Anders Lewén

Johan Wikström

Markus Fahlström

Per Enblad

Teodor Svedung Wettervik

Frontiers in Neurology

Svedung-Wettervik, Teodor

Fahlström, Markus

Wikström, Johan

Enblad, Per

Lewén, Anders

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Editorial: Moyamoya disease - natural history and therapeutic challenges

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TYPE EditorialPUBLISHED 05 September 2023DOI 10.3389/fneur.2023.1270197OPEN ACCESSEDITED AND REVIEWED BYYasushi Takagi,Tokushima University, Japan*CORRESPONDENCETeodor Svedung Wettervikteodor.svedung-wettervik@neuro.uu.seRECEIVED 31 July 2023ACCEPTED 24 August 2023PUBLISHED 05 September 2023CITATIONSvedung Wettervik T, Fahlström M, Wikström J,Enblad P and Lewén A (2023) Editorial:Moyamoya disease – natural history andtherapeutic challenges.Front. Neurol. 14:1270197.doi: 10.3389/fneur.2023.1270197COPYRIGHT© 2023 Svedung Wettervik, Fahlström,Wikström, Enblad and Lewén. This is anopen-access article distributed under the termsof the Creative Commons Attribution License(CC BY). The use, distribution or reproductionin other forums is permitted, provided theoriginal author(s) and the copyright owner(s)are credited and that the original publication inthis journal is cited, in accordance withaccepted academic practice. No use,distribution or reproduction is permitted whichdoes not comply with these terms.Editorial: Moyamoya disease –natural history and therapeuticchallengesTeodor Svedung Wettervik1*, Markus Fahlström2,Johan Wikström2, Per Enblad1 and Anders Lewén11Department of Medical Sciences, Section of Neurosurgery, Uppsala University, Uppsala, Sweden,2Department of Surgical Sciences, Section of Neuroradiology, Uppsala University, Uppsala, SwedenKEYWORDSmoyamoya disease, cerebral blood flow, revascularization, imaging, strokeEditorial on the Research TopicMoyamoya disease – natural history and therapeutic challengesMoyamoya disease (MMD) is characterized by gradual stenosis of the terminal internalcarotid artery with the development of fragile collateral vessels that partially restore thedistal blood flow. These patients are at high risk of developing brain ischemia because ofstenosis and intracranial hemorrhage (ICH) due to rupture of the fragile vessels (1). SinceMMD is a rare disorder (2), the level of evidence regarding epidemiology, natural history,pathophysiology, imaging, and treatments is limited (1). In this Research Topic; “Moyamoyadisease – natural history and therapeutic challenges”, we have received several contributionsthat have brought new insights into many different clinical domains of MMD.The natural history ofMMD remains elusive, but is important tomake a prognosis and todetermine the optimal timing for interventions. In this Research Topic, Cao et al. conducteda systematic review and meta-analysis on the predictors of MMD disease progression. Theauthors found younger age, family history, and contralateral vessel abnormalities to berisk factors. It was also interesting to read the study by Liu E. et al., who investigated therole of systemic inflammatory biomarkers, particularly certain blood cell ratios, in MMD.The authors found that patients with MMD exhibited pathological elevations in thesebiomarkers, but also that the level of elevation differed depending on the disease stage. Thus,biomarkers may play a role in improving the prognostication of MMD.Furthermore, radiological imaging plays a major part in the diagnosis and subsequentfollow-up of MMD. Positron emission tomography-computed tomography (PET-CT), oftenin conjunction with acetazolamide injection, has been the traditional method to studycerebral hemodynamics (3, 4), but other methods exist, such as arterial spin labelingmagnetic resonance imaging (ASL-MRI) (5). In a study by our group, we investigated thepotential role of evaluating cerebral hemodynamics with ASL-MRI with an acetazolamidetest and dynamic susceptibility contrast MR perfusion (Svedung Wettervik et al.). Wefound several strengths with the latter method, as it is radiation-free and provided uniqueinformation on capillary blood flow, which appeared pathological when the cerebrovascularreserve was exhausted. In another study (Fahlström et al.), we investigated the role ofthe ASL-MRI-derived coefficient of variation (CoVcbf), skewness, and kurtosis, whichhave previously been associated with cerebral hemodynamics in other conditions (6).However, in the current MMD study (Fahlström et al.), these variables did not carry anyhemodynamically relevant information.Frontiers inNeurology 01 frontiersin.orgSvedung Wettervik et al. 10.3389/fneur.2023.1270197Surgical treatment is warranted in MMD patients withexhausted cerebrovascular reserve (4). In a study by Chenet al., combined surgery with direct (superior temporal artery[STA] by-pass) and indirect (encephalo-dura-myosynangiosus)revascularization was often successful. The authors also foundpostoperative ultrasound examinations of the STA useful as anindicator of the success of indirect revascularization and inducedneoangiogenesis. Furthermore, patients with MMD occasionallydevelop ICHs, which may in some cases be caused by aruptured MMD-associated aneurysm. These aneurysms are oftenchallenging to treat due to their small size and deep location. Inthis Research Topic, Zhou et al. present a series of 11 patientswith rupturedMMD aneurysms that required endovascular parent-artery occlusion by means of coiling or Onyx. In their study,such treatment usually obliterated the aneurysm, and severeneurological complications were infrequent, suggesting that thisapproach may be an acceptable rescue therapy when isolatedaneurysm occlusion is not feasible. In addition to surgicaltreatments, certain medications may be beneficial in MMD,e.g., antiplatelets to counteract the formation of intraluminalthrombosis in the pathological MMD vessels. In the systematicreview and meta-analysis on antiplatelets in MMD by Liu T.et al., the authors found some support for such agents todecrease mortality and hemorrhagic (but not ischemic) stroke,while it also appeared to increase the by-pass patency followingdirect revascularization.Altogether, we have received many important contributionsin several different clinical MMD areas. While these bring usfurther insights into the natural history, adjunct diagnostic/follow-up modalities, and therapeutical options, the rarity of this diseasecontributes to great challenges in providing reliable answers toclinically important research questions. In future efforts, we needto collaborate to gather “big data” and proceed with clinical trials tofind optimal pharmacological agents and to refine the timing andchoice of surgical treatment.Author contributionsTS: Writing—original draft. MF: Writing—reviewand editing. JW: Writing—review and editing. PE:Writing—review and editing. AL: Writing—reviewand editing.Conflict of interestThe authors declare that the research was conducted in theabsence of any commercial or financial relationships that could beconstrued as a potential conflict of interest.Publisher’s noteAll claims expressed in this article are solely those of theauthors and do not necessarily represent those of their affiliatedorganizations, or those of the publisher, the editors and thereviewers. Any product that may be evaluated in this article, orclaim that may be made by its manufacturer, is not guaranteed orendorsed by the publisher.References1. Scott RM, Smith ER. Moyamoya disease and moyamoya syndrome. N Engl J Med.(2009) 360:1226–37. doi: 10.1056/NEJMra08046222. Baba T, Houkin K, Kuroda S. Novel epidemiological features of moyamoyadisease. J Neurol Neurosurg Psychiatry. (2008) 79:900–4. doi: 10.1136/jnnp.2007.1306663. Roder C, Bürkle E, Ebner FH, Tatagiba M, Ernemann U, Buck A, et al. Estimationof severity of moyamoya disease with [(15)O]water-positron emission tomographycompared with magnetic resonance imaging and angiography. World Neurosurg.(2018) 117:e75–81. doi: 10.1016/j.wneu.2018.05.1634. Bersano A, Khan N, Fuentes B, Acerbi F, Canavero I, Tournier-Lasserve E, et al.European stroke organisation (ESO) guidelines on moyamoya angiopathy endorsedby vascular european reference network (VASCERN). Eur Stroke J. (2023) 8:55–84. doi: 10.1177/239698732211440895. Noguchi T, Kawashima M, Irie H, Ootsuka T, Nishihara M, MatsushimaT, et al. Arterial spin-labeling MR imaging in moyamoya disease comparedwith SPECT imaging. Eur J Radiol. (2011) 80:e557–62. doi: 10.1016/j.ejrad.2011.01.0166. Mutsaerts HJ, Petr J, Václavu L, van Dalen JW, Robertson AD, Caan MW,et al. The spatial coefficient of variation in arterial spin labeling cerebral bloodflow images. J Cereb Blood Flow Metab. (2017) 37:3184–92. doi: 10.1177/0271678X16683690Frontiers inNeurology 02 frontiersin.org

Editorial: Moyamoya disease – natural history and therapeutic challenges

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