TY - JOUR
T1 - Burden of seizures and comorbidities in patients with epilepsy
T2 - a survey based on the tertiary hospital-based Epilepsy Syndrome Registry in Japan
AU - (Japan Rare Epilepsy Syndrome Registry Group: JRESG)
AU - Inoue, Yushi
AU - Hamano, Shin ichiro
AU - Hayashi, Masaharu
AU - Sakuma, Hiroshi
AU - Hirose, Shinichi
AU - Ishii, Atsushi
AU - Honda, Ryoko
AU - Ikeda, Akio
AU - Imai, Katsumi
AU - Jin, Kazutaka
AU - Kada, Akiko
AU - Kakita, Akiyoshi
AU - Kato, Mitsuhiro
AU - Kawai, Kensuke
AU - Kawakami, Tamihiro
AU - Kobayashi, Katsuhiro
AU - Matsuishi, Toyojiro
AU - Matsuo, Takeshi
AU - Nabatame, Shin
AU - Okamoto, Nobuhiko
AU - Ito, Susumu
AU - Okumura, Akihisa
AU - Saito, Akiko
AU - Shiraishi, Hideaki
AU - Shirozu, Hiroshi
AU - Saito, Takashi
AU - Sugano, Hidenori
AU - Takahashi, Yukitoshi
AU - Yamamoto, Hitoshi
AU - Fukuyama, Tetsuhiro
AU - Kuki, Ichiro
N1 - Publisher Copyright:
© 2022 Epileptic Disorders
PY - 2022/2
Y1 - 2022/2
N2 - Objective. 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. Methods. By analysing the cross-sectional data of patients registered in the tertiary hospital-based 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. Results. In total, 2,209 patients were registered. After excluding self-limited/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 (>90%), but some groups had relatively low proportions (<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. Significance. 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.
AB - Objective. 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. Methods. By analysing the cross-sectional data of patients registered in the tertiary hospital-based 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. Results. In total, 2,209 patients were registered. After excluding self-limited/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 (>90%), but some groups had relatively low proportions (<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. Significance. 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.
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U2 - 10.1684/epd.2021.1361
DO - 10.1684/epd.2021.1361
M3 - Article
C2 - 35118943
AN - SCOPUS:85123973979
SN - 1294-9361
VL - 24
SP - 82
EP - 94
JO - Epileptic Disorders
JF - Epileptic Disorders
IS - 1
ER -