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