Article Text


Late Niemann-Pick disease with neurovisceral storage: a classification problem.
  1. J McFarlane,
  2. L Murray,
  3. K Bradbury,
  4. P N Cowen
  1. Department of Pathology, University of Leeds.


    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.

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