Open AccessNeurological Manifestations of Niemann‐Pick Disease Type C in Cats
Author(s) -
Muñana Karen R.,
Luttgen Patricia J.,
Thrall Mary Anna,
Mitchell Thomas W.,
Wenger David A.
Publication year - 1994
Publication title -
journal of veterinary internal medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.356
H-Index - 103
eISSN - 1939-1676
pISSN - 0891-6640
DOI - 10.1111/j.1939-1676.1994.tb03208.x
Subject(s) - cats , medicine , dysmetria , ataxia , pathological , central nervous system , lysosomal storage disease , disease , pathology , niemann–pick disease, type c , niemann–pick disease , psychiatry
Seven Domestic shorthair cats with a lysosomal storage disorder analogous to human Niemann‐Pick disease type C, from a breeding colony were studied to characterize the neurological manifestations of this disorder. Affected cats were identified by means of liver biopsies at 4 to 6 weeks of age. Neurological examinations were performed at 2 week intervals from the onset of clinical signs. All cats displayed signs referrable to the cerebellum, with a subtle intention tremor noticed initially at 8 to 12 weeks of age; the disease was rapidly progressive. The tremor became more pronounced, menace response was lost, and severe dysmetria and ataxia developed. Three cats also had signs referrable to other areas of the central nervous system. Cats died or were euthanized between 12 and 43 weeks of age. Pathological findings included accumulation of substrate within neurons throughout the central nervous system, and axonal spheroid formation. The clinical and pathological findings in these cats are comparable to those in the human form of the disease.