Brown-Vialetto-Van Laere syndrome.
The clinical and pathological findings of a male with the Brown-Vialetto-Van Laere syndrome are described. This rare and fatal affection of the nervous system involves mainly the brain stem with the prominent and early manifestation of sensorineural deafness. Increased awareness and documentation of this disorder has added information on the mode of inheritance.
Francis
D A
DA
National Hospital for Nervous Diseases, London.
Ponsford
J R
JR
Wiles
C M
CM
Thomas
P K
PK
Duchen
L W
LW
D000293:Adolescent; D001306:Auditory Pathways / Q000473:pathology; D001921:Brain / Q000473:pathology; D010244:Bulbar Palsy, Progressive / Q000473:pathology*; D002531:Cerebellum / Q000473:pathology; D006319:Hearing Loss, Sensorineural / Q000473:pathology*; D006801:Humans; D008297:Male; D011689:Purkinje Cells / Q000648:ultrastructure; D013577:Syndrome; D014722:Vestibule, Labyrinth / Q000473:pathology