Amyotrophic lateral sclerosis (ALS), AKA "Lou Gehrig's Disease," is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.

1. Amyotrophic
Lateral Sclerosis

2. Epidemiology
 Prevalence: 5-7 per 100,000 worldwide
 More common in men by ratio of 1.5 to 1
 Ages affected: 50 to 70 years old

3. Etiology
 Idiopathic in most cases
 Autosomal dominant inheritance in 5-10% of
cases

4. Pathophysiology
 Upper and lower motor neuron degeneration
 Affects anterior horn cells
 Subtypes
 Progressive Bulbar Palsy
 Primary Lateral Sclerosis
 Spinal Muscular Atrophy

5. Symptoms
 Muscle aches and muscle cramps
 Weakness of distal upper limbs
 Weakness progresses inferiorly (towards feet)
 Dysarthria
 Dysphagia
 Drooling

6. Signs
 Muscle fibrillation and atrophy (upper limbs)
 Hyperreflexia
 Spasticity of lower limbs

7. Diagnosis:
Electromyogram (EMG)
 Muscle fibrillation on mechanical stimulation
 Increased duration and amplitude of action
potentials

8. Prognosis
 Majority of patients die within 1-3 years of
diagnosis
 Only 10% survive beyond 5 years

9. Treatment
 Riluzole 50 mg bid
 Anti-glutamate properties
 Only modest effect at best (extended life 3
months)
 Best effect if used early
 Vitamin E and Vitamin C
 Immunosuppressants not effective or
indicated

10. Supportive Management
 Treat at ALS center
 Physical Therapy
 Occupational Therapy
 Dietitian
 Neurologist
 Symptomatic treatment
 Progressive Pseudobulbar palsy
 Spontaneous laugh (Tricyclic Antidepressants)

11. Sources:
 Harrison’s
 Oxford
 Google
http://crisbertcualteros.page.tl