Amyotroph Lateral Scler. 2010 Aug;11(4):397-8.

Pontobulbar palsy and sensorineural deafness (Brown-Vialetto-van Laere syndrome): the first case from Libya.

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Arab Medical University, Benghazi, Libya. fathidakhil@live.com

Abstract

We report the first case of Brown-Vialetto-van Laere syndrome from Libya. A 27-year-old female patient with progressive pontobulbar palsy, sensorineural deafness and generalized muscle weakness is described. Although she was born to consanguineous first cousin parents, she was the only one affected among five brothers and six sisters. The first symptoms appeared at the age of 18 years with tinnitus, weakness and unsteadiness that progressed over the course of a few weeks to deafness, blurring of vision, dysphagia, and dysarthria. Over the last nine years her symptoms progressed slowly, although interrupted with periods of rapid deterioration. Fifty-eight cases have been reported since the first case was described in 1894.

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