Amyotrophic lateral sclerosis (ALS) has a prevalence of 5-7 per 100,000, more common in men aged 50 to 70, with most cases being idiopathic and some having autosomal dominant inheritance. It is characterized by upper and lower motor neuron degeneration leading to symptoms such as muscle weakness, dysarthria, and spasticity, with an overall poor prognosis as most patients die within 1-3 years of diagnosis. Treatment options are limited, primarily involving riluzole to modestly extend life, alongside supportive management through therapies and dietary assistance.

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