A proactive approach in symptomatic management of amyotrophic lateral sclerosis

Abstract

Amyotrophic lateral sclerosis (ALS) is an adult–onset motor neuron syndrome characterized by selective involvement of motor and cortical neurons in the frontotemporal lobe, brainstem, and spinal cord, eventually leading to multisystem neurodegeneration. Currently, it is not possible to alleviate progressive skeletal muscle wasting and weakness. In addition to the motor symptoms of ALS, non–motor symptoms such as pain, fatigue, cognitive impairment, anxiety, depression, pseudobulbar affect, sleep disturbances, and weight loss can occur. Although not all symptoms appear in all patients with ALS, it is necessary to know the characteristics of non–motor symptoms and practice multidisciplinary medical care to mitigate them as much as possible. Common symptomatic approaches include (1) prevention whenever possible, (2) listening actively to symptoms and assessing their nature and severity, (3) identifying the cause and contributing factors by physical examination, (4) communicating with the patient and caregivers in an easy–to–understand manner, and (5) considering from non–pharmacological therapy to medication. Morphine is effective against the intractable pain and heaviness as well as dyspnea associated with ALS, and can be considered even in the early stage of the disease. Based on the Japanese clinical practice guideline for ALS revised in 2023, I will review here an update on symptomatic management for patients with ALS. There is a great need to elucidate the pathophysiological basis and accumulate evidence for symptomatic treatment through intervention studies that focus on non–motor symptoms.