Disorder - Mitochondrial Myopathy

Organ Systems Involved
Nervous/sensory

Alternative Names
Kearns Sayre Syndrome (KSS)
Chronic Progressive External Opthalmopleigia (CPEO)
Mitochondrial Encephalopathy Lactic Acidosis and Stroke-like Episodes (MELAS)
Myoclonus Epilepsy Associated with Ragged -Red Fibres (MERRF)
Leigh's disease
Mitochondrial Cyopa

Brief Description

The cells of the body that supply energy to the muscle cells are dysfunctional, resulting in affected dogs being less able to exercise and the accumulation of waste products in the body following exercise.

Presenting Signs

Episodes of weakness develop frequently during short walks and subside following rest. The dog's posture at rest is normal. Following exercise, the hindlimb stride is shortened and the dog starts to 'bunny hop' (move both hind legs together when running) before collapsing. Affected dogs are unable to exercise normally; after walking a short distance they collapse, but after a rest of 30 seconds or so they recover and are able to walk another short distance. The condition progresses so that the distance traveled between rests keeps reducing. Some dogs with this disorder may also suffer heart problems such as heart murmur or disease of the heart muscle. Less commonly, the condition affects the brain, leading to epilepsy. Symptoms are present at the age of three months onwards.

Dogs at Risk

Young dogs at about three months.

Groups Affected
Gundog


Breeds Affected
Irish setter


Treatment

Medical

Related Disorders

Myopathy, congenital
Myopathy, progressive, subacute
Leigh's disease

PubMed References
Mitochondrial Myopathy

Further Reference Material [OMIA Number]
695


Contributor
Rina Harun