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Progressive Bulbar Paralysis Associated With Neural DeafnessA Nosological Entity
Román Alberca, MD;
Claudio Montero, MD;
Agustín Ibañez, MD;
Dolores I. Segura, MD;
Gonzalo Miranda-Nieves, MD
Arch Neurol. 1980;37(4):214-216.
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.
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.
Footnotes
Accepted for publication April 16, 1979.
Reprint requests to Ciudad Sanitaria Virgen del Rocío, Centro de Rehabilitacíon y Traumatología, Servicio de Neurología, Avda Manuel Siurot s/n (Dr Alberca).
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THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
Progressive Pontobulbar Palsy With Deafness: Clinical and Pathological Study of Two Cases
Brucher et al.
Arch Neurol 1981;38:186-190.
ABSTRACT
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