Anti-ganglionic acetylcholine receptor antibody causes prolonged megacolon in a patient with amyotrophic lateral sclerosis

A 54-year-old man who was diagnosed as having thoracic cord precedence type amyotrophic lateral sclerosis (ALS) received mandatory ventilation under tracheostomy 2.5 years after disease onset. Though mechanical ventilation increased the patient's gastrointestinal gas, oral ingestion was possible. Colon gas became conspicuous during the treatment, an enema was needed every day, and nutrition via enteron became difficult. Pupil abnormality, orthostatic hypotension, and abnormal sweating were not observed. After anti-ganglionic acetylcholine receptor antibody, status was determined to be positive, steroid pulse therapy was performed, and it decreased gastrointestinal gas significantly; however, the patient died of a repeated fungal infection. Pain and deterioration of the nutritional condition caused by meteorism made the activity of daily living and quality of life of this ALS patient decrease remarkably. Over three percent of ALS patients are reported to have anti-ganglionic acetylcholine receptor antibodies. We should consider autoimmune autonomic ganglionopathy in ALS patients.