Ponto-bulbar palsy with deafness (Brown-Vialetto-Van Laere syndrome)

The authors describe the clinical and electrophysiological features in 2 cases of ponto-bulbar palsy with deafness, and the clinical course and post-mortem findings in a sibling of one of these, who died in infancy, and who appears to have suffered from the same disease. The cases are compared with...

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Veröffentlicht in:	Journal of the neurological sciences 1981-05, Vol.50 (2), p.259-275
Hauptverfasser:	Gallai, V, Hockaday, J M, Hughes, J T, Lane, D J, Oppenheimer, D R, Rushworth, G
Format:	Artikel
Sprache:	eng
Schlagworte:	Adolescent Bulbar Palsy, Progressive - complications Bulbar Palsy, Progressive - diagnosis Bulbar Palsy, Progressive - genetics Deafness - complications Deafness - diagnosis Deafness - genetics Electromyography Female H-Reflex Humans Muscle Hypotonia - diagnosis Muscles - pathology Muscular Atrophy - diagnosis Neural Conduction Pons - pathology Syndrome
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Beschreibung
Zusammenfassung:	The authors describe the clinical and electrophysiological features in 2 cases of ponto-bulbar palsy with deafness, and the clinical course and post-mortem findings in a sibling of one of these, who died in infancy, and who appears to have suffered from the same disease. The cases are compared with 17 previously published cases, and the disease is discussed in relation to other progressive neurological diseases of childhood.
ISSN:	0022-510X
DOI:	10.1016/0022-510X(81)90172-6