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INTRODUCTION: Moyamoya disease (MD) is a progressive, occlusive disease of the arteries of the anterior cerebral circulation that may cause ischaemia or haemorrhage. Patient management aims to prevent new cerebrovascular events through surgical revascularisation and/or pharmacological treatment. MATERIALS AND METHODS: We studied a series of 17 patients with MD (n = 14) or moyamoya syndrome (MS; n = 3), who were evaluated between January 1989 and December 2016; 11 patients were women and 6 were men. Thirteen patients had definitive MD (76%), one had unilateral MD (5.2%), and 3 had MS (18%). The condition manifested as intraparenchymal haemorrhage (in 35.2% of patients), brain ischaemia (29.4%), subarachnoid haemorrhage (17.6%), seizures (11.7%), and headache with no associated haemorrhage (1 patient). RESULTS: Ten patients (58.8%) underwent revascularisation and 7 (41.2%) received pharmacological treatment. All patients were evaluated with the modified Rankin Scale (mRs) at admission and at the last consultation; mRs scores were significantly lower in the group undergoing surgery (P <  .04). During follow-up, none of the patients undergoing revascularisation experienced recurrences, whereas 2 patients receiving pharmacological treatment did experience a new vascular event (one ischaemic and one haemorrhagic) (P <  .05). No significant differences were observed between the treatment outcomes of different revascularisation techniques. CONCLUSIONS: Although our population has different demographic characteristics from those of other non-Asian populations, ours is the largest published series of Hispanic individuals with MD. Our results support the use of revascularisation procedures to improve these patients' neurological status and to prevent new cerebrovascular events.

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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.

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INTRODUCTION: Moyamoya disease was first believed to be confined to Japan, but over the years it has been reported all over the world. We report seven cases from the University Hospital of the Faculty of Medicine of Ribeirão Preto, University of São Paulo with a review of the previously described cases in Brazil. MATERIAL AND METHODS: The diagnosis was based on the diagnostic criteria of the Research Committee on Spontaneous Occlusion of the Circle of Willis. RESULTS: Five patients presented ischemic and two hemorrhagic events. The meta-analysis of the six cases previously described in Brazil showed that only two were true moyamoya disease according to the diagnostic criteria. CONCLUSION: We can assume that the real frequency of moyamoya disease has been underestimated in Brazil.

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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.

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