identified in pedigrees psychological, pathological, and PET studies. shared between all affected patients. The that show an autosomal dominant mode of [ABN] 203 Proceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons

accelerates pain amelioration after brachial employment difficulties were encountered A STUDY OF THE CAUSES OF LATE J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from plexus injury. Pain resolution usually by 12/33 and 5/33 respectively. Of those FUNCTIONAL DETERIORATION AFTER not preceded recovery of motor or sensory labelled "refractory epilepsy", 12 did PREVIOUS POLIOMYELITIS and 41 were rendered seizure D Kidd, RS Howard, E Chroni, FW function. have epilepsy [ABN] free or significantly improved by optimal use Heatley, A Williams, GT Spencer. St of "funny Thomas' Hospital, London, UK of AEDs or surgery. Diagnosis turns" was usually achieved on clinical except in patients with Many patients with previous poliomyelitis grounds alone syndromes" (PPS) in pseudoseizures on AEDs 58% of whom develop "postpolio A COMPREHENSIVE required investigation to refute the diagnosis which late functional deterioration follows a CLINICONEUROPHYSIOLOGICAL STUDY OF stability. The frequency WITH of epilepsy. period of relative IDIOPATHIC GENERALISED data show suboptimal diagnostic which PPS can be attributed to clearly These with ABSENCES IN ADULTS and management services for patients with defined causes remains uncertain. A review CP Panayiotopoulos, S Giannakodimos. St and newly referred patients with previous UK "funny turns" and epilepsy respectively of Thomas' Hospital, London, a strong case for expansion of spe- seen consecutively over a three provides poliomyelitis services and open access to multidisci- was undertaken. There were of age with cialist year period Eighty five patients over 16 years plinary epilepsy clinics. 156 patients (51 men, 105 women, mean typical absences (TAs) were studied. All had [ABN] age 52 (range 17-80) years) who had EEG confirmation, and 47% had additional developed acute paralytic poliomyelitis at a video-EEG documentation of TAs. mean age of 6 (0-5-42) years. Eighteen Prevalence of TAs was 22% among 410 patients had been ventilated, and 124 had adults with epilepsies and 63% among idio- had severe weakness during the acute pathic generalised epilepsies (IGE). REGIONAL CEREBRAL BLOOD FLOW DURING illness. After recovery, 22 were wheelchair The clinico-EEG manifestations of POSTERIOR SEIZURES: AN HMPAO SPECT bound and 31 mobile with severe disability. absences were syndrome related. 30 patients STUDY 140 patients (90%) developed symptoms of had juvenile myoclonic epilepsy; all had mild R Duncan, J Patterson, R Roberts, D functional deterioration at a mean time absences and myoclonic jerks on awakening, Hadley. Universities of Glasgow and interval of 42 (17-76) years after the and all but two had generalised tonic-clonic Dundee, UK paralytic illness. Functional deterioration seizures (GTCS). 10 patients had juvenile was associated with orthopaedic disorders absence epilepsy; all had severe absences and Ictal HMPAO SPECT was used to study five in 125 cases, neurological disorders in 16, all but one GTCS. 11 patients had eyelid patients whose clinical and surface video respiratory disorders in 12, and others in myoclonia with absences, a syndrome char- EEG data indicated seizure origin in the area 13. Treatment of bone and joint acterised by eyelid myoclonia with absences, of the temporoparieto-occipital junction abnormalities including surgery (26 cases) and photosensitivity. Seven patients had (TPOJ). Three patients had structural and provision of orthotic appliances (70 perioral myoclonia with absences, a syn- lesions in the area of the TPOJ. HMPAO cases) with physiotherapy, was associated drome characterised by TAs associated with injection was carried out from 30-120 sec- with functional improvement in 90% of ictal rhythmic perioral myoclonia. Four onds from clinical seizure onset. Acquisition those followed up. Other treatments patients had predominantly photo or pattern was carried out with a Strichmann 810 dedi- included weight loss, ventilatory support, induced TAs. Twelve of the remaining cated head imager. and pain relief. In this series progressive patients, showed remarkably similar clinico- Interictal SPECT showed perfusion postpolio muscular atrophy was not noted. EEG features, which were not fortuitous. A deficits in keeping with anatomical lesions in Functional deterioration was common and new syndrome characterised by "phantom three patients, hemispheric hypoperfusion associated with orthopaedic, neurological, absences" and GTCS, is proposed. The on the side of electrical seizure origin in a respiratory, and general medical factors name "phantom absences" has been coined fourth, and was normal in a fifth. Ictal which are potentially treatable. to denote TAs that are so mild that they are images showed hyperperfusion in the area of [ABN] inconspicuous to the patient and invisible to the TPOJ in all five patients. This hyper- the observer. Absences in adults constitute a perfusion extended anteriorly into the ipsi- diagnostic challenge to physicians and have lateral lateral temporal cortex and pole, with important implications in the syndromic hyperperfusion of a lesser degree in the ipsi- classification and management ofpatients. lateral mesial temporal cortex, and ipsilateral

[ABN] hyperperfusion of the basal ganglia in two http://jnnp.bmj.com/ EFFECT OF SURGICAL REPAIR ON PAIN RELIEF patients. In two patients there was ictal AFTER BRACHIAL PLEXUS INJURY IN ADULTS hypoperfusion of the rest of the ipsilateral JS Berman, M Taggart, P Anand, R Birch. as a change in asym- College hemisphere. Measured Royal London Hospital Medical metry index with respect to the interictal and Royal National Orthopaedic Hospital, DIAGNOSIS AND MANAGEMENT OF EPILEPSY CLINIC image, ictal hyperperfusion was most pro- London, UK AND "FUNNY TURNS" IN A SPECIALIST in five cases. D Smith, B Dafalla, DW Chadwick. Walton nounced at the TPOJ all It is concluded that ictal SPECT shows The characteristic pain after root avulsion Centre for Neurology and Neurosurgery, UK distinct patterns of perfusion in TPOJ

Liverpool, on September 29, 2021 by guest. Protected copyright. begins within weeks of brachial plexus seizures, and can distinguish them from injury in adults, and persists for many years, are often mesial temporal seizures. still being severe in 25% of patients after Patients with "funny turns" [ABN] four years. Surgical treatment of avulsion referred to neurologists with a previous or injuries led to preliminary observations that possible diagnosis of epilepsy. This study the surgery accelerated resolution of pain. was designed to assess the frequency of fac- This study examined the effect of surgery- tors contributing to and the consequences of EYELID MYOCLONIA WITH ABSENCES: A nerve grafting and transfer-on the duration an erroneous diagnosis of epilepsy, the out- ADULTS root avulsion pain. 487 come of patients referred with "refractory CLINICAL AND VIDEO-EEG STUDY IN and severity of St were studied prospectively over 10 seizures", and the relative roles of clinical S Giannakodimos, CP Panayiotopoulos. patients London, UK years with preganglionic root avulsions acumen and investigations in establishing Thomas' Hospital, or in combination with postganglionic the cause of "funny turns". alone is an ruptures or lesions in continuity (LIC). A retrospective analysis ofthe case records Eyelid myoclonia with absences (EMA) with pure ruptures of 324 patients was conducted. The sample idiopathic generalised epilepsy (IGE) syn- They were compared myoclonia and pure LIC. In the avulsion group, pain was divided into those exposed to antiepilep- drome, manifested by eyelid = and photosen- was reduced substantially at an average time tic drugs (AEDs) (n 184), of whom 92 associated with brief absences EEG shows of 175 days after operation, in comparison were said to have refractory seizures, and sitivity. The ictal generalised = wave with 68 days for the pure rupture group and those who had not received treatment (n 3-6 Hz spike/polyspike-slow activity, 58 days for the pure LIC group. Of the 39 140). mainly on eye closure. avulsion patients, only three reported The overall misdiagnosis rate was 26-1% This is the first clinical and video-EEG pure Their prevalence no improvement in their pain 20 months (46/184) with inadequate history taking and study of adults with EMA. was 2-7% among after surgery, the median time for misinterpretation of EEG equally respon- in the adult epilepsy clinic being 120 days. These sible. 19/40 reported side effects from AEDs 410 epileptic patients, 8-1% among patients improvement with findings support the view that nerve repair while unnecessary driving restrictions and with IGE, and 12-9% among patients 204 Proceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons IGE with absences. There were 11 patients (including the cases to be are AN ECONOMIC with presented here) ARGUMENT IN FAVOUR OF J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from EMA, all female, with a mean age of reported in the literature.' Five cases treated 30 9 years. Reported onset of absences ENDOSCOPIC THIRD AS A was surgically by one of the authors (TTK) are TREATMENT FOR OBSTRUCTIVE at a mean age of 7-8 years. Eyelid myoclonia reviewed. Three of these have with previously HYDROCEPHALUS absences was confirmed with video- been reported. Two new cases are EEG in 10 presented HS Ching, P Barlow. Southern General patients. The seizures consisted and term follow on the earlier cases of eyelid long up Hospital, Glasgow, UK myoclonia alone or preceding brief is reported. Intraoperatively these tumours (3-6s) absences. Ictal clinical and EEG often have a only small area of attachment to Objectives-To examine the health resource manifestations occurred mainly after eye the floor ofthe third ventricle and it is closure and were tempt- implications of performing endoscopic third inhibited by total darkness. ing to attempt to remove the tumour com- Only five patients showed photoparoxysmal ventriculostomy as an alternative to CSF pletely. In all cases the surgery was shunting in appropriate patients. responses as adults, although all had a pre- uneventful, yet three of the vious postoperatively Design-A retrospective study of case clinical and/or EEG documentation of five cases had significant morbidity, includ- records and photosensitivity. None of the patients radiographs of patients shunted had ing memory deficits, diabetes insipidus, and de novo at the Institute of self induced seizures. Infrequent generalised abnormalities of central Neurological temperature control. Sciences, Glasgow for the two year period tonic clonic seizures occurred in 10 patients, The place of in the treatment and were radiotherapy 1990-1. precipitated mainly by lights, sleep of these tumours is discussed.2 It is con- Outcome deprivation, fatigue, and menstruation. Mild measures-The number of repeat cluded that attempts at total surgical excision operations and extra in myoclonic jerks of the upper limbs occurred should be days hospital approached with caution as signif- required due to shunt com- in six patients. icant complications long term morbidity can follow such pared with the predicted extra operations Eyelid monoclonia with absences is often surgery and that the better resistant perhaps alter- and days in hospital for patients suitable for to medication and persists into native is subtotal removal followed by radio- endoscopic third adult life. therapy. ventriculostomy. [ABN] Results-One hundred and fifty new shunts 1 Iwasaki K, Kondo A, Takahashi JB, Yamanobe were inserted in the two years. Of these, 23 K. Intraventricular craniopharyngioma: report patients (15%) were judged suitable for of two cases and review of the literature. third Neurol Surg endoscopic ventriculostomy as an alter- 1992;38:294-301. native to CSF out of 23 2 Fischer EG, Welch K, Shillito J, et al. shunting. Eight Craniopharyngiomas in children. Long term patients required a total of 29 repeat opera- MEDULLOBLASTOMA: HAS THERE BEEN ANY effects of conservative surgical procedures tions and an extra 230 days in hospital due IMPROVEMENT IN FIVE YEAR SURVIVAL OVER combined with radiation therapy. Neurosurg to shunt an 1990;73:534-40. J complications. Assuming 80% THE PAST 20 YEARS? success (shunt free) rate for endoscopic third KM David, ATH Casey, RD Hayward, WFJ [SBNS] ventriculostomy, nine operations and 74 bed Harkness. Great Ormond Street Hospital for days per year could be saved by using this Sick Children, London, UK technique. Conclusions-In units undertaking a large The large number of cases of number of CSF shunt medulloblas- BLOOD TRANSFUSION AND SURVIVAL insertions, investment toma seen at Great Ormond Street allows a FROM in neuroendoscopic equipment has the unique INTRINSIC SUPRATENTORIAL HIGH GRADE opportunity to view longitudinally GLIOMAS potential to release significant resources for the effect that modem other uses. methods of investiga- JD Palmer, RM Pickering, DW Ellison, DA tion and treatment have on the prognosis. A [SBNS] cohort of Schilder, F Iannotti. Wessex Neurological cases treated over a 10 year period Centre, UK for whom a five year follow up was available Southampton, (1980-9) have been analysed. Eighty chil- dren were admitted Objectives-To measure the influence of during this time, of blood transfusion on VINCRISTINE AND CARBOPLATIN TREATMENT whom 75 went on to receive survival in patients with radiotherapy intracranial gliomas. FOR PROGRESSIVE LOW GRADE and/or chemotherapy (operative mortality ASTROCYTOMA OF 5%). Forty patients Design-Prospective data were examined BRAIN were alive and 38 disease from 175 patients who had entered EORTC MS Ashraf, P May, H McDowell. Royal free at five years after diagnosis-90% of studies. Transfusion Liverpool Children's those with favourable prognostic features glioma data were col- Hospital, Liverpool, (no lected retrospectively from case records. UK

perioperative spinal http://jnnp.bmj.com/ metastases, radical Patients-All were over 16 surgery) and 10% of those without. patients years old, steroids had been stopped by the 10th post- Five children (age range 4 months to 8 years Comparison with an earlier cohort of cases and 4 with from this operative day, survival was expected for months), progressive low grade institution (1965-74) gives the fol- more I and lowing information.' than eight weeks, and all had similar astrocytoma (grades II) were treated courses of with weekly vincristine and car- Modem methods of radiotherapy. (1-5 mg/M2) investigation and Outcome measures-Survival was established boplatin (500 mg/M2), every three weeks. treatment have clearly improved the outlook Response in terms of either for patients with by telephone interview of the next of kin. reduction in medulloblastoma-but the Results-Hazard ratios of tumour size or progression of was greatest advance remains the factors affecting disease, changes to survival were calculated a series of Cox achieved in all cases. The follow up is by period on September 29, 2021 by guest. Protected copyright. radiotherapy regimes introduced in the models. 20 to 10 months seven 1 960s. It is suggested that regression Variables significantly (mean months). there is unlikely to were Initial response combination be any further dramatic affecting survival age (P = 0-0016), suggests improvement until = 0 and chemotherapy with vincristine and carbo- radical changes can be made in either pathology (P 0075), tumour loca- the tion (P = 0 0075). After platin is effective in holding the disease and chemotherapy regimes (for example, high adjustment, Karnovsky index, tumour resection, or delaying the use of radiotherapy particularly dose chemotherapy with bone marrow res- in younger children. cue) or in adjunctive treatment had no significant immunological or gene mediated effect on therapies. survival. Blood transfusion was a [SBNS] significant protective factor for glioblastoma 1 McIntosh N. Medulloblastoma-a changing with a hazard ratio of 0-38 (95% confidence prognosis? Arch Dis Child 1979;54:200-3. intervals 0-22-0-67), had no effect for [SBNS] anaplastic astrocytoma 1-70 (0-58-4-94), EVALUATION OF MICROENVIRONMENTAL and was a risk factor for other pathologies HYPOXIA OF BRAIN TUMOURS: MODIFYING 4-56 (0-97-21-35). The different risk FACTORS between these groups is highly statistically GS Cruickshank, R Duckworth. Institute of significant (P = 0 0003). Neurological Sciences, Glasgow, UK Conclusions-In this group of patients trans- MORBIDITY OF SURGERY FOR fusion improved survival from glioblastoma. Objectives-To assess the of INTRAVENTRICULAR CRANIOPHARYNGIOMAS impact PaCO2 Suppression of cell mediated immunity is and blood pressure on oxygenation of MJ Davies, TT King. Royal London known to occur in patients with glioblastoma Hospital, London, UK tumours at operation measured similar to that seen in AIDS; further modula- by dynamic stepping micropolarography and tion by transfusion may play a part in microlaser Doppler. Craniopharyngiomas situated wholly within improving survival. stratification of the third ventricle are rare and Design-The patients based only 31 cases [SBNS] on the oxygen profile of their intracranial Proceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons 205 tumours may allow assessment ofthe impact Five patients (8%) developed CSF leak that tion (WBRT). Twenty nine patients received of chemical modifiers on radiation sensi- was treated operatively in four and by spinal SRT as primary treatment. Twenty three J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from tivity.' Characterisation of factors that influ- drainage in one. All patients with small patients were treated for recurrence after ence intracranial tumour hypoxia, is therefore tumours (<10 mm) had good recovery of surgery and WBRT and after WBRT alone. important. The question is whether the effect facial nerve function (those house grade IM); The SRT dose was 20 Gy in two fractions in of altered arterial PaCO2 and blood pressure 27% of patients with medium sized tumours most patients prescribed to the 90% isodose. on cerebral blood flow and brain oxygena- (11-25 mm), and 33% ofpatients with large The target volume encompassed the enhanc- tion devalue the significant level of hypoxia tumours (>26 mm) had a facial palsy. Ten ing tumour and a 2 mm margin. recorded in these tumours at operation. patients had some preserved hearing on the Local progression free survival (PFS) was Subjects-Twelve anaesthetised patients affected side at the time oftheir presentation. 61% at one year (27 months median PFS) were ventilated at an FiO2 of 0-25-0-3, and Postoperatively four were deaf, three had and the median survival was seven months. end tidal CO2 and PaO2 and PaCO2 were retained non-useful hearing, and three had Most patients had a functional improvement monitored. The Eppendorf PO2 histograph retained useful hearing. as measured by the Barthel index. Neuro- probe was used to measure PO2 profiles in Conclusion-The results compare favourably logical deterioration was noted in two tumours in these patients. Concurrently, with published data. Acoustic neuroma patients within one month of SRT. microlaser Doppler flow measurements were surgery can be performed safely in a small The results of SRT in patients with soli- made from brain, brain around tumour and neurosurgical unit. tary brain metastases in terms of local con- tumour. [SBNS] trol and survival seem equivalent to Outcome measures and results-Alteration in neurosurgical excision; SRT is a useful pal- PO2 indices and variations in erythrocyte flux liative treatment-it is well tolerated, non- were assessed in response to a fixed change COMPLICATIONS OF SKULL BASE GLOMUS invasive, and outpatient based and is in pCO2 or mean arterial blood pressure; for SURGERY: 10 YEARS OF OTONEUROSURGICAL particularly of value in patients whose sur- reproducibility and reversibility of effect, EXPERIENCE vival is determined by the systemic course of altering the PaCO2 from 37 mm Hg to 31 PC Whitfield, DA Moffat, DG Hardy. disease. The role of WBRT in combination mm Hg and back resulted in a reversible fall Addenbrooke's Hospital, Cambridge, UK with SRT remains to be defined. in median tumour PO2 of 4-2 (0-4) mm Hg, [SBNS] and in PO2 levels of less than 2-5 mm Hg Objectives-To review and evaluate the man- from 26-3% to 46-5%. Changes in micro- agement and outcome in patients under- regional blood flow (erythrocyte flux) going surgery for skull base glomus tumours. recorded in the tumour, peritumoural area, Design-Retrospective analysis of all patients INCIDENCE OF BRAIN METASTASES AND and distant brain illustrate a pronounced im- with large skull base glomus tumours treated RELATION TO SYSTEMIC TUMOUR SITES pairment ofreactivity in the peritumoural area. by combined otoneurosurgery during a 10 R Grant, D Collie. Westem General Conclusions-Altering PaCO2 has a repro- year period. Hospital, Edinburgh, UK ducible impact on tumour PO2 that may be Subjects-Fifteen patients with glomus jugu- due to direct or shunting effects on cerebral lare tumours, three patients with glomus There are no good epidemiological studies of circulation. The tumour ApO2/APaCO2 seen vagale tumours, and one patient with a glo- brain metastases in the United Kingdom. is less than that for normal brain and does mus hypoglossus. The incidence of brain metastases in the not overlap with tumour P02 data from Outcome measures-(a) Performance of addi- Lothian region, the frequency of single extracranial tumours, suggesting that tional procedures to treat post-operative metastasis and metastases of unknown hypoxia in intracranial tumours may be an complications. (b) Functional outcome on a primary, and the clinical relations with important factor in their resistance to treat- disability scale at long term follow up. primary site were examined. ment. Multichannel microlaser Doppler Results-An infra/transtemporal approach Incident cases of intracranial tumour techniques used in conjunction with tissue (n = 15) for Fisch grade C and D tumours, (1989 + 1990) in the Lothian region were oxygen measurement allows the factors con- or a tympanomastoid approach (n = 4) for identified by examining all CT reports from trolling tumour hypoxia to be evaluated and Fisch grade B tumours was performed. The the three scanners that cover SE Scotland, possibly manipulated therapeutically. facial nerve was transposed in 12 patients supplemented by multiple cross referenced 1 Rampling R, Cruikshank GS, Lewis AP, et al. (63%). Cranial nerves were necessarily tran- cancer registration and neurological data- Direct measurement of PO2 distribution and sected in eight cases (42%); in six of these bases. Case records were retrieved and bioreductive enzymnes in human malignant preoperative signs of cranial nerve involve- patient data analysed. brain tumours. J Radiat Oncol Biol Phys One hundred and eighty four people with 1994;29:427-3 1. ment were evident. Ten patients (53%) http://jnnp.bmj.com/ [SBNS] underwent surgery to treat complications; brain metastases were identified (incidence: teflon injection ofvocal cord (n = 5), tarsor- 12-6/100 000 pop/year). Brain metastases rhaphy (n = 3), wound re-exploration (n = accounted for 46-5% of all intracranial 3), surgery for facial palsy (n = 2), thyro- tumours; 37% of brain metastases were single; 34% of patients had no history of SAFE NEUROSURGERY? AN AUDIT OF plasty (n = 2), ventriculoperitoneal shunt = = systemic malignancy at neurological presen- ACOUSTIC NEUROMA SURGERY IN A SMALL (n 1), and squint surgery (n 1). At long term follow up (mean 3-24 years) 68% of tation and no systemic site was ever identi- NEUROSURGICAL UNIT fied in 15% (27). Lung (104), breast (22), PJ Kane, B Gendeh, M Hawthorne, FP patients had an excellent functional outcome, 11% a good outcome, and 21% a fair outcome. melanoma (14), GI tract (seven) and renal on September 29, 2021 by guest. Protected copyright. Nath. Middlesbrough General Hospital and (four) were the most commonly identified North Riding Infirmary, Middlesbrough, UK Conclusions-Most patients with a pre- existing lower cranial nerve palsy adapt to primary sites. All patients with brain meta- stases from breast and GI tumours and 13 Aims-To audit the results of acoustic neu- intraoperative cranial nerve transection well. A multidisciplinary team is re- with melanoma, had a history of malignancy roma surgery in a neurosurgical unit with extremely before neurological presentation. This pro- small catchment population and compare quired to achieve optimal results in patients with these complex skull base tumours. vides useful clinical information for investi- with published standards of outcome. gating patients with brain metastases. Design-Retrospective study of 64 patients [SBNS] [ABN] with acoustic neuroma operated on by a single neurosurgeon and neurootologist in the period 1986-94. All patients were oper- RADIOSURGERY/STEREOTACTIC ated on by the suboccipital retromastoid RADIOTHERAPY FOR SOLITARY BRAIN EFFECTS OF ARTERIOVENOUS approach. Peroperative facial nerve monitor- METASTASES: A NON-INVASIVE ALTERNATIVE MALFORMATIONS ON CORTICAL ing became available in 1992. Specific audit TO SURGERY ORGANISATION: IMPLICATIONS FOR SAFETY points included referral pattern; mortality; M Brada, RE Wurm, AP Warrington, RW OF RESECTION postoperative CSF leak; postoperative facial Laing, S Sardell, F Hines, JD Graham. Royal GM McKhann II, JM Eskridge, DL nerve function at six months; and hearing Marsden Hospital and Institute of Cancer Silbergeld, AB Harris, GA Ojemann, HR preservation. Research, London, UK Winn. University of Washington, Seattle, Results-Sixty nine per cent of patients were USA referred by otologists, the remainder from Fifty two patients with 57 solitary brain neurologists, general physicians, or general metastases (one to two lesions) were treated Objectives-Preoperative and intraoperative practitioners. Two patients (3%) died after with stereotactic radiotherapy/radiosurgery functional mapping can be used to identify surgery. Both had large tumours (>5-5 cm). (SRT) with or without whole brain irradia- the Rolandic and language cortex in patients Neurological Surgeons ofthe Association ofBritish Neurologists and the Society ofBritish 206 Proceedings OF HEAD INJURY to - 2%. CDE is an appreciable improve- IDMPLEMENTATION

(AVMs) J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from with arteriovenous malformations technology. Further G3UIDELINES: WHERE ARE WE AFTER TWO cortex. Using these techniques ment in colour TCD AUDIT? near essential is required to see whether c-YCLES OF A REGIONAL excision of AVMs, the evaluation f J Addison, P to facilitate safe could be used as an D Pickard, S Jackson, impact that these lesions have on cortical aneurysm "pulsatility" HK Richards. University of indicator of impending rupture. SSmielewski, organisation has been studied. CCambridge, Cambridge, UK Design-Utilising preoperative super- [ABN] selective amobarbital (WADA) testing and Guidelines for the management of head intraoperative stimulation mapping, the injuries have been widely adopted but may topographical organisation of functional cor- not be followed in practice.' A criterion investigated. dis- tex in patients with AVMs was SERUM LIPOPROTEIN (A) CONCENTRATIONS based audit was conducted in the eight Patients-Thirty eight consecutive patients CEREBROVASCULAR ANEURYSM t general hospitals of East Anglia to assess cortex IN FAMILIAL trict with AVMs near essential functional C Bolger, JP Phillips. Beaumont Hospital, tthe quality of documentation, investigation, were evaluated. and Walton Hospital, care of patients with head injury. the Dublin, Eire and Results-Individual variability exists in Liverpool, UK First cycle (1 October 1992-31 March topographical location of sensory and motor 1993)-4789 patients' notes (1458 admit- cortex and of sites essential for language investigate serum lipoprotein ted) were extracted and those that did not Most Objective-To function in patients with AVMs. (a) (Lp(a)) concentrations in patients with meet the guidelines were peer reviewed patients with AVMs reorganise functional familial cerebrovascular saccular aneurysms. anonymously in each hospital. Documen- cortex into atypical topographical configura- Design-Case identification from family his- tation was poor (no record of GCS 52%, BP tions by displacing Rolandic and/or language admitted with proved sub- 37%, or skull radiograph 71%). Failure at have tory of patients cortex. Two patients were found to arachnoid haemorrhage secondary to peer review for skull radiograph, admission motor cortex located within AVMs. saccular cerebrovascular aneurysm. Patients and/or CT criteria was 12% (range 8-15% Conclusions-There is variability and plastic- more first degree relatives with between hospitals): under investigation patients with two or ity ofhuman cortical organisation in proved aneurysmal subarachnoid haemor- 10%, over investigation 2-4%. with AVMs. Most AVMs displace functional rhage were recruited. They were screened Second cycle (1 December 1993-28 February cortex and can potentially be safely resected. angiography and fasting serum 1994)-a structured head injury proforma testing with cerebral of Preoperative superselective WADA Lp(a) was measured. was introduced in October 1993.2 A total and/or intraoperative stimulation mapping Patients-To date 15 patients (siblings) from 2718 notes (786 admissions) were audited are recommended for lesions near eloquent families have been identified (eight and peer reviewed. Documentation unre- two < cortex to determine those that may be males and seven females, mean age 37 9 improved by 37% to 89% (P 0-001). sectable. (10-2) years). Overall failure for skull radiograph, admis- [SBNS] Outcome measures-Serum Lp(a) concentra- sion, and/or CT criteria was not significantly relation to the presence or absence of reduced (13%; range 10%-19%). Over- tions in to saccular aneurysm. investigation was reduced from 2-4% Results-Four patients died from subarach- 11% (P < 0-001) but underinvestigation before enrolment into the increased from 10% to 12% (P < 0-001). OF INTRACRANIAL ANEURYSMS noid haemorrhage "DYNAMICS" one patient refused investigation. Of Conclusion-Improvement in documentation DEMONSTRATED BY COLOUR TRANSCRANIAL study; the remaining 10 patients: seven had asymp- and provision of a "structured" proforma DOPPLER ULTRASOUND identified on cerebral not yet improved the implementation of Wardlaw, J Cannon. University of tomatic aneurysms have JM angiogram. Six of these patients had serum guidelines agreed by regional consensus. Edinburgh, Edinburgh, UK above normal (> 30 Lp(a) concentrations 1 Suggestions from a group of neurosurgeons. mg/dl; mean (SD) 54-7 (11-8) mg/dl). Two for initial management after head It is possible to demonstrate intracranial and Guidelines using directionally patients had normal cerebral angiograms injury. BMJ 1984;288:983-5. aneurysms and AVMs was normal (10 2 Wallace SA, Bennett J, Perez-Avila CA, Gullan colour transcranial the Lp(a) in both patients injuries in the accident and sensitive conventional In the remaining patient RW, et al. Head Doppler ultrasound (CDI), which has been and 25 mg/dl). emergency department: are we using develop- (with an Lp(a) concentration of 64 mg/dl) resources effectively? Journal of Accident and available for several years. A recent Medicine 1994;11:25-31. Doppler technology "colour angiography was incomplete. Emergency ment of colour Conclusions-There is evidence of an associa- [SBNS] Doppler energy" (CDE) or "power of more sensitive to tion between raised serum concentration Doppler" is considerably aneurysms. http://jnnp.bmj.com/ blood. This technique has been Lp(a) and cerebral flowing [SBNS] applied to the detection of intracranial ASSESSMENT OF THE GtIASGOW HEAD INJURY aneurysms in patients presenting with sub- OUTCOME PREDICTION PROGRAM arachnoid haemorrhage (SAH). JJ Nissen, PA Jones, DF Signorini, JD As many patients as possible presenting to Miller. Westem General Hospital, with our regional neurosurgical service SPECTROSCOPIC Edinburgh, UK or LP proved) were A MAGNETIC RESONANCE recent SAH (either CT RELATED TO assessed by colour , STUDY OF PARKINSONISM Objectives-To compare the predicted v to BOXING the using the temporal bone windows, blind Barker, actual outcome after head injury using on September 29, 2021 by guest. Protected copyright. although not to the AJ Lees, CA Davie, Z Pirtosek, GJ program,' the result of angiography DA Miller. Institute of Glasgow head injury prediction on CT. DP Kingsley, how often predictions were distribution ofblood London, UK and to determine Thirty nine patients have been examined Neurology, available. so far (mean age 50), the first nine with CDI spectroscopy, Design-Retrospective data (1989-94) from 30 patients with CDE. Proton magnetic resonance with severe head injury, and the last the lentiform nucleus, was car- 325 patients were recognised as an area of localised to requiring evacuation and/or Aneurysms in three exprofessional boxers who haematoma where no normal artery was expected, ried out coma >six hours, with Glasgow outcome colour developed a parkinsonian syndrome, six fol- and on CDE by apparently greater "pulsatil- disease scale (GOS) scores at six to 24 months cardiac patients with idiopathic Parkinson's the proba- ity" than normal arteries during the controls. The three low up. The program calculates reduced the proportion off and in six age matched state (GOS 1 or cycle. CDE showed a pronounced reduction in bilities of death/vegetative with a poor bone window (10% with boxers all severe disability (GOS 3), or moderate patients the absolute concentration ofN-acetyl aspar- 2), CDE and 33% with CDI). CDE detected 26 with disability/good recovery (GOS 4 or 5) on angiograpil- tate (NAA), compared with the patients before evacua- of 29 aneurysms demonstrated and the control group. admission after severe injury, Parkinson's disease ~~~~~~ cally (90%), whereas CDI only detectedi l~~~~~~~~ tion of acute haematoma, or at 24 hours, t This reduction is likely to reflect neuronal those in coma. 55%. In five patients who underwen and globus three days, or seven days for to see thee loss occurring in the putamen were possible in 77%, aneurysm coiling it was possible the hypothesis that Results-Predictions inserted into the aneurysm and tcD pallidus, and supports 19%, 34%, and 53% of "suitable" coil being extra pyramidal syndrome, which occurs 96%, measure the residual neck with CDE. Th(e the patients at admission, operation, 24 hours, sectiona in exboxers, is distinct from idiopathic mean change in aneurysm cross findings will be three days, and seven days respectively. was 49OX10 Parkinson's disease. These meant that predictions area between systole and diastole on post trau- Paralysis/ventilation cross sectional area of arn related to the existing literature ranging from 15% of (± 28%) and in the Parkinson's syndrome. were often unavailable, adjacent normal artery was 18% (± 20%) matic those "suitable" at admission, to 76% at 24 mean of difference -30-8%, 99% CI -6 0 [ABN] Proceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons 207 hours. Seventy four per cent of predictions over long periods, an excellent frequency with phosphorus magnetic resonance spec- were correct (highest probability), 16% response, and a low measurement error. The troscopy, and vasospasm with angiography J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from pessimistic, and 10% optimistic. Of those three microtransducers, coded A, B, and C, and transcranial Doppler (TCD), in a 62 patients whose eventual outcome was either were examined in the pressure flow test rig year old woman with subarachnoid haemor- GOS 4/5 or GOS 1, 84% were correct; for designed for assessment of hydrocephalus rhage from a right middle cerebral artery those GOS 3, only 12% were correctly pre- shunts. All three microtransducers complied (MCA) aneurysm. dicted. Of 64 pessimistic predictions, 48% with their manufacturer's specifications, Results-On day 3, she was well. Spectro- were predicted to die or be vegetative, but giving high quality readings under test condi- scopy on the right showed acidosis (pH actually achieved a moderate or good outcome. tions. Some differences were noted, how- 6-35) suggesting anaerobic metabolism, but Conclusion-The Glasgow head injury pre- ever, 24 hour zero drifts were lower than 0-8 was normal contralaterally (pH 7-01, control diction program can be a useful adjunct in mm Hg and C had the lowest. The tempera- mean 7-02 (SD 0-01). On day 6, she became clinical decision making in some head ture drift was very low in B and C but A had drowsy and confused. Spectroscopy showed injured patients. a drift of 0-27 mm Hg/°C; A had a static decreased high energy phosphates, suggest- error of less than 0-3 mm Hg, B of less than ing cell death. SPECT showed pronounced 1 Murray LS, Teasdale GM, Murray GD, et al. Does prediction of outcome alter patient 2 mm Hg, and C of less than 8 mm Hg. The hypoperfusion of the right frontal lobe (CBF management? Lancet 1993;341:1487-91. limits of the frequency response were not relative to cerebellum 0-58 (SD 0-03) right, [SBNS] detectable in A and B (band width wider 0-87 (SD 0 02) left). There was no TCD than 50 Hz), whereas C had a band width evidence of vasospasm (right MCA velocity limited to 18 Hz. 43 (SD 1) cm/s, left 35 (SD 2, controls 58 Conclusion-Transducer B scored the best (SD 2). Angiography (day 7) did not show OUTCOME IN CHILDREN WITH SEVERE HEAD overall but all three behave satisfactorily on vasospasm. INJURY: IMPORTANCE OF CEREBRAL bench testing. The use of each transducer in Aneurysm clipping was complicated by PERFUSION PRESSURE everyday clinical practice requires evaluation. rebleeding, and postoperatively by vaso- PJ Kane, IR Chambers, A Davidson, L [SBNS] spasm (MCA velocity >120 cm/s). A recov- Treadwell, NV Todd, A Jenkins, PJ ery SPECT (day 25) showed hyperaemia Crawford, RP Sengupta, AD Mendelow. (0-98 (SD 0-03) in the right frontal lobe. Newcastle General Hospital, Newcastle- Conclusions-Angiography and TCD only upon-Tyne, UK A PROSPECTIVE COMPARISON OF SUBDURAL examine large and medium sized arteries. FIBREOPTIC AND FLUID FILLED SINGLE The SPECT finding of hypoperfusion, later Objectives-To determine what factors may LUMEN BOLT PRESSURE TRANSDUCERS IN reversing, without angiographic vasospasm, be important in predicting outcome after VENTILATED PATIENTS suggests that there may be changes in small severe head injury in children. S Bavetta, JC Sutcliffe, P Chumas, OCE vessels beyond angiographic resolution. The Design-Retrospective study of 50 children Sparrow, PJ Hamlyn. The Royal London existence of such changes could explain (age < 16) with a severe head injury Hospital and St Bartholomew's Hospital, discrepancies between ischaemia and angio- (Glasgow coma score on admission to the London, UK graphic vasospasm complicating subarach- neurosurgical unit < 8) who required noid haemorrhage. intracranial pressure (ICP) monitoring as a A clinical comparison was made between [SBNS] routine part of their management. Mean subdural fibreoptic and fluid filled subdural arterial blood pressure, ICP, and cerebral bolt pressure transducers in ventilated perfusion pressure (CPP) were measured patients with head injury. Twelve patients continuously. Outcome was determined at had both devices inserted in neighbouring sites. In two were FAMILIAL HEMIPLEGIC MIGRAINE AND THE six months with the Glasgow outcome scale. patients there obvious AGE RELATED SPECTRUM OF CEREBRAL Independent outcome is defined as those technical problems with the fibreoptic sys- tem and the results were therefore excluded AUTOSOMAL DOMINANT ARTERIOPATHY children who made a good recovery or were LINKED TO CHROMOSOME 19 moderately disabled. from further analysis. In the remaining 10 M Hutchinson, J O'Riordan, M Javed, E Results-Nine patients died. In the remain- patients 2167 pairs of simultaneous record- Quin, T Willcox, N Parfrey, D Macerlaine, der the outcome scores at six months after ings were made for up to nine days and the TG Nagy, E Toumier-Lasserve. St Vincent's injury were: seven severely disabled; 12 degree of correspondence studied. A quarter Hospital, Dublin, Eire, and Inserm U25, moderately disabled; 22 good recoveries. of paired readings differed by more than Paris, France Glasgow coma score on admission, injury 5 mm Hg. Neither device showed a predis- http://jnnp.bmj.com/ severity score, and average maximum ICP position to reading either higher or lower Cerebral autosomal dominant arteriopathy were not reliable predictors of outcome. As than the other, but in individual patients with subcortical infarcts and leucoence- with a previous study,' higher values of there was often a consistent bias. The pro- phalopathy (CADASIL) is a recently average minimum CPP were associated with portion of closely corresponding readings described familial cerebrovascular disorder better outcome: good recovery 58-5 (3 9) tended to diminish after four days, and when which has been shown to map to chromo- mm Hg; moderately disabled 58-4 (2 5) mm either device registered a very high or low some 19ql2. Familial hemiplegic migraine Hg; severely disabled 44-5 (4-3) mm Hg; value. Clinically relevant episodes, defined has also been shown to be linked to chromo- dead 45-3 (16-2) mm Hg. Average mini- as a reading of over 20 mm Hg by only one some 19 close to the CADASIL locus. The mum CPP values >50 mm Hg were associ- ofthe two monitors, occurred 221 times with CADASIL phenotype is defined, as a mini- on September 29, 2021 by guest. Protected copyright. ated with a significantly greater chance of both systems equally likely to produce higher mum, by brain MRI abnormalities in an at independent outcome (X2 P < 0.05). readings. Differences in treatment would be risk family member. By this criterion 10 of Conclusion-This study points to the impor- likelier if such values occurred on conse- 15 fully investigated members of a large Irish tance of CPP as a determinant of outcome cutive occasions, which happened, on aver- family had CADASIL. Brain MRI showed a after severe head injury in children. age, about every 30 hours. The relative of accuracy of the two devices remains uncer- leucoencephalopathy varying severity. 1 Mendelow AD, Allcut DA, Chambers IR, et al. there The spectrum of presenting features In: Avezaat CJJ, et al, eds. ICV VII. Berlin: tain, but is evidence suggesting that included familial hemiplegic migraine, com- Springer Verlag 1993:544-8. both are prone to errors. mon and classical migraine, transient cere- [SBNS] [SBNS] bral ischaemic attacks, recurrent strokes, and spinal cord infarction. One family member was asymptomatic; two members developed a pseudobulbar palsy and subcortical LABORATORY TESTING OF THREE EVIDENCE OF SMALL VESSEL CHANGES dementia. In five familial hemiplegic INTRACRANIAL PRESSURE CAUSING ISCHAEMIA AFTER SUBARACHNOID migraine started in childhood and four of MICROTRANSDUCERS HAEMORRHAGE these have subsequently developed the fea- M Czosnyka, Z Czosnyka, JD Pickard. JG Rowe, N Soper, R Ouwerkerk, RSC Kerr, tures of CADASIL. Familial hemiplegic University of Cambridge, Cambridge, UK B Rajagopalan. MRC Spectroscopy Unit and migraine has not been reported in associa- the Radcliffe Infirmary, Oxford, UK tion with CADASIL. Linkage analysis Three comparably priced ICP microtrans- showed significant lodscores (3-91 with ducers have become available on the United Objective-To follow cerebral blood flow D19S226) with markers close to the locus Kingdom market, each characterised by their (CBF) with single photon emission com- for the CADASIL gene. We postulate that manufacturer as having very low zero drift puted tomography (SPECT), metabolism familial hemiplegic migraine is, in this 208 Proceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons

family, a manifestation of the CADASIL allele was 0 59:0 41 in cases frequency and STATUS DYSTONICUS ET RIGIDITUS: A J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from gene inheritance, may which produce vari- 0-48:0-52 in controls (P = 0-01). The DD REPORT OF 10 CASES able, age related clinical manifestations. genotype conferred a relative risk of cere- H [ABN] Manji, RS Howard, NP Hirsch, DH brovascular disease of 2-4 (95% CI Miller, L Carr, NP Quinn, CD Marsden. 1-14-5-05; P = 0 02). The relation was inde- National Hospital for Neurology and pendent of age, sex, smoking history, dia- Neurosurgery and Great Ormond Street betes, or cholesterol but seemed to be Hospital for Sick Children, London, UK WHICH METHOD OF MEASUREMENT SHOULD mediated at least in part through hyper- BE USED TO ASSESS CAROTID ANGIOGRAMS? tension. Neither the DD genotype nor the Patients with primary and secondary dys- GR Young, PRD Humphrey, TE Nixon, D allele was positively associated with tonic syndromes may develop increasingly ETS Smith. Walton Centre for Neurology carotid stenosis or intima-media thickness. frequent and severe episodes of generalised and Neurosurgery, Liverpool, UK The DD genotype is a new risk factor for dystonia and rigidity, which may be refrac- cerebrovascular disease. It seems to exert its tory to conventional drugs and, in severe action at least In future, non-invasive techniques will obvi- partly through hypertension in cases, result in bulbar and ventilatory com- ate the need for digital subtraction angio- this age group, although not via extracranial promise. graphy (DSA) in the assessment of large vessel disease. In view of the lack of Ten cases (mean age 21-4 association with (range 6-39) extracranial internal carotid artery stenosis. carotid artery wall thickness years) are presented. The diagnoses were There is currently much debate concerning and stenosis it may act via small vessel dis- athetoid cerebral palsy (three), post- which measuring technique to adopt when ease, altered functional vessel responses, or traumatic dystonia (three), postencephalitic assessing the degree of carotid stenosis from by predisposing to cardiac disease. hemidystonia (one), relapsing/remitting angiograms. Using both DSAs and magnetic [ABN] dystonia-parkinsonism (one), infantile stri- resonance angiograms (MRAs), four atal necrosis (one), and myoclonic dystonia different methods-the "ECST", the (one). Possible precipitating factors included "NASCET", the "common carotid artery as infection (one) and starting clonazepam denominator" and the visual impression CONTINUED ISCHAEMIC DAMAGE AFTER (two). Eight patients were ventilated for a ("eyeballing") methods-were assessed and CEREBRAL INFARCTION IN HUMANS mean period of 61 (range 7-300) days. The their reproducibility determined. One hun- DE Saunders, FA Howe, A van den indications for ventilation were bulbar dred and ten DSAs and 74 MRAs were Boogaart, MA McLean, JR Griffiths, MM (four), respiratory (five), exhaustion (eight), assessed using all four methods by two inde- Brown. St George's Hospital Medical and metabolic disturbance as a result of pendent observers on two separate occa- School, London, UK rhabdomyolysis (one). sions. Measurements were made using Drug treatment used included benzhexol, vernier scale calipers. The mean (SD) of the The aim was to study the ischaemic penum- tetrabenazine, pimozide, baclofen (oral and differences were calculated for each method bra in humans, by measuring metabolic intrathecal), chlorpromazine, and carba- (the smaller the SD the better the agree- changes after middle cerebral artery territory mazepine. One patient improved with ben- ment). (MCA) infarction using proton magnetic zhexol and another with tetrabenazine. Two For DSA, the mean (SD) of the differ- resonance spectroscopy ('H-MRS; 1-5T sys- patients underwent -bilateral ences between observers were: ECST, 6-4 tem). Localised IH-MRS was carried out (no improvement); unilateral (improved). (8-9)%, NASCET, 9-6 (21-3)%; common within the area of infarction and contralat- Two patients died, four returned to their carotid, 6-5 (13-8)%, eyeballing, 0-1 (10-7)% eral hemisphere. Peak areas were obtained prestatus condition, two improved partially, respectively. The consistency improved by by variable projection time domain fitting and two eventually recovered. each technique as the degree of stenosis analysis. The unsuppressed water signal was Thus patients with status dystonicus et increased. Similar results were obtained for used as internal concentration standard. Ten rigiditus should be managed in intensive care MRA and intraobserver agreement. patients with acute MCA territory infarction units; standard drug treatment may be bene- Using calipers, the ECST and the com- were studied within 28 hours of stroke and ficial; paralysis and ventilation may be of mon carotid techniques show least variation for three months. Significant changes were benefit and the prognosis is guarded, partly in reporting. The NASCET technique seems seen in the initial infarct spectra compared depending on the underlying condition. the least reproducible. Interestingly, eye- with the contralateral spectra (table 1). [ABN] balling is a highly reproducible technique. Lactate, a marker of anaerobic metabolism, [ABN] was detected within the infarct but not in the

contralateral hemisphere. N-acetyl aspartate http://jnnp.bmj.com/ (NAA), a neuronal marker, and total crea- ABNORMAL MOTOR UNIT SYNCHRONISATION tine (Cr/PCr) were significantly reduced. BETWEEN ANTAGONIST MUSCLES IN Further ACE GENE reductions in NAA and Cr/PCr DYSTONIA POLYMORPHISM, occurred within the first week A fall CEREBROVASCULAR DISEASE, AND CAROTID (table 2). SF Farmer, G Sheean, JC Rothwell, CD in lactate was seen ATHEROMA within the infarct core Marsden. Institute of Neurology, London, over the first seven to 10 UK H Markus, J Barley, S R days. Jeffery, Lunt, JM In conclusion, neuronal loss is detected Bland, N Carter, MM Brown. King's non-invasively College School of Medicine and Dentistry by 'H-MRS within 28 hours Activity in networks of neurons that provide of stroke onset. The on September 29, 2021 by guest. Protected copyright. and St George's Hospital Medical School, continuing loss of neu- common presynaptic input to motoneurons London, UK rons may represent continued ischaemic produces synchronisation of motor unit dis- damage after MCA infarction. charges. Cross correlation of EMGs from The molecular basis of the genetic predispo- [ABN] pairs of synergistic muscles in humans may sition to stroke is largely unknown. A dele- tion polymorphism in intron 16 of the ACE gene has been reported as an independent Table 1 risk factor for myocardial infarction although Initial data (day 1) Contralateral the mechanism of this effect remains (n=7) Infarct (n=10) P value (t test) unknown. NAA (mmol/l) 14-7 (1-6) 88 (4 3) < 0 001 With the polymerase chain reaction the Cr/PCr (mmol/l) 11-6(1*0) 9-4(2 9) < 005 Cho (mmol/l) 2-1 insertion-deletion polymorphism was deter- (0-3) 1-9 (0-5) NS Lactate (mmol/1) 0 19-2 (0-5) < 0-001 mined in 101 white patients (mean age 64 8) and 137 age matched controls (mean age 63 9). All patients presented with a carotid territory acute ischaemic event and carotid Table 2 duplex was performed to assess degree of internal carotid artery stenosis and common Infarct Value day 1 (n=7) Value 7 days (n=7) P value (t test) carotid artery intima-media (IM) thickness. NAA Plasma ACE (mmol/l) 10-6 (3 4) 6-1 (4-1) < 0-05 concentrations were measured. Cr/PCr (mmol/l) 10-5 (2 6) 4-4 (2-8) < 0 01 The DD genotype was commoner in Cho(mmol/l) 1-8 (0-6) 1-7 (0-7) NS patients with cerebrovascular disease than in Lactate (mmol/l) 16-9 (9 3) 11-4 (7-2) NS controls (36/101 v 30/137, P = 0 02). D:I Values for tables 1 and 2 are means (SD). Proceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons 209

motor unit and thus of 21 patients with primary writing tremor ments can be developed which enhance show synchronisation J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from common presynaptic input. By contrast, (PWT) are described. This was first des- endogenous or transplanted oligodendrocyte antagonist motoneurons-for example, flexor cribed by Rothwell, et al (1979). Subse- repair. and extensor carpi radialis (FCR/ECR)-do quently there has been controversy as to [ABN] not produce synchronised discharges even whether PWT is a forme fruste of essential when a subject deliberately cocontracts the tremor or a variant ofwriter's cramp. muscles. Twenty one patients with PWT were Dystonia is characterised by involuntary studied using surface polymyography and IS THE VARIATION IN PREVALENCE OF cocontraction of antagonist muscles. One accelerometry. Seven had a family history of MULTIPLE SCLEROSIS WITHIN THE UNITED hypothesis concerning abnormal muscle co- PWT. In 13 patients the forearm reciprocal KINGDOM DUE TO MIGRATION, LATITUDE, contraction in dystonia is that it is produced inhibition curve ofthe median nerve H reflex OR GENES? by abnormal common presynaptic input to was also examined. The results of reciprocal PM Rothwell. Western General Hospital, motoneurons of antagonist muscles. Five inhibition were compared with those of 10 Edinburgh, UK patients with dystonia were studied (two normal controls and to those obtained from idiopathic, three symptomatic, four women, patients with writer's cramp. There is debate about the extent to which the ages 17-60). Simultaneous multiunit EMGs The median frequency of PWT was 5-5 worldwide latitudinal gradient in the preva- were recorded using concentric needle elec- Hz (range 4-1-7-3 Hz). Segregated EMG lence ofmultiple sclerosis is due to genetic or trodes inserted into FCR and ECR, while activity was typically alternating in forearm environmental factors. Northern Scotland patients cocontracted the muscles. For com- flexor/extensor pairs but co-contraction, has the highest prevalence in the world. parison, identical recordings were obtained extensor activation alone and varying EMG Recent findings of high rates in southern from four healthy subjects (three men, ages patterns were also recorded. Forearm recip- England, however, have cast doubt on the 28-33). The times of motor unit occurrence rocal inhibition was normal in PWT. The assumption of a latitudinal gradient within from FCR and ECR were cross correlated. patients were subclassified on clinical the United Kingdom. To assess this a preva- Cross correlograms of the healthy subjects grounds into type A (task induced) and type lence study was performed in south east were flat. Those of dystonic subjects con- B (positionally sensitive) PWT. The only dif- Scotland. In addition, as susceptibility to tained a peak at time zero (median size k = ferences between these two groups were that multiple sclerosis seems to be either inher- 1-6; median duration = 38 ms), indicating cocontracting tremor and tremor induced by ited or determined in childhood, mortality abnormal motor unit synchronisation. tendon taps to the volar aspect of the wrist from multiple sclerosis within England and It is concluded that in dystonia antagonist occurred in type B but not type A cases. Wales was analysed by place of birth as well motoneurons share abnormal common pre- Dominant hand writing speed was signifi- as place of death to detect any confounding synaptic inputs. Activity of these inputs may cantly reduced in PWT compared with age due to migration. produce abnormal muscle cocontraction. matched controls (PWT 73-1 letters/min; Between 1969 and 1974 all deaths regis- [ABN] controls 138-8 letters/nin (P < 0 0001). tered in England and Wales were linked with It is concluded that patients with PWT birth records. Place ofbirth and normal resi- can be distinguished from writer's cramp as dence before death were obtained for the both the disynaptic and presynaptic phases 3151 deaths in which multiple sclerosis was a EMG GUIDED TREATMENT OF TASK SPECIFIC normal in PWT DYSTONIA WITH BOTULINUM TOXIN A of reciprocal inhibition are cause and analysed within the nine standard GL Sheean, NMF Murray, AJ Lees, CD whereas the amount of presynaptic inhibi- regions. There was no latitudinal gradient by Marsden. National Hospital for Neurology tion is decreased in writer's cramp; PWT can place of death, but the proportion ofpatients and Neurosurgery, London, UK also be differentiated from hereditary essen- born outside the region in which they died tial tremor on clinical criteria. was higher in southern regions (41%-63%) The role of EMG in selection and placement [ABN] than northern regions (8%-18%). Re- of injections of botulinum toxin (BTX-A) in analysis by place ofbirth, however, still failed the treatment of limb dystonia is ill defined. to show a latitudinal gradient within EMG guided injections ofDysport) BTX-A England. There were fewer than expected deaths of people born in Wales (observed/ were used to treat 37 patients with task spe- IN AXO-GLIAL ADHESION MOLECULES expected = 0-64, 95% CI = 0 54-0-75). The cific dystonia, 30 with writer's cramp (14 INTERACTIONS simple, 16 dystonic), five with primary writ- preliminary prevalence rate of multiple scle- CE Shaw, R Milner, C ffrench-Constant, and Border regions of ing tremor and two with musician's dystonia. DAS Compston. University of Cambridge, rosis in the Lothian

in http://jnnp.bmj.com/ After clinical inspection during the relevant Cambridge, UK Scotland (population 850 000) was excess task, likely active muscles were sampled with of 200/100 000. This is similar to rates in and significantly higher a teflon coated, 23G monopolar needle With recent advances in immunotherapy for northern Scotland which allowed simultaneous EMG recording than the rates in southern England. These multiple sclerosis (MS) the possibility exists is no latitudinal gradi- and injection. Usually two or three, but up to for promoting remyelination by endogenous data suggest that there six, muscles were treated in each session. ent within either England or Scotland, but or transplanted oligodendrocytes. The of Commonly injected muscles included the molecular mechanisms regulating axo-glial that there are differences in the prevalence wrist flexors/extensors, thumb and deep fin- multiple sclerosis between England, interaction in developmental myelination and Wales. This most probably ger flexors, and index finger extensors. Initial and repair are largely unknown. To study the Scotland, of the on September 29, 2021 by guest. Protected copyright. doses ranged from 20-80 MU and follow up role of the adhesion molecules, myelin asso- reflects the genetic susceptibility was from 1-60 months. Initial evaluation ciated glycoprotein (MAG), and integrins in respective populations. and treatment generally took 45-60 minutes mediating this interaction an in vitro model [ABN] and up to 30 minutes at follow up. of myelination co-culturing rodent sensory Subjective improvement occurred in 86% of neurons and glia has been used. The regional patients (good to excellent in 76%). Four expression of MAG by oligodendrocytes is showed no benefit. Significant weakness under axonal influence and MAG protein SERIAL BRAIN AND SPINAL CORD MRI IN developed in nine and involved an adjacent, binds to axons confirming a specific ligand MULTIPLE SCLEROSIS non-injected muscle in one case. EMG is interaction. MAG expression occurs after JW Thorpe, D Kidd, IF Moseley, BE essential for accurate identification of target the initial recognition and ensheathment Kendall, WI McDonald, DH Miller. muscles (both before and after BTX-A) and takes place and blocking antibodies do not Institute ofNeurology, London, UK is particularly important for muscle selection inhibit myelin formation, suggesting that in tremor. MAG plays only a minor part in myelination. The dynamics of multiple sclerosis have [ABN] Integrins are heterodimeric adhesion been extensively studied by serial MRI of the molecules, composed of a and ,B subunits brain; less is known about the spinal cord. that recognise specific molecules in the Monthly gadolinium-enhanced brain and PRIMARY WRITING TREMOR extracellular matrix. With immunoprecipita- spinal cord MRI over a period of one year PG Bain, LJ Findley, TC Britton, MA tion of cross species co-cultures a6Af61 and were carried out in 10 patients with relaps- Gresty, JC Rothwell, PD Thompson, CD avfl5 were greatly upregulated during myeli- ing-remitting multiple sclerosis. Six patients Marsden. MRC Human Movement and nation, suggesting that they may have a role had a total of 11 clinical relapses of which Balance Unit, London, UK in mediating this event. With a better under- eight implicated the spinal cord. One hun- standing of the molecular mechanisms that dred and sixty seven active (enhancing or The clinical and neurophysiological features control myelination it is hoped that treat- new non-enhancing) lesions were detected in 21020Proceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons the brain, of which only one was symp- Compston. University of Cambridge, dendrocytes in vitro is encouraging for the tomatic; conversely six ofthe 19 active spinal Cambridge, UK prospects of repair in multiple sclerosis. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from cord lesions found were symptomatic. All [ABN] but seven of the active lesions were in the six Familial recurrence risks in multiple sclerosis patients who relapsed. Both cord and brain are becoming of increasing interest to geneti- activity were more common around the time cists in search of disease susceptibility genes of relapse. There was a strong association and to clinicians who counsel patients. Little CREATION OF COMPOSITE DATA SETS FOR between the cord and brain MRI activity. contemporary information is available for USE WITH THE ISG IMAGE DIRECTED No progressive cord atrophy was detected the United Kingdom, the population of SURGICAL SYSTEM from measurements of cord cross sectional which continues to exhibit an overall trend of PR Eldridge, JN Roberts, RHT Edwards. area. increasing prevalence and incidence; rates Walton Centre for Neurology and Neuro- In relapsing-remitting multiple sclerosis, derived from genetically and geographically surgery and the University of Liverpool, therefore, imaging the brain alone will detect distant populations may not be relevant. In Liverpool, UK 90% ofactive lesions. The lack ofprogressive addition crude risks, which have previously cord atrophy, suggesting that significant been used, may be misleading in younger Computed tomography and MRI provide axonal loss has not occurred, is in keeping populations because of the widely dis- information conceming anatomy, pathology, with their good recovery after relapse: this tributed age at onset profile of multiple scle- and function of the brain. Many data sets are contrasts with our previous findings in pro- rosis. The personal details and disease status created for a single patient. Image directed gressive multiple sclerosis in which increas- have been systematically recorded for more systems enable accurate surgical localisation ing cord atrophy developed whereas active than 11 000 relatives of 650 probands with from these data sets. Unfortunately the ISG lesions were rare in the absence of superim- probable or definite disease listed on the image directed system only utilises a single posed relapses. The concurrence of brain Cambridgeshire Multiple Sclerosis Register data set at a time. It would be desirable to and spinal cord lesions implies a systemic and prevalent on 1 July 1993 to provide make use ofinformation from more than one trigger for disease activity. crude and age adjusted recurrence risks for data set in surgery; such as different MR [ABN] first, second, and third degree relatives. sequences, or combinations ofMRI and CT. Maximal crude risks were found for sisters of A method combining data sets for the ISG female probands (5%) and age adjusted risks system is illustrated, using the image analysis for female offspring of male probands (7%). program "analyze". Raw CT and MRI data DAILY URINARY NEOPTERIN EXCRETION AS Risk was found to fall proportionately with are transferred to "analyze" using XDimage. AN IMMUNOLOGICAL MARKER OF DISEASE genetic distance from the proband but was Features of importance are segmented in ACTIVITY IN MULTIPLE SCLEROSIS still significantly raised in first cousins each data set. The data sets are combined G Giovannoni, M Lai, DH Miller, JW (1-0%) compared with background preva- and retumed to the ISG system for surgery. Thorpe, D Kidd, AJ Thompson, G Keir, lence (132 x 105) suggesting that the pheno- This process requires close involvement of EJ Thompson. Institute of Neurology, menon of familial aggregation in multiple the operating surgeon. London, UK sclerosis is likely to depend on genetic The technique has wide application. factors. Examples shown include intrinsic tumours, The excretion of neopterin, a marker of [ABN] acoustic neuroma; pituitary tumours, macrophage activity in patients with multiple caroticocavemous fistulas (utilising MR sclerosis was investigated. Daily urinary angiography). Bony detail, best obtained neopterin:creatinine ratios (UNCR) were from CT, can be combined with data con- measured by high performance liquid chro- ceming tumours best seen on MRI (acoustic, matography for a 12 week period in eight DO CELLS FROM THE ADULT HUMAN BRAIN pituitary, cavemoma, base of skull tumours). controls, eight patients with non-relapsing HAVE REMYELINATING POTENTIAL? The method compensates for the geometric progressive, and nine patients with relapsing DAS Compston, NJ Scolding, J Sussman, distortion of MRI data sets, which can result multiple sclerosis. Infection, relapses, day to CE Shaw. University of Cambridge, in inaccuracy twice as great as when using day fluctuations in neurological function and Cambridge, UK CT. Gd enhanced MRI were monitored. [SBNS] The mean UNCR was significantly higher Limited remyelination of some multiple scle- in patients with non-relapsing progressive rosis lesions is known to occur but recurrent (191 (86) umol/mol) and relapsing disease lesions lead to permanent demyelination and http://jnnp.bmj.com/ (558 (652) ,umol/mol) compared with con- disability. Advances in immunotherapy may EMBOLISATION OF INOPERABLE trols (119 (28) ,umol/mol, P = 0-001). One arrest the inflammatory process and the real INTRACRANIAL ANEURYSMS BY asymptomatic peak in the serial UNCRs was possibility exists for repairing areas of ENDOSACCULAR PACKING WITH PLATINUM found in one control subject compared with demyelination in an immunologically stable COILS an average 2-2 (2 3) peaks/patient/month in environment. Possible strategies for repair J Byme, A Molyneux, R Kerr, P Brennan, A non-relapsing progressive and 3-2 (1-3) include promoting endogenous remyelina- Hope, S Renowden. Radcliffe Infirmary, peaks/patient/month in patients with relaps- tion or transplantation of cells with myelinat- Oxford, UK ing multiple sclerosis (P = 0 002); two peaks ing potential. To determine which were temporally associated with a clinical oligodendrocytes derived from adult human Over a 30 month period 154 patients were on September 29, 2021 by guest. Protected copyright. relapse and clearly defined peaks and eleva- brain have remyelinating potential, their referred for endovascular treatment of inop- tions in the baseline were noted around 21 interaction with neuronal cultures was stud- erable intracranial aneurysms. Patients were days after an upper respiratory tract infection ied in vitro. Embryonic rat dorsal root gan- judged unsuitable for surgical clipping, being in three patients. Patients with day to day glion neurons were purified in culture and unfit for craniotomy because of the fluctuations in neurological function had cells dissociated from human temporal lobe aneurysm's size, location, and wall thickness. higher mean UNCR than patients without white matter were added. Co-culture with Six patients were not treated by the endovas- (526 (624) v 182 (69) pmol/mol, P = 0 05). axons improved oligodendrocyte survival but cular route because of unsuitable anatomy Mean UNCRs tended to be higher in did not lead to proliferation. Within one and 18 patients were treated for additional patients with MRI activity than patients week oligodendrocyte precursors were coincidental aneurysms. A total of 164 without activity (512 (631) v 203 (84) ,umol/ extending processes along axonal bundles aneurysms were therefore treated in 173 pro- mol, NS). and by four weeks there was extensive cedures using Guglielmi detachable coils Neopterin seems to be a promising ensheathment of axons by membranes posi- (Target Therapeutics). Most patients pre- immunological marker that can detect sub- tive for myelin basic protein. After six weeks, sented with subarachnoid haemorrhage clinical inflammation in patients with mul- there were loose wraps of myelin around (74%) and over 70% were treated within tiple sclerosis. axons by transmission electron microscopy three weeks ofthe ictus. Forty five per cent of [ABN] but compact myelin was not detected. This aneurysms were on vertebrobasilar arteries. confirms that isolated adult human oligo- The aneurysms were packed with multiple dendrocytes can correctly identify exposed electrolytically detached coils to form stable axons as the target for ensheathment but intraluminal thrombus and all patients have MULTIPLE SCLEROSIS: RECURRENCE RISKS species barriers may exist in this model that been offered follow up angiography at six FOR RELATIVES prevent compact myelin formation. This months. Procedural complications have N Robertson, J Deans, M Fraser, DAS attempt at remyelination by human oligo- included aneurysm rupture and coil fractures Proceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons 211

but the most common adverse event has Outcome measures-Concentrations of NPY, EFFECT OF ANGIOGRAPHY, ANAESTHESIA, been the formation of thromboemboli. As a VIP, SP, and CGRP measured by enzyme AND OPERATION ON INTRACRANIAL PRESSURE J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from result anticoagulants are now given during linked immunosorbent assay and expressed IN PATIENTS WITH ANEURYSMAL and for up to 48 hours after treatment. as pmol/g tissue. SUBARACHNOID HAEMORRHAGE The overall morbidity (permanent deficit) Results-Neuropeptide concentrations were SS Praharaj, IR Chambers, A Clark, PJ was 5% and mortality 4%. Complications significantly higher in the middle cerebral Crawford, A Jenkins, NV Todd, RP occurred with similar frequency in the ante- artery compared with the posterior cerebral Sengupta, A Gholkar, AD Mendelow. rior and posterior circulations. artery (NPY 13-53 (2-85) v 7-18 (1-39), University of Newcastle-upon-Tyne and [SBNS] P < 0 05; VIP 13-72 (2-93) v 3-34 (0 77), Regional Medical Physics Department, P <0-01; SP 8-83 (1-43) v 1-23 (0-46), Newcastle-upon-Tyne, UK P < 0-001; CGRP 26-93 (3 02) v 2-12 (0 32), P < 0-001). There was no difference Design-A total of 18 patients with suspected POSTERIOR CIRCULATION ANEURYSMS: TO between the concentrations in the middle aneurysmal subarachnoid haemorrhage COIL OR CLIP? cerebral artery compared with its trifurca- (SAH) had intracranial pressure (ICP) mon- KW Lindsay, E Teasdale, P Mathew. tion. There was no correlation between itoring measured during transfemoral digital Institute of Neurological Sciences, Glasgow, neuropeptide concentrations and either age subtraction angiography under local anaes- UK or postmortem delay. thesia. Five ofthese also had their ICP moni- Conclusion-Neuropeptide concentrations tored during anaesthesia and operation. ICP Objective-To determine the optimal man- are consistently higher in the middle cerebral was measured with a Camino pressure trans- agement of patients with posterior circula- artery than in the posterior cerebral artery in ducer. Simultaneously, blood pressure was tion aneurysms. humans. The concentrations are stable and measured either with a cuff or arterial line. Design-All patients with posterior circula- independent of postmortem delay. Post- Results-Angiography raised the ICP in all tion aneurysms undergoing treatment by mortem cerebral vessels are thus suitable for patients. Preangiographic ICP was 11-59 either direct clipping or by coil embolisation comparative studies ofneuropeptide concen- (1-54) mm Hg, whereas perangiographic were reviewed over a five year period from trations in disease. peak ICP was 23-61 (4 68) mm Hg. 1989 to 1994. [SBNS] Intubation during anaesthesia was the single Patients-Of 76 patients included in the most important variable affecting ICP, study, 66 underwent direct clipping and 10 which rose from between 3 and 87 mm Hg underwent coil embolisation. (average rise 24-5 (15-9) mm Hg). A corres- Outcome measures-Aneurysm features (size, PEROPERATIVE MEASUREMENT OF JUGULAR ponding rise in MAP, however, ensured a direction offundus, neck height, and width). VENOUS OXIMETRY DURING CAROTID relatively stable CPP. Outcome as assessed by the Glasgow out- ENDARTERECTOMY Conclusion-These that the AJ Davidson, AD IR findings suggest come scale. Complications during coiling Mendelow, Chambers. greatest haemodynamic disturbance to and occlusion rate. Newcastle General Hospital, Newcastle- patients with subarachnoid haemorrhage Results-Of 66 patients undergoing operative upon-Tyne, UK occurs during intubation. This strengthens clipping, 60 (90%) made a favourable out- the case for performing angiography under come; three patients died. In five of the six Objective-To measure jugular bulb oxygen local anaesthesia. patients making an unfavourable outcome saturation during carotid endarterectomy. [SBNS] (severe disability or death), the aneurysm lay Design-Prospective observational study. at the basilar bifurcation. Aneurysm features This was performed by means of a 40 cm 4 did not seem to relate to outcome. FG Opticath fibreoptic catheter connected In 10 patients, 32 coil embolisation proce- to an Oximetric 3 computer and recorded EXPERIENCES WITH AN ARTIFICIAL CERVICAL dures were performed. Two patients re-bled continuously on a paper hard copy and a JOINT during coiling, of whom one died. In- Psion computer. The fibreoptic catheter was BH Cummins. Frenchay Hospital, Bristol, UK advertent placement occurred in four positioned by the surgeon under direct vision patients, but only one developed a neurolog- via a facial vein tributary into the jugular Most surgical procedures on the cervical ical deficit. Complete occlusion was bulb on the operative side and removed at spine include fusion by bone, metal, or both. achieved in two patients and > 90% occlu- the end of the procedure. Other monitoring Over the years such fusion at one or more sion in four. Revascularisation occurred on included ECG, invasive blood pressure, cen- spaces may be associated with osteophytic three occasions. tral venous pressure, pulse oximetry, and degeneration at the adjacent levels, causing Conclusions-Coil embolisation is not per- end tidal capnography. radiculopathy or myelopathy. http://jnnp.bmj.com/ formed without some risk. The long term Patients-Twenty patients undergoing In 10 "last ditch" spaces of such disorder effects of incomplete occlusion remain carotid endarterectomy under either local or accompanying multiple levels of congenital unknown. Use should be restricted to techni- general anaesthesia depending on the prefer- or surgical fusion, an artificial joint designed cally difficult aneurysms, particularly those ence ofthe patient and surgeon. by the author and made by the engineering at the basilar bifurcation. Outcome-Assessed correlation of peropera- department ofthe hospital has been inserted to [SBNS] tive monitoring with neurological outcome. provide some degree offlexibility at that level. Results-The system provided a reliable, The joint is of stainless steel in two pieces, continuous, real time monitor of jugular the upper with a downward dome facing on September 29, 2021 by guest. Protected copyright. bulb saturation during surgery. Observation slightly smaller in radius than the shallower NEUROPEPTIDE CONCENTRATIONS IN HUMAN of changes in jugular bulb saturation and saucer of the upper face of the lower piece. CEREBRAL ARTERIES correlation with neurological deficits in those This allows rotation, limited flexion, and L Chadwick, P Milner, G Burnstock, PJ patients under local anaesthesia alone greatly extension and glide. Locking screws main- Hamlyn. St Bartholomew's Hospital, and aided in blood pressure manipulation and in tain the joint in position. University College, London, UK the decision as to the need to insert tempo- Technically this has proved satisfactory rary bypass shunts. Typical patterns seen and nine of the 10 joints have provided reso- Objectives-To document the concentrations included falls in jugular saturation associated lution ofsymptoms in these difficult cases. ofneuropeptide Y (NPY), vasoactive intesti- with hypotension and hence inadequate [SBNS] nal polypeptide (VIP), substance P (SP), and cerebral perfusion and profound falls in satu- calcitonin gene related peptide (CGRP) in ration on carotid cross clamping relieved by several arteries of the Circle of Willis in insertion oftemporary shunts. Equally useful humans and to determine whether there is were stable readings obtained during general BIOCOMPATIBLE OSTEOCONDUCTIVE any change in the concentrations with post- anaesthesia or cross clamping, thus avoiding POLYMER V ILIAC GRAFT IN ANTERIOR mortem delay. the potentially deleterious side effects that CERVICAL : A PROSPECTIVE Design-Documentation and comparison might occur with shunt insertion, fluid load- COMPARATIVE STUDY ON 110 PATIENTS between neuropeptide concentrations in the ing, or inotropic drug treatment given in an OPERATED ON FOR CERVICAL DISC DISEASE middle and posterior cerebral arteries and attempt to raise blood pressure. AA Madawi, M Powell, HA Crockard. The the middle cerebral trifurcation. Conclusions-Jugular venous oximetry is a National Hospital for Neurology and Subjects-Twenty nine male and 18 female valuable monitor to aid in the peroperative Neurosurgery, London, UK control cadavers (mean age 73-8 (1-71) years care ofpatients undergoing carotid endarter- and mean postmortem delay 27-91 (1-41)) ectomy. Donor site morbidity is a significant problem hours. [SBNS] in anterior cervical fusion. A biocompatible 21222Proceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons osteoconductive polymer (BOP) has been decompression was sufficient in 92% of with spinal meningiomas operated on over used in many centres to circumvent these patients and only one patient required more 20 years at the Brook Neurosurgical Unit. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from problems. A prospective study was per- than two levels to be decompressed. In 79% The age range was 22-91 (mean 61-5) years, formed to evaluate its use and long term of cases soft disc protrusions contributed to with 12 men (15%) and 66 women (85%), effectiveness. the compression whereas in 21 % osteo- followed up for a mean of 10-5 years. The Materials and methods-One hundred and phytes alone caused the compression. We clinical history was of less than one year in ten patients, 50 for iliac graft (IG) and 60 for believe that this simple technique is a suffi- 55 patients and less than six months in 26 BOP were recruited into this study. 72 cient surgical treatment for most cases ofcer- patients. There was poor correlation patients were radiculopathic, 20 myelo- vical degenerative disease. It does not require between presenting complaint and relation pathic, and 18 were radiculomyelopathic. a fusion and avoids the specific problems and to the spinal cord: 15 lay anteriorly, 15 The mean duration of symptoms was 20-3 complications associated with Cloward type anterolaterally, 23 laterally, 17 posterolater- months. The Smith Robinson technique was operations. A long term follow up study of ally, and eight posteriorly. Plain radiology used in 70, and the Cloward technique in 40 these patients is presently being carried out was normal in the entire intradural group (74 patients; 80 patients had one level, 30 had and to date no late problems have become patients) with only one example of tumour two level surgery. Patients were followed up apparent. calcification. Three out of the four extra- routinely at three to six month intervals [SBNS] dural group showed abnormalities on plain (range 3-24, mean 11 months) at which time radiology. Two tumours were purely extra- clinical and radiological assessment were dural and a further two both intradural and made, including CT and MRI. extradural. The dural attachment was ex- Results-The mean hospital stay was 5 9 A RANDOMISED CONTROLLED TRIAL cised in 20 cases. Full recovery offunction was days for those with IG, and 5-1 days for COMPARING AUTOMATED PERCUTANEOUS achieved in 57 patients (73%), and a further BOP. Complications were recorded as two LUMBAR DISCECTOMY AND LUMBAR 17 (22%) were improved to independent neck haematomas required evacuation, one MICRODISCECTOMY mobility. There was one recurrence, at 14 years iliac wound haematoma was managed con- G Findlay, S Chatterjee, P Foy. Walton after operation. By contrast with previous servatively, two graft non-unions with Centre for Neurology and Neurosurgery, series, histology showed no striking predomi- kyphus (IG) required revision and plating, Liverpool, UK nance of psammomatous meningiomas. two complete graft extrusions (one IG, one [SBNS] BOP) required revision. Partial graft extru- Much interest has been created by the sion occurred in 18 IG (2-5 mm, mean 3 advent of automated percutaneous lumbar mm) and seven BOP (2-4 mm, mean 2-6 discectomy (APLD) in the management of mm). Kyphus was found in 13 IG (3-30, contained lumbar disc hemiation. Several TRANSDURAL MICRONEUROSURGICAL mean 12-3 degrees) and in four BOP (mean papers have reported encouraging results but EXCISION OF PROLAPSED DORSAL DISCS 3-15, 10-3 degrees). The BOP sank into the to date attempts to perform controlled JM Rice Edwards, D Peterson. Charing adjacent endplates and the space collapsed studies of APLD have been hampered by Cross Hospital, London, UK after Smith Robinson procedures. Also scle- numbers too small to be statistically signifi- rosis was found around BOP within one to cant. We designed a randomised study with a A tenet of neurosurgical practice is that a two months, but even by 18 months there separate blind assessor to compare the laminectomy should not be done for the was no incorporation or degradation. results of APLD with conventional lumbar removal of dorsal discs. This belief is based Osseous union was seen to begin within one microdiscectomy. It was planned to recruit on historical evidence of operations per- to two months and had finished by one year 180 patients to the study and to complete a formed with rongeurs and of impossible in 48 cases after IG. two year follow up in all cases. During the attempts to remove anterior lesions extra- Conclusion-BOP can act as a good spacer study it became apparent to the non-blind durally. after anterior cervical fusion, with evidence participants that the results of APLD were An operation is described which involves of incorporation or degradation through the much worse than surgery and it was decided careful removal of laminae with a drill and a period offollow up. to analyse the results at that stage. transdural microapproach to the dorsal disc. [SBNS] This produced 70 patients who had small After removing the lamina the spinal cord contained disc hemiation proved by MRI (all is not usually displaced against the dorsal other types were excluded from the study). dura and a very adequate view of the anterior In the APLD group only eight of 30 (26 7%) disc can be obtained laterally without signifi- achieved excellent or good results, whereas cant retraction of the spinal cord. It has been EXTENDED ANTERIOR CERVICAL DISCECTOMY the corresponding figure for the micro- possible to completely remove the hard disc http://jnnp.bmj.com/ WITHOUT FUSION: A SIMPLE AND SUFFICIENT discectomy group was 32 of 40 (80%). prolapse in the 10 cases where this approach OPERATION FOR MOST CASES OF CERVICAL Moreover for patients in the APLD group has been used. DEGENERATIVE DISEASE who had unsatisfactory results and opted for The technique has the following advan- RS Maurice-Williams, N Dorward. Royal subsequent microdiscectomy (20 of 22 tages: the approach is familiar to neuro- Free Hospital and School of Medicine, patients), the success rate was only 13 of 20 surgeons whereas the anterolateral and London, UK (65%). Thus the eventual success rate of transthoracic approaches can be difficult those treated by APLD alone or APLD and unless practised often, the spinal cord can be Of 291 operations performed for cervical microdiscectomy was 21 of 30 (70%). for adhesions to the dura (which

inspected on September 29, 2021 by guest. Protected copyright. degenerative disease causing cord or root A recent controlled study comparing occurred in two cases with associated arach- involvement over a 12 year period 187 have APLD to chemonucleolysis also showed noiditis), it is safe providing that the disc been treated by extended anterior discec- poor results for APLD. Our study shows that protwsion is not of an extreme size. This can tomy without fusion, removing bone on in the chosen group of patients APLD is in- be judged from MRI. either side of the posterior disc space so as to effective. Our success rate for microsurgery [SBNS] give a wide exposure of the anterior spinal for all types of disc hemiations was found to and root dura. This technique has been used be 91% and better than the 80% we found for 73% of the cases operated on in the past for the selected group in our study. four years. Nine patients (4-8%) required It is considered that a trial is needed to additional posterior decompression for co- compare the results ofAPLD with conservative Poster Presentations existing spinal or root canal stenosis. By the treatment and that a third control group of first postoperative follow up at two to four patients who had neither surgery nor APLD ASYMPTOMATIC CNS ABNORMALITIES IN months 94 5% of patients showed clear neu- would have been valuable in this study. BEHCET'S DISEASE: A MAGNETIC RESONANCE rological or functional improvement, 3% [SBNS] STUDY were unchanged, 1 % were worse, and 1*5 % F Ahmed, JM Bamford, KS Blanshard, BA had died (the deaths were in elderly patients Noble. St James's University Hospital, and with severe myelopathy and intercurrent the General Infirmary, Leeds, UK disease). Minor treatable complications SPINAL MENINGIOMAS: A 20 YEAR REVIEW occurred in 3-2%. Only two patients (1%) AT King, B Doshi, RW Gullan, MM Sharr, Involvement of the CNS occurs in up to one complained of postoperative neck pain. JR Bartlett. Brook General Hospital, third ofpatients with Behcet's disease, desig- Patients were mobilised immediately after London, UK nated as neuro-Behcet's disease. Clinical surgery without a collar and most left hospi- features are either due to cerebral venous tal within one to four days. A single level This is a retrospective study of 78 patients thrombosis or parenchymal pathology. Proceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons 213

Prognosis of neuro-Behcet's disease is exhibiting a broad range of activity; and par- that some of the nerve fibres were not J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from poor and early intervention may modify the ticular effectiveness in more resistant forms conducting. There were no statistically sig- course of the disease. Early diagnosis is, ofidiopathic generalised epilepsy. nificant differences between pre and post- therefore, desired before the development of [ABN] operative values oflatency or duration on the disabling neurological sequelae. Asympto- left or any of the parameters on the right. matic abnormalities on MRI have been Follow up studies showed that in eight of reported but their prevalence is unknown. nine nerves with unobtainable responses The results of the initial assessment of a MAGNETIC RESONANCE SPECTROSCOPY IN postoperatively, the conduction returned in cohort of patients with MRI and angiogram IDIOPATHIC four to seven weeks. The who will PARKINSON'S DISEASE WITH findings suggested be followed prospectively are MOTOR OSCILLATIONS that the amplitude and area of the diaphrag- reported. K Ray-Chaudhuri, G C matic response are more sensitive than Eighteen patients Lemmens, Ellis, fulfilling the Inter- J Dawson, JD Parkes, S Williams, PN Leigh. latency in detecting phrenic nerve paresis national Study Group (ISG) criteria for the King's College School of Medicine and associated with open heart surgery. diagnosis were assessed. None had symp- Dentistry, London, UK [ABN] toms and signs on routine examination to suggest current or previous involvement of The mechanism of motor the nervous fluctuations in system. Their disease activity idiopathic Parkinson's disease (IPD) is not was assessed according to ISG recommenda- clear. The striatal neuronal and biochemical SPONTANEOUS INTRACRANIAL HYPOTENSION tions. alterations that have been in Magnetic resonance reported FROM A CSF LEAK IN A PATIENT WITH angiograms were Parkinson's disease are based mainly on normal in all cases. Thirteen patients had MARFAN'S SYNDROME postmortem, not in vivo studies. MRS was RJ Davenport, SJS Chataway, CP Warlow. normal MRI. Other scans showed high sig- used with regions of interest in the nal intensity in T2 weighted images, either as putamen University of Edinburgh, Western General and cerebellum to investigate eight patients Hospital, Edinburgh, UK single lesions >10 mm or multiple lesions with probable idiopathic Parkinson's disease (5-10 mm) in the cortex and periventricular (mean age 54 on region. (9) years) levodopa treat- A 21 year old girl with known Marfan's syn- ment with motor fluctuations. Results were It is concluded that asymptomatic MR drome first presented in 1992 with a short compared with results with six age matched history of severe postural headaches reminis- abnormalities in patients with Behqet's dis- controls 52 ease are healthy (mean age (10) years). cent of a postlumbar puncture (LP) head- uncommon and are not related to Spectra were collected using a STEAM duration of the disease or its activity in ache. Other than the features ofher Marfan's sequence at an echo time of 136 ms. Scans syndrome the examination was normal. A other systems. Whether these abnormalities were obtained in the "off" rather than "on" predict future neuro-Beh9et's disease is CT brain scan was normal but at LP the condition. opening pressure was unrecordable. She uncertain. There was a [ABN] significant reduction in puta- recovered spontaneously but two years later men NAA/creatine ratios in patients with presented with an identical headache of idiopathic Parkinson's disease (mean 1-104 such severity that she required admission to (SD 0-17)) compared with controls (mean hospital. 1-62 (SD 0-21), P < 0-001). NAA/choline Radioisotope cistemography showed an ratios were also A SURVEY OF THE USE OF LAMOTRIGINE AND lower in the putamen of abnormal accumulation of isotope at the TI VIGABATRIN IN CLINICAL PRACTICE patients with idiopathic Parkinson's disease level suggestive of a CSF leak; CT myelo- D Chadwick, G Schapel. University of (1-106 (SD 0-16)) compared with controls graphy confirmed this leak and multiple Liverpool, Liverpool, UK (1-52 (SD 0 36), P < 0 03). There were no arachnoid diverticulae around the lumbro- significant differences between values for sacral nerve roots were also identified. A survey was undertaken between these ratios in the cerebellum in idiopathic A saline epidural infusion led to an imme- September 1993 and February 1994 that Parkinson's disease compared with controls. diate and dramatic improvement in her identified 356 patients who had been pre- These findings suggest the presence of symptoms. This improvement has been scribed either vigabatrin or lamotrigine since axonal and/or neuronal damage in the puta- maintained after two thoracic epidural auto- men in late the licensing of these compounds from a idiopathic Parkinson's disease. logous blood patches and a subsequent comprehensive database of just over 2200 This may be related either to the disease radioisotope cistemogram has not shown patients with epilepsy attending the Mersey itself or to the effects of long term levodopa any leak. Regional Epilepsy Clinic since 1989. The treatment. It is proposed that the patient's headache http://jnnp.bmj.com/ population had a chronic refractory epilepsy; [ABN] was caused by a low CSF pressure secondary 90% were having more than one seizure per to a CSF leak from the spinal subarachnoid week and two thirds were receiving one drug. space, after rupture of one of the many Two hundred and twenty three patients demonstrated arachnoid diverticulae, which were exposed to lamotrigine for up to 56 A COMPREHENSIVE ELECTROPHYSIOLOGICAL are associated with Marfan's syndrome. The months and 217 patients to vigabatrin for up EVALUATION OF PHRENIC NERVE INJURY rupture may have been either spontaneous or to 72 months. The median maximum dose RELATED TO OPEN HEART SURGERY following minor unrecognised trauma. of Treatment lamotrigine achieved was 400 mg per day E Chroni, RL Patel, N Taub, GE Venn, RS with site specific epidural blood on September 29, 2021 by guest. Protected copyright. and that for vigabatrin was 2 g per day. Howard, CP Panayiotopoulos. St Thomas' patching has proved remarkably successful. Kaplan-Meier survival curves were plotted Hospital, London, UK [ABN] for time to withdrawal of the new drug. The probability of continuing on lamotrigine at Phrenic nerve damage is a widely recognised 40 months was 57% compared with 43% for complication of open heart surgery. A vigabatrin. The commonest adverse event prospective electrophysiological study of AN INVESTIGATION OF DIFFUSE AXONAL causing withdrawal of lamotrigine was skin phrenic nerve function was conducted in 59 INJURY OCCURRING AFTER CLOSED HEAD rash (5% of patients exposed), whereas patients undergoing cardiopuimonary TRAUMA adverse psychiatric consequences were the bypass. The right and left phrenic nerves CA Davie, R Greenwood, IF Moseley, WI commonest reason for withdrawal ofvigaba- were stimulated percutaneously at the neck McDonald, DH Miller. Institute of trin (8% of cases). Ten per cent of patients and the diaphragmatic responses were Neurology, London, UK exposed to lamotrigine became seizure free recorded with surface electrodes placed over compared with 6% exposed to vigabatrin. the 8th intercostal space. The latency, ampli- Pathological studies in patients with severe Most of the patients becoming seizure free tude, duration, and area of the evoked head injury often show changes of diffuse on lamotrigine were those with generalised responses were measured before and after axonal The abnormalities detected epilepsies and there was little shearing. difference the operation. Postoperatively no responses by MRI are often insufficient to explain the between the outcomes for the new drugs in were elicited in two patients bilaterally, in deficits of or partial epilepsies. memory changes in personality five from the left, and in two from the right that can occur. Proton magnetic resonance This survey indicates that there is little dif- phrenic nerve. Comparison ofthe postopera- spectroscopy be useful in ference in the efficacy of these two (MRS) may novel tive with the preoperative group values in the assessing the presence of axonal loss or drugs in the partial epilepsies. The side effect remaining patients showed that the ampli- dysfunction by observing changes in profile of the drugs seems radically different tude and area of the left phrenic nerve were N-acetylaspartate (NAA)-a neuronal as does the range of activity, lamotrigine lower in the postoperative study, indicating marker. MRI and MRS have been carried 214 4Proceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons

out in six patients with a history of severe patients with focal aphasic syndromes. The value. Peripheral nerve phenol injections J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from head trauma and in eight healthy controls. images were analysed with stereological grids have been shown to produce tone changes. MR images of the brain were collected fol- to determine the cross sectional area (A) and This effect may be of functional value in the lowed by long echo proton MRS from a cortical boundary length (B) on each image. management of spasticity. A myometric localised volume of normal appearing white Profile plots of the cross sectional areas (A) paradigm was employed alongside standard matter (NAWM) in the left frontal lobe. In and the complexity parameter B/ A of indi- measures of disability to assess the effect of five patients there was mild cerebral atrophy vidual lobes ofeach hemisphere were obtained. local phenol injection into different sites in with ventricular dilatation and in one The data for patients with focal aphasic 23 patients with spasticity. The amount of patient, more severe cerebral atrophy. There syndromes show distinct anterior or poste- force required to move the selected joint was a significant reduction in the concentra- rior patterns of morphological change in the through a predetermined range was made tion of NAA (median 8-22 mM, range temporal lobes. Interval scans show that sig- before and after injection and at one month 6-5-8-8 mM) from frontal NAWM in the nificant quantitative changes can on occa- after injection. Results showed significant patient group compared with healthy con- sion be precisely determined within a period tone reduction in ankle plantarflexion (n = trols (median 10-44 mM, range 8-76-10-81 of one year. The pattern of the changes has 14, P < 0-001), knee flexion (n = 14, P < mM, P < 0 006). There was no significant been related to the evolving pattern ofneuro- 0-01), leg adduction (n = 9, P < 0-01), difference in the concentrations of creatine psychological deficit. elbow flexion (n = 9, P < 0 05), and finger and choline containing compounds between It is concluded that stereology represents a flexion (n = 2, P < 0-001). The effect was the two groups. Proton MRS may be useful rapid and efficient method of determining maintained at one month after injection at in measuring the extent and degree of axonal morphological data to characterise the pat- the ankle (n = 7, P < 0-001), knee loss and/or dysfunction occurring after tern of atrophic change in cortical disease. (n = 14, P < 0 05), leg adduction (n = 9, P closed head injury. [ABN] < 0-01), and fingers (n = 2, P < 0-05) but [ABN] not at the elbow (n = 7, P = 0-51). Overall activities of daily living scores improved sig- nificantly after injection (n = 23, P < 0-01). INCREASED SOLUBLE VCAM-1 This study confirms the usefulness ofperiph- eral nerve blockade with phenol in the man- AUTOSOMAL DOMINANT DESMINOPATHY CONCENTRATIONS IN CSF OF PATIENTS WITH MULTIPLE SCLEROSIS agement of spasticity using a myometric WITH A DISTAL MYOPATHY AND HEART paradigm and standard measures of overall BLOCK AG Droogan, SA McMillan, JP Douglas, SA DJ Dick, JR Anderson. Norfolk and Norwich Hawkins. Royal Victoria Hospital, Belfast function. Hospital, Norwich, and Addenbrooke's and Belfast City Hospital, Belfast, UK [ABN] Hospital, Cambridge, UK Factors determining the composition and Three members of one family over two gen- temporal evolution of the inflammatory infil- erations were found to have heart block and trate in multiple sclerosis are unknown, but INTEROBSERVER VARIATION IN CLASSIFYING a distal myopathy. Neurological examination the profile of adhesion molecule expression MULTIPLE SCLEROSIS of the index case showed a distal pattern of at the site of the lesion may play a part. HL Ford, MH Johnson, AS Rigby. St muscle weakness with preserved reflexes and Activated cerebral vascular endothelial cells James's University Hospital, Leeds, UK normal sensation. Electromyography, ECG, express leucocyte, vascular cell, and intracel- serum creatine kinase, and an ischaemic lac- lular adhesion molecules (E-selectin, With the advent of new treatments for tic test were normal. A muscle biopsy VCAM-1, and ICAM-1), which facilitate multiple sclerosis, clinicians must be able to showed selective type 2 atrophy and mainly leucocyte adhesion to endothelium and use diagnostic criteria reliably and to define subsarcolemmal eosinophilic plaques of migration into inflammatory lesions. Serum the course of multiple sclerosis.The aim was cytoplasm. Immunohistochemical staining and CSF concentrations of soluble (s) E- to determine if the Poser diagnostic criteria showed that these deposits stained positively selectin, sVCAM-1, sICAM-1 were deter- could be applied consistently in practice and for desmin and to a lesser extent for ubiqui- mined by enzyme linked immunosorbent the course of multiple sclerosis defined. tin, actin, and glycogen. Electromicroscopy assay in 51 patients with clinically definite The case records of 85 consecutive out- of the deposits showed the presence ofglyco- multiple sclerosis, 28 patients with inflam- patients with multiple sclerosis were retro- gen granules and electron dense granular matory neurological disease (IND), and 39 spectively analysed by two independent material, which was often closely associated patients with non-inflammatory neurological observers. The cases were classified: clini- with a smeared Z band. disease (NIND). Concentrations of cally definite multiple sclerosis, laboratory http://jnnp.bmj.com/ These are the muscle biopsy features of a sVCAM-1 in CSF were significantly supported definite multiple sclerosis, clini- desminopathy, which in this family is inher- increased in multiple sclerosis (mean (SD), cally probable multiple sclerosis, laboratory ited as an autosomal dominant trait. This is a 18-3 (9 9) ng/ml) compared with IND (11-3 supported probable multiple sclerosis, sus- rare disorder in which there is accumulation (7 3) ng/ml, P= 0-001) and NIND (10-6 pected multiple sclerosis, and unable to of desmin in skeletal muscle and presumably (5-3) ng/ml, P = 0-0001). Serum and CSF classify; the course was defined as early there are similar abnormalities to be found in concentrations of sICAM-1 were normal in relapsing-remitting, benign, secondary pro- cardiac muscle or the conducting system. each group. Serum sE-selectin was de- gressive, primary progressive, and unable to neurofila- creased in multiple sclerosis compared with Desmin is one of the intermediate classify. on September 29, 2021 by guest. Protected copyright. ments and is a structural component of the NIND (45 5 (23-9) ng/ml v 55 0 (19 9) The statistical agreement between the two cytoskeleton of mature muscle. It anchors ng/ml, P = 0 03). In multiple sclerosis, high observers was measured by calculating the myofibrils in the transverse plane and to the CSF concentrations of sVCAM- 1 and K statistic. There was substantial agreement sarcolemma. The mechanism by which sICAM- 1 correlated with the presence of in classifying the cases (K = 0-65, 95% CI = desmin accumulates in this condition is not CSF oligoclonal bands (P = 0 004 and P = 0 52-0-78). The main disagreement lay in clear. 0-001 respectively). Measurement of soluble defining attacks of multiple sclerosis (K = Previously reported cases have been VCAM-1 in CSF therefore seems to provide a 0 30, 95% CI = 0-03-0 57). There was mainly sporadic and if cardiac involvement marker ofdisease activity in multiple sclerosis. moderate agreement for determining the occurred, it took the form of cardiomyo- [ABN] course of multiple sclerosis (K = 0-57, 95% pathy. CI = 0-43-0-71). The observers differed in [ABN] their definition of primary progressive and secondary progressive cases. This may be PERIPHERAL NERVE BLOCKADE WITH PHENOL due to the poor documentation of relapses IN SPASTICITY: A MYOMETRIC AND and remissions early in the disease course. A METHOD OF CHARACTERISING CEREBRAL FUNCTIONAL ASSESSMENT The efficacy of new treatments is being ATROPHY S Duckworth, N Jordan. Hunters Moor judged on relapse rate. Consensus definition M Doran, N Roberts, RHT Edwards. Regional Rehabilitation Centre, Newcastle- of an attack of multiple sclerosis is there- University of Liverpool, Liverpool, UK upon-Tyne, UK fore essential. Each attack should be well documented, as should subsequent clin- The aim was to evaluate the patterns and Spasticity remains a significant cause of ical improvement. The definition must be morphological characteristics of cerebral neurological disability. Oral pharmacological rigorously adhered to to allow consistent atrophy using volumetric MRI. treatment is associated with unwanted side application of diagnostic criteria in practice. Volumetric MRI was obtained from effects. Selective tone management is of [ABN] P-roceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons 215

ANTI-PURKINJE CELL CYTOPLASMIC mated lesion contouring technique was the of children had a hospital outpatient atten- J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from ANTIBODIES. ARE THEY NON-SPECIFIC most reproducible and should therefore be dance in the past year compared with only MARKERS OF CEREBELLAR CELL DAMAGE? considered for use in treatment trials. 31% of adults; children were also signifi- A Gibson, M Hadjivassiliou, A Milford- Further studies are needed to assess sensitiv- cantly more likely to have had diagnostic Ward, GAB Davies-Jones. Royal Hallamshire ity to changes in lesion load over time. investigations. and Northern General Hospitals, Sheffield, [ABN] The largest single cost to the health service UK was the drug cost, representing around 40% of the total; inpatient costs also accounted Anti-Purkinje cell antibodies that recognise for a sizeable proportion of costs. Uptake of discrete antigenic components within LNK, A NEW CLASS OF PROTEIN KINASE: HIGH medical services was significantly higher Purkinje cell cytoplasm have been desig- EXPRESSION LEVELS IN GLIOMAS AND DURING among patients with frequent seizures-for nated "anti-Yo" and shown by some authors HUMAN NERVOUS SYSTEM DEVELOPMENT example, mean number of inpatient admis- to be strongly associated with paraneoplastic NJ Gutowski, R Ludwig, C Proschel, M-J sions was 1-6 among adults with frequent cerebellar degeneration. The association is Blouin, M Noble. Ludwig Institute for seizures, compared with 0-01 among those not universally accepted and may merely Cancer Research, London, UK seizure free. People with frequent seizures reflect Purkinje cell damage. also had a much higher uptake of state bene- We have used an immunofluorescent Protein kinases that include growth factor fits. Improvements in the management of method, with monkey cerebellar slices as receptors are central in mediating division epilepsy may improve seizure control, which substrate, to detect the presence of a and differentiation in the nervous system is the key to reducing the direct and indirect Purkinje cell cytoplasmic antibody in and may also be important as cell lineage costs ofthis condition. patients with and without cerebellar signs as markers. [ABN] well as a group of age and sex matched From the cDNA library of a human healthy controls. glioblastoma multi forme cell line of the A total of 60 ataxic patients with mean age oligodendrocyte type 2 astrocyte (0-2A) lin- 49 years and a male to female ratio of unity eage a new protein kinase LNK was cloned. WEEKLY V MONTHLY BRAIN MRI FOR were compared with 28 neurological controls LNK represents a new class of protein DETECTION OF DISEASE ACTIVITY IN without cerebellar signs and 50 healthy con- kinase. Although it contains all conserved MULTIPLE SCLEROSIS trols with mean age 50 years and the same motifs in the kinase domain it has low overall HM Lai, T Hodgson, DG MacManus, male to female ratio. The proportions of amino acid homology to other kinases in this S Webb, WI McDonald, DH Miller. cytoplasmic antibody positives in the three domain. It also has two LIM domains in the Institute ofNeurology, London, UK groups were 40/60, 1/28, and 4/50 respec- amino terminal region possibly indicating a tively. Using analysis of proportions shows transcriptional role. By northern analysis on In therapeutic trials, MRI at monthly inter- that these differences are significant at the total RNA, the LNK 3.3 kb band has been vals is often used to evaluate efficacy. At such 0-001 level or less. found to be upregulated in many glioblas- an interval, 80% of new lesions on T2 It remains to be seen if the antibody toma primary cell populations. In the mouse weighted images enhance, and conversely, described here is the same as that described the highest expression of murine LNK is about 50% of new gadolinium enhancing elsewhere but further characterisation of the found in the nervous system. Expression of lesions are not associated with clear changes antigenic determinant with western blot LNK has been found in the developing on the T2 weighted image. Weekly T2 and analysis should resolve this issue. human nervous system by in situ hybridisa- gadolinium enhanced brain MRI were per- [ABN] tion. formed for three months in three patients These findings may be consistent with with clinically active relapsing progressive LNK having a regulatory role in human multiple sclerosis. CNS development, in gliomas, and also as a On weekly MRI 38 new enhancing lesions EVALUATION OF A COMPUTER ASSISTED cell lineage marker. were seen. On monthly scans there were 33 QUANTIFICATION OF MULTIPLE SCLEROSIS [ABN] new enhancing lesions but only 13 new T2 LESIONS IN CRANIAL MRI lesions. Twenty five lesions were followed up J Grimaud, M Lai, JW Thorpe, P Adeleine, from when they first enhanced until they had D Plunmmer, G Barker, PS Tofts, WI stopped enhancing. Of these 11 (44%) McDonald, DH Miller. Institute of UPTAKE OF SERVICES FOR EPILEPSY AND THE enhanced for less than four weeks (one to

Neurology, London, UK COSTS OF CARE three weekly scans). All new T2 lesions http://jnnp.bmj.com/ A Jacoby, G Baker, D Chadwick. showed initial enhancement. Several computer assisted techniques for Universities of Newcastle-upon-Tyne and Monthly gadolinium enhanced MRI is measuring multiple sclerosis lesion volume Liverpool, UK only slightly less sensitive than weekly scan- on MRI have been developed to provide an ning in detecting disease activity, and is still objective, quantitative, and sensitive means Epilepsy is a common neurological condition the preferred frequency for monitoring treat- for monitoring disease activity, particularly and it is estimated that in the United ment as it is easier to perform. It seems from in the context of treatment trials. We have Kingdom there are presently around 300000 weekly studies that virtually all new T2 evaluated three techniques: manual tracing people with active epilepsy. The resource lesions undergo an initial phase of enhance- (similar to that of the North American,B implications of caring for these patients are ment-as this may occasionally be as short as on September 29, 2021 by guest. Protected copyright. interferon trial), automated lesion contour- likely, therefore, to be significant. A recent one to two weeks, it is sometimes missed on ing (local lesion based threshold) and inten- community study in one health region in the monthly studies. sity based thresholding for the whole brain. United Kingdom has examined the uptake of [ABN] Contiguous 5 mm thick axial proton density medical services among people with epilepsy images ofthe brain were obtained on a 1-5 T and calculated the direct and indirect costs MR imager in eight patients with definite of their care. Patients included in the study multiple sclerosis. Analysis of the scans were were identified through the medical records performned twice, independently by three of a random sample of general practitioners X LINKED DOMINANT HEREDITARY MOTOR operators, using the three different tech- in the region. The pattern of care was very AND SENSORY NEUROPATHY WTH CX32 niques. different for adults and children, reflecting in MUTATION The table shows the results. The auto- part the natural history of epilepsy: thus 86% BRF Lecky, PK Thomas, S Malcolm. Walton Centre for Neurology and Neurosurgery, Liverpool, Royal Free Hospital School of Medicine, and Institute of Child Health, London, UK Intraobserver agreement Interobserver agreement Three generations of a family are described Techniques Min Max Mean (SD) Min Max Mean (SD) with hereditary motor and sensory neuro- Manual 0-4 18-5 9 0 (5-2) 4 9 21-7 11-0 (5-8) pathy (HMSN). Inheritance is consistent Contouring 0-1 7-7 2-48 (2-1) 1-8 6-6 4-5 (1-6) with X linked dominant transmission, which Thresholding 0-2 22 7-5 (6 9) 2-8 19-2 11-4 (4-9) is also suggested by the more severe clinical involvement of affected males. The onset of Coefficients ofvariation ofthe measurement techniques (0 0 = perfect reproducibility). symptoms is in early adolescence with distal 216216Proceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons wasting and weakness. A prominent symp- Patient 1, aged 47 had 18 months pain United Medical and Dental Schools and St J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from tom, not characteristic of HMSN types I or and wiggling of the right little toe. Injection Thomas' Hospital, London, UK II, is of painful cramping of the extremities of 150-250 MU Dysport three monthly into both on exercising affected muscles and abductor digiti quinti and the fourth It has been suggested that the postpolio syn- spontaneously at night. Disability in affected interosseous muscle has greatly improved drome may be due to persistence or reactiva- females is not severe whereas affected males involuntary movements and pain since 1989. tion of poliovirus infection in the CNS. In have considerable difficulty with both man- Patient 2 aged 50 had five years of wig- view of this we decided to search for ual function and gait. gling movements of all toes and severe pain poliovirus and other enteroviruses in the Nerve conduction studies show reduced in the left foot and lower leg. Infiltration of CNS of patients with previous poliomyelitis or absent sensory action potentials with mild 500 MU Dysport through the plantar fascia to determine whether or not these viruses to moderate slowing of motor conduction controlled movements for six months. Pain persist in the CNS in humans. Cerebrospinal velocities, although not to the extent usually cleared for one month, and for a further year fluid and peripheral blood was studied using found in HMSN type I. was reduced with an additional transcuta- a nested polymerase chain reaction for the The gene for the gap junction protein con- neous nerve stimulator. Further BTx-A presence of enterovirus RNA. Thirty nexin 32 is located on the long arm of the X injections controlled the movements but not patients have been studied. Enterovirus chromosome. This group of proteins is the pain. RNA was detected in CSF from one patient, known to connect the cytoplasmic compart- Patient 3, aged 37 had 18 years of pain in CSF and lymphocytes from a second ments of cells. Their precise role is not and movements of all toes and both feet. patient, and in serum only from a third known in neural tissue but Cx32 occurs in Dysport (250 MU) infiltrated into each foot patient. By contrast enterovirus RNA was mammalian peripheral nerve at the nodes of as for patient 2 suppressed the movements, not detected in 30 CSF samples from control Ranvier and in Schmidt-Lanterman but gave only two weeks of pain relief, and patients without evidence of infectious dis- incisures. Several different point mutations adjunctive measures were unsuccessful. ease. Four patients with previous poliomyeli- of Cx32 have been described. The present Booster doses of 300 MU/foot added no tis died from unrelated causes during the family shows a methionine for valine sub- benefit. study. Fresh postmortem samples were avail- stitution at position 139. More studies are needed, but BTx-A may able from three and enterovirus RNA was [ABN] control the movements and sometimes the detected in spinal cord tissue from two pain in patients with this syndrome. patients. Nucleotide sequencing studies are [ABN] underway to identify the enterovirus serotypes involved. It is unlikely that all patients in whom enterovirus was detected NEUROPHYSIOLOGICAL EXAMINATION OF had acute infections. If this point is con- RESPIRATORY MUSCLE FUNCTION IN HEADACHES IN THE ACCIDENT AND firmed by further study, these results suggest MYOTONIC DISORDERS EMERGENCY DEPARTMENT: ARE WE that enteroviruses do persist in the CNS of VP Misra, RS Howard, BD Youl. National OVERINVESTIGATING? patients with previous poliomyelitis, as Hospital for Neurology and Neurosurgery, S Moreea, S Goodacre, MH Johnson. recently demonstrated in patients with London, UK St James's University Hospital, Leeds, UK motor neuron disease. Further study is now required to understand the biological and Ventilatory failure in myotonic dystrophy is A prospective study was conducted on all clinical relevance of these findings. associated with weakness of the respiratory patients presenting to the accident and emer- [ABN] and bulbar muscles and a defect in central gency department with a primary complaint control. Previous reports have suggested the of headache. They were followed up after presence of myotonia in the primary and three months to reach a final diagnosis. accessory muscles of respiration but this has In three months, there were 93 cases out not been studied in detail. Four patients with of 21 187 attendances (0 44%). Serious AN ASSESSMENT OF DIETARY AND SERUM ow)-3 myotonia (three with myotonia dystrophica pathology was found in 10 patients: three POLYUNSATURATED FATTY ACIDS AND and one with myotonia congenita) were subarachnoid haemorrhages (3 2%), one ANTIOXIDANTS IN NEWLY DIAGNOSED studied. intracerebral haemorrhage, three meningitis, PATIENTS WITH MULTIPLE SCLEROSIS A concentric needle EMG examination and three cerebral neoplasms. 48 patients HES Mulholland, SA Hawkins, JJ Strain, IS was carried out by a standard technique. The (52%) were seen acutely by a neurologist, 31 Young, MBE Livingstone. Royal Victoria obliquus extemus abdominis, eighth inter- being admitted. This included all cases of Hospital, Belfast, Queen's University, costal, and diaphragm muscles yielded serious pathology except for one neoplasm. Belfast, and University of Ulster, Northern http://jnnp.bmj.com/ myotonic discharges in all patients. 39 patients (42%) described a headache of Ireland Additionally, all patients with myotonia dys- sudden onset, the remaining having a trophica showed abnormally polyphasic headache of gradual onset. Of the first, six There has been a longstanding interest in the motor units of short duration and low ampli- had serious pathology: the three subarach- relation of polyunsaturated fatty acids to the tude at all three loci. noid haemorrhages (7*7%), the intracerebral pathogenesis and treatment of multiple The findings confirm that myotonia may haemorrhage, one neoplasm, and one sclerosis. As soon as patients are given the affect respiratory muscles, including the meningitis. They were among the 18 (46%) diagnosis of multiple sclerosis most make five changes to their eating habits. Many studies diaphragm. In addition, myopathic changes patients admitted from this group. Forty on September 29, 2021 by guest. Protected copyright. are present in the diaphragmatic muscles of patients (48%) were referred by their general in the past, performed on patients with patients with myotonic dystrophy. practitioner. Serious pathology was found in established multiple sclerosis, have shown [ABN] two of the 18 describing a sudden headache low serum linoleic acid. There is little infor- (one neoplasm and one subarachnoid haem- mation on serum fatty acids in newly diag- orrhage) and two of the 27 with a headache nosed patients. The lipid profile and of gradual onset (both neoplasms). In all, 30 antioxidant status in 30 newly diagnosed (32%) had a head CT, eight in the acute patients in the Neurology Department in TREATMENT OF PAINFUL FEET AND MOVING phase. There were seven other subarachnoid Belfast were examined and compared with TOES WITH BOTULINUM TOXIN A haemorrhages presenting differently during controls matched for age, sex, race, socio- AP Moore, J Pilling. Walton Centre for the study period. economic group, and smoking habits. Neurology and Neurosurgery, Liverpool and The results highlight suboptimal use of Dietary intake was assessed using a seven day Norfolk and Norwich Hospital, Norwich, resources. Is there a case for headache cards dietary history and a self administered food UK similar to head injury cards used in the acci- frequency questionnaire. Patients and con- dent and emergency department? trols ate similar diets. Serum and red cell Treatment of an underlying cause may help [ABN] membrane fatty acids were determined by in the syndrome of painful feet and moving gas liquid chromatography. Vitamins A, E, toes, but conventional treatment is often and carotenoids were measured by high per- ineffective in idiopathic cases. Treatment formance liquid chromatography (HPLC). with botulinum toxin A (BTx-A) has not DETECTION OF ENTEROVIRUS RNA IN THE Lipid peroxidation was measured using the been previously reported. We describe three CNS OF PATIENTS WITH PREVIOUS PARALYTIC TBA reaction. No major differences in the women with painful feet and moving toes POLIOMYELITIS blood concentrations of the variables exam- unresponsive to conventional treatment, and P Muir, F Nicholson, J Ajetunmobi, M Li, ined were found. treated with local injections of BTx-A. GT Spencer, RS Howard, JE Banatvala. [ABN] Proceedings ofthe Association ofBritsh Neurologists and the Society ofBritish Neurological Surgeons 217

Hhal POLYMORPHISM IN THE CYP2D6 GENE IS FUNCTIONAL INTEGRITY OF CORTICAL sensible patient counselling, including NOT ASSOCIATED WITH PARKINSON'S DISEASE DYSPLASIA advice about preventative measures, without J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from IN CAUCASIANS MP Richardson, MJ Koepp, DJ Brooks, DR engendering undue alarm. DJ Nicholl, AC Williams, P Bennett, SL Ho, Fish, JS Duncan. Institute of Neurology and [ABN] DB Ramsden. University of Birmingham, MRC Cyclotron Unit, London, UK Birmingham, UK The extent of altered benzodiazepine recep- A genetic association between mutant alleles tor density in focal cortical dysgenesis was FELBAMATE INHIBITS VERATRIDINE INDUCED of the cytochrome P-450 (CYP2D6) gene determined. It was hypothesised that the GLUTAMATE RELEASE FROM MOUSE CORTEX and Parkinson's disease has been reported. area of functional abnormality in cortical J Srinivasan, A Richens, JA Davies. Polymorphisms in this gene, situated on dysgenesis exceeds the structural abnormal- University of Wales College of Medicine, chromosome 22, give rise to the autosomal ity seen on MRI. Cardiff, UK recessive, poor debrisoquine metaboliser "IC-Flumazenil PET was performed to phenotype, and to a more than twofold determine volume ofdistribution (IIC-FMZ- Felbamate, a new anticonvulsant drug, has increased risk of developing Parkinson's dis- Vd) in four patients. These scans were an affinity for the strychnine insensitive ease in Caucasian populations. Recently, a co-registered with high resolution MRI pixel glycine site on the NMDA receptor. This novel mutation, an Hhal polymorphism in by pixel. The extent of dysplasia was defined study compared the effects of felbamate and CYP2D6 exon 6, was shown to confer a five- on the MRI and regions of interest placed on 5,7 dichlorokynurenic acid (5,7 DCKA), a fold increased risk of Parkinson's disease in the abnormality, surrounding cortex, distant potent glycine site antagonist, on glutamate Japanese; we have studied this further in a cortex, and in corresponding areas in the release. Caucasian population where Parkinson's opposite hemisphere. The same set of Glutamate was released from coronal disease is more common. regions of interest was applied to the PET slices of mouse cortex by potassium (60 DNA was analysed by the polymerase and '1C-FMZ-Vd determined. The results mM) or veratridine (20 ,uM). Perfusate was chain reaction (PCR) in 96 patients with were compared with 10 normal controls assayed for glutamate by high performance sporadic Parkinson's disease and 60 con- and expressed as asymmetry indices. liquid chromatography. Basal release of trols, the products digested with Hhal Additionally, each of these same four glutamate was 3-5 pmol/mg tissue/2 min. restriction endonuclease, and the digest pat- patients' 'IC-FMZ-Vd scans were analysed The first pulse of potassium or veratridine tems studied after gel electrophoresis. by Statistical Parametric Mapping (SPM), induced a pronounced (eight to ninefold) Although the overall prevalence of this via automated volumetric normalisation, release of glutamate over basal and the sec- mutant allele was significantly higher in this analysis of covariance (ANCOVA), and ond pulse produced an average release of 90 population than in Japanese (36% v 12%, pixel-by-pixel t test in comparison with 24 (8%) and 67 (6%) respectively of the first X2= 63-2, P < 0 001), there was no statisti- normal controls. pulse. Felbamate (100 pM to 800 pM) had cally significant difference in the distribution The regions of interest based approach no effect on basal or potassium stimulated of the alleles between the patients with showed significant differences between release of glutamate. Veratridine stimulated Parkinson's disease and controls asymmetry indices in patients and controls release was significantly inhibited by felba- (Parkinson's disease: 42 wild type (wt), 41 for the lesion region (P < 0-01) and for the mate in concentrations of 400 pM (39-2 heterozygotes (he), 13 mutant homozygotes surrounding cortex (P < 0 02) but not for (6%), n = 12, P < 0-01) and 800 pM (37 9 (mu); v controls:23 wt, 30 he, 7 mu) (X 2 distant cortex. These findings were con- (5%), n = 8, P < 0-01). 5,7 DCKA (50 to (3 x 2 table) = 0-79; P = 0 68). Therefore, it firmed at the P < 0-00 1 level in SPM, which 200 pM) had no effect on glutamate release. seems that the Hhal polymorphism is not, by additionally showed the anatomical extent of The inhibitory effect of felbamate on glu- itself, ofmajor significance to the aetiology of changes in detail. It was concluded that the tamate release is probably unrelated to its Parkinson's disease in Caucasians. existence of more widespread functional affinity for the glycine site on the NMDA [ABN] compared with structural abnormality may receptor and may be due to inactivation of in part explain the poor outcome of focal sodium channels. resections in patients with focal dysplasia. [ABN] [ABN]

CLINICAL, CSF, AND MRI FINDINGS IN DEVIC'S NEUROMYELITIS OPTICA EUROPEAN COMMUNITY STROKE PROJECT: JI O'Riordan, H Gallagher, DPE Kingsley, NON-FATAL INJURIES IN AN UNSELECTED RESOURCE USE, COSTS, AND OUTCOME OF http://jnnp.bmj.com/ EJ Thompson, WI McDonald, DH Miller. POPULATION OF PATIENTS WITH EPILEPSY DIFFERENT CARE PACKAGES FOR STROKE Institute ofNeurology, London, UK D Smith, GA Baker, A Jacoby, DW J Stewart, R Howard, R Ross Russell, A Chadwick. Walton Centre for Neurology Rudd, C Wolfe. St Thomas' Hospital, Eleven patients with Devic's neuromyelitis and Neurosurgery, Liverpool, UK London, UK optica are described. The mean age of pre- sentation was 36-3 years and 10 patients The excess mortality associated with Stroke imposes a major burden on health were female. Severe deficits in ambulation epilepsy, and the factors responsible for this resources worldwide; in the United and vision were the rule rather than the are well documented. Epidemiological data Kingdom acute stroke care alone costs 4-6% exception with vision reduced to counting indicate that fatal accidents are over- ofthe NHS budget. There is a 10-fold varia- on September 29, 2021 by guest. Protected copyright. fingers or worse in nine patients and six represented as a cause of death in epilepsy. tion in age standardised mortality for stroke remaining wheelchair bound. There was Non-fatal accidents are much more common in people under 65 years across Europe, negative local synthesis of CSF oligoclonal but their incidence, and associated factors, which is not reflected in the incidence rates bands in nine. Two patients had evidence of are not known with any certainty. and therefore may be related to different pat- a connective tissue disorder and six had non- A postal questionnaire has been sent to a terns ofcare for stroke patients. specific increases of various autoantibodies. heterogenous, community based population The EC stroke project has been estab- Ten patients underwent MRI of brain and (n > 1000) ofpatients with epilepsy. In addi- lished to explore the relation between spinal cord. In nine there were diffuse abnor- tion to clinical and demographic details and resource use, costs, and outcome of stroke. malities involving both cervical and thoracic previously validated scales of psychosocial The study involving 24 hospitals from nine cords with extensive cord swelling. The wellbeing, this contains a series of questions European countries, commenced on remaining patient had linear signal change in about injuries; burns, head and dental 1 January 1993. Methodology has been the upper cervical and thoracic cords. A trauma, fractures, and submersion accidents developed to enable standardised data col- brain MRI was normal in six; in four there incurred in the previous 12 months. lection to describe stroke care in terms of were multiple deep white matter lesions. Data are available on 346 adults. Burns type and severity of stroke, hospital admis- Two patients had signal change in the optic and scalds, head injury, dental injury, frac- sion rates, bed type, duration of stay, use of nerves; one with associated swelling. Devic's ture, and submersion accidents are reported tests, amount of rehabilitation, and outcome neuromyelitis optica remains a disorder of in 14%, 18%, 8%, 5%, and 13% respec- at one year. unknown aetiology with poor outcome. tively. Initial resource data on 2438 stroke Thus MRI is a valuable tool in diagnosis, Multivariate analysis, designed to identify patients are reported for the first year. In two aiding the prognostic evaluation by the factors associated with these injuries, are Spanish centres, 81% of patients were physician. being conducted. These data should permit admitted to neurology beds compared with [ABN] estimates of individual risk, which facilitates 0% in two British centres. In one German 21828Proceedings ofthe Association ofBritish Neurologists and the Society ofBritish Neurological Surgeons centre, 94% of patients received brain imag- isolated myelopathy. All had presented with presented. Thirty eight of the 54 living ing, 80% carotid Dopplers, and 36% cerebral symptoms and signs referable to the spinal members of the family were examined; there J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.200 on 1 August 1995. Downloaded from angiography, compared with 30%, 0%, and cord or optic nerves. Eleven had a primary were 11 affected persons of the 26 members 1% respectively in one British centre. It will progressive course, seven relapsing-remitting over the age of 30. Both the severity of the be interesting to correlate this wide variation and only one secondary progressive. paraplegia and the age ofonset (20-50 years) in resource use with outcome at one year. Moderate or severe disability was the rule in varied considerably, with one asymptomatic [ABN] the primary progressive patients whereas the person at the age of 62 years. relapsing remitting patients were minimally One member died of a four year dement- disabled. All had at least one lesion visible in ing illness at the age of 62. Four other the spinal cord. In patients in whom the members of this sibship aged 62-70 had SPINAL MULTIPLE SCLEROSIS: A CLINICAL diagnosis of multiple sclerosis is not sup- neuropsychological examination; all showed AND MRI STUDY ported by abnormalities on brain MRI, evidence ofa dementia. Vocabulary, reading, JW Thorpe, D Kidd, IF Moseley, DAS imaging of the spinal cord can be of consid- and computational abilities were relatively Compston, WI McDonald, DH Miller. erable value. preserved by contrast with a pronounced Institute of Neurology, London and [ABN] deficit in perceptual and constructional University of Cambridge, Cambridge, UK ability. Verbal learning and memory were below expectation but not as dramatically Although MRI detects the white matter impoverished as visual recall. All four pro- lesions of multiple sclerosis within the brain "PURE" AUTOSOMAL DOMINANT SPASTIC files were remarkably similar, worsened with with high sensitivity, a minority of patients PARAPLEGIA WITH LATE ONSET DEMENTIA: A age, and all four tended to use overt compen- have normal brain imaging. Nineteen FAMILY STUDY satory verbal analysis in visuoconstructive patients are described, selected from over S Webb, J Hutchinson, M Hutchinson. St tasks. Magnetic resonance imaging showed a 170 who had undergone brain and spinal Vincent's Hospital, Dublin, Eire generalised atrophy. This dementia is diffi- cord imaging, in whom minimal (n = 9) or cult to classify; it differs from Alzheimer's no (n = 10) brain abnormalities were found. The phenotype of "pure" forms ofhereditary disease in having intact reasoning and rela- Eleven had clinically definite or laboratory spastic paraplegia (HSP) is highly variable. tively preserved verbal skills and is charac- supported definite multiple sclerosis accord- Familial dementia has not been reported in terised by a progressive predominantly ing to the Poser criteria; four had clinically either the pure or complicated forms ofHSP. parietal cognitive loss in the seventh decade. probable disease, and four had progressive A family with autosomal dominant HSP is [ABN] http://jnnp.bmj.com/ on September 29, 2021 by guest. Protected copyright.