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Progressive Bulbar Paralysis

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Progressive Bulbar Paralysis, Progressive Bulbar Palsy, Duchenne's Syndrome, Duchenne's Paralysis, Labioglossopharyngeal Paralysis, Labioglossolaryngeal Paralysis, Bulbar Paralysis

  • Epidemiology
  1. Onset age 50-70 years
  • Pathophysiology
  1. Subtype of Amyotrophic Lateral Sclerosis (ALS)
  2. Cranial Nerve motor nuclear atrophy, glial overgrowth
    1. Cranial Nerve 5
    2. Cranial Nerve 7
    3. Cranial Nerve 9
    4. Cranial Nerve 10
    5. Cranial Nerve 12
  3. Subcortical involvement of corticobulbar tracts
  • Symptoms
  1. Drooling
  2. Difficult chewing
  3. Dysphagia
  4. Dysarthria
  5. Nasal regurgitation
  • Signs
  1. Arm and leg spasticity
  2. Hyperactive reflexes
  3. Ophthalmoplegia
  4. Fasciculation of Tongue and lip muscles
  5. Emotional lability
  • Differential Diagnosis
  1. Acute Bulbar Paralysis
    1. Acute CNS vascular lesion (Hemorrhage or thrombosis)
    2. Acute Bulbar Polioencephalitis
  2. Chronic Bulbar Paralysis
    1. Syringobulbia
    2. Multiple Sclerosis
  • Course
  1. Progresses to Aspiration Pneumonia, respiratory arrest
  2. Death in 1 to 3 years from onset