MERRF

Myoclonic epilepsy and ragged red fibers (MERRF) is a maternally transmitted disease in which patients have myoclonus, a seizure disorder, and evidence of a myopathy.

Ataxia, associated with evidence of neuronal loss from the cerebellar system (including inferior olive in the medulla, cerebellar cortex, and outflow nuclei), is also a common component.

Two common mutations involve the mitochondrial tRNA, affecting protein synthesis within mitochodria.