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Article
April 1980

Progressive Bulbar Paralysis Associated With Neural Deafness: A Nosological Entity

Author Affiliations

From the Departments of Neurology (Drs Alberca and Ibañez) and Pathology (Drs Montero, Segura, and Miranda-Nieves), Ciudad Sanitaria Virgen del Rocio, Seville, Spain.

Arch Neurol. 1980;37(4):214-216. doi:10.1001/archneur.1980.00500530052007
Abstract

• A complete autopsy verification of progressive bulbar palsy associated with neural deafness was performed. Hearing loss and speech difficulties developed in a five-year-old girl. When she was 24 years old, clinical examination demonstrated deafness and bulbopontine paralysis together with retinitis pigmentosa, peripheral amyotrophies, pyramidal signs, and ataxia. The patient died at 27 years and the autopsy disclosed degenerative changes characterized by simple atrophy and loss of neurons accompanied by gliosis and loss of myelinated fibers. The structures principally affected were the anterior horns and the motor nuclei of the brain stem together with the eighth cranial nerve nuclei. Loss of myelinated fibers was found in the spinocerebellar and pyramidal tracts and in the fasciculus gracilis. Our study suggests that progressive bulbar paralysis with neural deafness should be considered as a nosological entity.

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