Cardiomyopathy with Hereditary Cerebellar Ataxia Report of an Autopsied Case

Here was described a 38-year-old man with cerebellar ataxia who developed Adams-Stokes syndrome, abnormal electrocardiographic findings including various arrhythmias, and congestive heart failure. His parents were first cousins. One of his sisters manifested convulsive seizures and ataxia. The patient died of congestive heart failure. Autopsy revealed atrophy of cerebellar cortex and degeneration of dentate, olivary and pontine nuclei, but no spinal cord lesion. The heart showed extensive myocardial fibrosis and fatty infiltration. Coronary arteries appeared normal. The relationship between neurological and myocardial changes in cerebellar ataxia was discussed.