Snapshot A 61-year-old male presents with left-sided hand weakness and trouble with walking. He is not sure why these symptoms occur. On physical exam, tongue fasciculations are appreciated. He has slow speech. The left upper extremity shows forearm atrophy and depressed reflexes. The right lower extremity is hypertonic, with 3+ reflexes, and positive Babinski sign. Introduction A progressive neurodegenerative disease affecting both upper and lower motor neurons login to view 3 more bullets Certain cases may be due to superoxide dismutase type 1 (SOD-1) mutations login to view 1 more bullet Epidemiology Age of onset: login to view 2 more bullets Presentation Symptoms login to view 13 more bullets Evaluation LMN signs determined by physical exam, electrophysiologic, or neuropathologic studies UMN signs determined by physical exam Symptom progression spread or signs No other disease process can explain the patient's findings EMG shows widespread denervation and fibrillation potential Differential Benign fasciculations Spinal muscular atrophy Poliomyelitis Multiple sclerosis Syringomyelia Treatment Riluzole login to view 2 more bullets Edaravone Supportive treatment login to view 2 more bullets Complications Weight loss Respiratory failure Sialorrhea Depression Prognosis Main cause of death is respiratory failure