Neuronal ceroid lipofuscinosis in an adult American Staffordshire Terrier.

Nolte, Anna; Bello, Aimara; Drögemüller, Michaela; Leeb, Tosso; Brockhaus, Eva; Baumgärtner, Wolfgang; Wohlsein, Peter (2016). Neuronal ceroid lipofuscinosis in an adult American Staffordshire Terrier. Tierärztliche Praxis. Ausgabe K - Kleintiere, Heimtiere, 44(6), pp. 431-436. Schattauer 10.15654/TPK-150766

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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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