PEDIATRICS Vol. 67 No. 4 April 1981, pp. 517-522
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Progressive Sensory Loss in Familial Dysautonomia

Felicia B. Axelrod MD1, Kamla Iyer MD1, Irving Fish MD1, John Pearson MD1, Mary Ellen Sein RN1, and Neil Spielholz PhD1

1 Department of Pediatrics, New York University Medical Center, New York

Clinical variability in sensory impairment was demonstrated among 75 patients with familial dysautonomia. Older patients had a greater tendency toward increased dysfunction in pain sensation, joint position and Romberg's sign, and vibratory sense. Significant worsening with increased age was supported by retesting of 53 patients after a five-year interval. Sensory and motor axon loss were indicated by electrodiagnostic testing of peripheral nerves and abnormal cortical somatosensory evoked potentials. Familial dysautonomia is a hereditary disease with variable penetrance which involves both failure of intrauterine development of neurons and their postnatal maintenance.

Submitted on April 2, 1980
Accepted on July 7, 1980




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