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Juvenile-onset bulbospinal muscular atrophy with deafness: Vialetta-van laere syndrome or madras-type motor neuron disease?

✍ Scribed by B. A. Summers; M. Swash; M. S. Schwartz; D. A. Ingram


Book ID
104718209
Publisher
Springer
Year
1987
Tongue
English
Weight
265 KB
Volume
234
Category
Article
ISSN
0340-5354

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✦ Synopsis


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. T is 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.