This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citing articles on Web of Science (12)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

Clinical and Pathological Study of Two Cases

Jean M. Brucher, MD; René Dom, MD; Alfons Lombaert, MD; Herwig Carton, MD

Arch Neurol. 1981;38(3):186-190.

Abstract
• Among the hereditary affections of the nervous system associated with deafness, a rare condition called "progressive pontobulbar palsy with deafness" has been described. In this slowly progressive condition, hearing loss and vestibular areflexia are almost always the first symptoms, occurring in late childhood or early adulthood. Only 18 cases—some sporadic, several familial—have been published without a full report of pathological findings. The clinical and pathological data of two new cases—one familial, one sporadic—are described here. There are differences from other forms of bulbar paralysis, lower motor neuron diseases, and some spinocerebellar hereditary affections. In view of the homochrony and homotypy in familial cases and the pathological findings, progressive pontobulbar palsy with deafness appears to be an abiotrophic process with autosomal recessive inheritance.

Author Affiliations
From the Laboratoire de Neuropathologie, Université de Louvain, Brussels (Dr Brucher), and the Kliniek voor Neurologie en Neurochirurgie, Universiteit Leuven, Belgium (Drs Dom, Lombaert, and Carton).

Footnotes
Accepted for publication April 2, 1980.

Reprint requests to Laboratoire de Neuropathologie, av E Mounier, 52 (ANPG 5260), 1200 Brussels, Belgium (Dr Brucher).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?