{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,16]],"date-time":"2026-03-16T20:38:56Z","timestamp":1773693536848,"version":"3.50.1"},"reference-count":38,"publisher":"IMR Press","issue":"9","license":[{"start":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T00:00:00Z","timestamp":1761782400000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Rev. Neurol."],"abstract":"<jats:p>Introduction: Moyamoya angiopathy is a cerebrovascular disease characterized by progressive stenosis of the intracranial internal carotid arteries. There is limited literature addressing epilepsy in this condition, especially in Western countries. Methodology: This was a retrospective study conducted in the public hospitals of Arag\u00f3n, analyzing data from all patients diagnosed with moyamoya angiopathy between 1981 and 2024. Epidemiological aspects of the disease were studied, as well as the presence of epilepsy and its management in this group of patients. Results: A total of 26 patients were included, with an estimated prevalence in Arag\u00f3n of 1.71 cases per 100,000 inhabitants. The mean age at diagnosis was 36.64 years, with an equal sex distribution. Half of the patients presented with moyamoya syndrome. Fifty percent of the patients experienced a seizure and 42.31% of the total met diagnostic criteria for epilepsy. Most seizures were focal (81.8%), with a predominance of frontal lobe semiology. Levetiracetam was the most commonly used treatment. Up to four of the twelve patients with epilepsy met diagnostic criteria for drug-resistant epilepsy. Conclusions: Although the prevalence of moyamoya in our series was lower than in Asian populations, the prevalence of epilepsy was significantly higher (50% of patients with seizures vs 0.9\u201318.9% in Asian series). In our cohort, epilepsy in moyamoya angiopathy was associated with the occurrence of syncope, cognitive impairment, affective disorders, and an earlier age at diagnosis. There are no other studies available addressing the percentage of drug-resistant epilepsy in these patients.<\/jats:p>","DOI":"10.31083\/rn37504","type":"journal-article","created":{"date-parts":[[2025,11,7]],"date-time":"2025-11-07T12:48:06Z","timestamp":1762519686000},"source":"Crossref","is-referenced-by-count":1,"title":["Epilepsy in Patients With Moyamoya Angiopathy"],"prefix":"10.31083","volume":"80","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6734-943X","authenticated-orcid":true,"given":"Mario","family":"Bautista-Lacambra","sequence":"first","affiliation":[{"name":"Servicio de Neurolog\u00eda, Hospital Universitario Miguel Servet, 50009 Zaragoza, Espa\u00f1a"},{"name":"Grupo de Investigaci\u00f3n en Neurociencias, Instituto de Investigaci\u00f3n Sanitaria (IIS) Arag\u00f3n, 50009 Zaragoza, Espa\u00f1a"},{"name":"Departamento de Medicina, Dermatolog\u00eda y Psiquiatr\u00eda de la Universidad de Zaragoza, 50009 Zaragoza, Espa\u00f1a"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vanesa","family":"Garayoa-Irigoyen","sequence":"first","affiliation":[{"name":"Servicio de Neurolog\u00eda, Hospital Universitario Miguel Servet, 50009 Zaragoza, Espa\u00f1a"},{"name":"Grupo de Investigaci\u00f3n en Neurociencias, Instituto de Investigaci\u00f3n Sanitaria (IIS) Arag\u00f3n, 50009 Zaragoza, Espa\u00f1a"},{"name":"Unidad de Epilepsia F\u00e1rmaco-Resistente, Hospital Universitario Miguel Servet, 50009 Zaragoza, Espa\u00f1a"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luisa-Fernanda","family":"Tique-Rojas","sequence":"first","affiliation":[{"name":"Servicio de Neurolog\u00eda, Hospital Universitario Miguel Servet, 50009 Zaragoza, Espa\u00f1a"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar\u00eda","family":"Seral-Moral","sequence":"first","affiliation":[{"name":"Servicio de Neurolog\u00eda, Hospital Universitario San Jorge, 22004 Huesca, Espa\u00f1a"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jes\u00fas","family":"Moles-Herbera","sequence":"first","affiliation":[{"name":"Servicio de Neurocirug\u00eda, Hospital Universitario Miguel Servet, 50009 Zaragoza, Espa\u00f1a"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amparo","family":"L\u00f3pez-Lafuente","sequence":"first","affiliation":[{"name":"Secci\u00f3n de Neuropediatr\u00eda y Neurometabolismo, Servicio de Pediatr\u00eda del Hospital Universitario Miguel Servet, 50009 Zaragoza, Espa\u00f1a"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rosario","family":"Barrena-Caballo","sequence":"first","affiliation":[{"name":"Unidad de Neurorradiolog\u00eda Intervencionista del Servicio de Radiolog\u00eda del Hospital Universitario Miguel Servet, 50009 Zaragoza, Espa\u00f1a"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1653-7112","authenticated-orcid":true,"given":"Carlos","family":"Tejero-Juste","sequence":"first","affiliation":[{"name":"Servicio De Neurolog\u00eda, Hospital Cl\u00ednico Universitario Lozano Blesa, 50009 Zaragoza, Espa\u00f1a"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marta","family":"Palac\u00edn-Larroy","sequence":"first","affiliation":[{"name":"Servicio de Neurolog\u00eda, Hospital Universitario Miguel Servet, 50009 Zaragoza, Espa\u00f1a"},{"name":"Grupo de Investigaci\u00f3n en Neurociencias, Instituto de Investigaci\u00f3n Sanitaria (IIS) Arag\u00f3n, 50009 Zaragoza, Espa\u00f1a"},{"name":"Departamento de Medicina, Dermatolog\u00eda y Psiquiatr\u00eda de la Universidad de Zaragoza, 50009 Zaragoza, Espa\u00f1a"},{"name":"Secci\u00f3n de Neurovascular, Servicio de Neurolog\u00eda, Hospital Universitario Miguel Servet, 50009 Zaragoza, Espa\u00f1a"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6506-1037","authenticated-orcid":true,"given":"Herbert","family":"Tejada-Meza","sequence":"first","affiliation":[{"name":"Servicio de Neurolog\u00eda, Hospital Universitario Miguel Servet, 50009 Zaragoza, Espa\u00f1a"},{"name":"Grupo de Investigaci\u00f3n en Neurociencias, Instituto de Investigaci\u00f3n Sanitaria (IIS) Arag\u00f3n, 50009 Zaragoza, Espa\u00f1a"},{"name":"Departamento de Medicina, Dermatolog\u00eda y Psiquiatr\u00eda de la Universidad de Zaragoza, 50009 Zaragoza, Espa\u00f1a"},{"name":"Unidad de Neurorradiolog\u00eda Intervencionista del Servicio de Radiolog\u00eda del Hospital Universitario Miguel Servet, 50009 Zaragoza, Espa\u00f1a"},{"name":"Secci\u00f3n de Neurovascular, Servicio de Neurolog\u00eda, Hospital Universitario Miguel Servet, 50009 Zaragoza, 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(Accedido: 12 Enero 2025)."},{"key":"ref12","doi-asserted-by":"publisher","unstructured":"Scheffer IE, Berkovic S, Capovilla G, Connolly MB, French J, Guilhoto L, et al. ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017; 58: 512\u2013521.","DOI":"10.1111\/epi.13709"},{"key":"ref13","doi-asserted-by":"publisher","unstructured":"Niu H, Tan C, Jin K, Duan R, Shi G, Wang R. Risk factors for early seizure after revascularization in patients with moyamoya disease. Chinese Neurosurgical Journal. 2022; 8: 44.","DOI":"10.1186\/s41016-022-00305-0"},{"key":"ref14","doi-asserted-by":"publisher","unstructured":"Mikami T, Ochi S, Houkin K, Akiyama Y, Wanibuchi M, Mikuni N. Predictive factors for epilepsy in moyamoya disease. Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association. 2015; 24: 17\u201323.","DOI":"10.1016\/j.jstrokecerebrovasdis.2014.07.050"},{"key":"ref15","doi-asserted-by":"publisher","unstructured":"Zhao M, Lin Z, Deng X, Zhang Q, Zhang D, Zhang Y, et al. Clinical Characteristics and Natural History of Quasi-Moyamoya Disease. Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association. 2017; 26: 1088\u20131097.","DOI":"10.1016\/j.jstrokecerebrovasdis.2016.12.025"},{"key":"ref16","doi-asserted-by":"publisher","unstructured":"Fujimura M, Tominaga T. Diagnosis of moyamoya disease: international standard and regional differences. Neurologia Medico-chirurgica. 2015; 55: 189\u2013193.","DOI":"10.2176\/nmc.ra.2014-0307"},{"key":"ref17","doi-asserted-by":"publisher","unstructured":"Bao XY, Duan L, Li DS, Yang WZ, Sun WJ, Zhang ZS, et al. Clinical features, surgical treatment and long-term outcome in adult patients with Moyamoya disease in China. Cerebrovascular Diseases (Basel, Switzerland). 2012; 34: 305\u2013313.","DOI":"10.1159\/000343225"},{"key":"ref18","doi-asserted-by":"publisher","unstructured":"Starke RM, Komotar RJ, Hickman ZL, Paz YE, Pugliese AG, Otten ML, et al. Clinical features, surgical treatment, and long-term outcome in adult patients with moyamoya disease. Clinical article. Journal of Neurosurgery. 2009; 111: 936\u2013942.","DOI":"10.3171\/2009.3.JNS08837"},{"key":"ref19","doi-asserted-by":"publisher","unstructured":"Ma Y, Zhao M, Zhang Q, Liu X, Zhang D, Wang S, et al. Risk Factors for Epilepsy Recurrence after Revascularization in Pediatric Patients with Moyamoya Disease. Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association. 2018; 27: 740\u2013746.","DOI":"10.1016\/j.jstrokecerebrovasdis.2017.10.012"},{"key":"ref20","doi-asserted-by":"publisher","unstructured":"Jin SC, Oh CW, Kwon OK, Hwang G, Bang JS, Kang HS, et al. Epilepsy after bypass surgery in adult moyamoya disease. Neurosurgery. 2011; 68: 1227\u201332; discussion 1232.","DOI":"10.1227\/NEU.0b013e31820c045a"},{"key":"ref21","doi-asserted-by":"publisher","unstructured":"Lagger I, Garino E, Martinez O, Knorre E, Ernst G, Burgue\u00f1o AL. Risk factors for drug-resistant epilepsy in adult patients. Medicina Clinica. 2023; 160: 547\u2013550.","DOI":"10.1016\/j.medcli.2023.03.006"},{"key":"ref22","doi-asserted-by":"publisher","unstructured":"Lattanzi S, Rinaldi C, Cagnetti C, Foschi N, Norata D, Broggi S, et al. Predictors of Pharmaco-Resistance in Patients with Post-Stroke Epilepsy. 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Neurology International. 2024; 16: 869\u2013879.","DOI":"10.3390\/neurolint16040065"},{"key":"ref26","doi-asserted-by":"publisher","unstructured":"Alotaibi AB, Alrashedi HB, Elsafi TS. Moyamoya Disease in a Patient With Sickle Cell Disease: A Case Report and Review of the Literature. Cureus. 2024; 16: e55592.","DOI":"10.7759\/cureus.55592"},{"key":"ref27","doi-asserted-by":"publisher","unstructured":"Liu E, Jin L, Tao W, Tan X, Liu C, Zhou S, et al. Analysis of Risk Factors for Epilepsy after Combined Re-Vascularization in Adult Patients with Moyamoya Disease. Neurology India. 2023; 71: 471\u2013475.","DOI":"10.4103\/0028-3886.378661"},{"key":"ref28","doi-asserted-by":"publisher","unstructured":"Abdul Rab S, Arabi TZ, Raheel HM, Sabbah BN, Zain AlAbidien NH, Alsemari A. Moyamoya syndrome secondary to mitochondrial disease in a patient with partial trisomy 13q14 and 13q31: A novel case report and literature review. Heliyon. 2023; 9: e13466.","DOI":"10.1016\/j.heliyon.2023.e13466"},{"key":"ref29","doi-asserted-by":"publisher","unstructured":"Gatti JR, Penn R, Ahmad SA, Sun LR. Seizures in Pediatric Moyamoya: Risk Factors and Functional Outcomes. Pediatric Neurology. 2023; 145: 36\u201340.","DOI":"10.1016\/j.pediatrneurol.2023.04.025"},{"key":"ref30","doi-asserted-by":"publisher","unstructured":"Das S, Ray BK, Dubey S. Temporal lobe epilepsy with nocturnal wandering leading to discovery of Moyamoya Angiopathy. Acta Neurologica Belgica. 2023; 123: 279\u2013281.","DOI":"10.1007\/s13760-021-01830-y"},{"key":"ref31","doi-asserted-by":"publisher","unstructured":"Alramadan A, Ul Haq A, Basindwah S, Alshail E. Seizure outcome in moyamoya after indirect revascularization in pediatric patients: Retrospective study and literature review. Surgical Neurology International. 2021; 12: 73.","DOI":"10.25259\/SNI_633_2020"},{"key":"ref32","doi-asserted-by":"publisher","unstructured":"Talbot J, Singh P, Puvirajasinghe C, Genomics England Research Consortiumgecip-help@genomicsengland.co.uk, Sisodiya SM, Rugg-Gunn F. Moyamoya and progressive myoclonic epilepsy secondary to CLN6 bi-allelic mutations - A previously unreported association. Epilepsy & Behavior Reports. 2020; 14: 100389.","DOI":"10.1016\/j.ebr.2020.100389"},{"key":"ref33","doi-asserted-by":"publisher","unstructured":"Lu J, Xia Q, Yang T, Qiang J, Liu X, Ye X, et al. Electroencephalographic features in pediatric patients with moyamoya disease in China. Chinese Neurosurgical Journal. 2020; 6: 3.","DOI":"10.1186\/s41016-019-0179-2"},{"key":"ref34","doi-asserted-by":"publisher","unstructured":"Nakayama C, Mikami T, Ukai R, Chiba R, Enatsu R, Suzuki H, et al. Moyamoya disease with epileptic nystagmus: A case report. Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia. 2019; 70: 251\u2013254.","DOI":"10.1016\/j.jocn.2019.08.069"},{"key":"ref35","doi-asserted-by":"publisher","unstructured":"Kuroda N, Fujimoto A, Okanishi T, Sato K, Nishimura M, Enoki H. Epilepsy surgery for a patient with neurofibromatosis type 1 concomitant with moyamoya syndrome. Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia. 2019; 61: 307\u2013310.","DOI":"10.1016\/j.jocn.2018.10.050"},{"key":"ref36","doi-asserted-by":"publisher","unstructured":"Garson SR, Monteith SJ, Smith SD, Keogh BP, Gwinn RP, Doherty MJ. Down syndrome associated moyamoya may worsen epilepsy control and can benefit from surgical revascularization. Epilepsy & Behavior Case Reports. 2018; 11: 14\u201317.","DOI":"10.1016\/j.ebcr.2018.09.008"},{"key":"ref37","doi-asserted-by":"publisher","unstructured":"Kodama N, Aoki Y, Hiraga H, Wada T, Suzuki J. Electroencephalographic findings in children with moyamoya disease. Archives of Neurology. 1979; 36: 16\u201319.","DOI":"10.1001\/archneur.1979.00500370046008"},{"key":"ref38","doi-asserted-by":"publisher","unstructured":"Frechette ES, Bell-Stephens TE, Steinberg GK, Fisher RS. Electroencephalographic features of moyamoya in adults. 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