Moya-moya disease is a very rare disease characterized by a progressive vasculopathy leading to stenosis of the main intracranial arteries. Cerebral blood flow is usually impaired by constriction of the vessels with collateral circulation developing around the blocked vessels to compensate for the blockage. This may lead to an ischaemic stroke as observed on the index case. The aim of this case report is to highlight moyamoya disease as a rare cause of ischaemic stroke in an elderly male. We report a case of a 70 year old Nigerian male who has hypertension and diabetes mellitus, and presented with features of right hemispheric ischaemic stroke. The patient was investigated further with cerebral angiography and was found to have moyamoya disease. Angiography of the index case showed numerous abnormal hazy looking vessels arising from the level of the circle of willis and equivalent non filling in the posterior supratentorial and infratentorial spaces typical of moya moya pattern. He responded to medical therapy administered in the acute phase and subsequently followed-up at hospice care. Patient showed some clinical improvement with significant residual motor deficit on the left side of the body. The acute management was mainly symptomatic and directed towards reducing elevated intracranial pressure, improving cerebral blood flow, and controlling seizures. The index patient was frail, unfit and therefore could not benefit from surgical revascularization procedure which is the main treatment option for moyamoya disease.
Published in | World Journal of Medical Case Reports (Volume 1, Issue 1) |
DOI | 10.11648/j.wjmcr.20200101.14 |
Page(s) | 9-12 |
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This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
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Copyright © The Author(s), 2020. Published by Science Publishing Group |
Moyamoya Disease, Ischaemic Stroke, Cerebral Angiography and Revascularization
[1] | Scott R. Michael; Smith, Edward R. (2009). "Moyamoya Disease and Moyamoya Syndrome" New England Journal of Medicine. 360 (12): 1226–1237. |
[2] | Duan L, Bao X, Yang W, Shi W, Li D, Zhang Z, Zong R, et al (2012). "Moyamoya disease in China". Stroke. 43 (1): 56-60. |
[3] | Suzuki J, Takaku A (1969) Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol 20: 288-299. |
[4] | Edward R, Smith. "Moyamoya Arteriopathy". Current Treatment Options in Neurology. 2012: 14 (6): 549–556. |
[5] | Lin R, Xie Z, Zhang J, Xu H, Su H, Tan X et al. clinical and immunopathological features of moyamoya disease. Plos one. 2012; 7. |
[6] | Janda P, Bellew J, Veerappan V (2009)."Moyamoya disease: case report and literature review". The Journal of the American Osteopathic Association. 109 (10): 547–553. PMID 19861596. |
[7] | Zhang H, Zheng L, Feng L. Epidemiology, diagnosis and treatment of moyamoya disease. Exp Ther Med 17: 1977-1984, 2019. |
[8] | Pioa J, Wu W, Yang Z, Yu J. Research Progress of Moyamoya Disease in children. Int J Med Sci 2015: 12 (7): 566-575. |
[9] | Cho WS, Chung YS, Kim JE, Jeon JP, Son YJ, Bang JS, et al. The natural clinical course of hemodynamically stable adult moyamoya disease. J Neurosurg 2015; 122: 82-89. |
[10] | Noh HJ, Kim SJ, Kim JS, Hong SC, Kim KH, Jun P, et al. Long term outcome and predictors of ischemic stroke recurrence in adult moyamoya disease. J Neurol Sci 2015; 359: 381-388. |
[11] | Abel makubi, D. soka, J. makani. Moyamoya disease, a rare cause of recurrent strokes in an African sickle cell child: Does hydroxyurea have a role in the context? Vol 1, No 1 (2012). |
[12] | Guet S, Herve D, Tounier-lasserve E, kossorotoff M. Moya moya disease and diagnosis: from genetic to clinical management. 2015; 8: 49-68. |
[13] | Houkin K, Kuroda S. Moyamoya disease: current concepts and future perspectives. Lancet Neurol. 2008; 27: 1056–66. 10. |
[14] | Houkin K, Nakayama N, Kuroda S, Ishikawa T, and Nonaka T. How does angiogenesis develop in pediatric moyamoya disease after surgery? Prospective study with MR angiography. Childs Nerv Syst. 2004; 20: 734–41. |
[15] | Kuroda S, Ishikawa T, Houkin K, Nanba R, Hokari M, Iwasaki Y. Incidence and clinical features of disease progression in adult moyamoya disease. Stroke 2005; 36: 2148-2153. |
[16] | Yamada I, Matsushima Y, Suzuki S (1992) Moyamoya disease: diagnosis with three-dimensional time-of-flight MR angiography. Radiology 184: 773-778. |
APA Style
Edeh Samuel, Fintan Ekochin, Okafor Umezurike, Ugwu Chidiebube. (2020). Moyamoya Disease in a 70 Year Old Nigerian Male: A Case Report. World Journal of Medical Case Reports, 1(1), 9-12. https://doi.org/10.11648/j.wjmcr.20200101.14
ACS Style
Edeh Samuel; Fintan Ekochin; Okafor Umezurike; Ugwu Chidiebube. Moyamoya Disease in a 70 Year Old Nigerian Male: A Case Report. World J. Med. Case Rep. 2020, 1(1), 9-12. doi: 10.11648/j.wjmcr.20200101.14
@article{10.11648/j.wjmcr.20200101.14, author = {Edeh Samuel and Fintan Ekochin and Okafor Umezurike and Ugwu Chidiebube}, title = {Moyamoya Disease in a 70 Year Old Nigerian Male: A Case Report}, journal = {World Journal of Medical Case Reports}, volume = {1}, number = {1}, pages = {9-12}, doi = {10.11648/j.wjmcr.20200101.14}, url = {https://doi.org/10.11648/j.wjmcr.20200101.14}, eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.wjmcr.20200101.14}, abstract = {Moya-moya disease is a very rare disease characterized by a progressive vasculopathy leading to stenosis of the main intracranial arteries. Cerebral blood flow is usually impaired by constriction of the vessels with collateral circulation developing around the blocked vessels to compensate for the blockage. This may lead to an ischaemic stroke as observed on the index case. The aim of this case report is to highlight moyamoya disease as a rare cause of ischaemic stroke in an elderly male. We report a case of a 70 year old Nigerian male who has hypertension and diabetes mellitus, and presented with features of right hemispheric ischaemic stroke. The patient was investigated further with cerebral angiography and was found to have moyamoya disease. Angiography of the index case showed numerous abnormal hazy looking vessels arising from the level of the circle of willis and equivalent non filling in the posterior supratentorial and infratentorial spaces typical of moya moya pattern. He responded to medical therapy administered in the acute phase and subsequently followed-up at hospice care. Patient showed some clinical improvement with significant residual motor deficit on the left side of the body. The acute management was mainly symptomatic and directed towards reducing elevated intracranial pressure, improving cerebral blood flow, and controlling seizures. The index patient was frail, unfit and therefore could not benefit from surgical revascularization procedure which is the main treatment option for moyamoya disease.}, year = {2020} }
TY - JOUR T1 - Moyamoya Disease in a 70 Year Old Nigerian Male: A Case Report AU - Edeh Samuel AU - Fintan Ekochin AU - Okafor Umezurike AU - Ugwu Chidiebube Y1 - 2020/10/13 PY - 2020 N1 - https://doi.org/10.11648/j.wjmcr.20200101.14 DO - 10.11648/j.wjmcr.20200101.14 T2 - World Journal of Medical Case Reports JF - World Journal of Medical Case Reports JO - World Journal of Medical Case Reports SP - 9 EP - 12 PB - Science Publishing Group SN - 2994-726X UR - https://doi.org/10.11648/j.wjmcr.20200101.14 AB - Moya-moya disease is a very rare disease characterized by a progressive vasculopathy leading to stenosis of the main intracranial arteries. Cerebral blood flow is usually impaired by constriction of the vessels with collateral circulation developing around the blocked vessels to compensate for the blockage. This may lead to an ischaemic stroke as observed on the index case. The aim of this case report is to highlight moyamoya disease as a rare cause of ischaemic stroke in an elderly male. We report a case of a 70 year old Nigerian male who has hypertension and diabetes mellitus, and presented with features of right hemispheric ischaemic stroke. The patient was investigated further with cerebral angiography and was found to have moyamoya disease. Angiography of the index case showed numerous abnormal hazy looking vessels arising from the level of the circle of willis and equivalent non filling in the posterior supratentorial and infratentorial spaces typical of moya moya pattern. He responded to medical therapy administered in the acute phase and subsequently followed-up at hospice care. Patient showed some clinical improvement with significant residual motor deficit on the left side of the body. The acute management was mainly symptomatic and directed towards reducing elevated intracranial pressure, improving cerebral blood flow, and controlling seizures. The index patient was frail, unfit and therefore could not benefit from surgical revascularization procedure which is the main treatment option for moyamoya disease. VL - 1 IS - 1 ER -