Brown-Vialetto-Van Laere syndrome
- Brown-Vialetto-Van Laere syndrome
The Brown-Vialetto-Van Laere syndrome is an exceptionally rare neurological disorder of unknown cause, characterized primarily by deafness and paralysis of the muscles of the face, neck, shoulders and limbs. The neurological manifestations develop insidiously: they usually begin with sensorineural deafness, progress inexorably to paralysis, and often culminate in respiratory failure. The syndrome affects children, adolescents, and younger adults; the age at onset of symptoms in the reported cases has ranged from infancy to the third decade of life. The prognosis is poor — most patients diagnosed with the syndrome die within 10 years. There is no cure.
The syndrome was first described by Charles Brown in 1894; further accounts by Vialetto and Van Laere followed in 1936 and 1966, respectively. There are fewer than 60 cases reported in the medical literature over the 100 odd years since its first description.
References
#Brown CH. Infantile amyotrophic lateral sclerosis of the family type. "J Nerv Ment Dis" 1894, 21:707-716.
#Vialetto E. Contributo alla forma ereditaria della paralisi bulbare progressive. "Riv Sper Freniat" 1936, 40:1-24.
#Van Laere J. Paralysie bulbo-pontine chronique progressive familiale avec surdité. Un cas de syndrome de Klippel-Trenaunay dans la même fratrie - problèmes diagnostiques et génétiques. "Rev Neurol" 1966, 115:289-295.
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