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一项关于伴有共济失调和感觉异常的慢性感觉性神经病的临床、电生理、形态学及免疫学研究。

A clinical, electrophysiological, morphological and immunological study of chronic sensory neuropathy with ataxia and paraesthesia.

作者信息

Mamoli A, Nemni R, Camerlingo M, Quattrini A, Castro L, Lorenzetti I, Canal N

机构信息

Neurology Division, Riuniti Hospital, Bergamo, Italy.

出版信息

Acta Neurol Scand. 1992 Feb;85(2):110-5. doi: 10.1111/j.1600-0404.1992.tb04008.x.

Abstract

We have observed 9 patients (8 men and 1 woman), 58 to 77 years of age with neuropathy with only sensory symptoms and insidious onset. Five of them (4 men and 1 woman) aged 65 to 77 years, had normal serum electrophoretic profiles, while the others (all men), 58 to 74 years, had IgM monoclonal gammopathy of undetermined significance (MGUS). Clinical data were consistent with a sensory neuropathy affecting predominantly the kinesthetic sense (position and vibration sensation). The electrophysiological data indicated predominant sensory axonal neuropathy. Morphological data confirmed the primary axonal damage. Western immunoblot showed that the IgG from a patient without MGUS reacted with a 55 kD protein of dorsal root ganglion homogenate. Three of four patients with IgM MGUS were serum reactive against chondroitin sulfate C (ChS-C) in double immunodiffusion. After absorption with ChS-C the monoclonal peak completely disappeared from two patients and was decreased in the third patient. Our data indicate that immunological abnormalities are part of the pathogenesis for a subgroup of chronic neuropathy with only sensory symptoms.

摘要

我们观察了9例患者(8例男性,1例女性),年龄在58至77岁之间,患有仅表现为感觉症状且起病隐匿的神经病变。其中5例(4例男性,1例女性)年龄在65至77岁之间,血清电泳图谱正常,而其他患者(均为男性),年龄在58至74岁之间,患有意义未明的IgM单克隆丙种球蛋白病(MGUS)。临床数据与主要影响运动觉(位置觉和振动觉)的感觉神经病变相符。电生理数据表明主要为感觉轴索性神经病变。形态学数据证实了原发性轴索损伤。蛋白质免疫印迹法显示,来自无MGUS患者的IgG与背根神经节匀浆中的一种55 kD蛋白发生反应。4例IgM MGUS患者中有3例血清在双向免疫扩散中对硫酸软骨素C(ChS-C)呈反应性。用ChS-C吸收后,两名患者的单克隆峰完全消失,第三名患者的单克隆峰降低。我们的数据表明,免疫异常是仅表现为感觉症状的慢性神经病变亚组发病机制的一部分。

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