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Brain, Vol. 118, No. 6, 1557-1563, 1995
© 1995 Guarantors of Brain

research-article

A new variant of sensory ataxic neuropathy with autosomal dominant inheritance

G. W. van Dijk1, J. H. J. Wokke1,2,, P. L. Oey3, H. Franseen3, P. F. Ippel4 and H. Veldman2

1Department of Neurology, University of Utrecht The Netherlands 2Department of Laboratory of Neuromuscular Disorders, University of Utrecht The Netherlands 3Department of Clinical Genetics, University of Utrecht The Netherlands 4Department of Clinical Genetics, University of Utrecht The Netherlands

Correspondence to: Correspondence to: Professor John H. J. Wokke, MD, University Hospital Utrecht. PO Box 85500, 3508 GA Utrecht, The Netherlands

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

hereditary; sensory ataxia; sensory neuropathy

Received March 6, 1995. Revised July 6, 1995. Accepted July 22, 1995.


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