What is progressive bulbar palsy (PBP)?

Updated: Apr 28, 2014
  • Author: Hidehiro Takei, MD; Chief Editor: Adekunle M Adesina, MD, PhD  more...
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Answer

Answer

PBP is a progressive degenerative disorder of the motor nuclei in the medulla (specifically involving the glossopharyngeal, vagus, and hypoglossal nerves) that produces atrophy and fasciculations of the lingual muscles, dysarthria, and dysphagia. In adults, because most of the cases presenting with these pure bulbar symptoms represent so-called bulbar-onset ALS and eventually develop widespread symptoms typically seen in ALS, some authors consider this disorder to be a subset of ALS.

Infantile PBP is a rare disorder that occurs in children and presents as the following 2 phenotypically associated forms:

  • Brown-Vialetto-Van Laere syndrome (pontobulbar palsy with deafness)

  • Fazio-Londe disease

Brown-Vialetto-Van Laere syndrome is characterized by bilateral sensorineural deafness that is followed by CN VII, CN IX, and CN XII palsies, whereas Fazio-Londe disease causes progressive bulbar palsy without deafness. Both disorders are genetically heterogeneous.


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