Childhood Moyamoya Disease and Moyamoya Syndrome: A Pictorial Review

Currie, Stuart; Raghavan, Ashok; Batty, Ruth; Connolly, Daniel; Griffiths, Paul D.

doi:10.1016/j.pediatrneurol.2011.02.007

Cited by 53 publications

(49 citation statements)

References 108 publications

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“…Cerebral angiography is the preferred method of diagnosis [1], but high-resolution MR angiography can suffice [2,3]. Both patients fulfilled the criteria for moyamoya attested by the presence of bilateral steno-occlusive changes in the terminal portion of the internal carotid artery, in the proximal portions of the anterior and middle cerebral arteries, and by the presence of abnormal vascular networks in the vicinity of the stenoocclusive vessels [1][2][3]. The disease has had a benign evolution in both patients, after neurosurgical intervention in the younger sister and after conservative treatment in the older.…”

Section: Discussionmentioning

confidence: 99%

“…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%

“…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]. The familial incidence of affected first-degree relatives is approximately 10% to 12% in Japan and 2% to 6% in the United States [4].…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Caucasian Familial Moyamoya Syndrome With Rare Multisystemic Malformations

Nzwalo

Santos

Gradil

et al. 2013

Pediatric Neurology

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“…In this way, the distal supraclinoid ICA sponse to the increasing hypoperfusion [8][9][10][11] . However, other descriptions refer to new anastomotic vessels 12 and true neoangiogenesis around the circle of Willis [13][14] .…”

Section: Introductionmentioning

confidence: 99%

Selective and Superselective Angiography of Pediatric Moyamoya Disease Angioarchitecture: The Anterior Circulation

et al. 2014

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The angioarchitecture of the so-called moyamoya vessels in children has not been explicitly analyzed. We aimed to investigate the precise anatomy of the vascular anastomotic networks in patients with childhood moyamoya disease. Six children diagnosed with moyamoya disease for the first time underwent an angiographic investigation with selective and superselective injections. We recorded the arterial branches feeding the moyamoya anastomotic networks, their connections and the recipient vessels. Depending on the level of the steno-occlusive lesion, the feeding vessels included the medial striate arteries, the perforators of the choroidal segment of the carotid, the uncal artery, the medial and lateral branches of the intraventricular segment of the anterior choroidal artery, perforators of the communicating segment, the superior hypophyseal arteries, the prechiasmal branches of the ophthalmic artery, the ethmoidal arteries and the dural branches of the cavernous carotid. Through connections, which are described, the recipient vessels were the lateral striate arteries and the middle cerebral, the medial striate arteries and the anterior cerebral, medullary arteries around the ventricular system, anterior temporal branches of the middle cerebral, orbitofrontal and frontopolar branches of the anterior cerebral, as well as other cortical branches of the anterior and middle cerebral territories. The use of high quality selective and superselective angiography enabled us to clearly demonstrate for the first time aspects of the microangiographic anatomy of the moyamoya anastomotic network previously only vaguely or incompletely described.

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A 15-Year-Old Boy With Trisomy 21 and Postoperative Weakness

Schwarz

Amlie‐Lefond

2016

JAMA Pediatr

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Childhood Moyamoya Disease and Moyamoya Syndrome: A Pictorial Review

Cited by 53 publications

References 108 publications

Caucasian Familial Moyamoya Syndrome With Rare Multisystemic Malformations

Caucasian Familial Moyamoya Syndrome With Rare Multisystemic Malformations

Selective and Superselective Angiography of Pediatric Moyamoya Disease Angioarchitecture: The Anterior Circulation

A 15-Year-Old Boy With Trisomy 21 and Postoperative Weakness

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