Moyamoya Vasculopathy Research Articles

Reader response: Cerebello-spinal tDCS in ataxia: A randomized, double-blind, sham-controlled, crossover trial.

In “Severe hyperhomocysteinemia manifesting as moyamoya vasculopathy and Henoch-Schonlein purpura,” Cho et al. described a 37-year-old woman with a history of Henoch-Schonlein purpura with leukocytoclastic vasculitis and immune mesangiopathic glomerulonephritis who presented with recurrent ischemic infarcts and was found to have hyperhomocysteinemia due to homozygous cystathionine β-synthase (CBS), then developed moyamoya vasculopathy requiring a direct bypass on the left and an encephalo-duro-arterio-synangiosis procedure on the right. The authors suggested that the cerebral, skin, and renal changes are all the result of CBS deficiency. In review of this case, Drs. Gaillard and Arquizan postulate that the underlying mechanism was likely a systemic vasculitis with CNS involvement and question whether (1) inflammatory markers, autoantibodies, and serologies were checked in serum or CSF, (2) a brain biopsy was obtained, or (3) a trial of steroids or immunosuppressants was given. Cho et al. respond that (1) serial cerebral angiograms showed progressive intracranial stenosis consistent with moyamoya vasculopathy, not with vasculitis, and (2) CSF showed no pleocytosis, and inflammatory and infectious tests were negative. They note that there was no recurrence of skin or renal events despite the absence of systemic therapy, making the diagnosis of secondary CNS vasculitis unlikely. While both Cho et al. and Drs. Gaillard and Arquizan agree that the high-resolution MRI was suggestive of vasculitis, Cho et al. believe it is consistent with a moyamoya-like vasculopathy and that this case represents a new phenotype of CBS deficiency.

Preeclampsia and Cerebrovascular Disease.

Preeclampsia and Cerebrovascular Disease.

Rare and Low-Frequency Variants in RNF213 Confer Susceptibility to Moyamoya Syndrome Associated with Hyperthyroidism

Moyamoya syndrome (MMS), distinguished from definite moyamoya disease (MMD), is characterized by moyamoya vasculopathy thought to develop secondary to underlying conditions (e.g., hyperthyroidism). Recent studies have shown that a proportion of East Asian (EAS) patients with MMS possess the p.R4810K variant of RNF213 (rs112735431), the foremost susceptibility variant among EAS patients with MMD. We evaluated the association between hyperthyroidism-associated MMS (hMMS) and sequence variants in RNF213. We performed next-generation sequencing of RNF213 in 15 patients with hMMS. Candidate coding variants for the association analysis were defined by allelic frequencies of <1%, based on the p.R4810K frequency in the Japanese population. The association with hMMS was tested using a collapsing method, and 260 unrelated EAS women from the 1000 Genomes Project served as population-based controls. All patients were female, reflecting female predominance in both moyamoya and hyperthyroid conditions. Five candidate missense variants in RNF213 were identified in 8 of 15 patients (53.3%): p.C118R, p.R4062Q, and p.R4810K as heterozygous; and p.A3468V and p.S3986N as compound heterozygous with p.R4810K. Among 260 EAS female controls, 36 (13.8%) had putatively functional variants. All identified variants were missense variants and were significantly overrepresented among patients compared with EAS controls (permuted P= 0.00010; odds ratio= 7.03; 95% confidence interval, 2.09-24.3). Rare and low-frequency missense variants in RNF213 confer susceptibility to both MMD and hMMS. This finding indicates that susceptibility variants in RNF213 may require additional clinical factors with an effect equivalent to hyperthyroidism in order to develop moyamoya vasculopathy.

Progressive stenosis and radiological findings of vasculitis over the entire internal carotid artery in moyamoya vasculopathy associated with graves\u2019 disease: a case report and review of the literature

BackgroundMoyamoya vasculopathy (MMV) associated with Graves’ disease (GD) is a rare condition resulting in ischemic stroke accompanied by thyrotoxicity. Radiological findings of vasculitis have been reported in the walls of distal internal carotid arteries (ICAs) in these patients; however, no reports have described in detail the processes of progression of the lesions in the proximal ICA. Moreover, treatments to prevent recurrence of ischemic stroke and progression of MMV have not yet been sufficiently elucidated.Case presentationWe report a progressive case of MMV associated with GD and review the literature to clarify relationships among recurrence, progression, thyrotoxicity and treatment. Our patient developed cerebral infarction during thyrotoxicity with no obvious stenosis of ICAs. Five months later, transient ischemic attacks recurred with thyrotoxicity. Antiplatelet therapy and intravenous methylprednisolone stopped the attacks. Stenosis of the left ICA from the proximal to distal portion and champagne bottle neck sign (CBN) were found. She declined any surgery. Afterward, gradual progression with mild thyrotoxicity was observed. Eventually, we found smooth, circumferential, concentric wall thickening with diffuse gadolinium enhancement of the left ICA from the proximal to the distal portion on T1-weighted imaging, suggesting vasculitis radiologically. The clinical and radiological similarities to Takayasu arteritis encouraged us to provide treatment as for vasculitis of medium-to-large vessels. In a euthyroid state and after administration of prednisolone and methotrexate, improved flow in the cerebrovascular arteries on magnetic resonance angiography was observed. Based on our review of the literature, all cases with recurrence or progression were treated with anti-thyroid medication (ATM) alone and accompanied by thyrotoxicity. CBN was observed in all previous cases for which images of the proximal ICA were available.ConclusionsWe report the details of progressive stenosis from a very early stage and radiological findings of vasculitis over the entire ICA in MMV associated with GD. Cerebral infarction can occur with no obvious stenosis of the ICA. We treated the patient as per vasculitis of a medium-to-large vessel. Management of GD by ATM alone seems risky in terms of recurrence. Adequate management of GD and possible vasculitis may be important for preventing recurrence and progression.

Cerebrovascular Reactivity Measured with ASL Perfusion MRI, Ivy Sign, and Regional Tissue Vascularization in Moyamoya

Arterial spin labeling (ASL) perfusion magnetic resonance imaging (MRI) may be used to determine brain regions at risk for ischemia in patients with moyamoya vasculopathy and to identify patients who may benefit from surgical revascularization. We aimed to investigate whether 1) the severity of moyamoya is related to the presence of leptomeningeal collaterals and cerebrovascular reactivity (CVR), 2) the presence of collaterals and ivy sign reflects disturbed CVR, and 3) arterial transit artefacts (ATAs) and ivy sign reflect the presence of collaterals. We determined severity of moyamoya on digital subtraction angiography (DSA) according to the modified Suzuki classification in 20 brain regions and scored regional tissue revascularization using a 4-point scale. Regional CVR and ATAs were assessed on ASL perfusion MRI, ivy sign on fluid attenuation inversion recovery MRI. In 11 patients (median age 36 years; 91% female), we studied 203 regions. ATAs were associated with the presence of collaterals on DSA (P < 0.01). Of all regions with clearly visible collateral vessels on DSA, however, only 24% had ATAs. Ivy sign was not related to the presence or absence of collaterals nor to CVR. In 10% of regions with good vascularization on DSA, CVR was poor or showed steal. ATAs were associated with the presence of collaterals on DSA. Although DSA vascularization scores correlated with CVR, 10% of regions with good vascularization on DSA had absent CVR or steal on ASL-MRI. DSA and ivy sign did not provide adequate information on the hemodynamic status of brain tissue in patients with moyamoya vasculopathy.

Abstract TP75: Distal Hyperintense Vessel Sign on FLAIR as a Predictor of Recurrent Stroke in Intracranial Arterial Stenosis

Introduction: The distal hyperintensity vessel (DHV) on FLAIR images is a linear/serpentine shaped hyperintensity that is seen not infrequently in acute ischemic stroke or transient ischemic attack (TIA). Previous studies reported that DHV could predict large vessel occlusion or stenosis and correlate with outcome. The purpose of this study is to evaluate whether presence of DHV is associated with high risk of recurrent ischemic stroke in symptomatic intracranial atherosclerotic severe stenosis. Methods: We retrospectively reviewed patients who presented with ischemic stroke or TIA to a University-affiliated Comprehensive Stroke Center from 2010 to 2015. Patients with etiology due to 70-99% atherosclerosis stenosis of intracranial carotid artery or middle cerebral artery were enrolled. Patients with atrial fibrillation or other cardiac embolism, ipsilateral extracranial carotid artery &gt; 50% stenosis, artery dissection, hypercoagulation state, Moyamoya or other non-atherosclerotic vasculopathy were excluded. FLAIR images were reviewed by two neuroradiologists and one vascular neurologist for the presence of DHV. A quantitative scoring system of maximum 7 based on ASPECTS distribution is applied to grade the extent of DHV. The primary measure is ischemic stroke in the territory of qualifying artery during follow up. Univariate and multivariate logistic regressions were used to identify predictors of recurrent ischemic stroke. Results: Out of 106 patients enrolled, 53 (50%) were found to have DHV (median score of 3.03 ± 1.47). There were more non-whites in DHV group than in non-DHV group (p=0.0314). During follow up, 17 (32%) patients in DHV group had recurrent ischemic stroke compared to 6 (11%) patients in non-DHV group (p=0.0087). The presence of DHV was the only factor associated with recurrent ischemic stroke with OR of 3.074 (95% CI 1.079 - 8.761, p=0.036) by multivariate logistic regression. There is a tendency of high risk of recurrent ischemic stroke for high DHV score with Pearson Correlation R value of 0.7. Conclusions: The presence of DHV is associated with higher risk of recurrent ischemic stroke in symptomatic intracranial atherosclerotic severe stenosis. Confirmation of this finding in large prospective studies is needed.

Wound healing complications after revascularization for moyamoya vasculopathy with reference to different skin incisions

OBJECTIVEMoyamoya vasculopathy (MMV) is a steno-occlusive cerebrovascular disease that can be treated by a surgical revascularization. All the revascularization techniques influence the blood supply of the scalp, with a risk for wound healing disorders. The authors' aim was to analyze the wound healing process in the patients who underwent a direct or combined bypass surgery with a focus on different skin incisions.METHODSThe authors retrospectively identified all the patients with MMV who were treated surgically in their institution. Subsequently, they analyzed demographic data, clinical symptoms, surgical treatment, and detailed history of complications. Based on the evolution of their surgical techniques and the revascularization strategy to be used, the authors applied the following skin incisions: linear incision, curved incision, incomplete Y incision, and complete Y incision. Group comparisons regarding wound healing disorders were performed with significance testing using Fisher's exact test.RESULTSThe authors identified 172 patients with MMV (61.6% moyamoya disease, 7% unilateral moyamoya disease, 29.7% moyamoya syndrome, and 1.7% unilateral moyamoya syndrome), of whom 124 underwent bilateral operations. One-quarter of the patients were juveniles. A total of 236 hemispheres were included in the analysis, of which 27.9% were treated by a combined procedure with encephalomyosynangiosis. Overall, 5.1% major and 1.7% minor wound complications occurred. The overall wound complication rate was lower in direct revascularization compared to combined revascularization (3% vs 15.2%). The lowest incidence of wound healing disorders was found in the linear incision group for the parietal superficial temporal artery branch (1.6%), followed by the incomplete Y incision group for the frontal branch of the superficial temporal artery (3.8%) in the direct bypass group. In the combined revascularization cohort, major or minor wound disorders appeared in 14.3% and 4.8%, respectively, in the complete Y incision group and in 4.2% (for both major and minor) in the curved incision group. The complete Y incision caused significantly more wound healing disorders compared to the remaining incision types (17.1% vs 3.1%, p = 0.007).CONCLUSIONSWound healing disorders are one of the major complications of revascularization surgery. Their incidence depends on the revascularization strategy and skin incision applied, with a complete Y incision giving the worst results.

Cognitive Functions in Children and Adults with Moyamoya Vasculopathy: A Systematic Review and Meta-Analysis.

Background and PurposePatients with moyamoya vasculopathy (MMV) may experience cognitive impairment, but its reported frequency, severity, and nature vary. In a systematic review and metaanalysis, we aimed to assess the presence, severity, and nature of cognitive impairments in children and adults with MMV. MethodsWe followed the MOOSE guidelines for meta-analysis and systematic reviews of observational studies. We searched Ovid Medline and Embase for studies published between January 1, 1969 and October 4, 2016. Independent reviewers extracted data for mean intelligence quotient (IQ) and standardized z-scores for cognitive tests, and determined percentages of children and adults with cognitive deficits, before and after conservative or surgical treatment. We explored associations between summary measures of study characteristics and cognitive impairments by linear regression analysis. ResultsWe included 17 studies (11 studies reporting on 281 children, six on 153 adults). In children, the median percentage with impaired cognition was 30% (range, 13% to 67%); median IQ was 98 (range, 71 to 107). Median z-score was –0.39 for memory, and –0.43 for processing speed. In adults, the median percentage with impaired cognition was 31% (range, 0% to 69%); median IQ was 95 (range, 94 to 99). Median z-scores of cognitive domains were between –0.9 and –0.4, with multiple domains being affected. We could not identify determinants of cognitive impairment. ConclusionsA large proportion of children and adults with MMV have cognitive impairment, with modest to large deficits across various cognitive domains. Further studies should investigate determinants of cognitive deficits and deterioration, and the influence of revascularization treatment on cognitive functioning.

359. Transient Ischemic attacks from Moya Moya disease associated with Graves disease during Pregnancy: A case report

Background Moya moya disease(MMD) is a rare idiopathic progressive vasculopathy,characterised by stenosis of the terminal portion of the internal carotid arteries and proximal portions of its major branches with the development of a thin collateral network of small vessels.It presents in young Asians with transient Ischemic attacks (TIAs), headaches,seizures,cognitive changes, ischemic or haemorrhagic strokes and is associated with many conditions including Graves disease. Case description 31 year old female with Graves disease (poorly controlled due to non-compliance) and previous 2 normal pregnancies presented during the third trimester of her 3rd pregnancy with intermittent headaches associated with right sided numbness of face and extremities lasting for few seconds without any features of Preeclampsia that were treated symptomatically.She had fluctuant thyroid function during her pregnancy and normal vaginal delivery at 40 weeks. She presented 2 months postpartum with severe headache and right sided numbness lasting for an hour without any features of Preeclampsia. She was thyrotoxic (FT4:51 pmol/L,FT3:16.6 pmol/L, TSH Although the Magnetic resonance imaging of the brain showed no infarcts and revealed features of MMD, the CT-Angiography of the Brain reported features suspicious of Takayasu’s arteritis with no evidence of it on Aortogram. She was treated with Aspirin, atorvastatin, carbimazole and a short course of Steroids. Discussion Awareness of the association between Graves’ disease and MMD in younger patients presenting with stroke like symptoms is important as early diagnosis and treatment of MMD in the setting of Graves Disease may prevent severe neurologic sequelae and eliminate the need for surgical intervention. Fluctuations in baseline thyroid function for patients with known Graves’ disease may be a potentiating factor in exacerbating Moyamoya vasculopathy. Most centres prefer caesarean delivery for patients with MMD to avoid haemodynamic instability.

Moyamoya vasculopathy with anti-SCL-70 antibodies: A case report and review of the literature

Moyamoya disease and syndrome constitute two distinct pathological entities with a primary finding of progressive distal internal carotid artery occlusion, which results in either an ischemic or hemorrhagic sequela. While the disease entity stands as a primary arterial vasculopathy, moyamoya syndrome represents a secondary vasculopathy associated with a systemic inflammatory process. We describe a patient with an ischemic stroke and angiographic findings of moyamoya in the setting of positive antinuclear antibodies and anti-Scl-70 antibodies with clinical features of scleroderma on exam. A review of current literature identified three similar cases where immunosuppression in addition to secondary stroke prevention led to reduced frequency of neurological sequelae. These cases plus our own demonstrate that patients with moyamoya vasculopathy in association with anti-Scl 70 antibodies exist on a spectrum with either predominant scleroderma features or neurological symptoms. There are a limited number of cases reported of moyamoya vasculopathy in association with anti-Scl 70 antibodies. This case report demonstrates that not only may the association be more common than we think, but that it exists on a dynamic spectrum with variable clinical presentation and course.

Prediction of Intracranial Arterial Stenosis Progression in Patients with Moyamoya Vasculopathy: Contrast-Enhanced High-Resolution Magnetic Resonance Vessel Wall Imaging

Moyamoya vasculopathy (MMV) is characterized by progressive stenosis of the intracranial arteries. MMV currently has no curative treatments, and cerebral ischemia and hemorrhage are the major outcomes. Evaluation of the stroke risk of each patient resulting from the progression of intracranial arterial stenosis is clinically important. We prospectively reviewed patients with intracranial arterial stenosis and already diagnosed MMV. High-resolution magnetic resonance imaging using contrast agent is the novel vessel wall imaging (VWI) technique for directly evaluating vascular walls and intracranial artery disease. All patients underwent high-resolution vessel wall imaging and magnetic resonance angiography at the time of registration, and they underwent follow-up magnetic resonance angiography. The Fisher exact test was used to assess associations between the degrees of wall enhancement and between stable and progressive intracranial arterial stenosis. A total of 24 patients (17 female; mean age, 36.1 ± 16.8 years; range 3-67 years) with MMV were consecutively recruited to this study. Progression of stenosis was shown in 6 lesions (66.6%) on strong enhancement, 2 lesions (12.5%) on mild enhancement, and 1 lesion (4.3%) on lack of enhancement. Arterial vessel wall enhancement in MMV patients correlated closely with progression of intracranial arterial stenosis (P= 0.002). Arterial vessel wall enhancement in MMV patients was closely related to progression of intracranial arterial stenosis. Strong enhancement of the intracranial vessel wall was associated with intracranial arterial stenosis progression, and lack of enhancement correlated with the stability of intracranial arterial stenosis.

Cockayne Syndrome Complicated by Moyamoya Vasculopathy and Stroke

Cockayne Syndrome Complicated by Moyamoya Vasculopathy and Stroke

Noninvasive Assessment of Hemodynamic Stress Distribution after Indirect Revascularization for Pediatric Moyamoya Vasculopathy.

Indirect revascularization surgery is an effective treatment in children with Moyamoya vasculopathy. In the present study, we hypothesized that DSC-PWI may reliably assess the evolution of CBF-related parameters after revascularization surgery, monitoring the outcome of surgical pediatric patients with Moyamoya vasculopathy. Thus, we aimed to evaluate differences in DSC-PWI parameters, including the hemodynamic stress distribution, in surgical and nonsurgical children with Moyamoya vasculopathy and to correlate them with long-term postoperative outcome. Pre- and postoperative DSC parameters of 28 patients (16 females; mean age, 5.5 ± 4.8 years) treated with indirect revascularization were compared with those obtained at 2 time points in 10 nonsurgical patients (6 females; mean age, 6.9 ± 4.7 years). We calculated 4 normalized CBF-related parameters and their percentage variance: mean normalized CBF of the MCA territory, mean normalized CBF of the proximal MCA territory, mean normalized CBF of cortical the MCA territory, and hemodynamic stress distribution. The relationship between perfusion parameters and postoperative outcomes (poor, fair, good, excellent) was explored using 1-way analysis of covariance (P < .05). A significant decrease of the mean normalized CBF of the proximal MCA territory and hemodynamic stress distribution and an increase of the mean normalized CBF of the cortical MCA territory were observed after revascularization surgery (P < .001). No variations were observed in nonsurgical children. Postoperative hemodynamic stress distribution and its percentage change were significantly different in outcome groups (P < .001). DSC-PWI indices show postoperative hemodynamic changes that correlate with clinical outcome after revascularization surgery in children with Moyamoya disease.

Cerebral Bypass Surgery: Level of Evidence and Grade of Recommendation.

Cerebral bypasses are categorized according to function (flow augmentation or flow preservation) and to characteristics: direct, indirect or combined bypass, extra-to-intracranial or intra-to-intracranial bypass, and high-, moderate- or low-capacity bypass. We critically summarize the current state of evidence and grades of recommendation for cerebral bypass surgery. The current indications for cerebral bypass are discussed depending on the function of the bypass (flow preservation or augmentation) and analyzed according to level of evidence criteria. Flow-preservation bypass plays an important role in managing complex intracranial aneurysms (level of evidence 4; grade of recommendation C). Flow-preservation bypass is currently only very rarely indicated in the treatment of cerebral tumors involving major cerebral arteries (level of evidence 5; grade of recommendation D). The trend has evolved in favor of partial resection and radiotherapy. To preserve the flow, the bypass is always a direct bypass.Flow-augmentation bypass is currently recommended for Moyamoya patients with ischemic symptoms and compromised hemodynamics (level of evidence 4; grade of recommendation C) and patients with hemorrhagic onset (level of evidence 1B; grade of recommendation A). Flow-augmentation bypass is currently not recommended for patients with recently symptomatic carotid artery occlusion, even in the setting of compromised cerebral hemodynamics (level of evidence 1A; grade of recommendation A), but may be considered in patients with hemodynamic failure and recurrent medically refractory symptoms as a final resort (level of evidence 5; grade of recommendation D). The results of recent randomized clinical trials narrow the indication for cerebral bypass in the setting of ischemic cerebrovascular disease. However, cerebral bypass is still very useful for managing complex intracranial aneurysms (not amenable to selective clipping or endovascular therapies) and is the only treatment option for managing symptomatic patients with Moyamoya vasculopathy and impaired brain hemodynamics.

Cerebrovascular blood pressure autoregulation monitoring and postoperative transient ischemic attack in pediatric moyamoya vasculopathy.

Children with moyamoya vasculopathy are at high risk of perioperative cerebral ischemia or hyperperfusion. Maintaining blood pressure within the range of functional cerebrovascular blood pressure autoregulation might reduce the risk of perioperative neurologic injury. We tested whether blood pressure autoregulation is associated with postoperative transient ischemic attack in a study of patients with pediatric moyamoya vasculopathy. We conducted an observational study of 15 pediatric patients undergoing surgical revascularization with pial synangiosis. Nine patients had bilateral moyamoya and 6 had unilateral moyamoya. We measured autoregulatory vasoreactivity intraoperatively and during the first postoperative night with the hemoglobin volume index, a value derived from near-infrared spectroscopy. We also identified the optimal mean arterial blood pressure at which autoregulation was most robust in each patient. Of the 15 children monitored, 3 with bilateral moyamoya and one with unilateral moyamoya experienced a transient ischemic attack. Poorer autoregulation during surgery was associated with postoperative transient ischemic attack among those with bilateral vasculopathy (P = .048, difference in hemoglobin volume index medians: 0.023, 95% confidence interval: 0.003-0.071). This relationship was not observed with postoperative autoregulation. The optimal mean arterial blood pressure was identifiable during surgery in all monitored patients, varied among patients, and often differed between the intraoperative and postoperative periods. Dysfunctional intraoperative autoregulation may increase the risk of TIA in patients with pediatric moyamoya vasculopathy. The blood pressure range that supports autoregulation appears to vary among patients. Using autoregulation monitoring to guide individualized blood pressure goals should be studied as a potential method to reduce perioperative neurologic morbidity in pediatric patients with moyamoya.

Posterior circulation involvement in pediatric and adult patients with moyamoya disease: a single center experience in 574 patients.

The importance of the posterior cerebral artery (PCA) involvement in moyamoya disease has been highlighted in recent years. However, few studies compared the impact of PCA lesions in moyamoya disease between pediatric and adult patients. We conducted this study to summarize the clinical features of moyamoya patients with PCA lesions and describe the difference between pediatric and adult patients. We reviewed the records of 696 consecutive moyamoya vasculopathy patients from 2009 to 2015. The Suzuki and the Miyamoto stages were used to evaluate the steno-occlusive lesions of the anterior and posterior arteries. Clinical and radiographic features were compared between those with and without PCA involvement, also between pediatric and adult patients. A total of 574 angiograms (140 pediatrics and 434 adults) were reviewed. The prevalence of PCA steno-occlusion did not differ significantly between pediatric patients and adult patients (35.0% vs. 30.4%, P=0.347). Pediatric patients had more advanced PCA stages compared to adult patients (P=0.045). There was a significant correlation of the PCA angiographic stages with the ipsilateral internal carotid artery (ICA) stages, both in pediatrics and in adults (both P<0.001). The frequency of ipsilateral cerebral infarction positively correlated with the advancement of PCA stages in adult patients (P<0.001), but not significant in pediatric patients (P=0.106). Pediatric patients tend to have more advanced PCA lesions than adult patients. The degree of PCA steno-occlusion positively correlates with the ipsilateral ICA stage, both in pediatrics and in adults.

Pregnancy and delivery in moyamoya vasculopathy: experience of a single European institution.

Moyamoya vasculopathy (MMV) is a stenoocclusive cerebrovascular disease frequently affecting adolescent females. Thus, the management of pregnancy is of major interest for physicians dealing with MMV patients. However, clinical data concerning pregnant MMV patients is lacking. Our aim was to analyze the course of pregnancy in our European MMV patient cohort. We retrospectively identified MMV patients with a history of pregnancy who were treated in our institution. We analyzed demographic data, clinical symptoms, treatment, and detailed history of pregnancies. We identified 31 MMV patients (81% moyamoya disease (MMD), 6% unilateral MMD, 13% quasi MMD) with a history of pregnancy resulting in a total of 60 pregnancies and 48 deliveries. Ninety-four percent of the patients became symptomatic by ischemic events and 6% by headache. Eighty-one and nineteen percent of the patients experienced their pregnancies prior to(first group) or after the diagnosis (second group), respectively. In the first group, the mean age at first delivery and bypass treatment were 24±4 and 42±10, respectively. Eight percent of these patients were diagnosed as MMV due to ischemic events during perinatal period. In the second group, the mean age at first delivery and bypass treatment were 26±7 and 23±6, respectively. No cerebral events were reported within perinatal period in this group. All previously operated patients continued their aspirin medication at least until the 3rd trimester. The majority of the pregnancies occurred prior to diagnosis in our European patient cohort. Our data indicates a protective effect of surgical treatment for avoiding cerebral events during pregnancy and delivery for already diagnosed patients.

The Collateral Circulation in Moyamoya Disease: A Single-Center Experience in 140 Pediatric Patients

BackgroundThe patterns and clinical significance of the presence of collaterals in moyamoya disease has not been elucidated thoroughly. We aimed to summarize the clinical features of collaterals in pediatric moyamoya disease. Basic proceduresWe reviewed consecutive pediatric patients with moyamoya vasculopathy who were admitted to our hospital from 2009 to 2015. Only pediatric moyamoya patients with digital subtraction angiography examinations were included in this study. We classified spontaneous collaterals into two groups by their origins, the internal carotid artery and vertebral artery (ICA-VA)-originated collaterals and the external carotid artery (ECA)-originated collaterals. Main findingsA total of 140 pediatric patients with moyamoya disease (mean age, 10.1 ± 3.7 years) were included in this study. We identified collaterals originated from ICA-VA in 122 (87.1%) pediatric patients (222 hemispheres). Patients in the ICA-VA collaterals group had similar Suzuki stages with patients without collaterals originated from ICA-VA (mean, 3.86 vs 3.50; P = 0.157). We identified collaterals originated from ECAs in 70 (50.0%) pediatric patients (106 hemispheres) with moyamoya disease. The infarctions were more common in patients with ECA-originated collaterals than in patients without ECA-originated collaterals (25 versus 12; P = 0.021). Principal conclusionsOur results demonstrate that collaterals originating from ECAs are associated with advanced Suzuki stages and infarction presentations in children with moyamoya disease.

Moyamoya Vasculopathy in PHACE Syndrome: Six New Cases and Review of the Literature.

PHACE syndrome (Posterior fossa malformations, large cervicofacial infantile Hemangiomas, Arterial anomalies, aortic coarctation and Cardiac abnormalities, and Eye abnormalities) is a neurocutaneous disorder including posterior fossa malformations, hemangiomas, arterial lesions, cardiac defects, and eye abnormalities. PHACE arteriopathies may be progressive and recently have been categorized based on the risk of acute ischemic stroke, increasing attention to the potentially devastating consequences of cerebrovascular complications in this syndrome. In contrast, the natural history of arteriopathy in PHACE syndrome remains poorly understood. At the moment, there are no established surgical guidelines for high-risk vasculopathies, including quasi-moyamoya, in this syndrome. We described the clinicoradiologic features of a small series of 6 patients with PHACE syndrome and quasi-moyamoya (5 female, age range 4 months to 12 years), focusing on the clinical course and surgical outcome of 3 children who were treated with encephaloduroarteriosynangiosis and encephalomyosynangiosis. In addition, we reviewed the radiologic, clinical, and surgical aspects of moyamoya vasculopathy in PHACE syndrome, providing information on 15 additional published cases. Although the natural history of arteriopathy in PHACE syndrome is poorly understood, patients with high-risk vasculopathies, such as quasi-moyamoya disease, may benefit of revascularization by using encephaloduroarteriosynangiosis and encephalomyosynangiosis.

Adolescents with moyamoya disease: clinical features, surgical treatment and long-term outcomes.

This study aimed to elucidate the clinical features, surgical treatment and long-term outcomes of adolescent patients with moyamoya disease. We reviewed consecutive patients with moyamoya vasculopathy who were admitted to our hospital from 2009 to 2015 to identify adolescent patients with moyamoya disease. Clinical features, surgical treatment and long-term outcomes were analyzed. Follow-up was performed by face-to-face or structured telephone interviews. Outcome measures were future stroke events. We performed univariate and multivariate time-to-event analyses to identify risk factors associated with future stroke events. A total of 95 adolescent patients with moyamoya disease (age at onset, 13.1±2.3years) were included in this study. During follow-up, 12 patients (12.6%) had stroke events. We found that the patients who underwent direct/combined bypass had a significantly lower risk of future strokes [hazard ratio (HR), 0.16; 95% confidence interval (CI), 0.03-0.74; P=0.019] compared to patients who underwent indirect bypass. Our results demonstrate that direct/combined bypass can be more effective in preventing future strokes than indirect bypass in adolescent patients with moyamoya disease.