Heredo-ataxia in a large Dutch pedigree. M.R.I. findings

One segment of a large Dutch pedigree with heredo-ataxia is presented. The clinical and genetic features of the disease, together with the M.R.I. findings, indicate the diagnosis of autosomal dominant late onset olivo-ponto-cerebellar atrophy. The clinical picture remained invariant through successive generations. Emphasis is put on the diagnostic usefulness of M.R.I. technique in the absence of neuropathological data.