Original Articles

Distinct presentation of Moyamoya disease in Iran

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Received: 5 May 2025
Accepted: 5 June 2025
Published: 25 August 2025
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Moyamoya Disease (MMD) is a rare cerebrovascular disorder characterized by stenosis of the internal carotid arteries. To date, no studies have specifically described the characteristics of Moyamoya among Iranian populations. This study aims to examine the clinical characteristics of a case series of Iranian patients with MMD. This study prospectively identified all patients diagnosed with MMD at an institutional tertiary hospital in Tehran from 2010 to 2020. Data on demographic characteristics, disease presentations, past medical history, type of vascular lesion, treatment approaches, and outcomes during follow-up were collected. A total of 15 patients with MMD were included. Of these, 9 (60%) were female. Age distribution at diagnosis showed 6 patients were younger than 40 years. No familial patterns of MMD were observed. All patients experienced Cerebrovascular Accidents (CVA) at disease onset. A notable prevalence of comorbid conditions was observed, including hypertension (33.3%), diabetes mellitus (20%), and seizure disorders (20%). Vascular lesions were unilateral in 46.6% of patients and bilateral in 53.4%. Direct bypass surgery was performed in 40% of cases. The mean follow-up period was 16.3±1.2 months. During this time, 2 patients died and 1 experienced a new CVA. Residual symptoms were present in 13.3% of patients. This study shows distinct characteristics of MMD in an Iranian cohort, including a female predominance, frequent ischemic symptoms, and a high rate of bilateral occlusions. Further prospective studies are warranted to assess the efficacy of medical and surgical interventions in preventing recurrent symptoms in this population.

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1. Berry JA, Cortez V, Toor H, et al. Moyamoya: An Update and Review. Cureus 2020;12:e10994. DOI: https://doi.org/10.7759/cureus.10994
2. Gupta A, Tyagi A, Romo M, et al. Moyamoya Disease: A Review of Current Literature. Cureus 2020;12:e10141. DOI: https://doi.org/10.7759/cureus.10141
3. Takagi Y, Kikuta KI, Nozaki K, et al. Histological features of middle cerebral arteries from patients treated for moyamoya disease. Neurol Med Chir (Tokyo) 2007;47:1-4. DOI: https://doi.org/10.2176/nmc.47.1
4. Takagi Y, Kikuta KI, Sadamasa N, et al. Caspase-3-dependent apoptosis in middle cerebral arteries in patients with moyamoya disease. Neurosurgery 2006;59:894-900. DOI: https://doi.org/10.1227/01.NEU.0000232771.80339.15
5. Bedini G, G. Blecharz K, Nava S, et al. Vasculogenic and Angiogenic Pathways in Moyamoya Disease. Curr Med Chem 2016;23:315-45. DOI: https://doi.org/10.2174/092986732304160204181543
6. Choi JW, Son SM, Mook-Jung I, et al. Mitochondrial abnormalities related to the dysfunction of circulating endothelial colony-forming cells in moyamoya disease. J Neurosurg 2018;129:1151-9. DOI: https://doi.org/10.3171/2017.5.JNS17147
7. Zhao M, Gao F, Zhang D, et al. Altered expression of circular RNAs in Moyamoya disease. J Neurol Sci 2017;381:25-31. DOI: https://doi.org/10.1016/j.jns.2017.08.011
8. Liu W, Hitomi T, Kobayashi H, et al. Distribution of Moyamoya Disease Susceptibility Polymorphism p.R4810K in RNF213 in East and Southeast Asian Populations. Neurol Med Chir (Tokyo) 2012;52:299-303. DOI: https://doi.org/10.2176/nmc.52.299
9. Suzuki J, Takaku A. Cerebrovascular “Moyamoya#x201D; Disease: Disease Showing Abnormal Net-Like Vessels in Base of Brain. Arch Neurol 1969;20:288-99. DOI: https://doi.org/10.1001/archneur.1969.00480090076012
10. Oki K, Katsumata M, Izawa Y, et al. Trends of Antiplatelet Therapy for the Management of Moyamoya Disease in Japan: Results of a Nationwide Survey. J Stroke Cerebrovasc Dis 2018;27:3605-12. DOI: https://doi.org/10.1016/j.jstrokecerebrovasdis.2018.08.030
11. Kim T, Oh CW, Bang JS, et al. Moyamoya disease: Treatment and outcomes. J Stroke 2016;18:21-30. DOI: https://doi.org/10.5853/jos.2015.01739
12. Guidelines for Diagnosis and Treatment of Moyamoya Disease (Spontaneous Occlusion of the Circle of Willis): Esearch Committee on the Pathology and Treatment of Spontaneous Occlusion of the Circle of Willis; Health Labour Sciences Research Grant for Research on Measures for intractable Diseases. Neurol Med Chir (Tokyo) 2012;52:245-66. DOI: https://doi.org/10.2176/nmc.52.245
13. Abbasi SH, Sundin Ö, Jalali A, et al. Ethnic Differences in the Risk Factors and Severity of Coronary Artery Disease: a Patient-Based Study in Iran. J Racial Ethn Heal Disparities 2018;5:623-31. DOI: https://doi.org/10.1007/s40615-017-0408-3
14. Sencer S, Poyanli A, Kiriş T, et al. Recent experience with Moyamoya disease in Turkey. Eur Radiol 2000;10:569-72. DOI: https://doi.org/10.1007/s003300050962
15. Salih MAM, Al-Jarallah AS, Abdel-Gader AGM, et al. Cardiac diseases as a risk factor for stroke in Saudi children. Saudi Med J 2006;27:S69-80.
16. Shoukat S, Itrat A, Taqui AM, et al. Moyamoya disease: A clinical spectrum, literature review and case series from a tertiary care hospital in Pakistan. BMC Neurol 2009;9:15. DOI: https://doi.org/10.1186/1471-2377-9-15
17. Tatli B, Ekici B, Sencer A, et al. Clinical features, prothrombotic risk factors, and long-term follow-up of eight pediatric Moyamoya patients. J Clin Neurol 2012;8:100-3. DOI: https://doi.org/10.3988/jcn.2012.8.2.100
18. Saeed M, Azam M, Saeed A, et al. Clinical features and outcome of moyamoya disease. Pakistan Paediatr J 2016;40:123-8.
19. Goren O, Hendrix P, Peled A, et al. Encephaloduroarteriosynangiosis with Dural Inversion for Moyamoya Disease in a Pediatric and Adult Population—a Single-Center 20-Year Experience. World Neurosurg 2021;149:e16–21. DOI: https://doi.org/10.1016/j.wneu.2021.02.102
20. Schwartzmann Y, Spektor S, Moscovici S, et al. Comparison between moyamoya disease and moyamoya syndrome in Israel. J Stroke Cerebrovasc Dis 2024;33:107635. DOI: https://doi.org/10.1016/j.jstrokecerebrovasdis.2024.107635
21. Wakai K, Tamakoshi A, Ikezaki K, et al. Epidemiological features of Moyamoya disease in Japan: Findings from a nationwide survey. Clin Neurol Neurosurg 1997;99:S1-5. DOI: https://doi.org/10.1016/S0303-8467(97)00031-0
22. Miyamoto S, Yoshimoto T, Hashimoto N, et al. Effects of extracranial-intracranial bypass for patients with hemorrhagic moyamoya disease: Results of the Japan adult moyamoya trial Stroke 2014;45:1415-21.
23. Noh HJ, Kim SJ, Kim JS, et al. Long term outcome and predictors of ischemic stroke recurrence in adult moyamoya disease. J Neurol Sci 2015;359:381-8. DOI: https://doi.org/10.1016/j.jns.2015.11.018
24. Scott RM, Smith ER. Moyamoya Disease and Moyamoya Syndrome. N Engl J Med 2009;360:1226-37. DOI: https://doi.org/10.1056/NEJMra0804622
25. Smith ER, Scott RM. Moyamoya: Epidemiology, Presentation, and Diagnosis. Neurosurg Clin N Am 2010;21:543-51. DOI: https://doi.org/10.1016/j.nec.2010.03.007
26. Agarwalla PK, Stapleton CJ, Phillips MT, et al. Surgical outcomes following encephaloduroarteriosynangiosis in North American adults with moyamoya. J Neurosurg 2014;121:1394-400. DOI: https://doi.org/10.3171/2014.8.JNS132176
27. Hori S, Kashiwazaki D, Yamamoto S, et al. Impact of interethnic difference of collateral angioarchitectures on prevalence of hemorrhagic stroke in Moyamoya disease. Clin Neurosurg 2019;85:134-45. DOI: https://doi.org/10.1093/neuros/nyy236
28. Yamamoto S, Hori S, Kashiwazaki D, Akioka N, et al. Longitudinal anterior-to-posterior shift of collateral channels in patients with moyamoya disease: An implication for its hemorrhagic onset. J Neurosurg 2019;130:884-90. DOI: https://doi.org/10.3171/2017.9.JNS172231
29. Guo DC, Papke CL, Tran-Fadulu V, et al. Mutations in smooth muscle Alpha-Actin (ACTA2) cause coronary artery disease, stroke, and Moyamoya disease, along with thoracic aortic disease. Am J Hum Genet 2009;84:617-27. DOI: https://doi.org/10.1016/j.ajhg.2009.04.007
30. Hayashi K, Horie N, Suyama K, et al. An epidemiological survey of moyamoya disease, unilateral moyamoya disease and quasi-moyamoya disease in Japan. Clin Neurol Neurosurg 2013;115:930-3. DOI: https://doi.org/10.1016/j.clineuro.2012.09.020
31. Kraemer M, Schwitalla JC, Diesner F, et al. Clinical presentation of Moyamoya angiopathy in Europeans: experiences from Germany with 200 patients. J Neurol 2019;266:1421-8. DOI: https://doi.org/10.1007/s00415-019-09277-1
32. Saarela M, Mustanoja S, Pekkola J, et al. Moyamoya vasculopathy – Patient demographics and characteristics in the Finnish population. Int J Stroke 2017;12:90-5. DOI: https://doi.org/10.1177/1747493016669847
33. Liu XJ, Zhang D, Wang S, et al. Clinical features and long-term outcomes of moyamoya disease: A single-center experience with 528 cases in China. J Neurosurg 2015;122:392-9. DOI: https://doi.org/10.3171/2014.10.JNS132369
34. Barrera D, Sercy E, Orlando A, et al. Associations of antithrombotic timing and regimen with ischemic stroke and bleeding complications in blunt cerebrovascular injury. J Stroke Cerebrovasc Dis 2020;29:104804. DOI: https://doi.org/10.1016/j.jstrokecerebrovasdis.2020.104804
35. Baba T, Houkin K, Kuroda S. Novel epidemiological features of moyamoya disease. J Neurol Neurosurg Psychiatry 2008;79:900-4. DOI: https://doi.org/10.1136/jnnp.2007.130666
36. Ahn IM, Park DH, Hann HJ,et al. Incidence, prevalence, and survival of moyamoya disease in Korea: A nationwide, population-based study. Stroke 2014;45:1090-5. DOI: https://doi.org/10.1161/STROKEAHA.113.004273

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How to Cite



Distinct presentation of Moyamoya disease in Iran. (2025). European Journal of Translational Myology, 35(4). https://doi.org/10.4081/ejtm.2025.13953