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JOURNAL OF NEUROLOGY

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PSYCHIATRY

Subacute spongiform and its H reflex studies in patients with Parkinson's disease

relation to Jakob-Creutzfeldt disease: report on J. G. MCCLEOD and C. WALSH ...... page 77 six cases Y. GOLDHAMMER, J. J. BUBIS, IDA SAROVA- PINHAS, and J. BRAHAM...... page 1 Cysticercosis of the central with amyotrophic lateral sclerosis: case report and Infantile spongy degeneration of the central review of the literature PAULINE KAHN....page 81 nervous system associated with glycogen storage Melanotic progonoma of the skull I. and markedly fatty liver Y. TAKEI and G. B. D. ANAGNOSTOPOULOS and G. J. H. EVERARD . .page 88 SOLITARE...... page 11 Basilar impression of the skull in patients with Peripheral neuropathy in chronic liver disease: adult coeliac disease and after gastric clinical, electrodiagnostic. and nerve biopsy L. J. HURWITZ and N. K. BANERJI ...... page 92 findings R. P. KNILL-JONES, C. J. GOODWILL, A. D. DAYAN, and ROGER WILLIAMS ...... page 22 The EEG in Huntington's : a clinical and

neuropathological study D. F. scorT, K. W. G. copyright. Biochemical studies in Tanzanian patients with HEATHFIELD, B. TOONE, and J. H. MARGERISON..... ataxic tropical neuropathy w. J. MAKENE and page 97 J. WILSON...... page 31 Return of EEG activity after electrocerebral Serum enzyme levels in patients with myasthenia silence: two case reports JOSEPH B. GREEN and gravis after aerobic and ischaemic exercise JERRY ANDRE LAUBER ...... page 103 KOLINS and JOHN GILROY...... page 34 Released involuntary laughter after temporal lobe http://jnnp.bmj.com/ infarction MICHAEL SWASH ...... page 108 A genetic study of infantile and juvenile myas- thenia gravis SARAH BUNDEY...... page 41 Axial enlargement of the 3rd ventricle, and dis- placement of the brain-stem in benign aqueduct Transmission block in terminal nerve twigs: a stenosis JAN JAKUBOWSKI and ANTONY JEFFERSON. single fibre electromyographic finding in man page 114 ERIC STALBERG and BARBARA THIELE .....page 52 The extensor digitorum brevis: histological and Spinal motor neurones in murine muscular histochemical aspects F. G. 1. JENNEKENS, B. E.

dystrophy and T. A. TOMLINSON, and J. N. WALTON ...... page 124

PAPAPETROPOULOS and W. G. BRADLEY....page 60 on September 27, 2021 by guest. Protected Anterior interbody fusion for cervical osteomye- Effect of voluntary contraction of the masseter and litis A. D. BARTAL, J. SCHIFFER, Y. D. HEILBRONN, other muscles upon the masseteric reflex in man and M. YAHEL ...... page 133 A. G. HANNAM ...... 66 page Proceedings of the Society of British Neurological The muscle silent period and reciprocal inhibition Surgeons ...... page 137

in man G. C. AGARWAL and G. L. GOTTLIEB.page 72 Book reviews ...... page 145

I LRITISH MEDICAL ASSOCIATION- TAVISTOCK SQUARE * LONDON * WCIH 9JR Contents J Neurol Neurosurg Psychiatry: first published as on 1 February 1972. Downloaded from No. 1 FEBRUARY 1972 Page Subacute spongiform encephalopathy and its relation to Jakob-Creutzfeldt disease: report on six cases Y. GOLDHAMMER, J. J. BUBIS, IDA SAROVAPINHAS, and J. BRAHAM ...... Infantile spongy degeneration of the central nervous system associated with glycogen storage and markedly fatty liver Y. TAKEI and G. B. SOLITARE ...... 1 Peripheral neuropathy in chronic liver disease: clinical, electrodiagnostic, and nerve biopsy findings R. P. KNILL- JONES, C. J. GOODWILL, A. D. DAYAN, and ROGER WILLIAMS ...... 22

Biochemical studies in Tanzanian patients with ataxic tropical neuropathy w. J. MAKENE and J. WILSON...... 31 Serum enzyme levels in patients with myasthenia gravis after aerobic and ischaemic exercise JERRY KOLINS and JOHN GILROY ...... 34 A genetic study of infantile and juvenile myasthenia gravis SARAH BUNDEY...... 41 Transmission block in terminal nerve twigs: a single fibre electromyographic finding in man ERIC STALBERG and BARBARA THIELE ...... 52 Spinal motor neurones in murine muscular dystrophy and spinal muscular atrophy T. A. PAPAPETROPOULOS and W. G. BRADLEY ...... 60 Effect of voluntary contraction of the masseter and other muscles upon the masseteric reflex in man A. G. HANNAM...... 66 The muscle silent period and reciprocal inhibition in man G. C. AGARWAL and G. L. GOTTLIEB ...... 72 H reflex studies in patients with Parkinson's disease J. G. MCLEOD and J. C. WALSH ...... 77 Cysticercosis of the central nervous system with amyotrophic lateral sclerosis: case report and review of the literature PAULINE KAHN ...... 81 Melanotic progonoma of the skull D. I. ANAGNOSTOPOULOS and G. J. H. EVERARD ...... 88 Basilar impression of the skull in patients with adult coeliac disease and after gastric surgery L. J. HURWITZ and N. K. BANERJI ...... 92 The EEG in Huntington's chorea: a clinical and neuropathological study D. F. SCOTT, K. W. G. HEATHFIELD, B. TOONE, and J. H. MARGERISON ...... 97

Return of EEG activity after electrocerebral silence: two case reports JOSEPH B. GREEN and ANDRE LAUBER... 103 Released involuntary laughter after temporal lobe infarction MICHAEL SWASH...... 108 Axial enlargement of the 3rd ventricle, and displacement of the brain-stem in benign aqueduct stenosis JAN copyright. JAKUBOWSKI and ANTONY JEFFERSON ...... 114 The extensor digitorum brevis: histological and histochemical aspects F. G. I. JENNEKENS, B. E. TOMLINSON, and J. N. WALTON ...... 124 Anterior interbody fusion for cervical osteomyelitis A. D. BARTAL, J. SCHIFFER, Y. D. HEILBRONN, and M. YAHEL 133 Proceedings of the Society of British Neurological Surgeons ...... 137

Book reviews ...... 145 http://jnnp.bmj.com/

No. 2 APRIL 1972 Effects of hyperkalaemia on the excitability of peripheral nerve K. N. SENEVIRATNE, 0. A. PEIRIs, and A. WEERASURIYA ...... 149 Permeability of blood nerve barriers in the diabetic rat K. N. SENEVIRATNE ...... 156

Conduction in unmyelinated fibres in experimental neuropathy A. P. HOPKINS and E. H. LAMBERT...... 163 on September 27, 2021 by guest. Protected Polyneuritis and herpes zoster A. D. DAYAN, E. OGUL, and G. S. GRAVESON ...... 170 Observations on cardiovascular and neuroendocrine disturbance in the Guillain-Barre syndrome A. G. DAVIES and H. R. DINGLE ...... 176 Subacute sclerosing panencephalitis as a cause of chronic and relapsing brain disorder M. DONNER, 0. WALTIMO, J. PORRAS, H. FORSIUS, and A.-L. SAUKKONEN ...... 180 Treatment of involuntary movement disorders with tetrabenazine MICHAEL SWASH, A. H. ROBERTS, HAZIM ZAKKO, and K. W. G. HEATHFIELD ...... 186 Contrasting patterns of memory span decrement in ageing and aphasia MARCEL KINSBOURNE ...... 192 Russell's diencephalic syndrome of early childhood MICHAEL A. SALMON ...... 196 Myopathy in association with primary hyperaldosteronism M. A. SAMBROOK, J. R. HERON, and G. M. ABER .... 202 X-linked scapuloperoneal syndrome P. K. THOMAS, D. B. CALNE, and C. F. ELLIOTT...... 208 Quantitative electromyography: a new analogue technique for detecting changes in action potential duration A. MOOSA and B. H. BROWN ...... 216 Muscle afferent potential ('A-wave') in the surface electromyogram of a phasic stretch reflex in normal humans ALEX M. CLARKE, PATRICIA T. MICHIE, and LEONARD C. T. GLUE ...... 221 Orbicularis oculi reflex in the Wallenberg syndrome: alteration of the late reflex by lesions of the spinal tract and nucleus of the trigeminal nerve JUN KIMURA and LYNN W. LYON ...... 228 Contents Page Habituation: effects of regular and stochastic stimulation M. R. DIMITRIJEVIC, J. FAGANEL, M. GREGORIC, P. W. J Neurol Neurosurg Psychiatry: first published as on 1 February 1972. Downloaded from NATHAN, and J. K. TRONTELJ ...... 234 Pneumoencephalo-roulette tomography of operated primary pontine haemorrhage with long survival: report of two cases M. KOWADA, K. YAMAGUCHI, Z. ITO, and S. MATSUOKA ...... 243 Injury of the basilar artery associated with closed head trauma CHENG-MEI SHAW and ELLSWORTH C. ALVORD, Jr. 247 Vascular responses in the hands of patients suffering from J. A. DOWNEY and D. B. FREWIN ...... 258 Experiences with the technique and complications of meglumine iothalamate (Conray) ventriculography SrEPHEN E. NATELSON, MARTIN P. SAYERS, and WILLIAM E. HUNT ...... 264 Average evoked responses and psychophysical performance in patients with pseudohypoparathyroidism MONTE BUCHSBAUM, CATHY KING, and ROBERT r. HENKIN ...... 270 Book reviews...... 277

No. 3 JUNE 1972 Raised and cerebral blood flow: 1. Cisterna magna infusion in primates I. H. JOHNSTON, J. 0. ROWAN, A. M. HARPER, and W. B. JENNETT ...... 285 Extradural haemorrhage-a hazard of ventricular drainage R. P. SENGUPTA and JOHN HANKINSON ...... 297 Cerebellar haematomas caused by angiomas in children G. ERENBERG, R. RUBIN, and K. SHULMAN ...... 304 Neurogenic muscle atrophy in myasthenia gravis BETTY BROWNELL, D. R. OPPENHEIMER, and J. M. K. SPALDING. 311 Neuropathy after alcohol withdrawal A. J. GARDNER ...... 323 Electrophysiological studies in alcoholism EILEEN BLACKSTOCK, GEOFFREY RUSHWORTH, and DENNIS GATH ..... 326 Nerve conduction during Wallerian degeneration in the baboon R. W. GILLIATT and R. J. HJORTH ...... 335 Effect of magnesium on nerve conduction velocity during regular dialysis treatment LAURA W. FLEMING, J. A. R. LENMAN, anld W. K. STEWART ...... 342

Stereotactic hypothalamotomy for behaviour disorders J. R. SCHVARCZ, R. DRIOLLET, E. RIOS, and 0. BETTI.... 356 A preclinical case of late adult metachromatic leukodystrophy? H. PILZ and H. C. HOPF ...... 360 Clinical manifestations of essential EDMUND CRITCHLEY ...... 365 Levodopa in : reduction in the electromyographic silent period and its relationship with tremor D. L. MCLELLAN ...... 373

Hereditary persistent distal cramps ANICA JUSIC, S. DOGAN, and v. STOJANOVIC ...... 379 copyright. associated with defects in neuromuscular transmission BERNARD M. PATTEN, AVERY HART, and ROBERT LOVELACE ...... 385 Depressed skull fracture: data, treatment, and follow-up in 225 consecutive cases R. BRAAKMAN ...... 395 Some aspects of the pathology and pathogenesis of the myelopathy caused by disc protrusions in the dog i. R. GRIFFITHS ...... 403 Stuttering: an investigation into cerebral dominance for speech GAVIN ANDREWS, P. T. QUINN, and W. A. SORBY 414 Book reviews ...... 419 http://jnnp.bmj.com/

No. 4 AUGUST 1972 Arthrogryposis multiplex congenita Part I: Clinical and electromyographic aspects E. P. BHARUCHA, S. S. PANDYA, and DARAB K. DASTUR ...... 425 Arthrogryposis multiplex congenita Part 2: Muscle pathology and pathogenesis DARAB K. DASTUR, ZOHRA A. RAZZAK, and E. P. BHARUCHA ...... 435

Structural changes in the early stages of Duchenne muscular dystrophy w. G. BRADLEY, P. HUDGSON, P. F. on September 27, 2021 by guest. Protected LARSON, T. A. PAPAPETROPOULOS, and M. JENKISON...... 451 Study of infantile with combined air and isotope ventriculography DOV FRONT, WILLEM J. OVERBEEK, and LOURENS PENNING ...... 456 Enlargement of the Sylvian aqueduct: a sequel of head injuries FRANCOIS C. BOLLER, MARTIN L. ALBERT, MARJORIE LeMAY, and ANDREW KERTESZ...... 463 Dynamics of the and the spinal dura mater ALBERT N. MARTINS, JOHN K. WILEY, and PAUL W. MYERS...... 468 Cerebrospinal fluid ascites: a complication of a ventriculoperitoneal shunt DAVID F. DEAN and IRVIN B. KELLER 474 Tonic vibration reflex in spasticity, Parkinson's disease, and normal subjects DAVID BURKE, COLIN J. ANDREWS, and JAMES W. LANCE ...... 477 Response of patients with postencephalitic Parkinsonism to levodopa ROGER C. DUVOISIN, JOAO LOBO-ANTUNES, and MELVIN D. YAHR ...... 487 Effect of tyramine in migraine: a double-blind study ADRIENNE MOFFETT, MICHAEL SWASH, and D. F. SCOTT.... 496 A trial of opipramol in the treatment of migraine HARRY JACOBS ...... 500 5-Hydroxytryptamine levels and platelet aggregation responses in subjects with acute migraine BARBARA P. HILTON and J. N. CUMINGS ...... 505 Huntington's chorea and tryptophan W. R. MCLEOD and D. J. de L. HORNE.510 Contents Page Amine metabolites in the cerebrospinal fluid in Huntington's chorea G. CURZON, JOHN GUMPERT, and DAVID J Neurol Neurosurg Psychiatry: first published as on 1 February 1972. Downloaded from SHARPE ...... 514 Effect of dexamethasone on experimental cerebral infarction in the gerbil M. J. G. HARRISON and R. W. ROSS RUSSELL ...... 520 Extracranial-intracranial bypass grafts G. KHODADAD...... 522 Rapid formation of giant aneurysm: case report LARRY C. FRIED and ANUNCIACION YBALLE ...... 527 Anterior interosseous nerve syndrome M. D. O'BRIEN and A. R. M. UPTON...... 531 Impairment of repetitive impulse conduction in experimentally demyelinated and pressure-injured nerves FLOYD A. DAVIS...... 537 Qualitative analysis of unilateral spatial neglect in relation to laterality of cerebral lesions G. GAINOTTI, P. MESSERLI, and R. TISSOT ...... 545 Primary cerebral rhabdomyosarcoma and the problem of medullomyoblastoma P. w. LEEDHAM...... 551 Syringomyelia in association with a neurofibroma of the filum terminale M. R. GOODING...... 560 Book reviews...... 565

No. 5 OCTOBER 1972 Isotope encephalography in the management of acute herpesvirus A. S. BLIGH, C. M. WEAVER, and C. E. C. WELLS ...... 569 Orbicularis oculi reflex in coma: clinical, electrophysiological, and pathological correlations LYNN W. LYON, JUN KIMURA, and WILLIAM F. MCCORMICK ...... 582

Voluntary and reflex control of the biceps brachii muscle in spastic-athetotic patients PETER D. NEILSON...... 589 Assessment of cyclobenzaprine in the treatment of spasticity PETER ASHBY, DAVID BURKE, SUDHAKAR RAO, and RICHARD F. JONES...... 599 Position sense and state of contraction; the effects of vibration G. EKLUND...... 606 Digital nerve action potentials in healthy subjects, and in carpal tunnel and diabetic patients E. B. CASEY and PAMELA M. LE QUESNE ...... 612 Electrophysiological evidence for a distal lesion in alcoholic neuropathy E. B. CASEY and PAMELA M. LE QUESNE 624 Effect of ischaemia on peripheral nerve function in patients with chronic renal failure undergoing dialysis treat-

ment P. CASTAIGNE, H.-P. CATHALA, L. BEAUSSART-BOULENGE, and M. PETROVER ...... 631 copyright. Recovery of nerve conduction after a pneumatic tourniquet: observations on the hind-limb of the baboon T. J. FOWLER, G. DANTA, and R. W. GILLIATT ...... 638 Pathology of peroneal muscular atrophy (Charcot-Marie-Tooth disease) J. TREVOR HUGHES and BETTY BROWNELL 648 Lipid changes in Duchenne muscular dystrophy B. P. HUGHES ...... 658 Fistula detection in cerebrospinal fluid leakage MARSHALL B. ALLEN, Jr., TAHER EL GAMMAL, MENARD IHNEN, and MORGAN A. COWAN ...... 664 http://jnnp.bmj.com/ Porencephaly diagnosed by isotope cisternography D. FRONT, J. W. F. BEKS, and L. PENNING ...... 669 Spontaneous cerebral ventriculostium: two cases G. C. KANJILAL...... 676 Effect of a delayed-action phenytoin preparation on blood phenytoin concentration F. BOCHNER, W. D. HOOPER, J. H. TYRER, and M. J. EADIE ...... 682 A case of GM2 gangliosidosis of late onset P. BUXTON, J. N. CUMINGS, R. B. ELLIS, B. D. LAKE, W. G. P. MAIR, J. R. ROBERTS, and E. P. YOUNG ...... 685 Rathke's cleft cyst STEVEN P. RINGEL and ORVILLE T. BAILEY ...... 693 WILLIAM G. DENNYSON ...... 698 Delayed traumatic intracerebral haemorrhage GOPAL BARATHAM and on September 27, 2021 by guest. Protected Alzheimer's disease in one monozygotic twin RICHARD HUNTER, A. D. DAYAN, and JOHN WILSON...... 707 Neurological abnormalities in the 'cri-du-chat' syndrome JACK COLOVER, MARY LUCAS, J. A. COMLEY, and A. M.ROE...... 711 Cerebrospinal fluid choline in extrapyramidal disorders S.-M. AQUILONIUS, B. NYSTROM, J. SCHUBERTH, and A. SUNDWALL ...... 720 Cerebrospinal fluid creatine kinase in acutely psychotic patients WILLIAM A. MARTIN, RICHARD E. GAREY, and ROBERT G. HEATH ...... 726 Blood viscosity in multiple sclerosis G. W. SCOTT BLAIR and R. H. MATCHETT...... 730 Malignant of the brain-stem GERALD S. GOLDEN, NITYA R. GHATAK, ASAO HIRANO, and JOSEPH H. FRENCH 732 Ocular bobbing and opsoclonus H. G. BODDIE ...... 739 Book reviews ...... 743

No. 6 DECEMBER 1972 Progressive cerebral poliodystrophy-Alpers' disease U. SANDBANK and P. LERMAN...... 749 Polygraphic sleep study in craniopagus twins H.-CG. LENARD and F. J. SCHULTE ...... 756 Non-communicating supratentorial subarachnoid cysts Y. S. BHANDARI ...... 763 Lumbar intraspinal extradural ganglion cyst ADAM BRISH and HUSHONG M. PAYAN.771 Coniteiits Page Tumours of the third ventricle in children BENNETT M. STEIN, RICHARD A. R. FRASER, and MICHAEL S. TENNER. 776 J Neurol Neurosurg Psychiatry: first published as on 1 February 1972. Downloaded from Evidence of genetic heterogeneity in Huntington's chorea D. C. WALLACE and A. C. HALL ...... 789 Myotonic dystrophy: investigation of the proposed defect in guanidoacetic acid synthesis C. E. BOLTON and

A. E. H. EMERY ...... 801 Operative treatment of aneurysms and Coanda effect: a working hypothesis J. L. ROBINSON and A. ROBERTS 804 Small in the carotid territory associated with a normal carotid arteriogram PETER BRADSHAW and JOHN GUMPERT ...... 810 Impairment of memory functions after acute head injury IRIS E. FODOR ...... 818 compression in Paget's disease NIALL E. F. CARTLIDGE, JOHN P. K. MCCOLLUM, and RAM D. A. AYYAR 825 Visual evoked responses in patients with multiple sclerosis NORMAN S. NAMEROW and NELSON ENNS ...... 829 Interval analysis of repetitive denervation potentials of human skeletal muscle B. CONRAD, F. SINDERMANN, and V. J. PROCHAZKA ...... 834 Quantitative electromyography: carrier detection in Duchenne type muscular dystrophy using a new automatic technique ALLIE MOOSA, BRIAN H. BROWN, and VICTOR DUBOWITZ ...... 841 A method for estimating the number of motor units in thenar muscles and the changes in motor unit count with ageing W. F. BROWN ...... 845 Interaction between voluntary contraction and tonic stretch reflex transmission in normal and spastic patients PETER D. NEILSON ...... 853 Nerve potentials and afferent conduction velocities in the differential diagnosis of amyotrophy of the hand ANICA JUSIC.and STANKO MILIC ...... 861 Enzyme response of traumatized tissue after intracortical injection into 5 day old rat brain N. ROBINSON ..... 865 Effect of dosage increments on blood phenytoin concentrations F. BOCHNER, W. D. HOOPER, J. H. TYRER, and M. J. EADIE...... 873 Corneal reflex in hemisphere disease R. T. ROSS...... 877 Subacute spongiform encephalopathy J. J. BUBIS, Y. GOLDHAM\1ER, and J. BRAHAM ...... 881 Pathology of radiation myelopathy R. J. BURNS, A. N. JONES, and J. S. ROBERTSON ...... 888 Vertebral artery occlusion by a cervical 'hour-glass' neurofibroma JAMES D. GEISSINGER, GEORGE GRUNER, and DANIEL RUGE...... 899 Klebsiella -report of nine cases D. J. E. PRICE and J. D. SLEIGH ...... 903 due to Haemophiliis aphrophilius: case report J. YAMASHITA, F. J. BONE, and EDWARD HITCHCOCK 909 copyright. Two-dimensional echoencephalography with electronic sector scanning H. A. C. KAMPHUISEN, J. C. SOMER, and W. A. OOSTERBAAN ...... 912 Proceedings of the Society of British Neurological Surgeons ...... 919 Book reviews ...... 926 http://jnnp.bmj.com/ on September 27, 2021 by guest. Protected