Slowly progressive, idiopathic neuro-degenerative disorder of upper and lower motor neurons
Risk Factors
Age onset 40-70
Family hx
Head trauma (e.g. military combat related)
Symptoms
Dysphagia
Laryngospasm
SOB
Can present w/predominantly bulbar symptoms (25%) including weak, low pitched, monotone, breathy speech, requiring extra effort, speech difficult to understand
Others present w/limb dominant symptoms (70%) including weakness, wasting, spasticiity
Difficulty swallowing
Diminished cough
Emotional lability including pathologic laughing
No sensory complaints
~1y from symptom onset to diagnosis
Avg 3-5y from diagnosis to death (highly variable); shorter for bulbar dz
Physical Exam Findings
Bulbar findings include: tongue fasciculations, tongue muscle wasting, loss of rise in palate w/"ahh," weak/breathy voice
Limb findings include: muscle weakness (flaccid or spastic), muscle wasting/atrophy often most apparent in hands, fasciculations of any muscle group, spasticity, hyper-reflexia, + Babinski
Normal sensation
Tests
EMG/NCS
MRI brain, Spine to exclude other CNS disease, cord compression