Moya-moya disease
DOI:
https://doi.org/10.36958/sep.v7i1.214Keywords:
Moyamoya, vasculopathy, cerebrovascular event, intracerebral hemorrhageAbstract
Moyamoya disease is a vasculopathy distinguished by progressive stenosis of cerebral arteries with the consequent formation of multiple fragile collateral vessels. Genetic studies identify RNF213 as the gene that provides susceptibility to develop the disease, but other genetic triggers are needed to express it. Moyamoya vessels are fragile and provide insufficient cerebral perfusion leading to a wide variety of clinical presentations such as: ischemic or hemorrhagic cerebrovascular events, epileptic seizures, cognitive impairment, disability or death.
The case of a male patient admitted for a hemorrhagic cerebrovascular event is presented. An arteriogram with digital subtraction showed severe stenosis at the level of the internal carotid arteries and collateral circulation from multiple basal and dural arteries, highlighting in the arterial phase that the collateral circulation emits an image similar to chimney smoke puffs.
In adults it occurs as intracranial hemorrhage due to hyperflow generated in anomalous vessels, which causes their rupture. Indirect revascularization by pial synangiosis or direct revascularization by bypass of the superficial temporal artery with the middle cerebral artery are the surgical treatments and and future ischemic or hemorrhagic cerebrovascular events should be prevented. The case is exposed because it is a pathology whose incidence is very low in Guatemala and an update on the pathophysiology and therapeutic approach will be provided.
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References
Demartini, Z., Jr, Teixeira, B. C., Koppe, G. L., Gatto, L. A. M., Roman, A., & Munhoz, R. P. (2022). Moyamoya disease and syndrome: a review. Radiologia brasileira, 55(1), 31–37. https://doi.org/10.1590/0100-3984.2021.0010 DOI: https://doi.org/10.1590/0100-3984.2021.0010
Fang, Y. C., Wei, L. F., Hu, C. J., & Tu, Y. K. (2021). Pathological Circulating Factors in Moyamoya Disease. International journal of molecular sciences, 22(4), 1696. https://doi.org/10.3390/ijms22041696 DOI: https://doi.org/10.3390/ijms22041696
Fujimura, M., Tominaga, T., Kuroda, S., Takahashi, J. C., Endo, H., Ogasawara, K., Miyamoto, S., Research Committee on Moyamoya Disease (Spontaneous Occlusion of Circle of Willis) of the Ministry of Health, Labor Welfare, Japan, & Guideline Committee 2021 of the Japan Stroke Society (2022). 2021 Japanese Guidelines for the Management of Moyamoya Disease: Guidelines from the Research Committee on Moyamoya Disease and Japan Stroke Society. Neurologia medico-chirurgica, 62(4), 165–170. https://doi.org/10.2176/jns-nmc.2021-0382 DOI: https://doi.org/10.2176/jns-nmc.2021-0382
Houkin, K., Ito, M., Sugiyama, T., Shichinohe, H., Nakayama, N., Kazumata, K., & Kuroda, S. (2012). Review of past research and current concepts on the etiology of moyamoya disease. Neurologia medico-chirurgica, 52(5), 267–277. https://doi.org/10.2176/nmc.52.267 DOI: https://doi.org/10.2176/nmc.52.267
Nathal, E., Serrano-Rubio, A., Maciel, E., & Arauz, A. (2018). Moyamoya disease in Mexico: Our experience. Enfermedad moyamoya en México. Experiencia institucional. Neurologia, S0213-4853(18)30179-8. Advance online publication. https://doi.org/10.1016/j.nrl.2018.05.006 DOI: https://doi.org/10.1016/j.nrl.2018.05.006
Scott, R. M., & Smith, E. R. (2009). Moyamoya disease and moyamoya syndrome. The New England journal of medicine, 360(12), 1226–1237. https://doi.org/10.1056/NEJMra0804622 DOI: https://doi.org/10.1056/NEJMra0804622
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Copyright (c) 2024 Ligia Ibeth Portillo Rivera, Sergio Raúl Chang Menéndez, María Ximena López Cervantes, Francisco Quijivix Cojulun, David Navarro Loarca, Juan Pablo Pineda
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