{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T01:05:40Z","timestamp":1779843940925,"version":"3.53.1"},"reference-count":46,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"21","license":[{"start":{"date-parts":[[2026,5,25]],"date-time":"2026-05-25T00:00:00Z","timestamp":1779667200000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Neurosurg Case Lessons"],"published-print":{"date-parts":[[2026,5,25]]},"abstract":"<jats:sec>\n                    <jats:title>BACKGROUND<\/jats:title>\n                    <jats:p>Aplastic or twig-like middle cerebral artery (Ap\/T-MCA) is a rare cerebrovascular anomaly characterized by unilateral stenosis of the proximal middle cerebral artery (MCA) with a plexiform arterial network along the horizontal (M1) segment, requiring distinction from moyamoya disease. Genetically confirmed cases remain rare.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>OBSERVATIONS<\/jats:title>\n                    <jats:p>\n                      We report the cases of 3 patients with Ap\/T-MCA carrying the\n                      <jats:italic>RNF213<\/jats:italic>\n                      (ring finger protein 213) variant. The first patient, a man in his 60s, presented with subarachnoid hemorrhage. The second patient, a woman in her 20s, developed involuntary movement of the left upper limb. The third patient, the older sister of the second patient, experienced transient left hemiparesis in her 40s. In all patients, MR angiography demonstrated unilateral MCA abnormalities, and digital subtraction angiography revealed M1 stenosis with a plexiform arterial network in the subarachnoid space. All patients were managed conservatively, and targeted sequencing confirmed the\n                      <jats:italic>RNF213<\/jats:italic>\n                      p.R4810K variant in each case. Only 2 Ap\/T-MCA patients with this variant have been previously reported.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>LESSONS<\/jats:title>\n                    <jats:p>\n                      The presence of the\n                      <jats:italic>RNF213<\/jats:italic>\n                      p.R4810K variant in all 3 patients supports a relationship between twig-like MCA and the\n                      <jats:italic>RNF213<\/jats:italic>\n                      variant. These findings suggest that Ap\/T-MCA may represent the\n                      <jats:italic>RNF213<\/jats:italic>\n                      -related vasculopathy spectrum, and the occurrence in two sisters indicates a hereditary predisposition.\n                    <\/jats:p>\n                    <jats:p>\n                      <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"https:\/\/thejns.org\/doi\/10.3171\/CASE26186\">https:\/\/thejns.org\/doi\/10.3171\/CASE26186<\/jats:ext-link>\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/case26186","type":"journal-article","created":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T14:52:35Z","timestamp":1779288755000},"source":"Crossref","is-referenced-by-count":0,"title":["Aplastic or twig-like middle cerebral artery with the RNF213 variant: illustrative cases"],"prefix":"10.3171","volume":"11","author":[{"given":"Ryo","family":"Tamura","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, National Hospital Organization Iwakuni Clinical Center, Iwakuni, Yamaguchi"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9266-2543","authenticated-orcid":true,"given":"Kodai","family":"Kume","sequence":"additional","affiliation":[{"name":"Department of Molecular Epidemiology, Research Institute for Radiation Biology and Medicine, Hiroshima University, Hiroshima"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-3277-6434","authenticated-orcid":true,"given":"Masato","family":"Kawakami","sequence":"additional","affiliation":[{"name":"Department of Neurological Surgery, Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences, Okayama, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1405-0901","authenticated-orcid":true,"given":"Hideshi","family":"Kawakami","sequence":"additional","affiliation":[{"name":"Department of Molecular Epidemiology, Research Institute for Radiation Biology and Medicine, Hiroshima University, Hiroshima"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8395-072X","authenticated-orcid":true,"given":"Satoshi","family":"Murai","sequence":"additional","affiliation":[{"name":"Neurovascular Research Unit, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Charlestown, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noboru","family":"Kusaka","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, National Hospital Organization Iwakuni Clinical Center, Iwakuni, Yamaguchi"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hayato","family":"Kotani","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, National Hospital Organization Iwakuni Clinical Center, Iwakuni, Yamaguchi"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takayuki","family":"Kakimoto","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, National Hospital Organization Iwakuni Clinical Center, Iwakuni, Yamaguchi"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuhiko","family":"Nishi","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, National Hospital Organization Iwakuni Clinical Center, Iwakuni, Yamaguchi"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hisakazu","family":"Itami","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, National Hospital Organization Iwakuni Clinical Center, Iwakuni, Yamaguchi"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinji","family":"Otsuka","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, National Hospital Organization Iwakuni Clinical Center, Iwakuni, Yamaguchi"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tsukasa","family":"Nishiura","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, National Hospital Organization Iwakuni Clinical Center, Iwakuni, Yamaguchi"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-0146-4198","authenticated-orcid":true,"given":"Kotaro","family":"Ogihara","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, National Hospital Organization Iwakuni Clinical Center, Iwakuni, Yamaguchi"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"issue":"9","key":"bib1","series-title":"Neuroradiology","first-page":"690","article-title":"Endovascular treatment of an \u201canterior cerebral artery\u201d aneurysm in a patient with \u201cembryonic unfused middle cerebral artery\u201d anomaly: a case report","volume":"47","author":"Cekirge","year":"2005","unstructured":"Cekirge HSPeynircioglu BSaatci I.Endovascular treatment of an \u201canterior cerebral artery\u201d aneurysm in a patient with \u201cembryonic unfused middle cerebral artery\u201d anomaly: a case report. Neuroradiology. 2005;47(9):690-694. doi:10.1007\/s00234-005-1407-3"},{"issue":"1","key":"bib2","series-title":"Cerebrovasc Dis","first-page":"1","article-title":"Aneurysms in twig-like middle cerebral artery","volume":"20","author":"Liu","year":"2005","unstructured":"Liu HMLai DMTu YKWang YH.Aneurysms in twig-like middle cerebral artery. Cerebrovasc Dis. 2005;20(1):1-5. doi:10.1159\/000086119"},{"issue":"6","key":"bib3","series-title":"J Neurosurg","first-page":"1718","article-title":"Radiological and clinical features of twig-like middle cerebral artery in comparison with moyamoya angiopathy: a multicenter retrospective study","volume":"137","author":"Goto","year":"2022","unstructured":"Goto YNanto MOka HRadiological and clinical features of twig-like middle cerebral artery in comparison with moyamoya angiopathy: a multicenter retrospective study. J Neurosurg. 2022;137(6):1718-1726. doi:10.3171\/2022.2.JNS212338"},{"issue":"7","key":"bib4","series-title":"PLoS One","first-page":"e22542","article-title":"Identification of RNF213 as a susceptibility gene for moyamoya disease and its possible role in vascular development","volume":"6","author":"Liu","year":"2011","unstructured":"Liu WMorito DTakashima SIdentification of RNF213 as a susceptibility gene for moyamoya disease and its possible role in vascular development. PLoS One. 2011;6(7):e22542. doi:10.1371\/journal.pone.0022542"},{"issue":"6","key":"bib5","series-title":"No Shinkei Geka","first-page":"533","article-title":"Ruptured aneurysm of an aplastic or twig-like middle cerebral artery with Ring Finger Protein 213 mutation: a case report","volume":"48","author":"Fukuyama","year":"2020","unstructured":"Fukuyama RYamamura KMurata HMiyatake SMatsumoto NAbe H.Ruptured aneurysm of an aplastic or twig-like middle cerebral artery with Ring Finger Protein 213 mutation: a case report. Article in Japanese. No Shinkei Geka. 2020;48(6):533-540. doi:10.11477\/mf.1436204224"},{"issue":"6","key":"bib6","series-title":"Interv Neuroradiol","first-page":"634","article-title":"Aplastic or twig-like middle cerebral artery and cardiogenic cerebral embolism mimicking moyamoya disease with RNF213 polymorphism: a case report","volume":"28","author":"Inoue","year":"2022","unstructured":"Inoue HOomura MNishikawa YMase MMatsukawa N.Aplastic or twig-like middle cerebral artery and cardiogenic cerebral embolism mimicking moyamoya disease with RNF213 polymorphism: a case report. Interv Neuroradiol. 2022;28(6):634-638. doi:10.1177\/15910199211062016"},{"issue":"3","key":"bib7","series-title":"J Stroke Cerebrovasc Dis","first-page":"107582","article-title":"Non-aneurysmal subarachnoid hemorrhage in aplastic or twig-like middle cerebral artery: a case report and literature review","volume":"33","author":"Kawakami","year":"2024","unstructured":"Kawakami MMurai SKusaka NNon-aneurysmal subarachnoid hemorrhage in aplastic or twig-like middle cerebral artery: a case report and literature review. J Stroke Cerebrovasc Dis. 2024;33(3):107582. doi:10.1016\/j.jstrokecerebrovasdis.2024.107582"},{"key":"bib8","series-title":"Clin Imaging","first-page":"31","article-title":"Twig-like middle cerebral arteries: clinical and radiological findings","volume":"73","author":"Onoue","year":"2021","unstructured":"Onoue KNguyen TNMian ADasenbrock HBedi HAbdalkader M.Twig-like middle cerebral arteries: clinical and radiological findings. Clin Imaging. 2021;73:31-37. doi:10.1016\/j.clinimag.2020.11.049"},{"issue":"4","key":"bib9","series-title":"Interv Neuroradiol","first-page":"584","article-title":"Twig-like middle cerebral artery: embryological persistence or secondary consequences?","volume":"27","author":"Ota","year":"2021","unstructured":"Ota TKomiyama M.Twig-like middle cerebral artery: embryological persistence or secondary consequences? Interv Neuroradiol. 2021;27(4):584-587. doi:10.1177\/15910199211024077"},{"issue":"6","key":"bib10","series-title":"Neurol Med Chir (Tokyo)","first-page":"261","article-title":"Anomalies of the middle cerebral artery","volume":"57","author":"Uchiyama","year":"2017","unstructured":"Uchiyama N.Anomalies of the middle cerebral artery. Neurol Med Chir (Tokyo). 2017;57(6):261-266. doi:10.2176\/nmc.ra.2017-0043"},{"issue":"4","key":"bib11","series-title":"AJNR Am J Neuroradiol","first-page":"585","article-title":"Angiographic and embryologic considerations in five cases of middle cerebral artery fenestration","volume":"23","author":"Gailloud","year":"2002","unstructured":"Gailloud PAlbayram SFasel JHBeauchamp NJMurphy KJ.Angiographic and embryologic considerations in five cases of middle cerebral artery fenestration. AJNR Am J Neuroradiol. 2002;23(4):585-587."},{"issue":"6","key":"bib12","series-title":"Neurosurgery","first-page":"1472","article-title":"Clinical and radiological features of patients with aplastic or twiglike middle cerebral arteries","volume":"70","author":"Seo","year":"2012","unstructured":"Seo BSLee YSLee HGLee JHRyu KYKang DG.Clinical and radiological features of patients with aplastic or twiglike middle cerebral arteries. Neurosurgery. 2012;70(6):1472-1480. doi:10.1227\/NEU.0b013e318246a510"},{"key":"bib13","series-title":"Surg Neurol Int","first-page":"192","article-title":"De novo formation of twig-like middle cerebral artery: an illustrative case","volume":"14","author":"Kajita","year":"2023","unstructured":"Kajita MYanaka KHanai SDe novo formation of twig-like middle cerebral artery: an illustrative case. Surg Neurol Int. 2023;14:192. doi:10.25259\/SNI_274_2023"},{"issue":"1","key":"bib14","series-title":"Neurosurg Rev","first-page":"82","article-title":"Adult unilateral moyamoya disease with familial occurrence in two definite cases: a case report and review of the literature","volume":"29","author":"Kusaka","year":"2006","unstructured":"Kusaka NTamiya TAdachi YAdult unilateral moyamoya disease with familial occurrence in two definite cases: a case report and review of the literature. Neurosurg Rev. 2006;29(1):82-87. doi:10.1007\/s10143-005-0406-5"},{"issue":"7","key":"bib15","series-title":"Neurol Med Chir (Tokyo)","first-page":"307","article-title":"Diagnostic criteria for moyamoya disease\u20142021 revised version","volume":"62","author":"Kuroda","year":"2022","unstructured":"Kuroda SFujimura MTakahashi JDiagnostic criteria for moyamoya disease\u20142021 revised version. Neurol Med Chir (Tokyo). 2022;62(7):307-312. doi:10.2176\/jns-nmc.2022-0072"},{"issue":"1","key":"bib16","series-title":"J Hum Genet","first-page":"34","article-title":"A genome-wide association study identifies RNF213 as the first moyamoya disease gene","volume":"56","author":"Kamada","year":"2011","unstructured":"Kamada FAoki YNarisawa AA genome-wide association study identifies RNF213 as the first moyamoya disease gene. J Hum Genet. 2011;56(1):34-40. doi:10.1038\/jhg.2010.132"},{"issue":"1","key":"bib17","series-title":"Neurol India","first-page":"35","article-title":"RNF213 polymorphism and moyamoya disease: a systematic review and meta-analysis","volume":"61","author":"Ma","year":"2013","unstructured":"Ma JLiu YMa LHuang SLi HYou C.RNF213 polymorphism and moyamoya disease: a systematic review and meta-analysis. Neurol India. 2013;61(1):35-39. doi:10.4103\/0028-3886.107927"},{"issue":"4","key":"bib18","series-title":"Life (Basel)","first-page":"555","article-title":"RNF213-associated vascular disease: a concept unifying various vasculopathies","volume":"12","author":"Hiraide","year":"2022","unstructured":"Hiraide TSuzuki HMomoi MRNF213-associated vascular disease: a concept unifying various vasculopathies. Life (Basel). 2022;12(4):555. doi:10.3390\/life12040555"},{"issue":"3","key":"bib19","series-title":"Medicina (Tokyo)","first-page":"590","article-title":"Genetic studies in cerebrovascular disease: RNF213-related vasculopathy","volume":"60","author":"Okazaki","year":"2023","unstructured":"Okazaki S.Genetic studies in cerebrovascular disease: RNF213-related vasculopathy. Medicina (Tokyo). 2023;60(3):590-594."},{"issue":"2","key":"bib20","series-title":"Circulation","first-page":"295","article-title":"Moyamoya disease susceptibility variant RNF213 p.R4810K increases the risk of ischemic stroke attributable to large-artery atherosclerosis","volume":"139","author":"Okazaki","year":"2019","unstructured":"Okazaki SMorimoto TKamatani YMoyamoya disease susceptibility variant RNF213 p.R4810K increases the risk of ischemic stroke attributable to large-artery atherosclerosis. Circulation. 2019;139(2):295-298. doi:10.1161\/CIRCULATIONAHA.118.038439"},{"issue":"8","key":"bib21","series-title":"Lancet Neurol","first-page":"747","article-title":"Moyamoya disease: diagnosis and interventions","volume":"21","author":"Ihara","year":"2022","unstructured":"Ihara MYamamoto YHattori YMoyamoya disease: diagnosis and interventions. Lancet Neurol. 2022;21(8):747-758. doi:10.1016\/S1474-4422(22)00165-X"},{"issue":"1","key":"bib22","series-title":"Sci Rep","first-page":"22163","article-title":"Ring finger protein 213 c.14576G>A mutation is not involved in internal carotid artery and middle cerebral artery dysplasia","volume":"11","author":"Murai","year":"2021","unstructured":"Murai YIshisaka EWatanabe ARing finger protein 213 c.14576G>A mutation is not involved in internal carotid artery and middle cerebral artery dysplasia. Sci Rep. 2021;11(1):22163. doi:10.1038\/s41598-021-01623-6"},{"issue":"11","key":"bib23","series-title":"Neurology","first-page":"803","article-title":"Homozygous c.14576G>A variant of RNF213 predicts early-onset and severe form of moyamoya disease","volume":"78","author":"Miyatake","year":"2012","unstructured":"Miyatake SMiyake NTouho HHomozygous c.14576G>A variant of RNF213 predicts early-onset and severe form of moyamoya disease. Neurology. 2012;78(11):803-810. doi:10.1212\/WNL.0b013e318249f71f"},{"issue":"9","key":"bib1","series-title":"Neuroradiology","first-page":"690","article-title":"Endovascular treatment of an \u201canterior cerebral artery\u201d aneurysm in a patient with \u201cembryonic unfused middle cerebral artery\u201d anomaly: a case report","volume":"47","author":"Cekirge","year":"2005","unstructured":"Cekirge HSPeynircioglu BSaatci I.Endovascular treatment of an \u201canterior cerebral artery\u201d aneurysm in a patient with \u201cembryonic unfused middle cerebral artery\u201d anomaly: a case report. Neuroradiology. 2005;47(9):690-694. doi:10.1007\/s00234-005-1407-3"},{"issue":"1","key":"bib2","series-title":"Cerebrovasc Dis","first-page":"1","article-title":"Aneurysms in twig-like middle cerebral artery","volume":"20","author":"Liu","year":"2005","unstructured":"Liu HMLai DMTu YKWang YH.Aneurysms in twig-like middle cerebral artery. Cerebrovasc Dis. 2005;20(1):1-5. doi:10.1159\/000086119"},{"issue":"6","key":"bib3","series-title":"J Neurosurg","first-page":"1718","article-title":"Radiological and clinical features of twig-like middle cerebral artery in comparison with moyamoya angiopathy: a multicenter retrospective study","volume":"137","author":"Goto","year":"2022","unstructured":"Goto YNanto MOka HRadiological and clinical features of twig-like middle cerebral artery in comparison with moyamoya angiopathy: a multicenter retrospective study. J Neurosurg. 2022;137(6):1718-1726. doi:10.3171\/2022.2.JNS212338"},{"issue":"7","key":"bib4","series-title":"PLoS One","first-page":"e22542","article-title":"Identification of RNF213 as a susceptibility gene for moyamoya disease and its possible role in vascular development","volume":"6","author":"Liu","year":"2011","unstructured":"Liu WMorito DTakashima SIdentification of RNF213 as a susceptibility gene for moyamoya disease and its possible role in vascular development. PLoS One. 2011;6(7):e22542. doi:10.1371\/journal.pone.0022542"},{"issue":"6","key":"bib5","series-title":"No Shinkei Geka","first-page":"533","article-title":"Ruptured aneurysm of an aplastic or twig-like middle cerebral artery with Ring Finger Protein 213 mutation: a case report","volume":"48","author":"Fukuyama","year":"2020","unstructured":"Fukuyama RYamamura KMurata HMiyatake SMatsumoto NAbe H.Ruptured aneurysm of an aplastic or twig-like middle cerebral artery with Ring Finger Protein 213 mutation: a case report. Article in Japanese. No Shinkei Geka. 2020;48(6):533-540. doi:10.11477\/mf.1436204224"},{"issue":"6","key":"bib6","series-title":"Interv Neuroradiol","first-page":"634","article-title":"Aplastic or twig-like middle cerebral artery and cardiogenic cerebral embolism mimicking moyamoya disease with RNF213 polymorphism: a case report","volume":"28","author":"Inoue","year":"2022","unstructured":"Inoue HOomura MNishikawa YMase MMatsukawa N.Aplastic or twig-like middle cerebral artery and cardiogenic cerebral embolism mimicking moyamoya disease with RNF213 polymorphism: a case report. Interv Neuroradiol. 2022;28(6):634-638. doi:10.1177\/15910199211062016"},{"issue":"3","key":"bib7","series-title":"J Stroke Cerebrovasc Dis","first-page":"107582","article-title":"Non-aneurysmal subarachnoid hemorrhage in aplastic or twig-like middle cerebral artery: a case report and literature review","volume":"33","author":"Kawakami","year":"2024","unstructured":"Kawakami MMurai SKusaka NNon-aneurysmal subarachnoid hemorrhage in aplastic or twig-like middle cerebral artery: a case report and literature review. J Stroke Cerebrovasc Dis. 2024;33(3):107582. doi:10.1016\/j.jstrokecerebrovasdis.2024.107582"},{"key":"bib8","series-title":"Clin Imaging","first-page":"31","article-title":"Twig-like middle cerebral arteries: clinical and radiological findings","volume":"73","author":"Onoue","year":"2021","unstructured":"Onoue KNguyen TNMian ADasenbrock HBedi HAbdalkader M.Twig-like middle cerebral arteries: clinical and radiological findings. Clin Imaging. 2021;73:31-37. doi:10.1016\/j.clinimag.2020.11.049"},{"issue":"4","key":"bib9","series-title":"Interv Neuroradiol","first-page":"584","article-title":"Twig-like middle cerebral artery: embryological persistence or secondary consequences?","volume":"27","author":"Ota","year":"2021","unstructured":"Ota TKomiyama M.Twig-like middle cerebral artery: embryological persistence or secondary consequences? Interv Neuroradiol. 2021;27(4):584-587. doi:10.1177\/15910199211024077"},{"issue":"6","key":"bib10","series-title":"Neurol Med Chir (Tokyo)","first-page":"261","article-title":"Anomalies of the middle cerebral artery","volume":"57","author":"Uchiyama","year":"2017","unstructured":"Uchiyama N.Anomalies of the middle cerebral artery. Neurol Med Chir (Tokyo). 2017;57(6):261-266. doi:10.2176\/nmc.ra.2017-0043"},{"issue":"4","key":"bib11","series-title":"AJNR Am J Neuroradiol","first-page":"585","article-title":"Angiographic and embryologic considerations in five cases of middle cerebral artery fenestration","volume":"23","author":"Gailloud","year":"2002","unstructured":"Gailloud PAlbayram SFasel JHBeauchamp NJMurphy KJ.Angiographic and embryologic considerations in five cases of middle cerebral artery fenestration. AJNR Am J Neuroradiol. 2002;23(4):585-587."},{"issue":"6","key":"bib12","series-title":"Neurosurgery","first-page":"1472","article-title":"Clinical and radiological features of patients with aplastic or twiglike middle cerebral arteries","volume":"70","author":"Seo","year":"2012","unstructured":"Seo BSLee YSLee HGLee JHRyu KYKang DG.Clinical and radiological features of patients with aplastic or twiglike middle cerebral arteries. Neurosurgery. 2012;70(6):1472-1480. doi:10.1227\/NEU.0b013e318246a510"},{"key":"bib13","series-title":"Surg Neurol Int","first-page":"192","article-title":"De novo formation of twig-like middle cerebral artery: an illustrative case","volume":"14","author":"Kajita","year":"2023","unstructured":"Kajita MYanaka KHanai SDe novo formation of twig-like middle cerebral artery: an illustrative case. Surg Neurol Int. 2023;14:192. doi:10.25259\/SNI_274_2023"},{"issue":"1","key":"bib14","series-title":"Neurosurg Rev","first-page":"82","article-title":"Adult unilateral moyamoya disease with familial occurrence in two definite cases: a case report and review of the literature","volume":"29","author":"Kusaka","year":"2006","unstructured":"Kusaka NTamiya TAdachi YAdult unilateral moyamoya disease with familial occurrence in two definite cases: a case report and review of the literature. Neurosurg Rev. 2006;29(1):82-87. doi:10.1007\/s10143-005-0406-5"},{"issue":"7","key":"bib15","series-title":"Neurol Med Chir (Tokyo)","first-page":"307","article-title":"Diagnostic criteria for moyamoya disease\u20142021 revised version","volume":"62","author":"Kuroda","year":"2022","unstructured":"Kuroda SFujimura MTakahashi JDiagnostic criteria for moyamoya disease\u20142021 revised version. Neurol Med Chir (Tokyo). 2022;62(7):307-312. doi:10.2176\/jns-nmc.2022-0072"},{"issue":"1","key":"bib16","series-title":"J Hum Genet","first-page":"34","article-title":"A genome-wide association study identifies RNF213 as the first moyamoya disease gene","volume":"56","author":"Kamada","year":"2011","unstructured":"Kamada FAoki YNarisawa AA genome-wide association study identifies RNF213 as the first moyamoya disease gene. J Hum Genet. 2011;56(1):34-40. doi:10.1038\/jhg.2010.132"},{"issue":"1","key":"bib17","series-title":"Neurol India","first-page":"35","article-title":"RNF213 polymorphism and moyamoya disease: a systematic review and meta-analysis","volume":"61","author":"Ma","year":"2013","unstructured":"Ma JLiu YMa LHuang SLi HYou C.RNF213 polymorphism and moyamoya disease: a systematic review and meta-analysis. Neurol India. 2013;61(1):35-39. doi:10.4103\/0028-3886.107927"},{"issue":"4","key":"bib18","series-title":"Life (Basel)","first-page":"555","article-title":"RNF213-associated vascular disease: a concept unifying various vasculopathies","volume":"12","author":"Hiraide","year":"2022","unstructured":"Hiraide TSuzuki HMomoi MRNF213-associated vascular disease: a concept unifying various vasculopathies. 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