Total intravenous anesthesia using remimazolam for primary lateral sclerosis

Primary lateral sclerosis (PLS) is an extremely rare neurodegenerative muscle disease of unknown cause. The choice of anesthesia for surgery in patients with neurodegenerative muscle disease is extremely important, as it may affect the patient’s prognosis. In this case report, we describe a 74-year-old man with PLS who underwent surgical closure of the larynx owing to repeated aspiration pneumonia. The surgery was performed without muscle relaxants and with total intravenous anesthesia using remimazolam, a relatively new, ultra-short-acting benzodiazepine sedative. The patient underwent surgery safely with no complications and had stable intraoperative hemodynamics. General anesthesia using remimazolam and remifentanil without muscle relaxants may be an appropriate anesthesia technique in patients with PLS who require surgery.