Conezyme Q is a mobile lipophilic electron carrier critical for electron transfer by the mitochondrial inner membrane respiratory chain. Conenzyme Q10 deficiencies, a type of Complex II deficiency mitochondrial disorder, is caused by mutations of any of the genes encoding proteins involved in the synthesis of coenzyme Q. Patients may show a favorable response to CoQ10 treatment. There are 5 major phenotypes
1. Encephalomyopathic form with seizures and ataxia
2. Multisystem infantile form with encephalopathy, cardiomyopathy, and renal failure
3. A cerebellar form with ataxia and cerebellar atrophy
4. Leigh syndrome with growth retardation
5. Isolated myopathic form
On imaging patients with ataxia may demonstrate cerebellar atrophy.