AAN GUIDELINES Bundle

Drug Therapies Î Riluzole should be offered to slow disease progression (A). Î In patients developing fatigue while taking riluzole, once risks of fatigue versus modest survival benefits have been discussed, withholding the drug may be considered (C). Î There are insufficient data at this time to support or refute treatment with lithium carbonate (U). Sialorrhea Î In patients who have medically refractory sialorrhea, botulinum toxin type B (BTxB) should be considered (B), and low-dose radiation therapy to the salivary glands may be considered (C). Remark: In ALS and other diseases, anticholinergic medications are generally tried first to reduce sialorrhea, although effectiveness is unproven. Pseudobulbar Affect Remark: Pseudobulbar affect—excessive laughing or crying or involuntary emotional expression disorder—affects 20%-50% of patients with ALS, especially in pseudobulbar palsy. Î If approved by the FDA, and if side effects are acceptable, a fixed-dose combination of dextromethorphan 20 mg/quinidine 10 mg should be considered for symptoms of pseudobulbar affect (B). UPDATE: FDA approved 2010 Cramps/Spasticity Î There are insufficient data to support or refute any specific intervention for the treatment of cramps (eg, quinine, gabapentin, vitamin E, and riluzole) (U). Î There are insufficient data to support or refute exercise or medication for treating spasticity (U). Remark: In multiple sclerosis and cerebral palsy, benzodiazepam, baclofen, dantrolene, and tizanidine are effective in reducing spasticity-related symptoms. Nutrition Management Î In patients with impaired oral food intake, enteral nutrition via percutaneous endoscopic gastrostomy (PEG) should be considered to stabilize body weight (B). However, there are insufficient data to support or refute specific timing of PEG insertion (U). Î PEG should be considered for prolonging survival (B). However, there are insufficient data to support or refute PEG for improving quality of life (U). Î Creatine, in doses of 5-10 g daily, should NOT be given as treatment because it is not effective in slowing disease progression (A). Î High-dose vitamin E should NOT be considered as treatment (B), while the equivocal evidence regarding low-dose vitamin E permits no recommendation (U). Selecting a Treatment Regimen

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