• Ahmad Sulaiman Alwahdy Neurology department of Fatmawati Central General Hospital
  • Fritz Sumantri Usman Neurology department of Fatmawati Central General Hospital



Angioplasty, moyamoya disease, neurocognitive, stroke


Moyamoya disease (MMD) is a rare idiopathic progressive vaso-occlusive disease causing multiple occlusion of cerebral vessels lead to ischemic stroke. Asian population is the most common race to be affected. We present a male patient 33-years old with suspected MMD with right hemiparesis and neurocognitive changes. On digital substraction angiography (DSA) there was appearance of ‘puff of smoke’ on his right hemisphere, stenosis middle cerebral arteries M1 bilaterally, stenosis of right opthalmic artery (OA), stenosis of left anterior cerebral artery (ACA) and aplasia of right ACA. Ballon angioplasty was performed on right OA that supply the contralateral symptomatic stenosis area (left A1) indirectly through anterior ethmoidal artery and anterior falcine artery (OA-ACA collateral). While no guidelines for the management of MMD, cerebral revascularization by using drug-eluting ballon (DEB) in right opthalmica artery is potentially effective treatment  that could allow the brain to have good blood supply (gives good collateral to both ACA), reduces burden of the fragile moya-moya vessels to be ruptured followed by  improvement of clinical results. Patient’s selection by understanding the stage, its progressivity and collateral formation are crucial before decision is made.


Kim J S. Moyamoya diseases: epidemiology, clinical features, and diagnosis. Journal of Stroke; 2016. 18(1); 2-11.DOI: 10.5853/jos.2015.01627.

Rodriguez G J, Kirmani J F, Ezzeddine M A, Qureshi A I. Primary percutaneous transluminal angioplasty for early moyamoya disease. J Neuroimaging; 2007. 17:48-53.DOI: 10.1111/j.1552-6569.2006.00075.x.

Suzuki J, Kodama N.Moyamoya disease:A review. Stroke; 1983. 14:104-109. DOI: 10.1161/01. str.14.1.104.

Gudepu R K, Qureshi M A, Qureshi I A, Rao L. Case report: A case report of moyamoya disease in a 36 year old African American women. F1000 Research; 2014. 3:297.DOI: 10.12688/f1000research.5859.1.

Leonard L L, Michael S, Sandra A, Cornelissen, Pervinder B, Anna S, et al. Angioplasty and stenting of adult onset moya-moya disease. J.inat; 2018.

DOI: 10.1016/j.inat.2018.10.013

Research Committee on the Pathology and Treatment of Spontaneous Occlusion of the Circle of Willis; Health Labour Sciences Research Grant for Research on Measures for Intrac¬table Diseases. Guidelines for diagnosis and treatment of moy¬amoya disease (spontaneous occlusion of the circle of Willis). Neurol Med Chir (Tokyo); 2012. 52:245-266.

DOI: 10.2176/nmc.52.245

Kang C G, Chun M H, Kang J A, Do K H, Choi S J. Neurocognitive dysfunction according to hypoperfusion territory in patients with moyamoya disease. Ann Rehabil Med; 2017. 41(1):1-8.

DOI: 10.5535/arm.2017.41.1.1

Penelope D Z, Peter K, Teresa E B, Gary K S, Leslie J D.Neurocognitive performance after cerebral revascularization in adult moyamoya disease. Stroke; 2017. 48:1514-1517. DOI: 10.1161/ STROKEAHA.116.016028

Lastri D, Alwahdy A. Clinical and radiologic approach to probable mixed dementia (vascular dementia and progressive supranuclear palsy). Malang Neurology Journal; 2020. 6(1): 46-50. DOI: 10.21776/ub.mnj.2020.006.01.10

Baaj AA, Agazzi S, Sayed ZA, Toledo M, Spetzler RF, van Loveren H. Surgical management of moyamoya disease: A review. Neurosurg Focus; 2009.26:1–7. DOI: 10.3171/2009.01.FOCUS08293

Kim T, Kwon O K, Chang W O, Jae S B, Hwang G, Lee Y J. Intracranial stenting using a drug-eluting stent for moyamoya disease involving suprclinoid internal carotid artery: A case report. Neurol Med Chir; 2014. 54:136-138. DOI: 10.2176/nmc. cr2012-0272

Khan N, Dodd R, Marks MP, Bell-Stephens T, Vavao J, Steinberg GK. Failure of primary percutaneous angioplasty and stenting in the prevention of ischemia in Moyamoya angiopathy. Cerebrovasc Dis; 2011. 31:147–153. DOI: 10.1159/000320253

Robert T, CicciÓ G, Sylvestre P, Chiappini A, Weil A G, Smajda S, et al. Anatomic and angiographic analyses of opthalmic artery collaterals in moyamoya disease. AJNR Am J Neuroradiol; 2018. 39:1121–26. DOI: 10.3174/ajnr.A5622.

Hariyanti T, Sunarti S, Vistiandini S. Cognitive impairment and depression are the most important risk factors for frailty. Malang Neurology Journal; 2020. 6(1): 20-23. DOI: 10.21776/ub.mnj.2020.006.01.4

Kelly ME, Bell-Stephens TE, Marks MP, Do HM, Steinberg GK. Progression of unilateral moyamoya disease: a clinical series. Cerebrovasc Dis; 2006. 22: 109–115. DOI: 10.1159/000093238.

Houkin K, Ito M, Sugiyama T, Shichinohe H, Nakayama N, Kazumata K, et al. Review of past research and current concepts on the etiology of moyamoya disease. Neurol Med Chir (Tokyo); 2012. 52:267–277.DOI:10.2176/nmc.52.267.

Gershlick A, Scheerder I D, Chevalier B, Lloyd A S, Camenzind E, Vrints C, et al.Inhibition of restenosis with a paclitaxel-eluting, polymer-free coronary stent. Circulation; 2004. 109:487-493.

DOI: 10.1161/01.CIR.0000109694. 8299.A0.

Suzuki J, Takaku A. Cerebrovascular ‘moyamoya’ disease: disease showing abnormal net-like vessels in base of brain. Arch Neurol; 1969. 20:288-299.

DOI: 10.1001/archneur.1969.00480090076012

Piao R, Oku N, Kitagawa K, Imaizumi M, Matsushita K, Yoshikawa T, et al. Cerebral hemodynamics and metabolism in adult moyamoya disease: Comparison of angiographic collateral circulation. Ann Nucl Med; 2004. 18:115-121. DOI: 10.1007/BF02985101.

Taki W, Yonekawa Y, Kobayashi A, Ishikawa M, Kikuchi H, Nishizawa S, et al. Cerebral circulation and metabolism in adults’ moyamoya disease: PET study. Acta Neurochir (Wien); 1989. 100:150-154. DOI: 10.1007/BF01403603.

Festa JR, Schwarz LR, Pliskin N, Cullum CM, Lacritz L, Charbel FT, et al. Neurocognitive dysfunction in adult moyamoya disease. J Neurol; 2010. 257:806-15. DOI: 10.1007/s00415-009-5424-8.

Calviere L, Catalaa I, Marlats F, Januel AC, Lagarrigue J, Larrue V. Improvement in cognitive function and cerebral perfusion after bur hole surgery in an adult with moyamoya disease: Case report. J Neurosurg; 2011. 115:347-9.

DOI: 10.3171/2011.3.JNS101117

Kim SK, Cho BK, Phi JH, Lee JY, Chae JH, Kim KJ, et al. Pediatric moyamoya disease: An analysis of 410 consecutive cases. Ann Neurol; 2010. 68:92-101. DOI: 10.1002/ana.21981.






Case Report