Juvenile-onset of Dejerine-Sottas disease in a Taiwanese woman.

ABSTRACT

Dejerine-Sottas disease is an uncommon hereditary neuropathy which has not been reported in Taiwan. We describe a 57-year-old woman who had slowly progressive weakness in her four limbs since adolescence. None of her close relatives had the disease and no consanguinity was noted. Neurologic examination showed severe weakness and vibratory sensation loss in the four limbs. The tendon reflexes were generally absent. Electrophysiologic studies suggested a systemic myelinopathic process. Light and electron microscopy of the sural nerve biopsy specimens revealed many onion-bulb shapes formed by the Schwann cell processes or basement membranes without any evidence of myelin sheaths around the axons, which are characteristic features of Dejerine-Sottas disease.