脳表ヘモジデリン沈着症の疫学

抄録

Superficial hemosiderosis (SHS) is a rare, neurodegenerative disease caused by toxic accumulation of hemosiderin on the surface of the brain and the spinal cord. With the Japanese guideline for the diagnosis and classification of SHS has been divided into three entities, according to the distribution of iron deposition and the clinical symptoms : “classical” type, “localized” type and “atypical” type. In “classical” type SHS. With our questionnaire survey to 792 medical institutes of the Japanese Society of Neurology, to collect information about SHS, estimated total number of patients with SHS in 2017 was 128 at 55 institutes. Among 122 patients with available data, 63% of patients had “classical” type (c–SHS), 24% had “localized” type (l–SHS), and 10% had “atypical” type (a–SHS). In this article, more detailed features of epidemiological findings about patients with SHS in Japan.