Motor Neuron Disease and Delicate Anesthesia Choices – Anesthesia for Motor Neuron Disease Patients

Abstract

Motor neuron diseases (MNDs), two major types of which are amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA), are caused by upper and/or lower motor neuron degeneration and death. They manifest with progressive skeletal muscle atrophy. Most ALS cases are idiopathic, whereas the cause of SMA is genetic. There is no cure for MNDs and anesthetic management is challenging due to patients’ respiratory dysfunction, abnormal response to muscle relaxants, and high risk of aspiration. General guidelines for this purpose state that intravenous administration of propofol and remifentanil are preferred. Muscle relaxants should be used sparingly due to their causing ventilatory depression, and depolarizing neuromuscular blockers should be avoided entirely for patients’ risk of hyperkalemia. This chapter discusses the etiology of MNDs, their clinical features, disease prognosis, palliative treatments, necessary surgical procedures, and preoperative and postoperative anesthetic management. It covers ALS, SMA, and other less common MNDs.