Understanding and treating moyamoya disease in children

2009 ◽  
Vol 26 (4) ◽  
pp. E2 ◽  
Author(s):  
Jodi L. Smith
Keyword(s):  
Moyamoya Disease ◽  
Treatment Options ◽  
Unknown Etiology ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Indirect Revascularization ◽  
Collateral Vessels ◽  
Revascularization Procedures ◽  
Disease Present

Moyamoya disease, a known cause of pediatric stroke, is an unremitting cerebrovascular occlusive disorder of unknown etiology that can lead to devastating, permanent neurological disability if left untreated. It is characterized by progressive stenosis of the intracranial internal carotid arteries and their distal branches and the nearly simultaneous appearance of basal arterial collateral vessels that vascularize hypoperfused brain distal to the occluded vessels. Moyamoya disease may be idiopathic or may occur in association with other syndromes. Most children with moyamoya disease present with recurrent transient ischemic attacks or strokes. Although there is no definitive medical treatment, numerous direct and indirect revascularization procedures have been used to improve the compromised cerebral circulation, with outcomes varying according to procedure type. Such techniques improve the long-term outcome of patients with both idiopathic and syndrome-associated moyamoya disease. This review provides a comprehensive discussion of moyamoya disease in children, with an emphasis on the most effective surgical treatment options.

Predictors of neoangiogenesis after indirect revascularization in moyamoya disease: a multicenter retrospective study

2020 ◽  
Vol 132 (1) ◽  
pp. 98-108 ◽  
Author(s):  
Yahui Zhao ◽  
Jiaxi Li ◽  
Junlin Lu ◽  
Qian Zhang ◽  
Dong Zhang ◽  
...  
Keyword(s):  
Moyamoya Disease ◽  
Bypass Surgery ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Scale Scores ◽  
The Status ◽  
Long Term Follow Up ◽  
Indirect Revascularization ◽  
Different Populations

OBJECTIVEThe effect of indirect revascularization to improve cerebral perfusion for moyamoya disease (MMD) is based on ingrowth of new vessels into the cortical brain. Preoperative indicators for neoangiogenesis would be helpful to the selection of appropriate procedures for MMD patients but have not yet been investigated. Our study aimed to identify potential predictors for neovascularization after indirect bypass surgery.METHODSThe authors reviewed consecutive cases with complete clinical and radiological documentation of patients who had undergone surgery between December 2010 and January 2018. Patients who were treated with indirect bypass surgery were included. Cerebrovascular characteristics were evaluated by catheter angiography. Neoangiogenesis after indirect bypass was determined as “good” or “poor” based on the Matsushima standard. Univariate and multivariate analyses were performed to identify predictors for neoangiogenesis after indirect bypass. Subgroup analyses by onset type and surgical type were carried out to identify specific predictors for different populations.RESULTSIn total, 231 hemispheres of 209 patients (mean ± SD age 23.06 ± 15.09 years, range 3–61 years) were retrospectively included. In 146 (63.2%) hemispheres, good neoangiogenesis was observed after indirect revascularization. Multivariate analysis showed that the status of ICA moyamoya vessels (p < 0.001, OR [95% CI] 3.242 [2.007–5.236]) is a predictor of favorable neoangiogenesis after indirect bypass surgery, whereas hemorrhagic onset (p < 0.001, OR [95% CI] 0.138 [0.054–0.353]) is a risk factor for poor neoangiogenesis. In addition, younger age was significantly associated with good neovascularization in patients with hemorrhagic onset (p = 0.027, OR [95% CI] 0.893 [0.808–0.987]), whereas age was not a significant predictor for neovascularization in non–hemorrhagic-onset patients (p = 0.955). Hemispheres with good revascularization had lower incidence of rebleeding, lower modified Rankin Scale scores, and more improvement of symptoms during long-term follow-up (p = 0.026, 0.006, and 0.013, respectively).CONCLUSIONSHemorrhagic onset predicts poor neovascularization after indirect bypass surgery for MMD patients. Abundant ICA moyamoya vessels indicate good neoangiogenesis after indirect bypass and vice versa, whereas absent ICA moyamoya vessels predict poor revascularization. Good neovascularization was associated with better long-term outcome. Future studies are needed to further address this issue and clarify the underlying pathophysiological mechanisms.

scholarly journals Clinical features, surgical treatment, and long-term outcome in adult patients with moyamoya disease

2009 ◽  
Vol 111 (5) ◽  
pp. 936-942 ◽  
Author(s):  
Robert M. Starke ◽  
Ricardo J. Komotar ◽  
Zachary L. Hickman ◽  
Yehuda E. Paz ◽  
Angela G. Pugliese ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Clinical Features ◽  
Moyamoya Disease ◽  
Adult Patients ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Baseline Status ◽  
Collateral Vessels

Object The object of this study was to report the clinical features, surgical treatment, and long-term outcomes in adults with moyamoya phenomenon treated at a single institution in the US. Methods Forty-three adult patients with moyamoya disease (mean age 40 ± 11 years [SD], range 18–69 years) were treated with encephaloduroarteriosynangiosis (EDAS). Neurologists examined patients pre- and postoperatively. Follow-up was obtained in person or by structured telephone interview (median 41 months, range 4–126 months). The following outcomes were collected: transient ischemic attack (TIA), infarction, graft collateralization, change in cerebral perfusion, and functional level according to the modified Rankin scale (mRS). Kaplan-Meier estimates of infarction risk were calculated for comparison of surgically treated and contralateral hemispheres. Results The majority of patients were women (65%), were Caucasian (65%), presented with ischemic symptoms (98%), and had bilateral disease (86%). Nineteen patients underwent unilateral and 24 patients bilateral EDAS (67 treated hemispheres). Collateral vessels developed in 50 (98%) of 52 hemispheres for which imaging was available and there was evidence of increased perfusion on SPECT scans in 41 (82%) of the 50 hemispheres evaluated. Periprocedural infarction (< 48 hours) occurred in 3% of the hemispheres treated. In the follow-up period patients experienced 10 TIAs, 6 infarctions, and 1 intracranial hemorrhage. Although the hemisphere selected for surgery was based upon patients' symptoms and severity of pathology, the 5-year infarction-free survival rate was 94% in the surgically treated hemispheres versus < 36% in the untreated hemispheres (p = 0.007). After controlling for age and sex, infarction was 89% less likely to occur in the surgically treated hemispheres than in the contralateral hemispheres (hazard ratio 0.11, 95% CI 0.02–0.56). Thirty-eight (88%) of 43 patients had preserved or improved mRS scores, relative to baseline status. Conclusions In this mixed-race population of North American patients, indirect bypass promoted adequate pial collateral development and increased perfusion in the majority of adult patients with moyamoya disease. Patients had low rates of postoperative TIAs, infarction, and hemorrhage, and the majority of patients had preserved or improved functional status.

scholarly journals Clinical Characteristics and Long-Term Outcome of Headaches Associated With Moyamoya Disease in the Chinese Population—A Cohort Study

2020 ◽  
Vol 11 ◽  
Author(s):  
Bin Gao ◽  
Kaijiang Kang ◽  
Jia Zhang ◽  
Dong Zhang ◽  
Xingquan Zhao
Keyword(s):  
Conservative Treatment ◽  
Moyamoya Disease ◽  
Chinese Population ◽  
Chinese Patients ◽  
Surgical Revascularization ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Long Term Follow Up

Background: Headache associated with Moyamoya disease (HAMD) in the Chinese population is not well-described. The long-term outcome of surgical revascularization and natural course of HAMD has not been disclosed either.Methods: A headache screening questionnaire in China based on the ICHD2 and a face-to-face interview performed by an experienced neurologist were used to investigate headache characteristics and frequency and pain intensity in the 3 months before admission, and a telephone interview was used for the follow-up of a large cohort of 119 Chinese patients with HAMD.Results: Headache intensity was rated as scores of 5.9 ± 2.0 on a visual analog scale (VAS), ranging from 0 to 10, in the 3 months before admission. Forty-six patients (38.6%) were categorized as having migraine-like headaches, 29 patients (24.3%) were categorized as having tension type-like headaches, and 44 patients (36.9%) had a combination of both. The majority of patients had migraine-like headaches (n = 34, 73.9%) with a migrainous aura. Both the frequency and intensity of the headache improved significantly in patients treated with surgical revascularization (n = 96, 80.7%) or the conservative treatment (n = 23, 19.3%) in a long-term follow-up.Conclusion: HAMD frequently presented with a migraine-like headache (75.5% in total). A tension type headache was present in 60.9% of patients. The symptom of dizziness is common in patients with HAMD (60.5%), and 19 of them (26.4%) met the diagnose of vestibular migraine. Both intensity and frequency of HAMD show a trend of spontaneous remission in a long-term follow-up, and there is no difference in long-term outcomes of HAMD between surgical revascularization and conservative treatment, which indicates that the effect of bypass intervention on HAMD may be a placebo effect.

scholarly journals 4035 Astrocyte LDLR-Related Protein 1 Increases Cytokine Sensitivity – The Role of Glia in Recovery after Brain Damage

2020 ◽  
Vol 4 (s1) ◽  
pp. 3-3
Author(s):  
Sadiya Ahmad ◽  
Pamela Reed ◽  
Shane Sprauge ◽  
Naomi Sayre
Keyword(s):  
Plasma Membrane ◽  
Treatment Options ◽  
Significant Loss ◽  
Null Mouse ◽  
Dependent Manner ◽  
Related Protein ◽  
Stroke Patients ◽  
Term Outcome ◽  
Long Term Outcome

OBJECTIVES/GOALS: The limited treatment options for ischemic stroke patients have resulted in stroke being a leading cause of death and the primary cause of long-term disability in the U.S. Finding effective treatment options requires a better fundamental understanding of the ongoing processes that contribute to poor long-term outcome. METHODS/STUDY POPULATION: Expression of Apolipoprotein E4 predisposes stroke patients to poor long-term outcome. This study aims to test one possible mechanism by which ApoE4 contributes to cognitive decline after stroke. Here, we examine the effect of a major ApoE4 receptor, low-density lipoprotein receptor related protein 1 (LRP1) on sensitivity to stress in astrocytes. LRP1 binds and moves extracellular ligands and plasma membrane proteins into the endocytic system. Others have shown that LRP1 regulates cell-surface TNF receptor (TNFR1) in non-astrocytic cells. We propose That LRP1 similarly regulates TNFR1 in the central nervous system to attenuate inflammatory response after stroke. Studies have shown that ApoE4 slows the recycling of endocytic LDL receptors. We hypothesize that ApoE4 inhibits the ability of LRP1 to remove TNFR1 from the plasma membrane. This is expected to increase cytokine sensitivity, resulting in worse outcome after stroke. We investigated the effect of LRP1 on astrocyte TNFα signaling and response in immortalized ApoE null mouse astrocytes subjected to lentiviral-mediated knockdown of LRP1. The astrocyte response to TNFα stimulation was tested in a time dependent manner using Western blotting of NFkB pathway components, which are the downstream mediators of TNFα signaling. We also tested astrocyte viability after prolonged TNFα stimulation using Alamar Blue reagent. We found that LRP1 deficient cells have increased phosphorylation of NFkB upon TNFα stimulation, and that loss of LRP1 resulted in significant loss of astrocyte viability after prolonged stimulation. RESULTS/ANTICIPATED RESULTS: Altogether, our results indicate that loss of LRP1 renders astrocytes more sensitive to TNFα. Future experiments will focus on testing the influence of LRP1 on recovery after middle cerebral artery occlusion in mice. DISCUSSION/SIGNIFICANCE OF IMPACT: These studies will elucidate how astrocyte-LRP1 contributes to outcome after stroke, and helps us to understand one potential way that ApoE4 exerts pathological effects. A better understanding of the long-term processes after stroke will allow identification of therapies which improve the morbidity and mortality associated with stroke. CONFLICT OF INTEREST DESCRIPTION: NA.

scholarly journals Long-term outcome of hemorrhagic moyamoya disease in 16 patients admitted to our hospital

Nosotchu ◽  
2004 ◽  
Vol 26 (3) ◽  
pp. 430-433
Author(s):  
Hiroaki Nomura ◽  
Takahiro Tomita ◽  
Kensuke Murakami ◽  
Noboru Takahashi ◽  
Yasuhiro Suzuki ◽  
...  
Keyword(s):  
Moyamoya Disease ◽  
Term Outcome ◽  
Long Term Outcome

scholarly journals Sex differences in traumatic brain injury: a multi-dimensional exploration in genes, hormones, cells, individuals, and society

2019 ◽  
Vol 5 (1) ◽  
Author(s):  
Cheng Ma ◽  
Xin Wu ◽  
Xiaotian Shen ◽  
Yanbo Yang ◽  
Zhouqing Chen ◽  
...  
Keyword(s):  
Traumatic Brain Injury ◽  
Cognitive Impairment ◽  
Brain Injury ◽  
Treatment Options ◽  
Term Treatment ◽  
Neuroprotective Effects ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Long Term Treatment

Abstract Traumatic brain injury (TBI) is exceptionally prevalent in society and often imposes a massive burden on patients’ families and poor prognosis. The evidence reviewed here suggests that gender can influence clinical outcomes of TBI in many aspects, ranges from patients’ mortality and short-term outcome to their long-term outcome, as well as the incidence of cognitive impairment. We mainly focused on the causes and mechanisms underlying the differences between male and female after TBI, from both biological and sociological views. As it turns out that multiple factors contribute to the gender differences after TBI, not merely the perspective of gender and sex hormones. Centered on this, we discussed how female steroid hormones exert neuroprotective effects through the anti-inflammatory and antioxidant mechanism, along with the cognitive impairment and the social integration problems it caused. As to the treatment, both instant and long-term treatment of TBI requires adjustments according to gender. A further study with more focus on this topic is therefore suggested to provide better treatment options for these patients.

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