Progressive Bulbar Paralysis

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

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II. Epidemiology

  1. Onset age 50-70 years

III. Pathophysiology

  1. Motor Neuron Disease related to Amyotrophic Lateral Sclerosis (ALS)
  2. Starts with speech and Swallowing difficulty (related to LMN deficits of CN 9, CN 10, CN 12)
  3. 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
  4. Subcortical involvement of corticobulbar tracts

IV. Symptoms

  1. Drooling
  2. Difficult chewing
  3. Dysphagia
  4. Dysarthria
  5. Nasal regurgitation

V. Signs

  1. Arm and leg spasticity
  2. Hyperactive reflexes
  3. Ophthalmoplegia
  4. Fasciculation of Tongue and lip Muscles
  5. Emotional lability

VI. 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

VII. Course

  1. Better prognosis (without Respiratory Failure) with disease duration >4 years, and segmental Muscle involvement
  2. Progresses to Aspiration Pneumonia, respiratory arrest
  3. Death in 1 to 3 years from onset

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