Motor

Amyotrophic Lateral Sclerosis

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Amyotrophic Lateral Sclerosis, ALS, Lou Gehrig's Disease

  • Epidemiology
  1. Prevalence: 5-7 per 100,000 worldwide
  2. Gender: More common in men by ratio of 1.5 to 1
  3. Ages affected: 50 to 70 years old
  • Etiology
  1. Idiopathic in most cases
  2. Autosomal Dominant inheritance in 5-10% of cases
  • Pathophysiology
  1. Upper Motor Neuron and Lower Motor Neuron degeneration
  2. Affects anterior horn cells
  3. Subtypes
    1. Progressive Bulbar Palsy
    2. Primary Lateral Sclerosis
    3. Spinal Muscular Atrophy
  • Symptoms
  1. Muscle aches and muscle cramps
  2. Weakness of distal upper limbs
  3. Weakness progresses inferiorly (towards feet)
  4. Dysarthria
  5. Dysphagia
  6. Drooling
  7. No sensory deficits
  • Signs
  1. Muscle fibrillation and atrophy (upper limbs)
  2. Hyperreflexia
  3. Spasticity of lower limbs
  1. Muscle fibrillation on mechanical stimulation
  2. Increased duration and amplitude of action potentials
  • Course
  1. Majority of patients die within 1-3 years of diagnosis
  2. Only 10% survive beyond 5 years
  • Management
  1. Riluzole 50 mg bid
    1. Anti-glutamate properties
    2. Only modest effect at best (extended life 3 months)
    3. Best effect if used early
    4. Very expensive ($700/month)
  2. Vitamin E and Vitamin C
    1. Shown effective in rats but not proven in humans
  3. Immunosuppressants not effective or indicated
  4. Treat at ALS center
    1. Physical Therapy
    2. Occupational Therapy
    3. Dietitian
    4. Neurologist
  5. Symptomatic treatment
    1. Progressive Pseudobulbar palsy
    2. Spontaneous laugh (Tricyclic Antidepressants)