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Brain, Vol. 112, No. 6, 1429-1443, 1989
© 1989 Guarantors of Brain

research-article

POLYNEUROPATHY IN FELINE NIEMANN-PICK DISEASE

PAUL A. CUDDON1,, ROBERT J. HIGGINS2, IAN D. DUNCAN1, STEPHEN P. F. MILLER3, JOANE M. PARENT4 and ANN B. MOSER5

1School of Veterinary Medicine, University of Wisconsin Madison, WI 2School of Veterinary Medicine, University of California-Davis Davis, CA 3National Institute of Neurological and Communicative Disorders and Stroke Bethesda, MD, USA 4Ontario Veterinary College, University of Guelph Guelph, Oniario, Canada 5Kennedy Institute for Handicapped Children Baltimore, MD, USA

Correspondence to: Correspondence to: Dr Paul Cuddon, Department of Medical Sciences, School of Veterinary Medicine, 2015 Linden Drive West, University of Wisconsin, Madison, WI 53706, USA

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.

Received July 26, 1988. Revised November 8, 1988. Accepted December 3, 1988.


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