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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 (cerebral Hemorrhage, Ischemic CVA)
    2. Acute Bulbar Polioencephalitis
  2. Chronic Bulbar Paralysis
    1. Syringobulbia
    2. Multiple Sclerosis
    3. Myasthenia Gravis
  • Course
  1. Progresses to Aspiration Pneumonia, respiratory arrest
  2. Death in 1 to 3 years from onset