{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T20:20:11Z","timestamp":1789417211049,"version":"build-2803163510"},"reference-count":50,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,2,20]]},"abstract":"\n OBJECTIVE<\/jats:title>\n This study was performed to clarify the clinical and radiological features of patients with moyamoya disease under 4 years of age in Japan.<\/jats:p>\n <\/jats:sec>\n \n METHODS<\/jats:title>\n For this retrospective multicenter cohort study, the MACINTOSH Study (Moyamoya Disease with Aggressive Clinical Course in Infants for Safety and Healthy Growth Study), patients were included if they were younger than 4 years of age, were hospitalized due to transient ischemic attack (TIA) or stroke between January 2008 and December 2018, and had a diagnosis of moyamoya disease or moyamoya syndrome. Patient clinical and radiological data were collected between January 1, 2021, and December 31, 2021. A Central Judgement Committee performed blind evaluations of imaging studies.<\/jats:p>\n <\/jats:sec>\n \n RESULTS<\/jats:title>\n Seventy-one patients were included in the study, 31 males and 40 females, whose mean age was 29.7 \u00b1 12.7 months (0\u201347 months). Patients under 1 year of age had a strong male predominance (p = 0.0344) and no family history of moyamoya disease. Clinical diagnosis at disease onset included TIA (n = 31) and ischemic stroke (n = 40). All patients under 1 year of age presented with ischemic stroke. Among all the patients with ischemic stroke, seizure occurred at a mean age of 11.8 \u00b1 9.9 months compared with 29.5 \u00b1 11.7 months in those without ischemic stroke (p < 0.001). Most patients (69\/71) had a diagnosis of bilateral moyamoya disease. Suzuki disease stage 2 was the most frequent (53\/142 hemispheres). Among the 140 diseased hemispheres, 79 had cerebral infarcts, which were categorized as watershed zone infarcts (n = 64) and branch territory infarcts (n = 15). The branch territory infarcts were most frequently observed in Suzuki stage 2 hemispheres.<\/jats:p>\n <\/jats:sec>\n \n CONCLUSIONS<\/jats:title>\n This study demonstrated that demographic, clinical, and radiological characteristics are very specific in patients with moyamoya disease under 4 years of age, especially those under 1 year. Even a very early disease stage (Suzuki stage 2) could provoke TIA and\/or ischemic stroke through the mechanism of artery-to-artery embolism as well as hemodynamic ischemia.<\/jats:p>\n \n Clinical trial registration no.: UMIN000040169 (\n www.umin.ac.jp<\/jats:ext-link>\n )\n <\/jats:p>\n <\/jats:sec>","DOI":"10.3171\/2025.10.peds25480","type":"journal-article","created":{"date-parts":[[2026,2,21]],"date-time":"2026-02-21T02:34:18Z","timestamp":1771641258000},"page":"429-439","source":"Crossref","is-referenced-by-count":2,"title":["Unique clinical and radiological features in patients with moyamoya disease under 4 years of age: a multicenter MACINTOSH Study in Japan"],"prefix":"10.3171","volume":"37","author":[{"given":"Satoshi","family":"Kuroda","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shoko","family":"Hara","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Institute of Science Tokyo;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takeshi","family":"Funaki","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Kyoto University Graduate School of Medicine, Kyoto;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshio","family":"Araki","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Nagoya University School of Medicine, Nagoya;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryuta","family":"Saito","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Nagoya University School of Medicine, Nagoya;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eika","family":"Hamano","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, National Cerebral and Cardiovascular Center, Suita;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroharu","family":"Kataoka","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, National Cerebral and Cardiovascular Center, Suita;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Murai","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Kawasaki Medical School, Kurashiki;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomohito","family":"Hishikawa","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Kawasaki Medical School, Kurashiki;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaki","family":"Ito","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Hokkaido University Graduate School of Medicine, 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Tokyo;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takakazu","family":"Kawamata","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Tokyo Women\u2019s Medical University, Tokyo;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shusuke","family":"Yamamoto","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daina","family":"Kashiwazaki","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emiko","family":"Hori","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ataru","family":"Nishimura","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koji","family":"Yoshimoto","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasushi","family":"Takagi","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Graduate School of Biomedical Sciences, Tokushima University, Tokushima;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoru","family":"Miyawaki","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Faculty of Medicine, The University of 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