• World Neurosurg · Apr 2023

    Clinical and genetic characteristics of patients with moyamoya disease who experienced both ischemic and hemorrhagic events.

    • Yudai Hirano, Satoru Miyawaki, Hideaki Imai, Hiroki Hongo, Yu Teranishi, Daiichiro Ishigami, Yu Sakai, Daisuke Shimada, Motoyuki Umekawa, Masafumi Segawa, Satoshi Koizumi, Hideaki Ono, Hirofumi Nakatomi, and Nobuhito Saito.
    • Department of Neurosurgery, Faculty of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan.
    • World Neurosurg. 2023 Apr 1; 172: e438e446e438-e446.

    ObjectiveCerebrovascular events in moyamoya disease are mainly classified into ischemic or hemorrhagic onset. It is rare for one patient to develop both ischemia and hemorrhage in moyamoya disease; detailed clinical course and genetic characteristics of such patients have not been elucidated. We aimed to clarify the clinical features of patients with both ischemic and hemorrhagic cerebrovascular events.MethodsWe analyzed the background factors, radiological features, and genotype of ring finger protein 213 c.14429 G > A (p.Arg4810Lys) of patients with moyamoya disease who visited our hospital between 1996 and 2020, and experienced both ischemic and hemorrhagic cerebrovascular events. Additionally, we analyzed factors that caused subsequent hemorrhage in adult-onset ischemic moyamoya disease.ResultsOf 262 patients, 12 presented with both ischemia and hemorrhage, of which, 4 exhibited pediatric onset and 8 had adult onset. In pediatric-onset subjects, ischemia was the initial event in all cases. Hemorrhagic events occurred at a median of 24.7 years postoperatively in patients who had undergone bypass surgery. In adult-onset subjects, ischemia preceded hemorrhage in 7 patients. In males, the interval to subsequent hemorrhage was significantly shorter for adult-onset ischemic moyamoya disease, and the hazard ratio for hemorrhagic events was 5.45. The ring finger protein 213 p.Arg4810Lys heterozygous variant was present in 9 patients.ConclusionsA majority of patients with moyamoya disease with both ischemia and hemorrhage experience an ischemic event first. Patients who developed ischemia in childhood may develop subsequent hemorrhage in approximately 20-25 years after bypass surgery. Male sex is a risk factor for a subsequent hemorrhagic event in adult-onset ischemic moyamoya disease.Copyright © 2023 Elsevier Inc. All rights reserved.

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