Moyamoya disease in Mexico: our experience.

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.

Enfermedad moyamoya en México. Experiencia institucional / Moyamoya disease in Mexico: Our experience

Introducción: La enfermedad moyamoya (EM) es una arteriopatía oclusiva y progresiva de la circulación cerebral anterior que puede producir eventos isquémicos o hemorrágicos. El objetivo terapéutico es la prevención de nuevos eventos a través de tratamiento médico o cirugía de revascularización.MétodosSe estudió a 17 pacientes con EM y síndrome de moyamoya, atendidos de enero de 1989 a diciembre del 2016. Trece pacientes tenían EM definitiva (76%), un paciente EM unilateral (5,2%) y 3 pacientes síndrome de moyamoya (18%). Once pertenecían al sexo femenino y 6 al masculino. Su forma de presentación fue hemorragia intraparenquimatosa (35,2%), isquemia cerebral (29,4%), hemorragia subaracnoidea (17,6%), crisis convulsivas (11,7%) y un paciente empezó con cefalea sin hemorragia.ResultadosEn 10 pacientes se realizó una cirugía de revascularización (58,8%) y 7 se manejaron médicamente (41,2%). La valoración neurológica, empleando la escala modificada de Rankin al ingreso y en su última consulta, mostró una diferencia significativa (p < 0,04) a favor del grupo quirúrgico. Ningún paciente del grupo quirúrgico presentó nuevos eventos vasculares, mientras que 2 pacientes del grupo médico desarrollaron uno nuevo (uno isquémico y otro hemorrágico). En nuestro estudio no hubo una diferencia significativa entre los métodos de revascularización, pero sí en comparación con los pacientes que se trataron médicamente (p < 0,05).ConclusionesSi bien nuestra población muestra diferencias con otras series reportadas fuera de Asia, esta es la serie reportada más numerosa para una población hispana. Nuestros resultados indican que los procedimientos de revascularización son útiles para prevenir nuevos eventos vasculares isquémicos o hemorrágicos. (AU)

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.MethodsWe studied a series of 17 patients with MD (n = 14) or moyamoya syndrome (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 moyamoya syndrome (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 (one patient).ResultsTen 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.ConclusionsAlthough 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. (AU)

Bypass temporosilviano: enfermedad Moyamoya / Temporosilvian Bypass: Moyamoya disease

Introducción: En 1957, Takeuchi y Shimizu describen una vasculopatía oclusiva que involucra la arteria carótida interna bilateral, con la formación de vasos colaterales. En 1969, Suzuki y Takaku denominan a la conexión vascular colateral en las imágenes de angiografía "moyamoya" que significa nube de humo.2,3 Objetivos: El propósito del siguiente video es la descripción detallada de una cirugía de revascularización directa a través de un bypass temporosilviano en paciente con enfermedad Moyamoya. Materiales y Métodos: Se describe el caso de un paciente masculino de 27 años de edad que presentó de accidente vascular cerebral hemorrágico derecho. En la angiografía se diagnosticó estenosis del 70% de la arteria carótida interna supraclinoidea derecha, acompañado de estenosis de la arteria cerebral media y cerebral anterior homolateral. Se realizó cirugía de revascularización cerebral directa con bypass temporosilviano derecho.4,5 Resultados: Luego de realizado el bypass se confirmó adecuada permeabilidad del mismo y en la angiografía postoperatoria se observó el desarrollo de circulación colateral a través de la anastomosis. El paciente no presentó déficit en el periodo postoperatorio. Conclusión: Aunque la incidencia de enfermedad de Moyamoya no es elevada, es una causa probable de stroke isquémico o hemorrágico en niños y adultos. El manejo adecuado es fundamental para mejorar el pronostico a largo plazo de los pacientes con esta rara patología.

Introduction: In 1957, Takeuchi and Shimizu describes an occlusive vasculopathy involving the bilateral internal carotid arteries, with the formation of collateral vessels. In 1969, Suzuki and Takaku designate the collateral vascular connections in the angiographical images "moyamoya" which means puff of smoke.2,3 Objectives: The purpose of the following video is the detailed description of a direct revascularization surgery through a temporosilvian bypass in a patient with Moyamoya disease. Materials and methods: We present a case of a 27-year-old male patient with a history of right hemorrhagic cerebral vascular accident. In the angiography, 70% stenosis of the right supraclinoid internal carotid artery was diagnosed, accompanied by stenosis of the middle and anterior homolateral cerebral artery. Direct cerebral revascularization surgery was performed with right temporosilvian bypass.4,5 Results: After performing the bypass, adequate permeability is confirmed and in the postoperative angiography the development of collateral circulation through the anastomosis was observed. The patient did not present a deficit in the postoperative period. Conclusion: Although the incidence of Moyamoya disease is not high, it is a probable cause of ischemic or hemorrhagic stroke in children and adults. Proper management is essential to improve the long-term prognosis of patients with this rare pathology.

Moyamoya disease in Mexico: Our experience. / Enfermedad moyamoya en México. Experiencia institucional.

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. METHODS: We studied a series of 17 patients with MD (n = 14) or moyamoya syndrome (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 moyamoya syndrome (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 (one 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.

Enfermedad moyamoya: presentación de un caso y revisión de la literatura / Moyamoya disease: case report and literature review

La Enfermedad Moyamoya es una arteriopatia oclusiva progresiva de los vasos cerebrales, específicamente de la porción distal de las arterias carótidas internas, por lo general con compromiso bilateral. Esta oclusión trae como consecuencia dilatación de los vasos sanguíneos colaterales con el objetivo de convertirse en vías de circulación colateral, de donde la enfermedad adopta su nombre, debido a la apariencia angiográfica de “nube de humo” de estos pequeños vasos dilatados. Clínicamente se manifiesta como eventos cerebrovasculares isquémicos debido a la oclusión de los vasos mencionados o como eventos hemorrágicos debido a la ruptura de los pequeños vasos sanguíneos dilatados. Presentamos el caso de una paciente femenina de 38 años, que acude al hospital en estado de coma, cuya tomografía computarizada revela hemorragia intraventricular que fue tratada con derivación ventricular externa más activador de plasminógeno tisular. Posterior a su estabilización clínica se realizó angiografía cerebral diagnóstica con hallazgos compatibles con Enfermedad Moyamoya. Se decidió realizar subduro-sinangiosis con el fin de inducir la formación de circulación colateral cerebral. Debido a la baja incidencia de la Enfermedad Moyamoya en Latinoamérica, generalmente no es incluida en el diagnóstico diferencial de los eventos cerebrales vasculares, por lo que recomendamos su sospecha en pacientes jóvenes y sin factores de riesgo que debutan con este tipo de eventos cerebrales.

Moyamoya disease is a progressive occlusive disease of the cerebral vessels, specifically the distal portion of the internal carotid arteries, usually with bilateral involvement. This occlusion results in dilatation of collateral blood vessels in order to form collateral circulation pathways, from which the disease takes its name due to the angiographic appearance of “puff of smoke” of these small dilated vessels. Clinically it manifests as ischemic cerebrovascular events due to occlusion of the vessel mentioned or hemorrhagic events due to rupture of small dilated blood vessels. We report the case of a female patient of 38 years old who was admitted to the hospital in coma state, the CT scan revealed intraventricular hemorrhage that was treated with an external ventricular derivation and tissue plasminogen activator. After clinical stabilization diagnostic cerebral angiography was performed with findings consistent with Moyamoya disease. We decided to perform a subduro-sinangiosis, in order to induce the formation of cerebral collateral circulation. Due to the low incidence of Moyamoya disease in Latin America, generally it is not included in the differential diagnosis of cerebral vascular events, we recommend suspect it in young patients without risk factors who present with this type of brain events.

Síndrome moyamoya y enfermedad de Graves en una mujer joven / Moyamoya syndrome and Graves' disease in a young woman

Resumen Introducción: la enfermedad y el síndrome moyamoya son entidades cerebrovasculares caracterizadas por la estenosis u oclusión de la porción terminal de la arteria carótida interna y la porción proximal de las arterias cerebrales media y anterior, acompañada por la formación de vasos colaterales en forma de red. La enfermedad de Graves es una tiroiditis autoinmune de origen desconocido y es la causa más común de hipertiroidismo. La asociación de estas dos enfermedades es rara. Caso clínico: mujer de 27 años de edad hospitalizada por un infarto cerebral e hipertiroidismo debido a enfermedad de Graves. Las imágenes diagnósticas identificaron un infarto cerebral extenso temporoparietal derecho en territorio de la arteria cerebral media. Además, se evidenció la estenosis de la porción supraclinoidea de la arteria carótida interna derecha y de la porción proximal de las arterias cerebral media y anterior derechas, sin presencia de vasos colaterales. Inicialmente, fue tratada con antitiroideos y esteroides. Requirió dos tratamientos con yodo radiactivo para controlar el hipertiroidismo. La lesión cerebral le dejó como secuelas déficit motor y sensitivo izquierdos y una epilepsia focal sintomática controlada con medicación. Actualmente recibe terapia de suplencia tiroidea y anticonvulsivante. El seguimiento angiográfico cerebral demostró aumento de la estenosis de los vasos cerebrales afectados sin evidencia de nuevas lesiones cerebrales. Conclusiones: los pacientes con hipertiroidismo secundario a la enfermedad de Graves y manifestaciones clínicas neurológicas focales agudas deben investigarse para un posible síndrome moyamoya. (Acta Med Colomb 2013; 38: 262-267).

Abstract Introduction: the disease and moyamoya syndrome are cerebrovascular entities characterized by stenosis or occlusion of the terminal portion of the internal carotid artery and the proximal portion of the middle and anterior cerebral arteries, accompanied by the formation of collateral vessels in the form of network. Graves' disease is an autoimmune thyroiditis of unknown origin and is the most common cause of hyperthyroidism. The association of these two diseases is rare. Case report: 27 year-old woman hospitalized for a stroke and hyperthyroidism due to Graves' disease. Diagnostic imaging identified a large right temporoparietal infarct in the territory of the middle cerebral artery. Additionally, stenosis of the supraclinoid portion of the right internal carotid artery and the proximal portion of the anterior and middle right cerebral arteries without the presence of collateral vessels, was evidenced. Initially, she was treated with antithyroid drugs and steroids. Two treatments with radioactive iodine to control hyperthyroidism were needed. The brain injury left sequelae of left motor and sensory deficits and symptomatic focal epilepsy, controlled by medication. She is currently receiving thyroid substitution therapy and anticonvulsant therapy. Angiographic follow-up showed increased vessel stenosis of the cerebral affected vessels without evidence of new brain lesions. Conclusions: patients with hyperthyroidism secondary to Graves' disease and acute focal neurological manifestations should be investigated for possible moyamoya syndrome. (Acta Med Colomb 2013; 38: 262-267).