Amyotrophic Lateral Sclerosis

Classification of Management Recommendations Classification Definition Strong (A) Established as effective, ineffective, or harmful (or established as useful/predictive or not useful/predictive) for the given condition in the specified population. (Level A rating requires at least two consistent Class I studies.)* Moderate (B) Probably effective, ineffective, or harmful (or probably useful/ predictive or not useful/predictive) for the given condition in the specified population. (Level B rating requires at least one Class I study or two consistent Class II studies.) Weak (C) Possibly effective, ineffective, or harmful (or possibly useful/ predictive or not useful/predictive) for the given condition in the specified population. (Level C rating requires at least one Class II study or two consistent Class III studies.) Insufficient (U) Data inadequate or conflicting; given current knowledge, treatment (test, predictor) is unproven. *In exceptional cases, one convincing Class I study may suffice for an "A" recommendation if 1) all criteria are met, 2) the magnitude of effect is large (relative rate improved outcome > 5 and the lower limit of the confidence interval is > 2). Abbreviations AAN, American Academy of Neurology; ALS, amyotrophic lateral sclerosis; bid, twice daily; BTxB, botulinum toxin type B; DM, dextromethorphan; FDA, Food and Drug Administration; FVC, forced vital capacity; h, hour(s); mg, milligram; MIE, mechanical insufflation/exsufflation; MIP, maximal inspiratory pressure; PCEF, peak cough expiratory flow; PEG, percutaneous endoscopic gastrostomy; Q, quinidine; q, every; SNP, sniff nasal pressure; TIV, tracheostomy invasive ventilation; VC, vital capacity Source Miller RG, Jackson CE, Kasarskis EJ, et al. Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review). Neurology. 2009; 73(15):1218-1226. [PMID: 19822872] Miller RG, Jackson CE, Kasarskis EJ, et al. Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review). Neurology. 2009;73(15):1227-1233. [PMID: 19822873] Endorsed by the American Association of Neuromuscular and Electrodiagnostic Medicine. Disclaimer This Guideline attempts to define principles of practice that should produce high-quality patient care. It is applicable to specialists, primary care, and providers at all levels. This Guideline should not be considered exclusive of other methods of care reasonably directed at obtaining the same results. The ultimate judgment concerning the propriety of any course of conduct must be made by the clinician aſter consideration of each individual patient situation. Neither IGC, the medical associations, nor the authors endorse any product or service associated with the distributor of this clinical reference tool. 5740 Executive Drive Suite 218 Baltimore, MD 21228 TEL: 410-869-3332 • FAX: 410-744-2150 For additional copies: orders@GuidelineCentral.com Copyright © 2012 All rights reserved

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