Long-Term Outcome of Revascularization Surgery for Moyamoya Disease in Korea

Author(s):  
Jeong Eun Kim ◽  
Chang Wan Oh
2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Jie Song ◽  
Yu Lei ◽  
Long Chen ◽  
Chao Gao ◽  
Wei Ni ◽  
...  

Objective. To evaluate whether hemodynamic factors are risk factors for prognosis in moyamoya disease (MMD). Materials and Methods. The retrospective study reviewed a single-center MMD cohort in Huashan Hospital from August 2017 to January 2020. Stroke events in 30 days and follow-up modified Rankin Scale (mRS) grade were recorded. Systematic assessments with perioperative mean arterial pressure (MAP), red blood cell (RBC) parameters, and fluid management were also conducted. Logistic regressions were applied to evaluate the predictors of worse outcomes. Data was analyzed using SPSS 24.0. Results. Admission to neurological intensive care unit (NICU) totalled about 347 after revascularization surgery. The result showed that the higher the postoperative MAP level (favorable group 95.7 ± 11.4  mmHg vs. unfavorable group 103.6 ± 10.4  mmHg, p < 0.001 ) and the greater the MAP variability (favorable group 0.26 ± 13.2 vs. unfavorable group 7.2 ± 13.5 , p = 0.006 ) were, the higher the patient’s follow-up mRS grade was. What is more, a higher early postoperative Hb level also seemed to predict a worse long-term clinical outcome (favorable group 116.9 ± 17.1  g/L vs. unfavorable group 123.7 ± 13.0  g/L, p = 0.03 ), but the difference disappeared after adjusting sex and age. Logistic regression analyses showed that a higher level of postoperative MAP ( β = 0.024 , 95% CI (0.004, 0.044), and p = 0.02 ) within the first 24 h in NICU might be the short-term risk factor. For long-term outcome, a higher level ( β = 1.058 , 95% CI (1.022, 1.096), and p = 0.001 ) and a greater variability ( β = 30.982 , 95% CI (2.112, 454.414), and p = 0.01 ) of postoperative MAP might be the negative predictors of mRS grade. Conclusions. The early postoperative hemodynamic management might be extremely critical for patients with MMD. Both high postoperative MAP levels and large MAP variability might affect the prognosis. What is more, we also found that a higher postoperative Hb level might be related with a worse outcome.


2020 ◽  
Vol 11 ◽  
Author(s):  
Bin Gao ◽  
Kaijiang Kang ◽  
Jia Zhang ◽  
Dong Zhang ◽  
Xingquan Zhao

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.


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

2012 ◽  
Vol 35 (1) ◽  
pp. 67-76 ◽  
Author(s):  
Guglielmo Mario Actis Dato ◽  
Fabrizio Sansone ◽  
Paola Omedé ◽  
Edoardo Zingarelli ◽  
Roberto Flocco ◽  
...  

2016 ◽  
Vol 124 (5) ◽  
pp. 1221-1227 ◽  
Author(s):  
Eun-Hee Kim ◽  
Mi-Sun Yum ◽  
Young-Shin Ra ◽  
Jun Bum Park ◽  
Jae Sung Ahn ◽  
...  

OBJECT Moyamoya disease (MMD) is an idiopathic cerebrovascular occlusive disorder prevalent in East Asia. In the pathogenesis of MMD, the important role of genetic factors is being elucidated, and RNF213 has recently been identified as a susceptibility gene for MMD. The aim of this retrospective study was to investigate the RNF213 genotype in patients with MMD and to determine their genotype-phenotype associations. METHODS The study involved 165 Korean MMD patients from 155 unrelated families who were diagnosed with MMD at a single center from 1995 to 2013. Their demographic, radiological, and clinical findings were evaluated. Direct sequencing of the major RNF213 single nucleotide polymorphisms was performed. The association of the common RNF213 variant with MMD risk was evaluated using historical controls for comparison. Correlations between RNF213 genotype and phenotype were statistically analyzed. RESULTS The c.14429G>A (p.R4810K) variant was identified in 125 (75.8%) of 165 MMD patients. Most patients (112) were heterozygous, and 13 patients had 2 copies of the c.14429G>A variant. A novel heterozygous variant, c.12086A>G (p.Q4029R), was found in 1 additional patient. The minor allele frequency of the c.14429G>A variant was significantly higher in the MMD group (138 [41.8%] of 330 patients) than in the control group (8 [1.36%] of 588 subjects; p < 0.001). The c.14429G>A (p.R4810K) variant significantly increased the risk of MMD in Korean patients, with an OR of 52.11 (p < 0.001) compared with controls. Moreover, c.14429G>A (p.R4810K) genotypes occurred more frequently in patients with a family history of MMD. The homozygous variant was highly associated with early-onset MMD (age at onset < 5 years), cerebral infarction at diagnosis, and cognitive impairment in long-term outcome. CONCLUSIONS The findings indicate that the c.14429G>A (p.R4810K) allele of RNF213 is strongly associated with Korean patients with MMD. The homozygous c.14429G>A (p.R4810K) variant is particularly related to early-onset MMD, severe symptomatic manifestations at diagnosis, and poor prognosis. This genotypic variant may be a useful biomarker for early-onset MMD or unstable MMD with cerebral infarction, which requires early diagnosis and revascularization treatment.


2015 ◽  
Vol 359 (1-2) ◽  
pp. 381-388 ◽  
Author(s):  
Hyun Jin Noh ◽  
Suk Jae Kim ◽  
Jong Soo Kim ◽  
Seung-Chyul Hong ◽  
Keon Ha Kim ◽  
...  

2009 ◽  
Vol 26 (4) ◽  
pp. E2 ◽  
Author(s):  
Jodi L. Smith

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.


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