Acute Adenosine Receptor Antagonism to Promote Breathing Plasticity in ALS
The purpose of this research study is to determine the effects of a medication, istradefylline, in conjunction with breathing air with reduced oxygen for short periods of time (called acute intermittent hypoxia, or AIH), on breathing. This project will study breathing in people with amyotrophic lateral sclerosis (ALS) and unaffected, age-matched adults. Istradefylline is prescribed to increase movement in people with other neuromuscular conditions. A recently completed study found that people with ALS took deeper breaths, 60 minutes after using AIH.
• Non-smoking adults aged 21-80 years will be eligible to participate.
• \- Upon screening, eligible patients will have an
• ALS diagnosis (El Escorial diagnostic classifications of probable/definite ALS),
• vital capacity (VC) \> 60% of predicted value, and
• ALS Functional Rating Scale (ALSFRS-R) scores of 2 or greater for bulbar and respiratory items: swallowing, speech, salivation, dyspnea, orthopnea, and respiratory insufficiency.
• Additionally, patients taking riluzole and/or edaravone must be on a stable dose for \>30 days.
• Unaffected control subjects will be eligible if they have a vital capacity (VC) \> 60% of predicted value.