Neuropathological Findings in Cerebellar Ataxia With Neuropathy and Vestibular Areflexia Syndrome

Abstract

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is characterized by neuropathological changes such as loss of Purkinje cells and degeneration of the posterior column of the spinal cord. In the peripheral nervous system, CANVAS is associated with loss of ganglion cells in the dorsal root and vestibular ganglia. Some patients may show degeneration of the inferior olivary nucleus, corticospinal tracts, and the facial and trigeminal ganglia. Further research is warranted to determine whether differences in lesion distribution are attributable to differences in genetic abnormalities and their combinations. To date, aggregates of abnormal proteins such as intranuclear inclusion bodies characteristic of this disease have not been identified in the nervous system.