Motor
Amyotrophic Lateral Sclerosis
search
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 Potential
s
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 Antidepressant
s)
Type your search phrase here