Neuronal Ceroid-lipofuscinosis in a Japanese Domestic Shorthair Cat

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A female Japanese domestic shorthair cat showed gait abnormality at 5 months of age, and head shaking and tremor became apparent from 6 months of age. Serum biochemistry at 13 months of age revealed markedly elevated ALT and ALP. The cat died at 16 months of age. Histopathologic examination revealed prominent cytoplasmic swelling of neurons with accumulation of yellowish pigments. The storage pigments stained positively with periodic acid Schiff reaction, Schmorl method, and Oil red O stain. Ultrastructurally, the neuronal storage consisted of aggregates of dense materials, similar to the granular osmiophilic deposits in infantile ceroid-lipofuscinosis in humans. Hepatocytes were markedly swollen and contained faintly eosinophilic inclusion. To our knowledge this case is the sixth case of feline ceroid-lipofuscinosis, which is characterized by granular osmiophilic dense bodies in the neurons and prominent involvement of hepatocytes.