{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,9]],"date-time":"2025-12-09T08:29:40Z","timestamp":1765268980144,"version":"build-2065373602"},"reference-count":33,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2024,8,20]],"date-time":"2024-08-20T00:00:00Z","timestamp":1724112000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neurology International"],"abstract":"<jats:p>Moyamoya disease is a cerebrovascular pathology characterized by progressive stenosis of the internal carotid arteries and their branches, leading to ischemic and\/or hemorrhagic disorders of the cerebral circulation, primarily affecting children and young adults. We present a case of a 23-year-old woman with a history of recurrent cerebrovascular accidents since childhood. Despite experiencing focal motor seizures and transient ischemic attacks, her condition remained undiagnosed until 2006, when, at the age of 7, a digital subtraction angiography revealed characteristic bilateral internal carotid artery occlusions. Subsequent diagnostic challenges and treatments preceded a worsening of symptoms in adulthood, including generalized tonic\u2013clonic seizures. Upon presentation to our clinic, the patient exhibited upper motor neuron syndrome and occipital lobe syndrome, consistent with the disease\u2019s pathophysiology, neuroimaging, and clinical manifestations. Imaging studies confirmed multiple ischemic lesions throughout the cerebral vasculature. Treatment adjustments were made due to the increased incidence of seizures, and the dose of her anti-seizure medication\u2014divalproex sodium\u2014was increased. This case underscores the diagnostic complexities and challenges in managing moyamoya disease, emphasizing the importance of early recognition and prompt intervention.<\/jats:p>","DOI":"10.3390\/neurolint16040065","type":"journal-article","created":{"date-parts":[[2024,8,20]],"date-time":"2024-08-20T01:38:45Z","timestamp":1724117925000},"page":"869-879","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Clinical Case of a 23-Year-Old Patient with Moyamoya Disease and Epilepsy in Bulgaria"],"prefix":"10.3390","volume":"16","author":[{"given":"Ekaterina","family":"Viteva","sequence":"first","affiliation":[{"name":"Department of Neurology, Faculty of Medicine, Medical University of Plovdiv, 4000 Plovdiv, Bulgaria"},{"name":"Clinic of Neurology, UMHAT \u201cSv. Georgi\u201d, 4000 Plovdiv, Bulgaria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Petar","family":"Vasilev","sequence":"additional","affiliation":[{"name":"Department of Neurology, Faculty of Medicine, Medical University of Plovdiv, 4000 Plovdiv, Bulgaria"},{"name":"Clinic of Neurology, UMHAT \u201cSv. Georgi\u201d, 4000 Plovdiv, Bulgaria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3280-5060","authenticated-orcid":false,"given":"Georgi","family":"Vasilev","sequence":"additional","affiliation":[{"name":"Clinic of Neurology, UMHAT \u201cSv. Georgi\u201d, 4000 Plovdiv, Bulgaria"},{"name":"Medical Faculty, Sofia University St. Kliment Ohridski, 1 Kozyak Str., 1407 Sofia, Bulgaria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4636-1384","authenticated-orcid":false,"given":"Kostadin","family":"Chompalov","sequence":"additional","affiliation":[{"name":"Department of Neurology, Faculty of Medicine, Medical University of Plovdiv, 4000 Plovdiv, Bulgaria"},{"name":"Clinic of Neurology, UMHAT \u201cSv. Georgi\u201d, 4000 Plovdiv, Bulgaria"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,8,20]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1226","DOI":"10.1056\/NEJMra0804622","article-title":"Moyamoya Disease and Moyamoya Syndrome","volume":"360","author":"Scott","year":"2009","journal-title":"N. Engl. J. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1001\/archneur.1969.00480090076012","article-title":"Cerebrovascular \u201cmoyamoya\u201d disease: Disease showing abnormal net-like vessels in base of brain","volume":"20","author":"Suzuki","year":"1969","journal-title":"Arch. Neurol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"S238","DOI":"10.1016\/S0303-8467(97)00082-6","article-title":"Guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis (\u2018moyamoya\u2019 disease)","volume":"99","author":"Fukui","year":"1997","journal-title":"Clin. Neurol. Neurosurg."},{"key":"ref_4","first-page":"e141","article-title":"Stroke Neuroimage: Hyperventilation Arterial Spin Labeling in Moyamoya Disease","volume":"53","author":"Parlak","year":"2022","journal-title":"Stroke"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/S0010-9452(88)80014-5","article-title":"Visual apperceptive agnosia: A clinico-anatomical study of three cases","volume":"24","author":"Warrington","year":"1988","journal-title":"Cortex"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1016\/S0010-9452(13)80125-6","article-title":"Apperceptive and associative forms of prosopagnosia","volume":"27","author":"Faglioni","year":"1991","journal-title":"Cortex"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"2197","DOI":"10.1093\/brain\/awr103","article-title":"Do children really recover better? Neurobehavioural plasticity after early brain insult","volume":"134","author":"Anderson","year":"2011","journal-title":"Brain"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1080\/09602010443000074","article-title":"Cognitive training and cognitive rehabilitation for people with early-stage Alzheimer\u2019s disease: A review","volume":"14","author":"Clare","year":"2004","journal-title":"Neuropsychol. Rehabil."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"722","DOI":"10.1080\/13607863.2012.667783","article-title":"Memory training interventions for older adults: A meta-analysis","volume":"16","author":"Gross","year":"2012","journal-title":"Aging Ment. Health"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"900","DOI":"10.1136\/jnnp.2007.130666","article-title":"Novel epidemiological features of moyamoya disease","volume":"79","author":"Baba","year":"2008","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"S58","DOI":"10.1016\/S0303-8467(97)00042-5","article-title":"Moyamoya disease in Europe, past and present status","volume":"99","author":"Yonekawa","year":"1997","journal-title":"Clin. Neurol. Neurosurg."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"3193","DOI":"10.1161\/STROKEAHA.107.513408","article-title":"Moyamoya disease in Europeans","volume":"39","author":"Kraemer","year":"2008","journal-title":"Stroke"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"e364","DOI":"10.1161\/STR.0000000000000375","article-title":"2021 Guideline for the Prevention of Stroke in Patients with Stroke and Transient Ischemic Attack: A Guideline from the American Heart Association\/American Stroke Association","volume":"52","author":"Kleindorfer","year":"2021","journal-title":"Stroke"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1421","DOI":"10.1007\/s00415-019-09277-1","article-title":"Clinical presentation of Moyamoya angiopathy in Europeans: Experiences from Germany with 200 patients","volume":"266","author":"Kraemer","year":"2019","journal-title":"J. Neurol."},{"key":"ref_15","first-page":"e19812","article-title":"Western Moyamoya Phenotype: A Scoping Review","volume":"13","author":"Miller","year":"2021","journal-title":"Cureus"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"e694","DOI":"10.1542\/peds.2005-0568","article-title":"Moyamoya syndrome associated with Down syndrome: Outcome after surgical revascularization","volume":"116","author":"Jea","year":"2005","journal-title":"Pediatrics"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"211","DOI":"10.3171\/PED\/2008\/1\/3\/211","article-title":"Surgical treatment of moyamoya syndrome in patients with sickle cell anemia: Outcome following encephaloduroarteriosynangiosis","volume":"1","author":"Hankinson","year":"2008","journal-title":"J. Neurosurg. Pediatr."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"932","DOI":"10.1212\/01.wnl.0000257095.33125.48","article-title":"Moyamoya following cranial irradiation for primary brain tumors in children","volume":"68","author":"Ullrich","year":"2007","journal-title":"Neurology"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"237","DOI":"10.2174\/156720206778018730","article-title":"New vessel formation in the central nervous system during tumor growth, vascular malformations, and Moyamoya","volume":"3","author":"Lim","year":"2006","journal-title":"Curr. Neurovasc. Res."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1092","DOI":"10.3171\/jns.1983.59.6.1092","article-title":"Moyamoya syndrome in only one of two identical twins: Case report","volume":"59","author":"Tanghetti","year":"1983","journal-title":"J. Neurosurg."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1038\/jhg.2010.132","article-title":"A genome-wide association study identifies RNF213 as the first Moyamoya disease gene","volume":"56","author":"Kamada","year":"2011","journal-title":"J. Hum. Genet."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"12","DOI":"10.5853\/jos.2015.01760","article-title":"The Pathophysiology of Moyamoya Disease: An Update","volume":"18","author":"Bang","year":"2016","journal-title":"J. Stroke"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"2148","DOI":"10.1161\/01.STR.0000182256.32489.99","article-title":"Incidence and clinical features of disease progression in adult moyamoya disease","volume":"36","author":"Kuroda","year":"2005","journal-title":"Stroke"},{"key":"ref_24","first-page":"142","article-title":"Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis","volume":"100","author":"Scott","year":"2004","journal-title":"J. Neurosurg."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"936","DOI":"10.3171\/2009.3.JNS08837","article-title":"Clinical features, surgical treatment, and long-term outcome in adult patients with moyamoya disease. Clinical article","volume":"111","author":"Starke","year":"2009","journal-title":"J. Neurosurg."},{"key":"ref_26","first-page":"49","article-title":"Moyamoya disease and syndromes: From genetics to clinical management","volume":"8","author":"Guey","year":"2015","journal-title":"Appl. Clin. Genet."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1148\/radiology.184.3.1509066","article-title":"Moyamoya disease: Diagnosis with threedimensional time-of-flight MR angiography","volume":"184","author":"Yamada","year":"1992","journal-title":"Radiology"},{"key":"ref_28","doi-asserted-by":"crossref","unstructured":"Zhang, J., Wang, J., Geng, D., Li, Y., Song, D., and Gu, Y. (2013). Whole-brain CT perfusion and CT angiography assessment of Moyamoya disease before and after surgical revascularization: Preliminary study with 256-slice CT. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0057595"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1177\/23969873221144089","article-title":"European Stroke Organisation (ESO) Guidelines on Moyamoya angiopathy Endorsed by Vascular European Reference Network (VASCERN)","volume":"8","author":"Bersano","year":"2023","journal-title":"Eur. Stroke J."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"644","DOI":"10.1054\/jocn.2002.1103","article-title":"Effects of indirect bypass surgery for occlusive cerebrovascular diseases in adults","volume":"9","author":"Isono","year":"2002","journal-title":"J. Clin. Neurosci."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1055\/s-2005-868160","article-title":"Surgical management of moyamoya syndrome","volume":"15","author":"Smith","year":"2005","journal-title":"Skull Base"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1007\/s00381-004-1118-9","article-title":"Revascularisation surgery for paediatric moyamoya: A review of the literature","volume":"21","author":"Fung","year":"2005","journal-title":"Childs Nerv. Syst."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1007\/s003810000407","article-title":"Perioperative management protocols for children with moyamoya disease","volume":"17","author":"Nomura","year":"2001","journal-title":"Childs Nerv. 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