Moyamoya disease: a review with emphasis on its surgical treatment / Enfermedad de Moyamoya: una revisión con énfasis en su tratamiento quirúrgico

La enfermedad de Moyamoya (EMM) se caracteriza por la estenosis progresiva y la oclusión de las arterias carótidas internas en la base del cráneo. Se observó por primera vez en 1957 por Takeuchi y Shimizu en arteriografías anormales del cerebro. Esto representa el 6 por ciento de los accidentes cerebrovasculares en los niños, y es una enfermedad cerebrovascular importante en este grupo de edad en Japón. La mayoría son casos esporádicos de la EMM, pero hay también una variante familiar que se produce en aproximadamente el 8 por ciento de las veces. El tratamiento de la EMM depende de la presentación clínica del paciente y la etapa de la enfermedad. Las opciones de tratamiento incluyen la observación y el seguimiento, el tratamiento médico y el tratamiento quirúrgico, o incluso combinaciones de ellos. En general, no existe una recomendación establecida en relación con la gestión de la enfermedad, una vez que hay una ausencia de estudios prospectivos aleatorizados con seguimiento razonable. En este artículo realizamos una revisión de la literatura acerca de la EMM, con énfasis en su tratamiento quirúrgico.

Moyamoya disease (MMD) is characterized by progressive stenosis and occlusion of the internal carotid arteries at the base of the skull. It was first observed in 1957 by Takeuchi and Shimizu in abnormal arteriograms of the brain. It accounts for 6 percent of strokes in children, and it is a major cerebrovascular disease in this age group in Japan. Most are sporadic cases of MMD, but there is also a familiar variant that occurs in approximately 8 percent of the times. The treatment of MMD depends on the patient’s clinical presentation and the stage of the disease. Treatment options include observation and monitoring, medical treatment and surgical treatment, or even combinations of them. In general, there is no established recommendation regarding the management of the disease, once there is an absence of prospective randomized trials with reasonable follow-up. In this article we perform a review on the literature about the MMD, with emphasis on its surgical treatment.

Cerebrovascular Disease and Stroke in Korean Male Adolescent

PURPOSE: This study aimed to estimate the prevalence and incidence of cerebrovascular disease (CVD) and stroke in Korean male adolescents. MATERIALS AND METHODS: The authors reviewed all medical certificates, medical records, and radiologic images from the examinations of Korean military conscription from January 2008 to May 2011. RESULTS: Of the 101156 examinees, 40 had CVD and stroke during adolescence. The overall prevalence and incidence of CVD and stroke was 39.54 cases per 100000 adolescents and 2.08 cases per 100000 adolescents per year, respectively and these were similar to the worldwide data. There were 3 cases of aneurysm, 3 cases of dural arteriovenous fistula, 11 cases of arteriovenous malformation, 4 cases of cavernous hemangioma, 4 cases of cerebrovascular infarction, 16 cases of Moyamoya disease, and 1 case of missing data. The incidence of arteriovenous malformation (0.57 cases per 100000 adolescents per year) was lower than the incidence for the worldwide general population. The incidence of Moyamoya disease was higher than that in any other country (15.82 cases per 100000 adolescents, vs. 0.83 cases per 100000 adolescents per year). CONCLUSION: We observed ischemic and hemorrhagic stroke, each accounting for approximately half of cases, and high incidence of Moyamoya disease with low incidence of arteriovenous malformation in Korean male adolescents.

Moyamoya induced acute paraplegia in a child with epilepsy

Moyamoya disease [MMD] is a chronic, occlusive, cerebrovascular disorder of unknown pathogenesis, characterized by progressive stenosis of the bilateral supraclinoid internal carotid arteries, with concomitant formation of tortuous arterial collateral vessels at the base of the brain, which reconstitute distal branches of the cerebral circulation. In Japanese, "Moyamoya" means "hazy puff of smoke" and refers to the angiographic appearance of the abnormal network of vessels that develop at the base of the brain and basal ganglia to supply of collateral route of blood flow. We report here the case of Moyamoya disease in a 5 year-old girl with normal mentality with a one year history of epilepsy, with Todd's paralysis. This condition is rare and most patients are diagnosed in childhood. With this report we aim to underscore the possibility that usual neurological sign could be associated with unusual neurological disorders

Enfermedad de moyamoya en el Perú / Moyamoya diseases in Peru

Presentamos tres casos de enfermed de moyamoya encontrados en el Servicio de Neurocirugía "Estebán D. Rocca" del Hospital Nacional "Guillermo Almenara Irigoyen" en Lima de 1947 a 1990. La enfermedad de moyamoya es un desorden vascular cerebral oclusivo crónico, de presentación infrecuente y evolución progresiva, la cual fue por primera vez descrita según algunos autores por Shimizu et al. en 1955 y según otros por Kudo et al. en 1956 como un tipo de enfermedad oclusiva de las arterias carótidas internas, posteriormente Suzuki et. al en 1969 la establecen como entidad nosológica en la literatura médica. Los cuadros clínicos son variables. La imagen angiográfica cerebral es típica. Este trabajo tiene por objeto presentar los tres únicos casos encontrados en nuestro servicio, todos ellos en pacientes adultos, dos varones y una mujer, edad promedio 28 años y con cuadro clínico hemorragia subaracnoidea a repetición y hemiparesia. Se trataría del primer reporte de enfermedad de moyamoya en el Perú.

Moyamoya disease in Korea

Thirty eight cases of moyamoa disease, 21 children, 17 adults were encountered during a 16-year period at Yonsei University Medical Center. Clinical manifestations, together with computed tomography (CT) and angiographic findings were analyzed with a review of the literature. The mean age was 6.3 +/- 3.5 years in children and 36.8 +/- 9.9 years in adults. The majority of attacks occurred in spring in both adults and children. The most common chief complaint on admission was hemiparesis followed by convulsion in children, while in adults, loss of consciousness was most common followed by headache. Of transient neurologic deficits, hemiplegia was most common in children, while cranial nerve involvement was common in adults. Hemiplegia, also was the most common permanent neurologic manifestation in children, while hemiparesis and intellectual deterioration were the most common in adults. Of the children, 90.6% showed infarction on CT, while 88.2% of adults had hemorrhage. Bilateral occlusion of the carotid arteries was the most common site of lesions in both adults and children on cerebral angiogaphy.