Medium chain acyl-CoA dehydrogenase deficiency (MCADD), a fatty acid oxidation disorder, is an inherited disease affecting the CKCS breed. This condition prevents the normal use of fat as an alternative source of energy during times of fasting or increased metabolic demands. Affected animals cannot burn fat for energy when their bodies run out of glucose, and as a result they may be affected by low blood sugar, altered central nervous system function, coma, or sudden death. With an early diagnosis, MCADD can be managed successfully by diet.
In a recent study, affected dogs showed complex focal seizures with prolonged lethargy, lower responsiveness and proprioceptive ataxia. These conditions occurred several times a week and lasted from 20 minutes up to 24 hours. Urine and blood analysis showed an elevated level of medium chain fatty acids. The symptoms improved under medical treatment and diet change towards low fat diet leading to several month without major seizures. The allele frequency within the CKCS population tested in the publication was 23.5%, which means that 5.52% of the tested CKCS were affected.
