{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:35:06Z","timestamp":1775003706245,"version":"3.50.1"},"reference-count":13,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2022,2,15]],"date-time":"2022-02-15T00:00:00Z","timestamp":1644883200000},"content-version":"vor","delay-in-days":14,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epileptic Disorders"],"published-print":{"date-parts":[[2022,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:italic>Objective<\/jats:italic>. To examine the current medical and psychosocial status of patients with epilepsy, aiming to facilitate appropriate application of the Intractable\/Rare Diseases Act of Japan.<\/jats:p><jats:p><jats:italic>Methods<\/jats:italic>. By analysing the cross\u2010sectional data of patients registered in the tertiary hospital\u2010based Epilepsy Syndrome Registry of Japan, we investigated the proportion of patients who met the severity criteria as defined by the Act (seizure frequency of at least once a month, or presence of intellectual\/neurological\/psychiatric symptoms, or both) and whether there are candidate syndrome\/diseases to be added to the existing list in the Act.<\/jats:p><jats:p><jats:italic>Results<\/jats:italic>. In total, 2,209 patients were registered. After excluding self\u2010limited\/idiopathic epilepsies, 1,851 of 2,110 patients (87.7%) met the severity criteria. The patients were classified into eight main epilepsy syndromes (594 patients), 20 groups based on aetiology (1,078 patients), and three groups without known aetiology (427 patients). Most of the groups classified by syndrome or aetiology had high proportions of patients satisfying the severity criteria (&gt;90%), but some groups had relatively low proportions (&lt;80%) resulting from favourable outcome of surgical therapy. Several small groups with known syndrome\/aetiology await detailed analysis based on a sufficiently large enough number of patients registered, some of whom may potentially be added to the list of the Act.<\/jats:p><jats:p><jats:italic>Significance<\/jats:italic>. The registry provides data to examine the usefulness of the severity criteria and list of diseases that are operationally defined by the Act. Most epilepsy patients with various syndromes\/diseases and aetiology groups are covered by the Act but some are not, and the list of designated syndromes\/diseases should be complemented by further amendments, as suggested by future research.<\/jats:p>","DOI":"10.1684\/epd.2021.1361","type":"journal-article","created":{"date-parts":[[2022,2,4]],"date-time":"2022-02-04T11:53:42Z","timestamp":1643975622000},"page":"82-94","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Burden of seizures and comorbidities in patients with epilepsy: a survey based on the tertiary hospital\u2010based Epilepsy Syndrome Registry in Japan"],"prefix":"10.1002","volume":"24","author":[{"given":"Yushi","family":"Inoue","sequence":"first","affiliation":[{"name":"NHO Shizuoka Institute of Epilepsy and Neurological Disorders  Shizuoka Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shin\u2010ichiro","family":"Hamano","sequence":"additional","affiliation":[{"name":"Division of Neurology, Saitama Children's Medical Center  Saitama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masaharu","family":"Hayashi","sequence":"additional","affiliation":[{"name":"Department of Brain Development and Neural Regeneration Tokyo Metropolitan Institute of Medical Science  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroshi","family":"Sakuma","sequence":"additional","affiliation":[{"name":"Department of Brain Development and Neural Regeneration Tokyo Metropolitan Institute of Medical Science  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shinichi","family":"Hirose","sequence":"additional","affiliation":[{"name":"Department of Pediatrics School of Medicine, Fukuoka University  Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Atsushi","family":"Ishii","sequence":"additional","affiliation":[{"name":"Department of Pediatrics School of Medicine, Fukuoka University  Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryoko","family":"Honda","sequence":"additional","affiliation":[{"name":"Department of Pediatrics NHO Nagasaki Medical Center  Nagasaki Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akio","family":"Ikeda","sequence":"additional","affiliation":[{"name":"Department of Epilepsy, Movement Disorders and Physiology Kyoto University Graduate School of Medicine  Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katsumi","family":"Imai","sequence":"additional","affiliation":[{"name":"NHO Shizuoka Institute of Epilepsy and Neurological Disorders  Shizuoka 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