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[遗传性复发性单神经病(腊肠样神经病)]

[Hereditary neuropathy with recurrent mononeuropathy (tomaculous neuropathy)].

作者信息

Serena M, Bardin P G

机构信息

Reparto di Neurologia, Ospedale Civile Regionale, Treviso.

出版信息

Riv Neurol. 1988 May-Jun;58(3):97-105.

PMID:3175463
Abstract

Clinical and neurophysiological studies were carried out in 15 members out of 5 families affected by recurrent peripheral nerve and brachial plexus palsies. Nerve biopsy showed typical "tomacula". While in 3 families a slight development of the symptomatology was observed in the other 2 families there was an evolution toward a severe symmetric neuropathy. Motor and sensory conduction velocity were, in some cases, slowed even in clinically unaffected nerves. Electrophysiological changes were more important at entrapment sites. Such abnormalities could be found also in unaffected family members.

摘要

对5个受复发性周围神经和臂丛神经麻痹影响的家庭中的15名成员进行了临床和神经生理学研究。神经活检显示典型的“腊肠样结构”。在3个家庭中观察到症状有轻微发展,而在另外2个家庭中则发展为严重的对称性神经病。在某些情况下,即使在临床上未受影响的神经中,运动和感觉传导速度也减慢。电生理变化在卡压部位更为明显。在未受影响的家庭成员中也可发现此类异常。

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