A case of wolfram syndrome associated with dysphagia

Abstract

A case of Wolfram syndrome associated with dysphagia is described hereinafter. In 1938, Wolfram reported four patients with diabetes mellitus and optic atrophy. Since then, this syndrome has been recorded over 100 cases, often in association with diabetes insipidus and deafness. Various symptoms are shown in this syndrome, dysphagia or vertigo is however rarely seen. Atrophy of hypothalamic nuclei that progresses gradually, degeneration of the optic nerve, its chiasm and tract, and totally unexpected degeneration of the pons and cerebellum are considered as the pathogenesis of this syndrome. The patient, twenty-seven-year-old female, has been suffering from optic atrophy since the age of fourteen and diabetes mellitus since the age of twenty-three. Dysphagia has developed since the age of seventeen. Dysphagia and dysequilibrium revealed brain stem lesion. As the treatment for dysphagia, cricopharyngeal myotomy was fairly effective. The specimen of the muscle obtained in the operation revealed neurogenic atrophy of muscle. © 1988, The Oto-Rhino-Laryngological Society of Japan, Inc. All rights reserved.