scholarly journals Transcriptomic Profile of Distal Middle Cerebral Artery from Moyamoya Disease Patients Reveals a Potential Unique Pathway

2020 ◽  
Vol 8 (A) ◽  
pp. 956-961
Author(s):  
Yulius Hermanto ◽  
Kent Doi ◽  
Ahmad Faried ◽  
Achmad Adam ◽  
Tondi M. Tjili ◽  
...  
Keyword(s):  
Gene Expression ◽  
Middle Cerebral Artery ◽  
Moyamoya Disease ◽  
Cerebral Artery ◽  
Superficial Temporal Artery ◽  
Rna Extraction ◽  
Temporal Artery ◽  
The Novel ◽  
Ras Pathway ◽  
Gene Probes

BACKGROUND: Moyamoya disease (MMD) is a peculiar disease, characterized by progressive steno-occlusion of the distal ends of bilateral internal carotid arteries and their proximal branches. Numerous studies of MMD investigated as a singular pathway, thus overlooked the complexity of MMD pathobiology. AIM: In this study, we sought to investigate the gene expression in the involved arteries to reveal the novel mechanism of MMD. MATERIALS AND METHODS: Eight middle cerebral artery (MCA) specimens were obtained from six patients underwent surgical procedure superficial temporal artery to MCA (STA-MCA bypass) for MMD and two control patients. We performed RNA extraction and microarray analysis with Agilent Whole Human Genome DNA microarray 4x44K ver.2.0 (Agilent Tech., Inc., Wilmington, DE, USA). RESULTS: From 42,405 gene probes assayed, 921 gene probes were differentially regulated in MCA of patients with MMD. Subsequent pathway analysis with PANTHER database revealed that angiogenesis, inflammation, integrin, platelet-derived growth factor (PDGF), and WNT pathways were distinctly regulated in MMD. Among genes in aforementioned pathways, SOS1 and AKT2 were the mostly distinctly regulated genes and closely associated with RAS pathway. CONCLUSION: The gene expression in MCA of patients with MMD was distinctly regulated in comparison with control MCA; presumably be useful for elucidating MMD pathobiology.

Prolonged/delayed cerebral hyperperfusion in adult patients with moyamoya disease with RNF213 gene polymorphism c.14576G>A (rs112735431) after superficial temporal artery–middle cerebral artery anastomosis

2020 ◽  
pp. 1-8
Author(s):  
Ryosuke Tashiro ◽  
Miki Fujimura ◽  
Masahito Katsuki ◽  
Taketo Nishizawa ◽  
Yasutake Tomata ◽  
...  
Keyword(s):  
Genetic Analysis ◽  
Gene Polymorphism ◽  
Intracerebral Hemorrhage ◽  
Middle Cerebral Artery ◽  
Moyamoya Disease ◽  
Cerebral Artery ◽  
Superficial Temporal Artery ◽  
Temporal Artery ◽  
Adult Patients ◽  
Cerebral Hyperperfusion

OBJECTIVESuperficial temporal artery–middle cerebral artery (STA-MCA) anastomosis is the standard surgical management for moyamoya disease (MMD), whereas cerebral hyperperfusion (CHP) is one of the potential complications of this procedure that can result in delayed intracerebral hemorrhage and/or neurological deterioration. Recent advances in perioperative management in the early postoperative period have significantly reduced the risk of CHP syndrome, but delayed intracerebral hemorrhage and prolonged/delayed CHP are still major clinical issues. The clinical implication of RNF213 gene polymorphism c.14576G>A (rs112735431), a susceptibility variant for MMD, includes early disease onset and a more severe form of MMD, but its significance in perioperative pathology is unknown. Thus, the authors investigated the role of RNF213 polymorphism in perioperative hemodynamics after STA-MCA anastomosis for MMD.METHODSAmong 96 consecutive adult patients with MMD comprising 105 hemispheres who underwent serial quantitative cerebral blood flow (CBF) analysis by N-isopropyl-p-[123I]iodoamphetamine SPECT after STA-MCA anastomosis, 66 patients consented to genetic analysis of RNF213. Patients were routinely maintained under strict blood pressure control during and after surgery. The local CBF values were quantified at the vascular territory supplied by the bypass on postoperative days (PODs) 1 and 7. The authors defined the radiological CHP phenomenon as a local CBF increase of more than 150% compared with the preoperative values, and then they investigated the correlation between RNF213 polymorphism and the development of CHP.RESULTSCHP at POD 1 was observed in 23 hemispheres (23/73 hemispheres [31.5%]), and its incidence was not statistically different between groups (15/41 [36.6%] in RNF213-mutant group vs 8/32 [25.0%] in RNF213–wild type (WT) group; p = 0.321). CHP on POD 7, which is a relatively late period of the CHP phenomenon in MMD, was evident in 9 patients (9/73 hemispheres [12.3%]) after STA-MCA anastomosis. This prolonged/delayed CHP was exclusively observed in the RNF213-mutant group (9/41 [22.0%] in the RNF213-mutant group vs 0/32 [0.0%] in the RNF213-WT group; p = 0.004). Multivariate analysis revealed that RNF213 polymorphism was significantly associated with CBF increase on POD 7 (OR 5.47, 95% CI 1.06–28.35; p = 0.043).CONCLUSIONSProlonged/delayed CHP after revascularization surgery was exclusively found in the RNF213-mutant group. Although the exact mechanism underlying the contribution of RNF213 polymorphism to the prolonged/delayed CBF increase in patients with MMD is unclear, the current study suggests that genetic analysis of RNF213 is useful for predicting the perioperative pathology of patients with MMD.

scholarly journals Paradoxical association of symptomatic cerebral edema with local hypoperfusion caused by the ‘watershed shift’ after revascularization surgery for adult moyamoya disease: a case report

2019 ◽  
Vol 12 ◽  
pp. 175628641987834 ◽  
Author(s):  
Jin Yu ◽  
Miao Hu ◽  
Lei Yi ◽  
Keyao Zhou ◽  
Jianjian Zhang ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Middle Cerebral Artery ◽  
Moyamoya Disease ◽  
Cerebral Artery ◽  
Cerebral Edema ◽  
Superficial Temporal Artery ◽  
Temporal Artery ◽  
Neurological Dysfunction ◽  
Fluid Replacement ◽  
Emission Computed Tomography

Superficial temporal artery–middle cerebral artery anastomosis is generally considered as an effective method in improving damage associated with intracerebral occlusions in moyamoya disease. Hemodynamic changes caused by revascularization are the cause of many postoperative complications. Of the 186 consecutive surgeries for moyamoya disease at our hospital from 2015, we herein presented one case of adult-onset moyamoya disease that manifested symptomatic local cerebral edema and local hypoperfusion caused by the ‘watershed shift’. A 67-year-old woman presented with limb numbness on the right side and underwent superficial temporal artery–middle cerebral artery anastomosis, resulting in neurological dysfunction and the formation of a reversible high-signal lesion at left frontotemporal lobes on T2-weighted images along with a decrease in perfusion values on 123I N-isopropyl-p-iodoamphetamine single-photon emission computed tomography, while the anastomotic vessel was patent on magnetic resonance angiography. This phenomenon of hypoperfusion area (left frontotemporal lobe) remote to anastomotic site (left temporal lobe area) led to the diagnosis of the ‘watershed shift’ phenomenon. In light of the hypoperfusion induced by ‘watershed shift’, the patient was treated with fluid replacement. With the gradual recovery of perfusion, the patient presented significantly improvement both on the magnetic resonance imaging findings and neurological symptoms. In conclusion, regional cerebral edema with hypoperfusion, possibly due to cerebral ischemia and the ‘watershed shift’ phenomenon, may be another novel entity that needs to be considered as a potential complication after extracranial–intracranial bypass for moyamoya disease.

scholarly journals Superficial temporal artery to middle cerebral artery bypass: past, present, and future

2008 ◽  
Vol 24 (2) ◽  
pp. E2 ◽  
Author(s):  
Marcelo D. Vilela ◽  
David W. Newell
Keyword(s):  
Middle Cerebral Artery ◽  
Skull Base ◽  
Cerebral Vasospasm ◽  
Moyamoya Disease ◽  
Cerebral Artery ◽  
Superficial Temporal Artery ◽  
Temporal Artery ◽  
Skull Base Tumors ◽  
Intracranial Atherosclerotic Disease

Object The aim of this study was to review the historical developments and current status of superficial temporal artery (STA) to middle cerebral artery (MCA) bypass. Method A literature review was performed to review the origins and current uses of the STA bypass procedure in neurosurgery. Results The idea of providing additional blood supply to the brain to prevent stroke and maintain neurological function has been present in the mind of neurosurgeons for many decades. In 1967 the first STA–MCA bypass was done by M. G. Yaşargil, and an enormous step was made into the field of microneurosurgery and cerebral revascularization. During the decades that followed, this technique was used as an adjuvant or a definitive surgical treatment for occlusive disease of the extracranial and intracranial cerebral vessels, skull base tumors, aneurysms, carotid–cavernous fistulas, cerebral vasospasm, acute cerebral ischemia, and moyamoya disease. With the results of the first randomized extracranial–intracranial (EC–IC) bypass trial and the development of endovascular techniques such as angioplasty for intracranial atherosclerotic disease and cerebral vasospasm, the indications for STA–MCA bypass became limited. Neurosurgeons continued to perform EC–IC bypasses as an adjuvant to clipping of aneurysms and in the treatment of skull base tumors and moyamoya disease; the procedure is less commonly used for atherosclerotic carotid artery occlusion (CAO) with definite evidence of hemodynamic insufficiency. The evidence that patients with symptomatic CAO and “misery perfusion” have an increased stroke risk has prompted a second trial for evaluating EC–IC bypass for stroke prevention. The Carotid Occlusion Surgery Study is a new trial designed to determine whether STA–MCA bypass can reduce the incidence of stroke in these patients. New trials will also reveal the role of the STA–MCA bypass in the prevention of hemorrhages in moyamoya disease. Conclusions The role of STA–MCA bypass in the management of cerebrovascular disease continues to be refined and evaluated using advanced imaging techniques and by performing randomized trials for specific purposes, including symptomatic CAO.

Patients with moyamoya disease presenting with movement disorder

2010 ◽  
Vol 6 (6) ◽  
pp. 559-566 ◽  
Author(s):  
Paritosh Pandey ◽  
Teresa Bell-Stephens ◽  
Gary K. Steinberg
Keyword(s):  
Middle Cerebral Artery ◽  
Movement Disorder ◽  
Moyamoya Disease ◽  
Cerebral Artery ◽  
Movement Disorders ◽  
Artery Bypass ◽  
Superficial Temporal Artery ◽  
Temporal Artery ◽  
Subcortical Region ◽  
Clinical Records

Moyamoya disease is a rare cerebrovascular disease characterized by idiopathic bilateral stenosis or occlusion of bilateral internal carotid arteries and the development of characteristic leptomeningeal collateral vessels at the base of the brain. Typical presentations include transient ischemic attacks or stroke, and hemorrhage. Presentation with movement disorders is extremely rare, especially in the pediatric population. The authors describe the cases of 4 children with moyamoya disease who presented with movement disorders. Among 446 patients (118 pediatric) with moyamoya disease surgically treated by the senior author, 4 pediatric patients had presented with movement disorders. The clinical records, imaging studies, surgical details, and postoperative clinical and imaging data were retrospectively reviewed. The initial presenting symptom was movement disorder in all 4 patients: chorea in 2, hemiballismus in 1, and involuntary limb shaking in 1. All the patients had watershed infarcts involving the frontal subcortical region on MR imaging. Additionally, 1 patient had a ganglionic infarct. Single-photon emission computed tomography studies showed frontoparietal cortical and subcortical hypoperfusion in all patients. Three patients had bilateral disease, whereas 1 had unilateral disease. All the patients underwent superficial temporal artery–middle cerebral artery bypass. Postoperatively, all 4 patients had complete improvement in their symptoms. The SPECT scans revealed normal perfusion in 3 patients and a small residual perfusion deficit in 1. Movement disorders are a rare presenting feature of moyamoya disease. Hypoperfusion of the frontal cortical and subcortical region was seen in all patients, and the symptomatology was attributed to ischemic dysfunction and imbalance in the cortical-subcortical-ganglionic-thalamic-cortical circuitry. Combined revascularization with superficial temporal artery–middle cerebral artery bypass and encephaloduroarteriosynangiosis leads to excellent results.

Focal hyperperfusion after superficial temporal artery—middle cerebral artery anastomosis in a patient with moyamoya disease

2004 ◽  
Vol 100 (1) ◽  
pp. 128-132 ◽  
Author(s):  
Kazuhide Furuya ◽  
Nobutaka Kawahara ◽  
Akio Morita ◽  
Toshimitsu Momose ◽  
Shigeki Aoki ◽  
...  
Keyword(s):  
Middle Cerebral Artery ◽  
Moyamoya Disease ◽  
Cerebral Artery ◽  
Single Photon ◽  
Superficial Temporal Artery ◽  
Photon Emission ◽  
Temporal Artery ◽  
Neurological Deterioration ◽  
Perfusion Reserve ◽  
Frontal Operculum

✓ Superficial temporal artery—middle cerebral artery (STA—MCA) anastomosis is a standard surgical therapeutic option in patients with moyamoya disease. Most patients experience improvement in their clinical symptoms immediately after surgery. The authors report on the case of a 39-year-old man with moyamoya disease who suffered from temporary and frequent neurological deterioration after undergoing a left STA—MCA anastomosis. Hemodilution and hypervolemia therapies did not improve his course. Technetium-99m hexamethylpropyleneamine oxime single-photon emission tomography scans demonstrated focal intense accumulation of the tracer in the frontal operculum on the side of the surgery. Although diffusion-weighted magnetic resonance (MR) imaging demonstrated no abnormalities except for the postoperative change, perfusion-weighted MR images and MR digital subtraction angiography revealed focal hyperperfusion in the left frontal operculum where the cerebral perfusion reserve was severely disturbed preoperatively. This evidence strongly supports the notion that focal hyperperfusion observed after STA—MCA anastomosis could occur in the poor perfusion reserve area preoperatively and could cause temporary neurological deterioration.

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