MAGNETIC-RESONANCE-IMAGING IN TYPICAL AND LATE-ONSET FRIEDREICHS-DISEASE AND EARLY-ONSET CEREBELLAR-ATAXIA WITH RETAINED TENDON REFLEXES

MRI makes it possible to study the in vivo brain and spinal cord morph ology of patients with hereditary ataxia, We performed T1- and T2-weig hted studies in eleven patients with Friedreich's disease (FD), Jive w ith ''late onset'' FD and ten with early onset cerebellar ataxia with retained tendon reflexes (EOCA), Cervical cord atrophy was constant in FD and ''late onset'' FD and often associated with atrophy of the cer ebellum and of the brainstem; T2-weighted studies showed posterior col umn degeneration in the cervical cord, The most frequent finding in EO CA was cerebellar atrophy, pure or associated with cervical cord or br ainstem atrophy; the cerebellar atrophy was marked in a few cases and was related to disease duration.