CLINICAL, HEMATOLOGIC, AND BIOCHEMICAL FEATURES OF A SYNDROME IN BERNESE-MOUNTAIN-DOGS CHARACTERIZED BY HEPATOCEREBELLAR DEGENERATION

Seven related Bernese Mountain Dogs developed a syndrome characterized by progressive cerebellar and hepatic disease. Clinically, stiffness in the hind limbs, mild incoordination, and a slight head tremor were first noticeable when pups were 4 to 6 weeks old. The condition progre ssed, causing pups to assume a wide-based stance. Other signs included head bobbing, spontaneous nystagmus, and, finally, paresis. Hematolog ic findings included leukocytosis with a left shift; normocytic, normo chromic anemia; hypoproteinemia, low serum creatinine, and urea nitrog en concentrations; excessive fasting plasma ammonia concentration; and an increase in concentration of serum bile acids. Portal venography p erformed on 1 dog revealed a small liver and extensive extrahepatic va ricosities. Necropsy revealed cerebellar hypoplasia, nodular liver, ex tensive abdominal varicosities, and ascites. Histologically, degenerat ion and depletion of Purkinje's cells and vacuolation, degeneration, a nd nodular regeneration of hepatic tissues were evident. Preliminary a nalysis of the pedigree was suggestive of an autosomal recessive patte rn of inheritance.