A 60-year old man with a 10-year history of multiple system atrophy (MSA) was found in respiratory arrest. After 4 months of respiratory support with two episodes of septic shock, he died. Autopsy disclosed severe atrophy of the mesencephalon, brainstem, medulla oblongata and cerebellum. Gallyas-Braak, alpha-synuclein and ubiquitin-positive inclusions in the cytoplasm of glial cells were evident, despite the severe ischaemic damage due to respiratory arrest and subsequent respiratory support for 4 months. The cause of respiratory arrest was not identified, but could be explained by the natural history of MSA. The bereaved family, who had suspected malpractice, was satisfied with the explanation based on the investigation performed by eight expert doctors, one expert nurse, two coordinator nurses and two lawyers in the model project promoted by the Japanese government.