Autosomal recessive spastic paraplegia: evidence for demyelination

Abstract
Neurological evaluation of a family living in rural Louisiana [USA] showed that 6 living members have spasticity, hyperactive stretch reflexes and pathological reflexes. Deceased family members (10) were similarly affected. All affected persons were offspring of consanguineous marriages, and sex distribution was equal, indicating that the inheritance was autosomal recessive. Although undetected clinically, conduction abnormalities suggestive of demyelination in the visual pathways were revealed by special tests.

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