Case study: improvement in amyotrophic lateral sclerosis symptoms in a patient receiving edaravone infusions

Abstract

Background: HB is a 52-year-old female who was diagnosed with amyotrophic lateral sclerosis (ALS) in February 2017. Her symptoms began in September 2016 with right-hand weakness, and they rapidly declined as demonstrated in decreased ALS Functional Rating Scale-Revised (ALSFRS-R) assessment score results from 27/48 in April 2017 to 18/48 in August 2017. HB was prescribed riluzole at the time of diagnosis and began receiving edaravone infusions in November 2017. During the initial visit for the edaravone infusions, the visiting nurse evaluated the patient to be bedbound with no movement of any extremities. She was unable to facially express any emotions except for crying, and she was nonverbal but able to communicate using a speech-generating device. Edaravone has been proven to slow the progression of ALS symptoms. However, after receiving several cycles of edaravone infusions, HB has demonstrated an improvement in specific categories of her ALS symptoms.Objectives: The purpose of this case study is to describe the potential improvement of ALS symptoms in a patient receiving edaravone infusions.Methods: A retrospective review of the patient’s medical documentation from July 2017 to December 2017 was performed, which comprised of the prescriber’s office visit notes, the home infusion company’s multi-disciplinary assessments, and interviews with the patient’s family members.Results: HB received a total of three infusion cycles with edaravone. Her ALSFRS-R scores improved from 16/48 to 21/48. Improvements were noted in the following categories: ability to turn in bed, dyspnea, orthopnea, and respiratory insufficiency. After completion of the first cycle, the patient’s family members reported that she had more energy and was no longer napping throughout the day. While being transferred from the bed to a wheelchair, the patient was able to assist by using her upper and lower extremities. Most notable, however, were the observations made by the visiting nurse. When returning for the second cycle of edaravone infusions, the patient was sitting up in a chair and greeted the nurse with a smile and said “Hi.” This was the first clear verbal communication the patient had since becoming nonverbal in August 2017.Conclusions: Though edaravone has only been clinically proven to slow the progression of ALS symptoms, HB has demonstrated symptom reversal in several categories, including respiratory function and mobility of her extremities. Data will continue to be collected and evaluated with each edaravone cycle.