Moyamoya Disease and Moyamoya Syndrome

Scott, R. Michael; Smith, Edward R.

doi:10.1056/nejmra0804622

Cited by 1,514 publications

(1,456 citation statements)

References 80 publications

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“…Known factors associated with moyamoya syndrome include sickle cell disease, NF1, therapeutic cranial irradiation, Down syndrome, congenital cardiac anomaly, renal‐artery stenosis, giant cervicofacial hemangiomas, and hyperthyroidism 3. A study based on 54 published cases of radiation‐induced moyamoya syndrome found that 29 (53.7%) arose following a diagnosis of optic glioma.…”

Section: Discussionmentioning

confidence: 99%

“…Besides stroke and TIA, lacunar lesions, vascular occlusive disease including moyamoya syndrome, vascular malformations including aneurysm, and hemorrhage also can be induced by radiation 2. Moyamoya vasculopathy is characterized by progressive stenosis of the intracranial internal carotid arteries and their proximal branches 3. When presenting with known associated risk factors, such as prior radiotherapy, patients with moyamoya vasculopathy are said to have moyamoya syndrome.…”

Section: Introductionmentioning

confidence: 99%

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Incidence and long‐term outcome of postradiotherapy moyamoya syndrome in pediatric patients with primary brain tumors: a single institute experience in Taiwan

Chang

Liang

et al. 2016

Cancer Medicine

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We aimed to investigate the incidence and long‐term outcome of moyamoya syndrome in pediatric patients with primary brain tumors after receiving cranial radiotherapy (RT) in a single institute in Taiwan. The complete medical records, medical images, and RT notes of 391 pediatric patients with primary brain tumors treated with cranial RT between January 1975 and December 2005 in Taipei Veterans General Hospital (TVGH), Taiwan, were entered into an electronic registry and reviewed. Eight (2%) cases of post‐RT moyamoya syndrome were identified in the sample of 391 patients. The median latency was 3 years post‐RT. Among the eight patients, three had craniopharyngioma, two had optic glioma, two had medulloblastoma, and one had a suprasellar astrocytoma. The prescribed physical doses of RT were in the range of 40–54 Gy. The incidence was highest in those with optic glioma (0.039/person‐year), followed by craniopharyngioma (0.013/person‐year), astrocytoma (0.003/person‐year), and medulloblastoma (0.002/person‐year). No patients died of vasculopathy. No difference in crude incidence was found between our results and those of other series. The incidence of moyamoya syndrome was diagnosis dependent, with the highest incidence among patients with optic glioma. No regional difference in incidence was found. Long‐term, stable neurological function may be achieved following timely surgical intervention.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Incidence and long‐term outcome of postradiotherapy moyamoya syndrome in pediatric patients with primary brain tumors: a single institute experience in Taiwan

Chang

Liang

et al. 2016

Cancer Medicine

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“…It is reported that the ratio of quasi-MMD is higher in western country [11]. Regarding with clinical manifestation of MMD, it is well known that the pediatric patients present with ischemic attack and adult patients tend to suffer from intracranial hemorrhage [12,13]. brain tumor/irradiation in 7 (7.5%), autoimmune disease in 7 (7.5%), hyperthyroidism in 7 (7.5%) [16].…”

Section: Discussionmentioning

confidence: 99%

A Case of Neurofibromatosis Type 1 Complicated with Repeated Intracerebral Hemorrhage due to Quasi-Moyamoya Disease

Hayashi

Morofuji

Horie

et al. 2015

Journal of Stroke and Cerebrovascular Diseases

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“…It includes disorders such as Down syndrome, polycystic kidney disease, neurofibromatosis, fibromuscular dysplasia, and meningitis among others. When these conditions are present, moyamoya is then named moyamoya syndrome or "quasi-moyamoya" [1][2][3]. Most affected patients present with ischemic symptoms.…”

Section: Introductionmentioning

confidence: 99%

“…Most affected patients present with ischemic symptoms. However, in a variable proportion of cases, more often in adults, cerebral hemorrhage can occur [1][2][3][4]. Headache, epilepsy, dyskinesia, and cognitive disturbances are other manifestations of moyamoya [1][2][3].…”

Section: Introductionmentioning

confidence: 99%

Caucasian Familial Moyamoya Syndrome With Rare Multisystemic Malformations

Nzwalo

Santos

Gradil

et al. 2013

Pediatric Neurology

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Moyamoya disease is an idiopathic progressive steno-occlusive disorder of the intracranial arteries located at the base of the brain. It is associated with the development of compensatory extensive network of fine collaterals. Moyamoya disease is considered syndromic when certain genetic or acquired disorders such as polycystic kidney disease, neurofibromatosis, or meningitis are also present. Although the genetic contribution in moyamoya is indisputable, its cause and pathogenesis remain under discussion. Herein, we report a rare occurrence of moyamoya syndrome in two European Caucasian siblings in association with unusual multisystemic malformations (polycystic kidney disease in one, and intestinal duplication cyst in the other). The karyotype was normal. No mutation in the RFN213 gene was found, and none of the HLA types linked to moyamoya disease or described in similar familial cases were identified. By describing these multisystemic associations, polycystic kidney disease for the second time, and intestinal malformation for the first time in the literature, our report expands the phenotypic variability of moyamoya syndrome. The coexistence of disparate malformations among close relatives suggests an underlying common genetic background predisposing to structural or physiological abnormalities in different tissues and organs.

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Moyamoya Disease and Moyamoya Syndrome

Cited by 1,514 publications

References 80 publications

Incidence and long‐term outcome of postradiotherapy moyamoya syndrome in pediatric patients with primary brain tumors: a single institute experience in Taiwan

Incidence and long‐term outcome of postradiotherapy moyamoya syndrome in pediatric patients with primary brain tumors: a single institute experience in Taiwan

A Case of Neurofibromatosis Type 1 Complicated with Repeated Intracerebral Hemorrhage due to Quasi-Moyamoya Disease

Caucasian Familial Moyamoya Syndrome With Rare Multisystemic Malformations

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