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Etiology Unknown. Superoxide dismutase mutations may have a key role. Diagnosis Electrophysiology Electromyography (EMG) and electroneurography. Neurologic examination Early spastic weakness of the upper and lower extremities, typical subcutaneous muscle fasciculations, and bulbar involvement. Treatment No curative treatment is currently available. Symptomatic treatment . Rilutek(Riluzole) The only medicine approved by the FDA. Lived an average of 2-3 months longer than placebo. May protect nerve cells from exposure to too much glutamate by decreasing the release of glutamate. Ubidecarenone Coenzyme Q10 has been used for heart problems. Also been used for breast cancer, gum disease or muscle problems. Anesthetic Considerations Bulbar involvement and respiratory muscle weakness leads to a risk for aspiration and pulmonary complications. May increase sensitivity to the respiratory depressant effects of sedatives and hypnotics. Neuroaxial Anesthesia Can be safely performed since it avoids manipulation of the airways and respiratory complications. Onders RP, Carlin AM, Elmo M, Sivashankaran S, Katirji B, Schilz R. Combined spinal-epidural block in a patient with amyotrophic lateral sclerosis: case report. Am J Surg. 2009 Mar;197(3):386-90. Still controversy about the possible exacerbation by local anesthetics. General Inhalational Anesthesia Without muscle relaxant With muscle relaxant General inhalational Anesthesia With muscle relaxant Succinylcholine should be avoided. Risk for hyperkalemia as a result of denervation and immobilization. Nondepolarizing neuromuscular blocking agents should be used with great caution. May cause prolonged and pronounced neuromuscular blockade. Low dose rocuronium appears feasible and safe. Kim TK, Kwon JE, Kim YL. General anesthesia in a patient with amyotrophic lateral sclerosis. Korean J Anesthesiol. 2007;51:S18–9. Total intravenous Anesthesia Total intravenous anesthesia (TIVA) without muscle relaxant. Propofol is believed
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