Progressive bulbar palsy

Clinic

• PBP belongs to a group of disorders known as motor neuron diseases.
• PBP is a disease that attacks the nerves supplying the bulbar muscles.
• These disorders are characterized by the degeneration of motor neurons in the cerebral cortex, spinal cord, brain stem, and pyramidal tracts.
• This specifically involves the glossopharyngeal nerve (IX), vagus nerve (X), and hypoglossal nerve (XII).
• It should not be confused with pseudobulbar palsy or progressive spinal muscular atrophy.
• ICD-11 lists progressive bulbar palsy as a variant of ALS

Signs / Symptoms

• Glossopharyngeal nerve
  • Reduced gag reflexes
• Vagus nerve
  • Weak palatal movements
• Hypoglossal nerve:
  • Fasciculations
  • Progressive difficulty with talking and swallowing
  • Weak movement of the facial muscles and tongue
  • Difficulty with pronunciations, particularly lateral consonants (linguals) and velars
  • Problems with drooling saliva
• If corticobulbar tract is affected a pseudobulbar affect with emotional changes may occur

Related disease

• Pseudobulbar palsy

• Gagging and choking increases the risk of pneumonia
• 25% of patients eventually develop ALS