Moyamoya Disease in China

Zhou

et al. 2016

JNS

M oyamoya vasculopathy (MMV) is a disorder that is characterized by stenosis or occlusion of the terminal internal carotid artery (ICA) and the proximal portion of the anterior and/or middle cerebral arteries. Additionally, it is accompanied by net-like collateral vessel formations, which are termed the "moyamoya" vessels.14,18 Moyamoya disease (MMD) is defined by the presence of bilateral MMV with an unknown etiology or the presence of unilateral MMV at 1 side with obvious terminal contralateral ICA stenosis.4 Unilateral MMD is defined by typical angiographic evidence of MMD unilaterally with normal or equivocal contralateral findings. 4The concept that unilateral MMD is an early form of bilateral MMD remains controversial, and the pathogenesis of moyamoya disorders is not fully understood. 7,11,14,18 MMD has been reportedly associated with various entities. 2,8,9,13,16,24 Analysis of the coexisting diseases that occur with moyamoya may be helpful in exploring moyamoya pathogeneses. Several reports have noted MMD in association with autoimmune diseases, including autoim- obJective This study explored whether there were differences between the autoimmune disease prevalence rates in unilateral and bilateral moyamoya disease (MMD). methods The authors performed a retrospective review of data obtained from the medical records of their hospital, analyzing and comparing the clinical characteristics and prevalence rates of all autoimmune diseases that were associated with unilateral and bilateral MMD in their hospital from January 1995 to October 2014. results Three hundred sixteen patients with bilateral MMD and 68 with unilateral MMD were identified. The results indicated that patients with unilateral MMD were more likely to be female than were patients with bilateral MMD (67.6% vs 51.3%, p = 0.014, odds ratio [OR] 1.99). Overall, non-autoimmune comorbidities tended to be more prevalent in the unilateral MMD cases than in the bilateral MMD cases (17.6% vs 9.8%, p = 0.063, OR 1.97, chi-square test). Autoimmune thyroid disease and other autoimmune diseases also tended to be more prevalent in the unilateral MMD cases than in the bilateral MMD cases (19.1% vs 10.8%, p = 0.056, OR 1.96 and 8.8% vs 3.5%, p = 0.092, OR 2.77, respectively, chi-square test). The overall autoimmune disease prevalence in the unilateral MMD cases was significantly higher than in the bilateral MMD cases (26.5% vs 13.6%, p = 0.008, OR 2.29, 95% CI 1.22-4.28, chi-square test). Multiple logistic regression analysis showed that autoimmune disease was more likely to be associated with unilateral than with bilateral MMD (p = 0.039, OR 10.91, 95% CI 1. 13-105.25). coNclusioNs This study indicated a higher overall autoimmune disease prevalence in unilateral than in bilateral MMD. Unilateral MMD may be more associated with autoimmune disease than bilateral MMD. Different pathogenetic mechanisms may underlie moyamoya vessel formation in unilateral and bilateral MMD.

Section: Discussionmentioning

confidence: 86%

Increased prevalence of autoimmune disease in patients with unilateral compared with bilateral moyamoya disease

Chen

Zhou

et al. 2016

JNS

“…1,3) However, the differences between the clinical profiles of Korean and Japanese patients are unlikely to be explained by genetic differences. Specific factor(s) other than the genetic factor are needed to explain the variations in the clinical phenotypes and low penetrance among carriers.…”

Section: Discussionmentioning

confidence: 99%

Distribution of Moyamoya Disease Susceptibility Polymorphism p.R4810K in RNF213 in East and Southeast Asian Populations

Neurol. Med. Chir.(Tokyo)

Hitomi

Kobayashi

et al. 2012

115

“…7 All data were collected through March 2015. The study protocol was approved by the institutional ethics committee.…”

Section: Retrospective Chart Reviewmentioning

confidence: 99%

“…25 There is wide controversy regarding the merits and shortcomings of the 2 surgical approaches for adults with MMD. 2,5,7,23,31 Starke et al reviewed the literature concerning the surgical treatment and concluded that both direct and indirect bypass were effective. 30 Since there is a lack of randomized trials, it was noted that the selection of the surgical modalities depended on the preference and surgical technique mastered by the cerebrovascular neurosurgeon.…”

Section: Surgical Treatmentmentioning

confidence: 99%

See 1 more Smart Citation

Surgical outcomes following encephaloduroarteriosynangiosis in adult moyamoya disease associated with Type 2 diabetes

Ren

Zhang

et al. 2016

JNS

Self Cite

M oyaMoya disease (MMD), a cerebral vascular disease, is characterized by progressive stenosis and occlusion of the terminal portion of the bilateral internal carotid arteries, leading to the formation of an abnormal vascular network composed of collateral pathways at the base of the brain. MMD can cause a reduction in the blood supply to the brain, and there are 2 major etiological categories of symptoms: those due to brain ischemia (i.e., stroke, transient ischemic attack, and seizure) and those due to the deleterious consequences of the compensatory mechanisms in response to ischemia (i.e., hemorrhage from fragile collateral vessels and headache aBBreViatiONS CBF = cerebral blood flow; DM = diabetes mellitus; EDAS = encephaloduroarteriosynangiosis; MCA = middle cerebral artery; MMD = moyamoya disease; mRS = modified Rankin Scale; PCA = posterior cerebral artery; STA = superficial temporal artery; T2DM = Type 2 DM. SuBmitted January 29, 2015. OBJectiVe Debate exists regarding the merits and shortcomings of an indirect bypass procedure for treating adult patients with moyamoya disease (MMD). Considerable variation in neovascularization occurs among different organs in patients with diabetes mellitus. Here, the effect of encephaloduroarteriosynangiosis on MMD associated with Type 2 diabetes mellitus (T2DM) is evaluated. methOdS A retrospective and 1:2 matched case-control study was conducted in moyamoya patients with or without T2DM (n = 180). Postoperative collateral formations were graded according to the Modified Collateral Grading System that originated from the Matsushima Angiographic Stage Classification. Neurological function outcomes before and after the operation were evaluated according to the modified Rankin Scale. Univariate and multivariate logistic regression analyses were performed to determine the risk factors for clinical outcome. reSultS There was no statistically significant difference in the constituent ratios of initial symptom and preoperative Suzuki stage between patients with and without T2DM. Progression of angiopathy around the circle of Willis was postoperatively observed in bilateral internal carotid arteries in both groups. Patients with T2DM had a higher postoperative Suzuki stage (p < 0.01) and more frequent development of collateral angiogenesis germinating from the external carotid after indirect revascularization procedures in the surgical cerebral hemisphere (82.7% vs 72.2%; p < 0.05). The extent of postoperative collateral formation in patients with diabetes mellitus was significantly higher (p < 0.01). Postoperative clinical improvement in the diabetes group was more common after revascularization procedures (p < 0.05), and the diabetes group had lower modified Rankin Scale scores (p < 0.05) in comparison with the nondiabetes group. Late postoperative stroke and posterior cerebral artery involvement were identified as predictors of unfavorable clinical outcome in both groups, while T2DM was associated with a favorable clinical outcome. cONcluSiONS Encephaloduroarteriosynangio...