A progressive neurodegenerative disease affecting upper and lower motor neurons, leading to muscle weakness, atrophy, spasticity, and ultimately respiratory failure. Average survival 3-5 years from symptom onset. No cure.

• 1Neurological examination
• 2MRI/CT imaging
• 3EEG
• 4EMG/NCV
• 5Lumbar puncture
• 6Genetic testing
• 7Neuropsychological testing

Median survival is 3-5 years from symptom onset. 10% survive >10 years (slow progressors). Respiratory failure is the primary cause of death. Riluzole extends survival by 2-3 months. Non-invasive ventilation (BiPAP) extends survival by 12-18 months when started at appropriate time. Gastrostomy feeding prevents malnutrition. Early multidisciplinary care improves quality of life and survival. Bulbar onset has worse prognosis (2-3 years) than limb onset (3-5 years).

• Head protection
• Cardiovascular health
• Cognitive stimulation
• Physical exercise
• Healthy diet
• Avoidance of toxins
• Regular monitoring