Clinical Trial Nuedexta in Subjects With ALS

Inclusion Criteria

• ALS diagnosed as possible, laboratory-supported probable, probable, or definite as defined by revised El Escorial criteria
• Age 18 years or older
• Exhibits bulbar dysfunction manifested by dysarthria and/or dysphagia, according to PI judgment, exhibits a score of 55 or above on the CNS-Bulbar Function Scale
• Capable of providing informed consent and following trial procedures
• Geographic accessibility to the site
• Women must not be able to become pregnant for the duration of the study and must be willing to be on two contraceptive therapies
• Slow vital capacity (SVC) measure ≥50% of predicted for gender, height, and age at the screening visit
• Must be able to swallow capsules throughout the course of the study, according to PI judgment
• Subjects must not have taken riluzole for at least 30 days or be on a 50mg BID dose of riluzole for at least 30 days prior to randomization (subjects how have never taken riluzole are permitted in the study)
• Subjects taking anti-sialorrhea medication(s) must be on a stable dose for at least 30 days prior to randomization (anti-sialorrhea naïve subjects are permitted in the study)
• Must be able to safely swallow at least 30 milliliters (mLs) of water for the water swallowing test

Exclusion Criteria

• Prior use of Nuedexta®
• Current use of dextromethorphan, quinidine, quinine, mefloquine or opioids
• History of quinidine, quinine, or mefloquine-induced thrombocytopenia, hepatitis, or other hypersensitivity reactions
• History of known sensitivity or intolerability to dextromethorphan
• Use of an mono amine oxidase inhibitor (MAOI) or within 14 days of stopping an MAOI
• Prolonged QT interval, congenital long QT syndrome, history suggestive of torsades de pointes, or heart failure
• Complete atrioventricular (AV) block without implanted pacemaker, or subjects at high risk of complete AV block
• Concomitant use with drugs that both prolong QT interval and are metabolized by cytochrome P 2D6 (CYP2D6) (i.e., thioridazine or pimozide)
• Exposure to any other experimental agent (off-label use or investigational) within 30 days prior to Baseline Visit
• Invasive ventilator dependence, such as tracheostomy
• Any history of either substance abuse within the past year, unstable psychiatric disease, cognitive impairment, or dementia, according to PI judgment
• Placement and/or usage of feeding tube
• Pregnant women or women currently breastfeeding
• Unable to turn diaphragm pacing device off during swallowing tests
• Salivatory Botox within 90 days (3 months) of screening
• Salivatory radiation within 180 days (6 months) of screening