Polyneuropathy in feline niemann-pick disease

Paul A. Cuddon, Robert Higgins, Ian D. Duncan, Stephen P.F. Miller, Joane M. Parent, Ann B. Moser

Research output: Contribution to journalArticlepeer-review

16 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1429-1443
Number of pages15
JournalBrain
Volume112
Issue number6
DOIs
StatePublished - Dec 1 1989

ASJC Scopus subject areas

  • Clinical Neurology

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