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Complications following Encephalo-Duro-Arterio-Myo-Synangiosis in a case of Moyamoya disease.

ABSTRACT: We report a case of an 18-year-old girl diagnosed to have Moyamoya disease (MMD), who underwent bilateral encephalo-duro-arterio-myo-synangiosis. Literature search has clearly inferred that in comparison to an adult patient, children with MMD can have a good prognosis if early diagnosis and active surgical intervention are achieved. Evidence has demonstrated that active surgical management, including indirect bypass surgery, may improve the cerebral circulation on a relatively larger scale than direct bypass surgeries, which can only improve the cerebral circulation in the area of the vascular reconstruction.

SUBMITTER: Hazra DN

PROVIDER: S-EPMC7045537 | biostudies-literature | 2020 Jan-Mar

REPOSITORIES: biostudies-literature

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Complications following Encephalo-Duro-Arterio-Myo-Synangiosis in a case of Moyamoya disease.

Hazra Darpa Narayan DN Ghosh Amit Kumar AK

Brain circulation 20200101 1

We report a case of an 18-year-old girl diagnosed to have Moyamoya disease (MMD), who underwent bilateral encephalo-duro-arterio-myo-synangiosis. Literature search has clearly inferred that in comparison to an adult patient, children with MMD can have a good prognosis if early diagnosis and active surgical intervention are achieved. Evidence has demonstrated that active surgical management, including indirect bypass surgery, may improve the cerebral circulation on a relatively larger scale than ...[more]

PMID: 32166202

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Project description:BackgroundMoyamoya disease is a rare chronic steno-occlusive cerebrovascular disease. It may have variable clinical symptoms associated with cerebral stroke, including motor paralysis, sensory disturbances, seizures, or headaches. However, patients with moyamoya disease rarely present with involuntary movement disorders, including limb-shaking syndrome, with no previous reports of limb-shaking syndrome occurring after revascularization procedures for this disease. Although watershed shifts can elicit transient neurological deterioration after revascularisation, symptoms originating from the contralateral hemisphere following the revascularization procedure are rare. Here, we report the case of moyamoya disease wherein the patient developed limb-shaking syndrome derived from the contralateral hemisphere after unilateral revascularisation.Case descriptionA 16-year-old girl presented with transient left upper and lower limb numbness and headache. Based on digital subtraction angiography, she was diagnosed with symptomatic moyamoya disease. Single-photon emission computed tomography (SPECT) showed decreased cerebral blood flow (CBF) on the right side, and she underwent direct and indirect bypasses on this side. Involuntary movements appeared in her right upper limb immediately postoperatively. SPECT showed decreased CBF to the bilateral frontal lobes. Subsequently, the patient was diagnosed with limb-shaking syndrome. After performing left-hemispheric revascularisation, the patient's symptoms resolved, and SPECT imaging confirmed improvements in CBF to the bilateral frontal lobes.ConclusionRevascularization for moyamoya disease can lead to watershed shifts, which can induce limb-shaking syndrome derived from abnormalities in the contralateral hemisphere of the revascularized side. For patients with new-onset limb-shaking syndrome after moyamoya revascularisation procedures, additional revascularization may be warranted for treatment of low perfusion areas.

Project description:Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by progressive spontaneous bilateral occlusion of the intracranial internal cerebral arteries (ICA) and their major branches with compensatory capillary collaterals resembling a "puff of smoke" (Japanese: Moyamoya) on cerebral angiography. These pathological alterations of the vessels are called Moyamoya arteriopathy or vasculopathy and a further distinction is made between primary and secondary MMD. Clinical presentation depends on age and population, with hemorrhage and ischemic infarcts in particular leading to severe neurological dysfunction or even death. Although the diagnostic suspicion can be posed by MRA or CTA, cerebral angiography is mandatory for diagnostic confirmation. Since no therapy to limit the stenotic lesions or the development of a collateral network is available, the only treatment established so far is surgical revascularization. The pathophysiology still remains unknown. Due to the early age of onset, familial cases and the variable incidence rate between different ethnic groups, the focus was put on genetic aspects early on. Several genetic risk loci as well as individual risk genes have been reported; however, few of them could be replicated in independent series. Linkage studies revealed linkage to the 17q25 locus. Multiple studies on the association of SNPs and MMD have been conducted, mainly focussing on the endothelium, smooth muscle cells, cytokines and growth factors. A variant of the RNF213 gene was shown to be strongly associated with MMD with a founder effect in the East Asian population. Although it is unknown how mutations in the RNF213 gene, encoding for a ubiquitously expressed 591 kDa cytosolic protein, lead to clinical features of MMD, RNF213 has been confirmed as a susceptibility gene in several studies with a gene dosage-dependent clinical phenotype, allowing preventive screening and possibly the development of new therapeutic approaches. This review focuses on the genetic basis of primary MMD only.

Dataset Information

Complications following Encephalo-Duro-Arterio-Myo-Synangiosis in a case of Moyamoya disease.

Publications

Complications following Encephalo-Duro-Arterio-Myo-Synangiosis in a case of Moyamoya disease.

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