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Proceedings of the Association of Britishneurologists from the Joint 20424ournal ofNeurology, Neurosurgery, anid Psychiatry 1997;62:204-215 Proceedings of the Association of British Neurologists from the J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.62.2.204 on 1 February 1997. Downloaded from joint meeting with the British Society for Clinical Neurophysiology, Charing Cross Hospital, 18-20 September 1996 CRYPTIC COELIAC( D)ISlIASE PRESENTING VI''H intraperitoneal (1 mg/kg, twice daily) or each comprising 32 transverse optical NEUROMUSC'UIAR DYSFUNCTION subcutaneous (10 mg/kg, once daily) routes sections of the optic disc at a depth of 1 5 to A Gibson, M Hadjivassiliou, AK from the time of immunisation, it prevented 4-0. A topographic map is created. A Chattopadhyay, GAB Davies-Jones, Royal the development of the neurological deficits contour is drawn around the swollen disc to Hallamshire Hospital, Sheffield, UK and histological changes observed in vehicle enable comparison of subsequent images to treated controls. Subcutaneous treatment baseline. Eight patients are described who presented from either day 7, or from the onset of the Eight patients were examined regularly with neuromuscular abnormalities of disease, again reduced disease severity. over a six to 12 month period. Five patients obscure aetiology despite thorough BB1 101 did not inhibit the development of with symptomatic improvement on medical investigation. None had evidence of CNS an immune response, as measured by the treatment showed significant reduction in involvement at presentation. All patients had production of antimyelin antibodies. It is papilloedema (neuroretinal rim volume) and antigliadin antibodies in their serum. Distal concluded that MMP inhibitors may have nerve fibre layer thickness. Four of these had duodenal biopsies in all patients fulfilled the potential as therapeutic agents in peripheral resolution of visual field defects; one was histopathological criteria for coeliac disease demyelinating diseases such as GBS. unreliable at perimetry. Three patients with (CD). Patients ranged in age from 18-67 chronic disc swelling and mild field loss (median 62) years and there were five men MNEASURING SINGLE COGNIlIVE EVENTS WITH showed no improvement or worsening of and three women. Neuromuscular disease WHOLE BRAIN FUNCTIONAI. M%lAGNETIC papilloedema and nerve fibre layer had been present for 1-18 (median four) RESONANCE IMAGING thickness; disc changes were not evident years before the diagnosis of CD. M Humberstone, S Clare, J Hykin, PG clinically. Automated Humphrey 300 All patients had nerve conduction studies Morris, GV Sawle, University of perimetry was used. and EMG and two patients had muscle Nottingham, Nottingham, UK This technique is a sensitive measure of biopsy. Three patients had sensorimotor optic disc changes, quantifies swelling, and neuropathy, one had motor neuropathy, one A technique has been developed for is a useful adjunct to perimetry in monitor- had myositis and sensorimotor neuropathy, studying single cognitive events with ing idiopathic intracranial hypertension, and one had myositis and motor functional MRI. The technique uses echo- particularly if perimetry is unreliable. neuropathy. The neuropathic abnormalities planar imaging at a field strength of 3 Tesla Several methods of displaying images are in these six patients were distally and generates one functional image of the demonstrated. predominant and implied axonal pathology. whole brain every three seconds. A signal One other patient had mononeuritis averaging technique was used to create time MANAGING ID)IOPATHIC INTRACRANIAL. multiplex, and one patient had sweeps of functional signal change after a HYPERTENSION: COORDINATED FOLLOW UP IS neuromyotonia. single cognitive event. A t test is used to ESSENTIAI. Cryptic CD may initially present with identify areas and times of significant J Craig, D Mulholland, JM Gibson, Royal neuromuscular dysfunction. CD merits early activation without making any a priori Victoria Hospital, Belfast, UK consideration in patients with assumptions about the spatial or temporal neuromuscular abnormalities of obscure pattern of response; thus allowing both to be The optimum management of patients with http://jnnp.bmj.com/ aetiology, especially if introduction of a characterised. This technique was used to idiopathic intracranial hypertension (IIH) is gluten free diet results in symptomatic analyse functional activity during simple unclear and there is uncertainty on how and improvement. motor tasks in six normal subjects. Widely when to treat visual complications. A distributed patterns of functional activity casenote review was performed to examine appeared transiently 3-6 seconds after a management of patients with IIH (as per AN INHIBI'1'OR OF MAIRIX single externally cued press of a button with modified Dandy criteria), between 1991 and METALLOPROlEIINASE ACTIVITY ANI) T'NFa the right thumb. Activation was seen in 1995 at the Royal Victoria Hospital. Forty RELEASE IS AN lEFF} EC'TIVE I'REATMEN' IN primary and in premotor cortex in all three patients were identified corresponding EXl'ERIMENTAI. AU'TOIMMUNE NEURI'L'IS subjects. The time course of activation can to an average annual incidence rate per on September 27, 2021 by guest. Protected copyright. KJ Smith, EJ Redford, M Davies, N be examined in real time using video. This 100 000 of 0 6 for the total and 0 9 for the Gregson, P Hughes, AJH Gearing, K Miller, technique can be used to analyse and female population. Casenotes in full were RAC Hughes, Guy's Hospital, London, and compare the separate tasks which constitute available for 38 patients (88%). Almost two Neures Ltd, Abingdon, UK a cognitive paradigm. Comparison between thirds had presented to the ophthalmology decisions to move and decisions not to move department, the most commonly reported The factors involved in the genesis of the in a "go", "no-go" paradigm discloses symptoms being headache (81%), transient inflammatory autoimmune demyelinating hierarchical organisation of the mesial visual obscurations (65%), and sustained disease Guillain-Barre syndrome (GBS) premotor cortex and identifies "decision visual loss (34%). Analgesia regularly remain poorly understood, although there is making" and "motor execution" areas. prescribed in 63% of patients was effective evidence that the pro-inflammatory cytokine in none, acetazolamide produced tumour necrosis factor-a (TNFa) may be MONIT1'ORING PAPILLOEDEMA WITH symptomatic improvement in 44%, and involved. Because TNFa release is CONFOCAL. IASER TOMOGRAPHY IN CSF opening pressure improvement in 22%. dependent upon a metalloproteinase IDIOPA'I'HIC IN'I'RACRANIAI. HYPER'I'ENSION Repeated lumbar puncture, although (MMP)-like enzyme, and since the DA Mulholland, J Craig, JM Gibson, SJA offering acute symptomatic relief (58%), did extracellular degrading MMPs are also Rankin, Royal Victoria Hospital, Belfast, not prevent visual deterioration. Seven implicated in the pathogenesis of UK patients (18%) underwent thecoperitoneal autoimmune disease, it was examined shunting to preserve vision or to control whether an inhibitor (BB1 101) of this class This study measured the efficacy of confocal headache. Visual acuity deteriorated in two of enzymes is effective in the therapy of an laser tomography at assessing optic disc (5%), visual fields in seven (18%), and two animal model of GBS. Experimental allergic swelling in patients with idiopathic (5%) developed optic atrophy despite neuritis (EAN) was induced in 116 Lewis intracranial hypertension. Eight patients therapy. Almost two thirds were solely rats using bovine peripheral nerve myelin. with papilloedema were studied. At every followed up by neurologists, only 50% When BB 1 101 was administered bv examination three images were acquired, having more than one formal visual field Proceedings 205 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.62.2.204 on 1 February 1997. Downloaded from assessment in the year after diagnosis. Visual subsequent X was significantly impaired for in the forearm flexor muscles. Magnetic functioning as measured by visual fields and > 1600 ms afterwards. Age-matched control brain stimulation, somatosensory evoked visual acuity, rather than by repeated CSF subjects showed a normal attentional blink potentials, and stretch reflex studies did not pressure monitoring, should guide therapy. which was significantly less protracted and provide evidence for delayed conduction Until IIH is better understood and less profound than patients. within central pathways. Forearm stretch controlled, and trials analysing treatment This investigation demonstrates an reflexes were present but their latencies were options are available, coordinated impairment of temporal components of prolonged. Somatosensory evoked potentials neurological and ophthalmological follow up attention in visuospatial neglect. were obtained in most patients but were is mandatory. delayed. Wrist tremor could be modulated DIFFERENCES IN DECISION MAKING BETWEEN by mechanical perturbations or median INVASIVE MENINGOCOCCAL DISEASE IN UNITED KINGDOM AND UNITED STATES nerve electrical shocks. Simple voluntary PLYMOUTH NEUROLOGISTS wrist movements were of normal duration T Ahmed, AY Al-Memar, D Dance, R PJ Martin, B Vickrey, D Gifford, S and peak velocity, but the kinematic profile Hopkins, S Harrison, I Bray, DC Thrush, Delrahim, T Belin, D Smith, DW was asymmetric. Each movement was Derriford Hospital and University of Chadwick, Walton Centre for Neurology associated with a triphasic EMG
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