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亚急性特发性脱髓鞘性多发性神经根神经病

Subacute idiopathic demyelinating polyradiculoneuropathy.

作者信息

Hughes R, Sanders E, Hall S, Atkinson P, Colchester A, Payan P

机构信息

Department of Neurology, United Medical School, Guy's Hospital, London, United Kingdom.

出版信息

Arch Neurol. 1992 Jun;49(6):612-6. doi: 10.1001/archneur.1992.00530300044009.

Abstract

Seven cases of subacute idiopathic demyelinating polyradiculoneuropathy had a monophasic illness characterized by progressive weakness of all four limbs that evolved during 4 to 8 weeks. Neurophysiological investigations implied demyelination in all seven cases. In two patients, sural nerve biopsy specimens that were taken showed macrophage-associated demyelination. All patients made substantial or complete recoveries with oral prednisolone (four cases) or without treatment (three cases). None of the patients required ventilation or had autonomic complications. These cases provide a link between the acute idiopathic demyelinating form of Guillain-Barré syndrome and chronic idiopathic demyelinating polyradiculoneuropathy.

摘要

7例亚急性特发性脱髓鞘性多发性神经根神经病患者呈单相病程,其特征为四肢进行性无力,在4至8周内进展。神经生理学检查提示所有7例均有脱髓鞘表现。2例患者的腓肠神经活检标本显示有巨噬细胞相关的脱髓鞘改变。所有患者经口服泼尼松龙治疗(4例)或未经治疗(3例)均有显著恢复或完全恢复。所有患者均无需通气支持,也未出现自主神经并发症。这些病例为吉兰-巴雷综合征的急性特发性脱髓鞘形式与慢性特发性脱髓鞘性多发性神经根神经病之间建立了联系。

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