Doença de Moyamoya: impacto no desempenho da linguagem oral e escrita / Moyamoya disease: impact on the performance of oral and written language

RESUMO A Doença de Moyamoya (DMM) é uma forma incomum de doença cerebrovascular oclusiva que acomete artérias do sistema nervoso central, acarretando alterações adquiridas de linguagem e dificuldades na aprendizagem. O objetivo foi descrever habilidades de linguagem oral/escrita e cognitivas em menina com diagnóstico de DMM de sete anos e sete meses. A avaliação constou de entrevista com pais, Observação do Comportamento Comunicativo, Teste de Vocabulário por Imagem Peabody, Teste de Desempenho Escolar, Perfil de Habilidades Fonológicas, Wechsler Intelligence Scale for Children, Teste Gestáltico Visomotor Bender, Wisconsin Card Sorting Test. Até os seis anos e cinco meses, ocorreram dois episódios de acidente vascular encefálico (AVE) em região temporoparietal esquerda e direita e área frontal esquerda. Realizou cirurgia para revascularização e tratamento medicamentoso. Avaliações audiológicas e oftalmológicas indicaram normalidade. Cursa o segundo ano do ensino fundamental. Apresentou alterações na linguagem oral e escrita (silábico-alfabética); não nomeação de todos os grafemas; escrita e aritmética média-inferior e leitura inferior à primeira série; habilidades do processamento fonológico em nível pré-escolar. A avaliação psicológica indicou nível intelectual satisfatório, porém prejuízo no desempenho cognitivo em tarefas verbais e de execução, limitações nas competências gráfico-percepto-motoras e na organização sequencial lógica. Os episódios de AVEs trouxeram interferências no desempenho dos processos de aprendizagem pelas especificidades das áreas afetadas, interferindo na análise, integração e interpretação de informações auditivas e visuais relevantes para os processos de aprendizagem.

ABSTRACT Moyamoya disease is an unusual form of occlusive, cerebrovascular disorder that affects the arteries of the central nervous system, causing acquired language alterations and learning difficulties. The study aim was to describe the oral/written language and cognitive skills in a seven-year-and-seven-month-old girl diagnosed with Moyamoya disease. The assessment consisted of interviews with her parents and application of the following instruments: Observation of Communicative Behavior, Peabody Picture Vocabulary Test, Academic Performance Test, Profile of Phonological Awareness, Raven’s Progressive Matrices Test, Special Scale, Wechsler Intelligence Scale for Children, Bender Visual Motor Gestalt Test, and Wisconsin Card Sorting Test. Two episodes of stroke in the left and right temporal-parietal and left frontal areas occurred until the age of six years and five months. Revascularization surgery and medication treatment were conducted. The audiologic and ophthalmologic assessments indicated normality. At the time of the study, the girl was attending the second grade of elementary school. She presented changes in oral and written language (syllabic-alphabetic), non-naming of all graphemes, low arithmetic and writing means, reading skill below first grade level and psycholinguistic delay, and pre-school level phonological processing skills. The psychological evaluation indicated satisfactory intellectual level; however, it also showed cognitive performance impairment in verbal and execution tasks and limitations on graphic-perceptual-motor skills and sequential logic organization. The stroke episodes influenced the performance of learning processes, affecting the analysis, integration, and interpretation of relevant visual and auditory information.

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.

Enfermedad de Moyamoya: reporte de un caso / Moyamoya disease: a case report

La enfermedad de Moyamoya es una patología caracterizada por la estenosis progresiva de la arteria carótida interna y sus ramas principales. Es de etiología desconocida, tiene como forma de presentación a la enfermedad cerebrovascular isquémica o hemorrágica, siendo la primera más frecuente, y afecta en mayor proporción a niños y adultos jóvenes constituyendo un reto diagnóstico. Su presencia se confirma mediante la angiografía por sustracción digital (ASD) y el manejo es médico y/o quirúrgico, siendo el último el que se asocia a un mejor pronóstico. Comunicamos el caso de un paciente peruano de ascendencia japonesa, sin factores de riesgo, con una hemorragia intracraneal cuyo diagnóstico final fue enfermedad de Moyamoya...

Moyamoya disease is characterized by progressive stenosis of the internal carotid artery and its main branches. The cause of the disease is unknown, ischemic or hemorrhagic stroke are the main manifestations (the former is more common) that disproportionately affect children and young adults, and is consider a diagnostic challenge. Its presence is confirmed by digital subtraction angiography (DSA) and the management may be medical or surgical, being the latter associated with a better prognosis. We report the case of a Peruvian male of Japanese ancestry without risk factors, with an intracranial hemorrhage who was finally diagnosed with Moyamoya disease...

Fanconi anemia associated with moyamoya disease in Saudi Arabia

We report a 10-year-old Saudi girl who has Fanconi anemia [FA] and was admitted due to acute hemiplegia, of the right side. She had a previous attack of left side hemiplegia that resolved spontaneously. The brain magnetic resonance angiography showed a cerebrovascular pattern of moyamoya disease. She underwent partially matched related donor stem cell transplantation [SCT], but unfortunately died 3 months later with post SCT complications. The association of moyamoya disease with FA is uncommon, and is rarely reported in the literature. Although this condition may be acquired, it is considered a truly congenital defect in FA, and to identify the etiology of this association furthermore genetic mutation analysis is needed

Pregnancy Outcomes in Women with Moyamoya Disease: Experiences at a Single Center in Korea

PURPOSE: Moyamoya disease (MMD) occurs predominantly in Korean and Japanese women. The aim of this study was to investigate clinical features and pregnancy outcomes in women with MMD. MATERIALS AND METHODS: We conducted a retrospective chart review of women with MMD who visited our Department of Obstetrics and Gynecology between January 2005 and October 2013. For all study subjects, clinical features, demographic characteristics, and perinatal outcomes were recorded. RESULTS: We identified 28 pregnancies in 22 patients who had been diagnosed with MMD. The mean maternal age at delivery was 31.9+/-3.5 years old. The mean gestational age at delivery was 38.0+/-0.9 weeks. Among the 28 pregnancies, 25 (92.5%) underwent cesarean section; 19 (76.0%) of them were performed under regional anesthesia and six (24.0%) under general anesthesia. The mean newborn weight was 3233.7+/-348.2 g. The 5-minute Apgar score in 85% of the newborns was higher than 8, with no other apparent complications. During the puerperal period, transient ischemic attack symptom or seizure occurred in 4 cases, although patients recovered within a few days. CONCLUSION: For pregnant women with MMD, it is important to control blood pressure and prevent hyperventilation during the intrapartum period, and the best methods of delivery and anesthesia should be considered to avoid unfavorable sequelae. Additionally, a multidisciplinary approach (i.e., neurosurgery) is necessary to constantly manage underlying diseases.

Revascularização cerebral na doença de moyamoya / Cerebral revascularization in moyamoya disease

Objetivo: Relatar uma série de casos de doença moyamoya/síndrome de moyamoya (DMM/SMM) tratados por revascularização cerebral no período de 2001 a 2013. Método: Estudo retrospectivo de 12 pacientes portadores de DMM/SMM submetidos à revascularização cerebral. Resultados: Trêspacientes foram operados por meio de by-pass de alto fluxo, três com by-pass de baixo fluxo e seis por revascularização indireta (encefaloarteriossinangiose associada à galeossinangiose). Realizamosdurante o seguimento: análise da ocorrência de novos acidentes vasculares cerebrais (AVC), avaliação funcional (utilizando a Escala de Rankin Modificada) e das complicações cirúrgicas. O tempo de acompanhamento para o grupo de by-pass de alto fluxo foi de quatro a dez anos, para o grupo de baixo fluxo e revascularização indireta de três meses a três anos. Nenhum paciente apresentou outro AVC no hemisfério operado tampouco piora funcional. As taxas de morbimortalidade e de infecção foram nulas. Conclusão: A revascularização cerebral foi efetiva, prevenindo a ocorrência de novos AVC e evitando piora funcional.

Objective: To report a case series of moyamoya disease/moyamoya syndrome (DMM/SMM) treated by cerebral revascularization in the period 2001-2013. Method: Retrospective study of twelve patients with DMM/SMM submitted to cerebral revascularization. Results: Three patients were operated through high-flow by-pass, three with low flow by-pass and six with indirect revascularization (encephaloarterio-sinangiosis associated with galeo-sinangiosis). Analyzed during follow-up: the occurrence of new strokes, functional assessment (using the modified Rankin scale) and surgical complications. The follow up to the group of high-flow by-pass was 4-10 years for the group of low flow and indirect revascularization of three months to three years. No patient had another cerebrovascular accident (CVA) in the hemisphere operated nor functional worsening. Rates of morbidity and mortality and infection were nil. Conclusion: Cerebral revascularization was effective, preventing the occurrence of new strokesand preventing functional deterioration.

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.

Intracranial Vasculopathy in a Patient with Systemic Sclerosis: Atherosclerotic or Moyamoya-Like Disease?

No abstract available.

Escolar com doença de Moyamoya tratada com pialsinangiose / School with Moyamoya disease treated with pialsynangiosis

É apresentado um caso de doença de Moyamoya que ocorreu em escolar do sexo feminino e que foi submetida a tratamento cirúrgico com a técnica de pialsinangiose com sucesso pós-cirúrgico. Enfatiza-se a imprtância da revascularização cerebral com a técnica mencionada para reverter total ou parcialmente os déficits gerados pela doença e para evitar a progressão das isquemias cerebrais.

Síndrome moyamoya en paciente con síndrome de Down y déficit de antitrombina III / Moyamoya disease in a girl with Down syndrome. Report of one case

Moyamoya disease is a unique chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation. It can be primary or secondary to genetic syndromes such as Down syndrome. We report a seven year-old girl with a Down syndrome that presented with a disturbance of consciousness, seizures and a right hemiparesia at the age of five. Magnetic resonance imaging showed old cortical ischemic lesions in both cerebral hemispheres and a recent infarction in the territory of the ¡eft middle cerebral artery. Brain angiography showed a proximal stenosis of both medial cerebral arteries and a net of collateral vessels, consistent with the diagnosis of moyamoya syndrome. The patient had also an antithrombin III deficiency. Aspirin was indicated and a surgical correction was recommended. However, prior to the procedure, the patient had a new infarction in the territory of the right middle cerebral artery, which caused a severe disability.

Selected malformations of the optic nerve head - morphology etiology and associations

Although individually rare, congenital malformations of the optic nerve head can be associated with significant visual morbidity, other ocular malformations, and systemic disorders, some of which life-threatening. This paper reviews the clinical features that allow the differentiation of typical colobomas of the optic nerve head from the morning glory disk anomaly. The former can be inherited and are associated with a variety of systemic syndromes and malformations; the latter are not inherited and are associated notoriously with basal encephaloceles and with Moyamoya disease of the carotid vascular system. Finally, the papillo-renal syndrome and the PHACE syndrome are briefly reviewed and their associated optic nerve malformations delineated

Enfermedad de Moyamoya, a propósito de dos casos / Moyamoya disease, 2 case-report

Moyamoya Disease is a progressive occlusive cerebrovascular disease of both internal carotid arteries and their branches, with compensatory development of a fine collateral vascular network. The etiology of the disease is unknown; however, several studies have suggested involvement of genetic and environmental factors in its pathogenesis. In children, it causes transient ischemic attacks and cerebral infarction, while bleeding is more frequent in adults. In Japan, where the disease is most frequently observed, the development of magnetic resonance with angiography has allowed diagnosis in asymptomatic Moyamoya Disease. Revascularization surgery is believed to reduce the incidence of new ischemic events and improve long term prognosis in these patients. We present 2 children with Moyamoya Disease treated in our Clinic between 1998 and 2007.

La enfermedad de Moyamoya es una enfermedad cerebrovascular oclusiva, progresiva de ambas arterias carótidas internas o sus ramas, compensada por el desarrollo de una fina red vascular colateral. Su etiología es desconocida, aunque distintos estudios han asociado su patogénesis a factores genéticos y ambientales. En Pediatría se manifiesta generalmente como crisis isquémicas transitorias o infartos cerebrales, mientras que en adultos es más frecuente la hemorragia cerebral. En países donde es más frecuente, como Japón, se ha logrado hacer un diagnóstico precoz en pacientes aún asintomáticos, gracias al desarrollo de la Resonancia magnética con angiografía cerebral. La cirugía de revascularización lograría reducir la incidencia de nuevos episodios isquémicos y mejorar el pronóstico a largo plazo de los pacientes que padecen esta patología. Presentamos a continuación dos casos que han llegado a nuestro centro en un período de 9 años.

Moyamoya en la población infantil no asiática. Ampliación de casuística / Moyamoya disease in non Asian population

La enfermedad o síndrome de moyamoya es una rara afección que provoca una oclusión de las carótidas intracraneales y determina una red vascular colateral anormal en la base del cráneo y que, por su aspecto arteriográfico, hace ya más de 50 años un grupo de neurocirujanos japoneses denominaron moyamoya, y de la cual persisten muchas interrogantes sin resolver. Se realizó un estudio descriptivo y longitudinal de 19 niños con síndrome o enfermedad de moyamoya. La edad promedio de inicio fue de seis años. Del total de niños incluidos, doce debutaron en forma aguda con infarto cerebral; dos pacientes (con antecedentes de epilepsia y retraso) lo hicieron, uno con infarto y otro con hemorragia cerebral, otros dos con epilepsia y retraso, uno con trastorno del lenguaje, siendo en los dos restantes un hallazgo casual. Entretanto doce pacientes se consideraron idiopáticos (portadores de enfermedad de moyamoya) y siete asociados a enfermedades sistémicas (síndrome de moyamoya). El diagnóstico inicial se confirmó por angiorresonancia en diez casos y por arteriografía convencional en nueve. El seguimiento promedio fue de cinco años, once evidenciaron progresión clínica y ocho estabilidad, con un seguimiento promedio de siete y dos años respectivamente. Todos mostraron progresión angiográfica. En siete niños se realizaron diez cirugías de revascularización sin complicaciones y con buenos resultados clínicos y angiográficos. Dos niños fallecieron (uno por hemorragia cerebral y otro por un infarto expansivo) y catorce presentaron secuelas leves a moderadas. Resulta importante identificar los niños que se pueden beneficiar con la cirugía de revascularización, una de las pocas opciones terapéuticas para evitar la progresión y complicaciones de esta grave enfermedad.

Moyamoya's disease or syndrome is a rare illness wich causes symptoms of cerebral ischemia due to intracranial arteries' stenosis, with secondary abnormal vasculature networks at the base of the brain. Since the first description of moyamoya disease done by a group of Japanese neurosurgeons more than 50 years ago, this rare illness is still considered an intriguing disease. The clinical features, treatment, imaging findings and outcomes of a series of not Asian children with Moyamoya disease are described in this study. A total of 19 six year old patients were analyzed. The initial presentation in 12 patients was infarctions, 2 with epilepsy and mental retardation, another 2 who had personal history of epilepsy and mental retardation developed symptoms of ischemic stroke (one of them), and hemorrhagic stroke the other one; 1experienced language problems, while in the last two the diagnosis was casual. On the other hand, 12 patientes were considered idiopathic cases (moyamoya's disease) and 7 were related to systemic illnes (moyamoya's syndrome). Initial diagnosis was achieved by magnetic resonance angiography in 10 cases, and conventional angiography in 9. All patients were followed up for a mean period of 5 years. While 11 patients experienced clinical worsening of symptoms, 6 asymptomatic. However, all of them showed angiographic progression. Seven patients underwent bypass surgery with no further complications, 2 passed away and 14 remained with moderate handicaps. A number of surgical procedures have been developed for revascularization, improving the outcome of some of these patients by preventing ischemic and hemorrhagic stroke. Due to the fact that moyamoya disease is not a silent disorder and its progress cause complications, surgical revascularization should always be considered in the management of these patients.

Moyamoya disease associated with positive findings for rheumatoid factor and myeloperoxidase-anti-neutrophil cytoplasmic antibody / La enfermedad de moyamoya asociada con hallazgos positivos en relación con el factor reumatoideo y los anticuerpos citoplasmáticos antineutrófilos mieloperoxidasa

A 39-year old female suddenly fell into a state of unconsciousness. She had no significantpast medical history. A computed tomography scan of the head demonstrated a massive left putaminal haemorrhage with a ventricular perforation, low density areas in the right frontal lobe, corona radiata and occipital lobe. A single emergency burr hole drainage of the haematoma was performed. Bilateral common carotid arteriograms showed stenosis of the right internal carotid artery and a complete obstruction of left internal carotid artery which were both accompanied by moyamoya vessels. The biochemical studies indicated serological positive findings for RF and MPO-ANCA. She was transferred to another hospital for nursing care in a vegetative state on the 163nd hospital day. This case indicates that immunological factors, inflammation or vasculitis might have possibly been associated with the development of either an obstruction or stenosis of the intracranial internal carotid arteries.

Moya Moya cases treated with encephaloduroarteriosynangiosis.

OBJECTIVE: To study neurological outcome of Moya Moya disease treated surgically with Encephaloduroarteriosynengopsis (EDAS). DESIGN: Prospective observational study. SETTINGS: Community and General with tertiary care facility. SUBJECTS: Eight children diagnosed with Moya Moya disease by Magnetic Resonance Angiogramover 4 years of period were selected for EDAS. Children who were not able to sustain surgery excluded from study. METHODS: Treatment modality selected were surgery in form of EDAS. After surgery subjects were followed up for minimum of 2 year period to know neurological out come. Outcome was reported as poor, fair, good and excellent. No statistical analysis performed due to small sample size. RESULTS: After surgery no episode of stroke or TIA was observed in any patient during 2 year follow up period and all patients are living without any new neurological deficit. CONCLUSION: Long term outcome of EDAS is promising.

Moyamoya disease--magnetic resonance imaging and magnetic resonance angiographic features.

A rare case of moyamoya disease in a 9-year-old female presented with behavioural disturbances and recurrent hemiparetic attacks is reported. Moyamoya disease is an uncommon form of arteritis which affects the cerebrovascular circulation. Magnetic resonance angiography is a non-invasive technique for evaluation of the carotid and vertebrobasilar circulation. The magnetic resonance angiography findings are typical and characteristic of this disease.

Postoperative Electroencephalogram for Follow up of Pediatric Moyamoya Disease

It is well known that the electroencephalographic finding in patients with moyamoya disease demonstrates the characteristic "re-build up" phenomenon a few minutes after hyperventilation. To evaluate the usefulness of an electroencephalogram (EEG) in the postoperative management of children with moyamoya disease, we studied the presence or absence of improvement in the clinical, single photon emission computed tomography (SPECT) and EEG findings, before and after surgery. Twenty-two patients, who underwent indirect revascularization surgery for moyamoya disease, were included in our study. Clinical improvement was assessed as the disappearance or decrease of a transient ischemic attack or headache. The findings on the EEG and SPECT were considered improved when the re-build up phenomenon was absent and when there was improvement in the cerebrovascular reserve as a result of the acetazolamide challenge test. The statistical correlation analysis for both clinical and EEG improvement were consistent (kappa value=0.409, p< 0.05). However, the result from the clinical and SPECT improvement as well as that from EEG and SPECT improvement were not statistically significant. Our results suggest that EEG can be used as a noninvasive and simple follow-up test for moyamoya disease after indirect revascularization surgery if the hyperventilation procedure is effectively performed during EEG recording.

Anomalias congênitas do disco óptico associadas à doença de Moyamoya: relato de caso / Congenital anomalies of the optic disc associated with Moyamoya disease: case report

As anomalias congênitas do disco óptico podem estar associadas a anormalidades vasculares intracranianas. Relatamos o caso de um paciente de 9 anos com anomalia do disco óptico tipo morning glory em um olho e coloboma do disco óptico e coróide infrapapilar no outro. O paciente apresentava história pregressa de ataque isquêmico transitório e angioressonância cerebral consistente com doença de moyamoya. O presente relato ilustra a importante associação das anomalias congênitas do disco óptico com a doença de moyamoya. O exame fundoscópico de pacientes suspeitos pode trazer subsídios ao diagnóstico clínico, abrindo a possibilidade do reconhecimento precoce das anormalidades vasculares intracranianas encontradas no moyamoya, aumentando a perspectiva de eventual intervenção profilática frente às potenciais complicações.

Role of magnetic resonance perfusion studies in moyamoya disease.

Moyamoya disease, Japanese for 'puff of smoke', is a rare disease that presents most commonly with recurrent TIAs (transient ischemic attacks) / stroke in childhood. Ischemic symptoms in patients with moyamoya disease are usually due to hemodynamically-mediated perfusion failure. Identification of abnormal tissue perfusion is an important aspect of the evaluation of these patients. We present the radiological features including the Magnetic Resonance (MR) Perfusion findings illustrating the hemodynamic changes of cerebral ischemia in a case of moyamoya disease.