Polyneuropathy in feline Niemann-Pick disease

Brain. 1989 Dec:112 ( Pt 6):1429-43. doi: 10.1093/brain/112.6.1429.


Two related cats, aged 5 months and 7 months, and 1 unrelated cat, aged 4 months, presented with signs of a progressive neuromuscular disease. Detailed electrophysiological studies suggested a primary demyelinating polyneuropathy, which was confirmed by muscle and nerve biopsies and on necropsy examination. Light and electron microscopic findings indicated a lysosomal storage disease, which was diagnosed as sphingomyelinase deficiency (Niemann-Pick disease) by enzyme analysis and lipid fractionation, although significant biochemical differences existed between the 2 related cats and the third cat. Several lines of evidence suggest that these 2 related cats were affected with a variant of type A Niemann-Pick disease, whereas cat 3 represented classic Niemann-Pick disease type A.

Publication types

  • Case Reports

MeSH terms

  • Animals
  • Cat Diseases / physiopathology*
  • Cats
  • Central Nervous System / pathology
  • Chemical Fractionation
  • Electrophysiology
  • Histocytochemistry
  • Lipid Metabolism
  • Lysosomes / enzymology
  • Muscles / metabolism
  • Niemann-Pick Diseases / complications
  • Niemann-Pick Diseases / pathology
  • Niemann-Pick Diseases / veterinary*
  • Peripheral Nerves / metabolism
  • Peripheral Nerves / pathology
  • Peripheral Nervous System Diseases / etiology
  • Peripheral Nervous System Diseases / physiopathology
  • Peripheral Nervous System Diseases / veterinary*