Paroxysmal Dyskinesia in Border Terriers: Clinical, Epidemiological, and Genetic Investigations.

BACKGROUND: In the last decade, a disorder characterized by episodes of involuntary movements and dystonia has been recognized in Border Terriers. OBJECTIVES: To define clinical features of paroxysmal dyskinesia (PD) in a large number of Border Terriers and to study the genetics of the disease. ANIMALS: 110 affected and 128 unaffected client-owned Border Terriers. METHODS: A questionnaire regarding clinical characteristics of PD was designed at Utrecht University and the University of Helsinki. Thirty-five affected Border Terriers underwent physical examination and blood testing (hematology and clinical biochemistry). Diagnostic imaging of the brain was performed in 17 affected dogs and electroencephalograms (EEG) between episodes were obtained in 10 affected dogs. A genomewide association study (GWAS) was performed with DNA of 110 affected and 128 unaffected dogs. RESULTS: One hundred forty-seven questionnaires were included in the study. The most characteristic signs during episodes were dystonia, muscle fasciculations, and falling over. The majority of owners believed that their dogs remained conscious during the episodes. A beneficial effect of anti-epileptic therapy was observed in 29 of 43 dogs. Fifteen owners changed their dogs' diet to a hypoallergenic, gluten-free diet, and all reported reasonable to good improvement of signs. Clinical examinations and diagnostic test results were unremarkable. The GWAS did not identify significantly associated chromosome regions. CONCLUSIONS AND CLINICAL IMPORTANCE: The survey results and EEG studies provided further evidence that the observed syndrome is a PD rather than epilepsy. Failure to achieve conclusive results by GWAS indicates that inheritance of PD in Border Terriers probably is complex.

Transient hyperammonemia due to urea cycle enzyme deficiency in Irish wolfhounds.

BACKGROUND: Irish Wolfhounds frequently have a congenital portosystemic shunt, but a considerable proportion of the 6- to 8-wk-old pups has hyperammonemia in the absence of portosystemic shunting. This hyperammonemia causes no signs and is transient, normalizing at the age of 3-4 months. HYPOTHESIS: Transient hyperammonemia has a metabolic basis in Irish Wolfhounds. ANIMALS: Two related (same sire) litters of Irish Wolfhounds (17 pups) and their parents were studied. METHODS: Integrity of the portal circulation was examined by ultrasonography and scintigraphy. Absence of parenchymal liver disease was verified by liver biopsy. Amino acid profiles were measured in 4 pups and repeated in 2 of these pups when ammonia concentrations had normalized. The amino acid profiles were compared with those of healthy Irish Wolfhound pups. RESULTS: Fasting venous ammonia concentrations were high (113-622 microg/dL, 65-345 micromol/L) in all pups, whereas bile acids were within reference range in all but 1. The ammonia and bile acid concentrations from all parents were within reference range. Portosystemic shunting was excluded in all but 1 pup. Liver biopsy excluded significant lesions in all 10 pups examined. Hypercitrullinemia was found and persisted even when ammonia had normalized, at the expense of an increase in glutamine and asparagine. CONCLUSIONS AND CLINICAL IMPORTANCE: Citrulline concentrations are controlled by the urea cycle enzymes argininosuccinase and argininosuccinate synthetase, and a defect in either of these enzymes may be responsible for the transient hyperammonemia in Irish Wolfhounds. Resolution of the hyperammonemia is associated with increased activity of alternative metabolic pathways forming glutamine and asparagine. Confirmation requires measurement of enzyme activities in liver tissue.

[Ehrlichia and Babesia infections in dogs in The Netherlands]. / Ehrlichia- en Babesia-infecties bij de hond in Nederland.

A retrospective study was performed at the Department of Clinical Sciences of Companion Animals at Utrecht University amongst 75 dogs diagnosed with a Babesia canis and/or an Ehrlichia canis infection. The majority of the dogs had visited an endemic area (most often the Mediterranean area or the Dutch Antilles), but two dogs became infected with Babesia in the Netherlands. Babesia infections were associated with a stay in an endemic area and an incubation period that are both significantly shorter (less than 3 months) than those for Ehrlichia and co-infections (more than 3 months). Reasons for the owner to seek veterinary attention (lethargy, anorexia, fever), findings from the physical examination (pale mucous membranes, hepato-/splenomegaly) and laboratory results (anemia, thrombocytopenia, hypo-albuminemia) were highly aspecific, making serology or PCR mandatory for diagnosing infections. Antigenic stimulation by the parasite sometimes resulted in immune-mediated diseases such as immune-mediated hemolytic anemia, thrombocytopenia, glomerulonefritis, and polyarthritis and in the case of ehrlichiosis in hypergammaglobulinemia. Specific therapy (imidocarb-diproprionate and/or doxycycline) was necessary, and because combined infections were common, it was considered appropriate to administer both drugs while the definitive diagnosis was being established. The prognosis was reasonably good, with almost half of all patients showing no clinical signs after treatment, although Babesia and co-infections were associated with a significantly longer survival sometimes resulted than Ehrlichia infections.