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世界核心医学期刊文摘（神经病学分册） 2005年第4期1卷 49-50页

作者： Ramirez C. De Sè ze J. stojkovic.t. 王晓琳 Servicede Neurologie D Hpital R. Salengro CHRU 59037 LilleCedex France

Introduction. Acute or sub- acute pure dysautonomia is *** report a case of sub- acute pure pandysautonomia with favorable outcome after intravenous immunoglobulin *** report. A 29- year- old right- handed student, with an uneventful medical history presented, for one month, bilateral loss of visual acuity and digestive disorders, associating diarrhea,vomiting and anorexia. Physical examination revealed bilateral intrinsec oculomotor nerve palsy, a dryness syndrome and severe orthostatic hypotension. Ophthalmologic examination showed bilateral diffuse parasympathic impairment associating an Argyll Robertson pupil and full pupil light reflex abolition. Elevated protein level (0.93g/l)was the only cerebrospinal fluid anomaly. Serum tests were negative for anti- gangliosides antibodies. the patient improved slowly after two series of intravenous immunoglobulin infusions. Conclusion. Clinical course and laboratory findings suggest that acute or sub- acute pure pandysautonomia events are likely to be related to acute polyradiculoneuritis. therefore intravenous polyvalent immunoglobulin infusions should be attempted, even if their efficacy needs to be confirmed.

关键词：失调综合征植物神经免疫球蛋白瞳孔对光反射交感神经损伤眼科检查动眼神经麻痹消化功能紊乱体位性低血压干燥综合征

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世界核心医学期刊文摘（神经病学分册） 2005年第3期1卷 60-60页

作者： Delval A. stojkovic.t. DeS`eze J. 赵正卿 Serv. de Neurol. Gen./Pathol. I. H.opital Salengro 59037 Lille Cedex France

Introduction. Polyneuropathies associated with IgM monoclonal gammopathy were recently recognized. Antibodies can react with glycoproteins such as myelin asso ciated glycoprotein (MAG), or gangliosides containing one sialosyl epitope such as GM1 or several sialosyl epitopes (polysialyted gangliosides) including GD2, G D3, Gt1b, Gt1a, GQ1b. Methods. We report on three patients presenting oculomotor dysfunction, chronic sensitive ataxic polyneuropathy, high sedimentation rate, IgM monoclonal paraprotein of unknown signification and antidisialosyl IgM antib odies and for two of them cold agglutinins. Such features have been previously d escribed under the acronym “CANOMAD”(chronic ataxic neuropathy with ophthalmop legia, M protein, agglutination and disialosyl antibodies). Results. One of the patients presents extra membranous glomerulopathy and severe motor disability as sociated with this syndrome. the pathophysiology of the glomerulopathy seems to be linked with the polyneuropathy. Patients were treated either by intravenous i mmunoglobulin, corticosteroids or cyclophosphamid. Response to treatment differs in the three cases and there is currently no consensus. Conclusion. Our study d emonstrates that spectrum of polyneuropathy associated with monoclonal polyneuro pathy may be larger than originally described.

关键词： disialosylated 多发性神经病单克隆蛋白冷凝集反应抗原决定簇神经节苷脂眼球运动障碍共济失调眼肌麻痹肾小球病

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格林-巴利综合征伴幻觉与onirism:被低估的伴随症状(法国)

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世界核心医学期刊文摘（神经病学分册） 2005年第5期1卷 55-55页

作者： Le Rhun E. De Sèze J. stojkovic.t. 王晓琳 Clinique Neurologique Hpital Roger Salengro CHRU de Lille 59037 Lille Cedex France

Introduction. Guillain-Barré Syndrome (GBS) is generally related to perip heral nervous system involvement, but certain variants with central nervous syst em manifestations have been described. Case report. In the present study we repo rt 2 patients with GBS associated with hallucinations and onirism. two men (age 64 and 49 years) presented GBS without proven infectious origin who required int ensive care because of respiratory problems. the disease progressed and manifest ations of encephalitis (hallucinations and onirism) appeared. the sensorimotor s igns and encephalitis manifestations evolved in parallel with full recovery in t he first patient and death after 11 months of intensive care in the second. Conc lusion. GBS may be associated with stereotypic central nervous system symptoms, mimicking delirium tremens. the manifestations would be related to the severity of the initial period, but not to long-term prognosis.

关键词： onirism 精神错乱中枢系统重症监护感觉运动完全康复中枢神经系统进展过程周围神经系统感染源

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