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.
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)