JUVENILE-ONSET BULBOSPINAL MUSCULAR-ATROPHY WITH DEAFNESS - VIALETTA-VANLAERE SYNDROME OR MADRAS-TYPE MOTOR-NEURON DISEASE

被引：27

作者：

SUMMERS, BA ^{[1
]}

SWASH, M ^{[1
]}

SCHWARTZ, MS ^{[1
]}

INGRAM, DA ^{[1
]}

机构：

[1] LONDON HOSP, DEPT NEUROL, LONDON E1 1BB, ENGLAND

来源：

JOURNAL OF NEUROLOGY | 1987年 / 234卷 / 06期

关键词：

D O I：

10.1007/BF00314095

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

A girl with rapid-onset, bulbospinal muscular atrophy and deafness is described. The patient''s mother and brother showed EMG features consistent with subclinical involvement. This bulbospinal form of spinal muscular atrophy associated with deafness described by Vialetto and van Laere closely resembles the Madras type of motor neuron disease, also associated with deafness, described by Jagganathan and colleagues.

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页码：440 / 442

页数：3

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