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  • Title: Neuronal ceroid lipofuscinosis in an adult American Staffordshire Terrier.
    Author: Nolte A, Bello A, Drögemüller M, Leeb T, Brockhaus E, Baumgärtner W, Wohlsein P.
    Journal: Tierarztl Prax Ausg K Kleintiere Heimtiere; 2016 Dec 05; 44(6):431-437. PubMed ID: 27778018.
    Abstract:
    A female, 5-year-old American Staffordshire Terrier with severe progressive neurological deficits, particularly in terms of ataxia and keeping balance, was examined pathomorphologically and a genetic analysis was performed. In neurons of various localizations of the central nervous system an accumulation of a finely granular pale eosinophilic or light brown material was found. In addition, the cerebellum revealed marked degeneration and loss of Purkinje and inner granule cells. The accumulated PAS-positive, argyrophilic, autofluorescent material showed ultrastructurally a lamellar appearance suggestive of lipofuscin. Genetic analysis revealed the presence of a sequence variant in the ARSG gene encoding the lysosomal enzyme arylsulfatase G. This case report describes an adult-onset of a neuronal ceroid lipofuscinosis that shows similarities with a human disorder termed Kufs disease.
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