A NEW VARIANT OF SENSORY ATAXIC NEUROPATHY WITH AUTOSOMAL-DOMINANT INHERITANCE

We describe a Dutch family with sensory ataxia in two generations, lat e onset of symptoms (over the age of 40 years) and slow progression. C linical, electrophysiological and sural nerve biopsy findings revealed a sensory polyneuropathy due to axonal degeneration of myelinated ner ve fibres in four of five investigated siblings. Other neurological ab normalities in the affected family members consisted only of mild eye movement disturbances, probably due to cerebellar involvement. Five ot her family members were investigated and found unaffected. As the dise ase is inherited from the affected father to his sons and daughters, t his is the first description of a probably autosomal dominant form of late onset hereditary sensory neuropathy with predominant sensory atax ia and minor other neurological abnormalities.