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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。

相似文献

1
[Hereditary neuropathy with recurrent mononeuropathy (tomaculous neuropathy)].[遗传性复发性单神经病(腊肠样神经病)]
Riv Neurol. 1988 May-Jun;58(3):97-105.
2
[Hereditary neuropathy with liability to pressure palsies (tomaculous neuropathy). Clinical, electrophysical and molecular study of two affected families].[易患压迫性麻痹的遗传性神经病(腊肠样神经病)。两个患病家族的临床、电生理及分子研究]
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[Hereditary neuropathy with liability to pressure palsy].[易患压迫性麻痹的遗传性神经病]
Harefuah. 1989 Apr 2;116(7):345-7.
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A rare genetic disorder in the differential diagnosis of the entrapment neuropathies: hereditary neuropathy with liability to pressure palsies.一种在卡压性神经病鉴别诊断中的罕见遗传性疾病:遗传性压力易感性神经病。
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Electrophysiological findings in hereditary motor and sensory neuropathy type I and II--a conduction velocity study.遗传性运动和感觉神经病I型和II型的电生理发现——一项传导速度研究。
Electromyogr Clin Neurophysiol. 1998 Mar;38(2):95-101.
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Central nervous system involvement in hereditary neuropathy with liability to pressure palsies: description of a large family with this association.遗传性压力易感性周围神经病的中枢神经系统受累:一个与之相关的大家族的描述。
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Deletion in the CMT1A locus on chromosome 17p11.2 in hereditary neuropathy with liability to pressure palsies.17号染色体p11.2区域CMT1A位点缺失与遗传性压力易感性周围神经病相关。
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