Hereditary sensory neuropathy with spinal cord disease

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Abstract
Report of a family with hereditary sensory neuropathy (HSN) and spinal cord disease. Thirteen cases were found among 47 members of this family in 4 generations. The early manifestation of the disease is a spastic paraparesis which shows up before the age of 10 years. Later on, a peripheral sensory neuropathy develops. Mode of inheritance is dominant. The presence of spinal cord disease concomitant with HSN is unique in this family. A review of the literature does not show similar cases. The pathology HSN of one subject studied by autopsy does not explain the findings in this family. From the clinical evidence available, the pathology must extend beyond the dorsal root ganglia.

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