Dr. Ankita Shah, Dr. Anagha Joshi, Dr. Aakash Vaswani, Dr. Amol Rathod


The moyamoya syndrome is a cerebrovascular condition that predisposes affected patients to stroke due to progressive stenosis of the intracranial
internal carotid arteries and their proximal branches. It can be seen in association with several inherited disorders. However, its association with
thalassemia is very rare.

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Scott RM, Smith ER. Moyamoya disease and moyamoya syndrome. New England Journal of Medicine. 2009 Mar 19;360(12):1226-37.

Matsushima Y, Aoyagi M, Nariai T, et al: Long-term intelligence out- come of post-encephalo-duro-arteri-synangiosis in childhood moy- amoya patients. Clin Neurol Neurosurg 1997;99(Suppl 2):S147–S150.

Fujisawa I, Asato R, Nishimura K, Togashi K, Itoh K, Noma S, Sagoh T, Minami S, Nakano Y, Yonekawa Y. Moyamoya disease: MR imaging. Radiology. 1987 Jul;164(1):103-5

Tarasów E, Kułakowska A, Łukasiewicz A, Kapica-Topczewska K, Korneluk-Sadzyńska A, Brzozowska J, Drozdowski W. Moyamoya disease: diagnostic imaging. Polish journal of radiology. 2011 Jan;76(1):73

Suzuki J, Kodama N: Moyamoya disease—Review. Stroke 1983;14: 104–109.

Ohene-Frempong K, Weiner SJ, Sleeper LA, et al: Cerebrovascular acci- dents in sickle cell disease: Rates and risk factors. Blood 1998;91:288–294.

Ganesan V, Prengler M, McShane MA, et al: Investigation of risk factors in children with arterial ischemic stroke. Ann Neurol 2003;53:167–173.

Tokunaga Y, Ohga S, Suita S, et al: Moyamoya syndrome with sphe- rocytosis: Effect of splenectomy on strokes. Pediatr Neurol 2001;25:75–77.

Cohen N, Berant M, Simon J: Moyamoya and Fanconi’s anemia. Pedi- atrics 1980;65:804–805.

Butrum MW, Williams LS, Golomb MR: A child with Diamond-Blackfan anemia, methylenetetrahydrofolate reductase mutation, and perinatal stroke. J Child Neurol 2003;18:800–802.

Kaysser TM, Wandersee NJ, Bronson RT, Barker JE: Thrombosis and secondary hemochromatosis play major roles in the pathogenesis of jaundiced and spherocytic mice, murine models for hereditary spherocytosis. Blood 1997;90:4610–4619.

Göksel BK, Ozdogu H, Yildirim T, Oğuzkurt L, Asma S. Beta-thalassemia intermedia associated with moyamoya syndrome. Journal of Clinical Neuroscience. 2010 Jul 1;17(7):919-20.

Olivieri NF: The beta-thalassemias. N Engl J Med 1999;341:99–109.

Cappellini MD, Robbiolo L, Bottasso BM, et al. Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia. Br J Haematol. 2000; 111:467–473.

Taher A, Isma’eel H, Mehio G, et al. Prevalence of thromboembolic events among 8,860 patients with thalassae- mia major and intermedia in the Mediterranean area and Iran. Thromb Haemost. 2006;96:488–491.

Eldor A, Durst R, Hy-Am E, et al. A chronic hypercoagulable state in patients with beta-thalassaemia major is already present in childhood. Br J Haematol. 1999;107: 739–746.


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