Which clinical history findings are characteristic of progressive bulbar palsy (PBP)?

Updated: Apr 28, 2014
  • Author: Hidehiro Takei, MD; Chief Editor: Adekunle M Adesina, MD, PhD  more...
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Brown-Vialetto-Van Laere syndrome typically presents with progressive sensorineural hearing loss. The age of onset of the initial symptoms reportedly ranges from infancy to the third decade of life. [11] The hearing loss is usually followed by other symptoms, including abnormalities of lower CNs VII-XII and LMN signs in the limbs, with an interval of several years. This interval has been reported to be shorter in males (mean, approximately 5 y) than in females (mean, almost 11 y). [11] Abnormalities of CNs II-VI occur much less frequently.

Fazio-Londe disease manifests itself in childhood as rapidly progressive weakness of the tongue, face, and pharyngeal muscles, as well as progressive upper limb weakness. No hearing impairment is seen.

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