Clinical Features, Surgical Treatment, and Long-term Outcome of Adult Moyamoya Patients

Starke, Robert M.; Komotar, Ricardo J.; Hickman, Zachary L.; Paz, Yehuda; Pugliese, Angela; Otten, Marc L.; Garrett, Matthew C.; Merkow, Maxwell B.; Elkind, Mitchell S.V.; Marshall, Randolph S.; Meyers, Philip M.; Festa, Joanne R.; Connolly, Edward S.

doi:10.1227/01.neu.0000333471.90507.f8

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“…In our series, the 10-year cumulative risk of recurrent stroke was 3.5%, and the 5-year risk (2.6%) was lower than the rates (5.8%-15%) reported by other groups. 7,8,16,33,39 This suggests that EDAS is effective for pediatric MMD and can improve local cerebral hemodynamics, increase cerebral blood supply, reduce the risk of stroke, and improve neurological function. Longterm outcomes were better in patients without infarction and with lower Suzuki stages.…”

Section: Discussionmentioning

confidence: 99%

Encephaloduroarteriosynangiosis for pediatric moyamoya disease: long-term follow-up of 100 cases at a single center

Zhang

Bao

Duan

et al. 2018

Journal of Neurosurgery: Pediatrics

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OBJECTIVEThe object of this study was to summarize the long-term effect of encephaloduroarteriosynangiosis (EDAS) for the treatment of pediatric moyamoya disease (MMD) and to investigate factors influencing the clinical outcomes of EDAS.METHODSClinical features, angiographic findings, and clinical outcomes were analyzed among MMD patients younger than 18 years who had been treated with EDAS between 2002 and 2007 at the authors’ institution. The Kaplan-Meier method was used to estimate stroke risk after EDAS. Predictors of neurological outcome were assessed.RESULTSOne hundred fifteen patients were identified. The mean age at symptom onset was 7.3 ± 4.0 years. The incidence of familial MMD was 11.3%. The female/male ratio was 1:1.16. A total of 232 EDAS procedures were performed, and the incidence of postoperative complications was 3%. Postoperative digital subtraction angiography was performed in 54% of the patients, and about 80% of the hemispheres showed good or excellent results. Neovascularization showed significant correlations with delay time (from symptom onset to first operation), Suzuki stage, and preoperative stroke (all p < 0.05). Clinical follow-up was available in 100 patients with a mean follow-up of 124.4 ± 10.5 months. Ten-year cumulative survival was 96.5% after surgery, and the risk of stroke was 0.33%/person-year. An independent life with no significant disability was reported by 92% of the patients. A good outcome correlated with a low Suzuki stage (p = 0.001). Older children and those without preoperative stroke had better clinical outcomes (p < 0.05).CONCLUSIONSOn the basis of long-term follow-up data, the authors concluded that EDAS is a safe and effective treatment for pediatric MMD, can reduce the risk of subsequent neurological events, and can improve quality of life. The risk of ischemia-related complications was higher in younger patients, and older children showed better outcomes. Compensation was greater with more prominent cerebral ischemia. The long-term clinical outcome largely depended on the presence and extent of preoperative stroke.

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Section: Discussionmentioning

confidence: 99%

Encephaloduroarteriosynangiosis for pediatric moyamoya disease: long-term follow-up of 100 cases at a single center

Zhang

Bao

Duan

et al. 2018

Journal of Neurosurgery: Pediatrics

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“…Among United States adult case series, the clinical features variously include motor deficits, speech disturbances, headache, and seizure [5,10,23]. Surgical revascularization is the most frequent interventional procedure for MMD, but specific indications for treatment in adults are not well established [10,21,28].…”

Section: Introductionmentioning

confidence: 99%

Neurocognitive dysfunction in adult moyamoya disease

et al. 2009

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We wanted to determine the neurocognitive profile of adult patients with moyamoya disease prior to neurosurgical intervention. The experience of three United States medical centers, Columbia University, University of Illinois at Chicago, and the University of Texas Southwestern Medical Center at Dallas, were combined. Clinical data from adult patients (N = 29) referred for neuropsychological evaluation from 1996 to 2008 were reviewed. Neurocognitive functioning was assessed using standardized neuropsychological tests and all data were converted to z-scores. Memory, attention, processing speed, verbal memory, visuo-spatial, language, and executive functions were examined. Cognitive dysfunction was defined as performance in two or more cognitive domains 1.5 standard deviations below age-corrected normative means OR one or more cognitive domains two standard deviations below age-corrected normative means. Manual strength and dexterity, as well as depressive symptoms, were also assessed. Two-thirds of patients demonstrated neurocognitive dysfunction. A large proportion of patients were found to have pronounced cognitive dysfunction (>2 SD below the mean) on tests of processing speed (29%), verbal memory (31%), verbal fluency (26%) and executive function (25%). Manual strength and dexterity were also affected in many patients, with impairment found in 36-58% of patients. Twenty-eight percent of patients reported moderate to severe depression, but depressive symptoms did not correlate with neurocognitive findings. A large proportion of adults with moyamoya disease demonstrate disruption of neurocognition in a broad range of functions, particularly those mediated by subcortical and frontal regions. The pattern of deficits suggests a mechanism of diffuse small vessel disease possibly caused by chronic hypoperfusion.

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Clinical Features, Surgical Treatment, and Long-term Outcome of Adult Moyamoya Patients

Cited by 2 publications

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Encephaloduroarteriosynangiosis for pediatric moyamoya disease: long-term follow-up of 100 cases at a single center

Encephaloduroarteriosynangiosis for pediatric moyamoya disease: long-term follow-up of 100 cases at a single center

Neurocognitive dysfunction in adult moyamoya disease

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