Moyamoya Syndrome Manifesting with Choreiform Movements

Filho, Pedro Tadao Hamamoto; Lira, Carlos Cézar Sousa de; Zanini, Marco Antônio

doi:10.1055/s-0037-1607967

Cited by 5 publications

(2 citation statements)

References 2 publications

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“…Enlarging collateral vessels, in particular lenticulostriate vessels, that pierce and perfuse the basal ganglia, have been associated with the development of complex movement disorders. Hemiballismus-type movements with subthalamic nucleus involvement have been documented [11]. It is unclear if the development of these complex movement disorders is due to enlarging vessels disrupting connections between fibers and nuclei or if it is caused by micro-ischemic changes from diminishing perfusion due to progressive stenosis.…”

Section: Review Pathologymentioning

confidence: 99%

Moyamoya: An Update and Review

Berry¹,

Cortez²,

Toor³

et al. 2020

Cureus

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This article is a clinical review of Moyamoya disease (MMD) and Moyamoya syndrome (MMS). We review the incidence, epidemiology, pathology, historical context, clinical and radiographic findings, diagnostic imaging modalities, radiographic grading systems, the effectiveness of medical, interventional, and surgical treatment, and some of the nuances of surgical treatment options. This article will help pediatricians, neurologists, neurosurgeons, and other clinical practitioners who are involved in caring for patients with this rare clinical entity. MMD is an intrinsic primary disease process that causes bilateral progressive stenosis of the anterior intracranial circulation with the involvement of the proximal portions of the intracranial internal carotid artery (ICA) extending to involve the proximal portions of the anterior cerebral artery (ACA) and middle cerebral artery (MCA); posterior circulation involvement is very rare. This causes a compensatory response where large numbers of smaller vessels such as the lenticulostriate arteries begin to enlarge and proliferate, which gives the angiographic appearance of a "Puff of Smoke", which is translated into Japanese as "Moyamoya”. MMS is a secondary process that occurs in response to another underlying pathological process that causes stenosis of intracranial blood vessels, such as radiation. For example, an external source of radiation causes stenosis of the ICA with a compensatory response of smaller blood vessels, which then enlarge and proliferate in response and has the same "Puff of Smoke" appearance on the diagnostic cerebral angiogram (DCA). Histological findings include an irregular internal elastic lamina with luminal narrowing, hyperplasia of the tunica media, and intimal thickening with vacuolar degeneration in smooth muscle cells in the tunica media. Compensation for diminishing blood supply occurs through angiogenesis, which causes the proliferation and enlargement of smaller collateral blood vessels to increase blood supply to under-perfused areas of the brain. MMD is rare in the United States, with just 0.086 newly diagnosed cases per 100,000 individuals per year, which is approximately one per million new cases annually. Risk factors for MMD include Eastern Asian ancestry and predisposing conditions such as neurofibromatosis and Down's syndrome. Clinically, patients often present with stroke signs and symptoms from cerebral ischemia. The proliferation of collateral blood vessels within the basal ganglia can produce movement disorders. Catheter-based DCA is the current gold standard for obtaining a diagnosis. CT perfusion allows preoperative identification of ischemic vascular territories, which may be amenable to surgical intervention. MRI enables rapid detection of acute ischemic stroke using diffusion-weighted Imaging (DWI) and apparent diffusion coefficient (ADC) sequences to assess for any diffusion restriction. Non-contrast CT of the head is used to rule out acute hemorrhage in the presentation...

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Section: Review Pathologymentioning

confidence: 99%

Moyamoya: An Update and Review

Berry¹,

Cortez²,

Toor³

et al. 2020

Cureus

View full text Add to dashboard Cite

show abstract

“…In individual moyamoya patients, neurological symptoms secondary to moyamoya included laryngomalacia [ 118 ], hypotension encephalopathy [ 117 ], behavioral changes [ 119 ], oral and written language deficits [ 119 , 128 ], transient psychosis [ 121 , 129 , 173 ], hemiplegia [ 126 , 131 ], hearing loss [ 132 ], and cognitive difficulties [ 120 ]. Movement disorders as symptoms of moyamoya included chorea [ 122 , 123 ], dystonia [ 124 , 125 ], hemifacial spasms [ 130 ], and hemiballismus [ 127 ]. Moreover, Kraemer et al analyzed the incidence of movement disorders among moyamoya patients in Europe.…”

Section: Reviewmentioning

confidence: 99%

Western Moyamoya Phenotype: A Scoping Review

Miller¹,

Unda²,

Holland³

et al. 2021

Cureus

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Moyamoya, a rare angiographic finding, is characterized by chronic and progressive stenosis at the terminal end of the internal carotid artery, followed by collateralization of the cerebral vasculature at the base of the skull. Coined by Suzuki and Takaku in 1969, the term “moyamoya” means a “puff of smoke” in Japanese, a reference to the angiographic appearance of moyamoya collateralization. Moyamoya is most commonly found in East Asian countries, where much governmental and civilian effort has been expended to characterize this unique disease process. However, despite its rarity, the occurrence of moyamoya in Western countries is associated with significant divergence regarding incidence, gender, sex, age at diagnosis, clinical presentation, and outcomes. Here, we attempted to review the Western literature on moyamoya presentation using the PubMed database to characterize the Western phenotype of moyamoya. We were guided by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for scoping reviews (PRISMA-ScR). We reviewed papers generated from a search with keywords “moyamoya case report,” those reported from a Western institution, and those reported on a relevant association. Our scoping review demonstrated various clinical associations with moyamoya. Moreover, we summarized the demographic profile and clinical symptomatology, as well as reported disease associations to better elucidate the Western phenotype of moyamoya.

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