Late Niemann-Pick disease with neurovisceral storage: a classification problem.
A 51 year old man presented in 1969 with slowly progressive cerebellar ataxia of unknown origin. He was admitted to hospital aged 68 after a fall, and a ruptured spleen was removed at laparotomy. Histological analysis of the spleen suggested Niemann-Pick disease, which was subsequently confirmed. He deteriorated and died of bronchopneumonia shortly afterwards: subdural haemorrhage with storage material in neurones was found at necropsy. This late onset case of Niemann-Pick disease with neurovisceral storage is unusual and may represent a variant.
McFarlane
J
J
Department of Pathology, University of Leeds.
Murray
L
L
Bradbury
K
K
Cowen
P N
PN
D000367:Age Factors; D001921:Brain / Q000473:pathology; D006801:Humans; D008055:Lipids / Q000032:analysis; D008297:Male; D008875:Middle Aged; D009542:Niemann-Pick Diseases / Q000145:classification* / Q000473:pathology; D013154:Spleen / Q000473:pathology
D008055:Lipids