HEREDITARY ATAXIA WITH SENSORY NEURONOPATHY - BIEMONDS ATAXIA

We report the case of a man with late-onset hereditary ataxia and sens ory loss. Three of his sisters were affected by a similar disorder; to date no other members of his family have developed symptoms. The clin ical features of this family are similar to a rare form of autosomal d ominant hereditary ataxia, recently classified as SCA4. Postmortem fin dings indicate that this syndrome is marked by degeneration of cerebel lar Purkinje cells, dorsal root sensory ganglion neurons, and the asce nding posterior columns. Similar clinical and pathologic findings were reported by Biemond in 1954.