Neuronal ceroid-lipofuscinosis in a mature dog.

The neuronal ceroid-lipofuscinoses are a group of diseases characterized by progressive accumulation of autofluorescent lipopigments within neurons with associated neuronal damage." Variations of these diseases occur which include accumulations of lipopigments in viscera. The diseases long have been recognized in man where earlier studies of the pathogenesis suggested an increased rate of peroxidation of unsaturated fats with accumulation of lipopigments in secondary lysosomes." Recent work, however, suggests that at least some forms of the human diseases involve disorders in the metabolism of dol ichol~ .~ .~ Ceroid-lipofuscinosis has been described in the dog,l.3.6.X.I0 cat: cattle," and sheep.' We describe a case of ceroid-lipofuscinosis in a mature dog associated with behavioral disturbance. The affected temer-cross bred male dog had progressively increasing aggressive tendencies from about four months of age. The dog was killed at nine years of age when circling and a head tilt developed and aggressive tendencies were severe. The dog was necropsied and histologic examination was done on liver, spleen, kidney, and a series of sections of brain. Lesions were limited to the brain with slight yellow discoloration grossly. The cerebral cortex had prominent gyri and sulci suggesting some brain atrophy. Histologically there was accumulation of periodic acid-Schiff (PAS)-positive, autofluorescent granular material in neurons throughout the brain. Neuronal deposits varied in different anatomic areas. The most pronounced lesions were in the hippocampus and the temporal cortex where the lesion was general and focally severe (table I). With hematoxylin and eosin (HE) stain, severely affected neurons were swollen with the perikaryon