Moyamoya disease: functional and neurocognitive outcomes in the pediatric and adult populations

Weinberg, David G.; Rahme, Rudy J.; Aoun, Salah G.; Batjer, H. Hunt; Bendok, Bernard R.

doi:10.3171/2011.3.focus1150

Cited by 64 publications

(63 citation statements)

References 57 publications

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“…2,13,16,21,32 MMD has variable clinical presentations. The disease causes ischemic stroke, intracranial hemorrhage, headache, seizures, and transient ischemic attacks (TIAs).…”

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confidence: 99%

Different aspects of dysexecutive syndrome in patients with moyamoya disease and its clinical subtypes

Fang

Hua

Zhang

et al. 2016

JNS

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obJective Dysexecutive syndrome is common in patients with moyamoya disease (MMD), a chronic cerebrovascular disease that is characterized by stenosis of the bilateral internal carotid arteries and progressive collateral revascularization, and MMD can be classified as ischemic or hemorrhagic according to the disease presentation and history. In this study, the authors aimed to determine which aspects of executive function are impaired in patients with MMD, in addition to the specific dysexecutive functions present among its clinical subtypes and the mechanisms underlying dysexecutive function in these patients. [SART]) to 49 patients with MMD and 47 IQ-, age-, education-, and social status-matched healthy controls. The dysexecutive questionnaire (DEX) was also used to assess participants' subjective feelings about their executive function. A total of 39 of the patients were evaluated by CT perfusion (CTP) before the assessments were performed, and the correlations among the performances of the patients on the above tests with the parameters of cerebral blood volume, cerebral blood flow (CBF), mean transit time (MTT), and time-to-peak (TTP) in the frontal lobes of these patients were also analyzed. results Many aspects of executive function in the patients with MMD were significantly poorer than those in the healthy controls, and the patients performed particularly poorer on the VF test, HSCT, N-back test, and SART. The patients with hemorrhagic MMD exhibited worse executive inhibition, executive processing, and semantic inhibition compared with those with ischemic MMD, but the latter group presented a worse working memory and poorer sustained attention. There were no significant differences in the DEX scores between the patients with MMD and healthy controls. The other findings were as follows: CBF was significantly positively correlated with the number correct on part B of the HSCT (r = 0.481, p = 0.01) and accuracy on the 0-back task of the N-back (r = 0.346, p = 0.031); MTT was significantly positively correlated with accuracy on the 2-back task of the N-back (r = 0.349, p = 0.034) and factor 5 of the DEX (r = 0.359, p = 0.032); and TTP was significantly positively correlated with the number correct on part B of the HSCT (r = 0.402, p = 0.034) and the 1-back reaction time of the N-back (r = 0.356, p = 0.026). coNclusioNs The patients with MMD exhibited impairments in semantic inhibition, executive processing, working memory, and sustained attention, but they were not aware of these deficits. Moreover, differences in dysexecutive function existed between the different subtypes of MMD. Hypoperfusion of the frontal lobe may be related to working memory and semantic inhibition impairments in patients with MMD.

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“…2,13,16,21,32 MMD has variable clinical presentations. The disease causes ischemic stroke, intracranial hemorrhage, headache, seizures, and transient ischemic attacks (TIAs).…”

mentioning

confidence: 99%

Different aspects of dysexecutive syndrome in patients with moyamoya disease and its clinical subtypes

Fang

Hua

Zhang

et al. 2016

JNS

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“…[18,24] In this report we described a cerebral revascularization technique for patients with MMV which enables increasing CBF in three different vascular territories. The described procedure permits to: 1) achieve an immediate unilateral MCA flow augmentation by performing a STA-MCA bypass and reinforce the blood supply by promoting neoangiogenesis in the MCA territory by means of EDMS, and 2) revascularize the ACA territories bilaterally by means of EDPS.…”

Section: Discussionmentioning

confidence: 99%

“…Next to revascularization in the MCA territory, re-establishing CBF in the anterior cerebral artery (ACA) area is most likely to be of importance, in particular in children with MMV [20,[23][24]; neurocognitive development has been correlated to frontal lobe CBF. [18,24] In this report we described a cerebral revascularization technique for patients with MMV which enables increasing CBF in three different vascular territories.…”

Section: Discussionmentioning

confidence: 99%

“…However, revascularization in the frontal territory is receiving gradually more attention: a considerable part of the ischemic presentation of (pediatric) moyamoya patients consists of neurocognitive disorders as well as lower extremity function, caused by frontal hypoperfusion. [2,5,12,24] Re-establishing cerebral blood flow (CBF) in the frontal territory may prevent, stabilize or improve neurocognitive decline. [6][7]15,[17][18][19][20]24] In this paper, we describe a combined one-staged procedure for revascularization of the MCA territory unilaterally by a direct STA-MCA bypass and the bifrontal territory by an encephalo-duro-periosteal-synangiosis (EDPS).…”

Section: Introductionmentioning

confidence: 99%

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Combined Bypass Technique for Contemporary Revascularization of Unilateral MCA and Bilateral Frontal Territories in Moyamoya Vasculopathy

Kronenburg

Esposito

Fierstra

et al. 2014

Acta Neurochirurgica Supplement

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Moyamoya vasculopathy (MMV) leads to chronic hypoperfusion predominantly in the middle cerebral artery (MCA) and anterior cerebral artery (ACA) territories. Most revascularization techniques focus on revascularization of the MCA territory. Augmentation of blood flow in the frontal area is important for neurocognition and lower extremity function. In this article we describe a new combined (direct and indirect) one-stage bypass technique consisting of a superficial temporal artery to middle cerebral artery (STA-MCA) bypass with encephalo-duro-synangiosis (EDS) for unilateral MCA revascularization, along with an encephalo-duro-periosteal-synangiosis (EDPS) for bifrontal blood flow augmentation. The strength of this technique is the revascularization of three vascular territories during a single surgical intervention: the MCA unilaterally; and the frontal territories bilaterally. Bifrontal EDPS may also be considered as a supplementary independent procedure for patients who previously underwent revascularization treatment in the MCA territory, but develop symptoms due to frontal hypoperfusion. bypass with an encephalo-duro-myo-synangiosis (EDMS) for unilateral MCA revascularization, along with a encephalo-duro-periosteal-synangiosis (EDPS) for bifrontal blood flow augmentation. The strength of this technique is the revascularization of 3 vascular territories during a single surgical intervention: the MCA unilaterally and the frontal territories bilaterally. Bifrontal EDPS can also be considered as a supplementary independent procedure for patients who previously underwent revascularization treatment in the MCA territory, but develop symptoms due to frontal hypoperfusion.

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“…[2][3][4][5][6][7] In addition to symptoms that can be ascribed to the MCA territory, children may present with lower extremity motor weakness and neuropsychological dysfunctions probably due to involvement of the frontal lobes. [8][9][10][11][12] In pediatric MMV, the cerebral blood flow (CBF) in the bifrontal areas as well as in the anterior watershed territory may continue to worsen despite good collateral formation or successful revascularization of the MCA territory. [9][10][11][12] Therefore, it is important to consider timely revascularization of the frontal areas, to prevent neurocognitive decline in pediatric patients.…”

Section: Introductionmentioning

confidence: 99%

“STA-MCA bypass with encephalo-duro-myo-synangiosis combined with bifrontal encephalo-duro-periosteal-synangiosis” as a one-staged revascularization strategy for pediatric moyamoya vasculopathy

et al. 2015

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PURPOSE Moyamoya vasculopathy progressively compromises cerebral blood flow resulting in chronic hypoperfusion. The middle cerebral artery (MCA) territory and the bifrontal areas are the regions most frequently affected. Although most techniques aim to only revascularize the MCA territory, augmentation of blood flow of the bifrontal areas is of importance in the pediatric moyamoya population since these regions play an important role in cognition, intellectual development, and in lower extremity and sphincter function. We recently described a one-staged surgical procedure combining revascularization of three regions, the MCA territory unilaterally and the frontal areas bilaterally. The purpose of this article is to report our surgical experience in eight children and to emphasize the rational for bifrontal revascularization. METHODS We report a case series consisting of eight children where the following surgical strategy was applied: (1) a direct superficial temporal artery-to-middle cerebral artery (STA-MCA) bypass with encephalo-duro-myo-synangiosis (EDMS) for unilateral MCA revascularization; in combination with (2) a bifrontal encephalo-duro-periosteal-synangiosis (EDPS) for bifrontal revascularization. Patients' characteristics and 30-day follow-up data are reported. RESULTS The patient group consisted of six girls and two boys (mean age 10.0, range 4.2-17.5 years): six children presented with moyamoya disease, two with moyamoya syndrome. We performed a one-staged revascularization of one MCA territory and both frontal areas in all patients. No significant complications occurred. Two patients experienced postoperative focal seizures, successfully treated with anti-epileptic medication. CONCLUSIONS The single-staged STA-MCA bypass with EDMS combined with bifrontal EDPS allowed revascularization of three regions (the MCA territory unilaterally and the frontal areas bilaterally) and may serve as an alternative and safe treatment option for pediatric moyamoya patients. Originally published at: Esposito, Giuseppe; Kronenburg, Annick; Fierstra, Jorn; Braun, Kees P J; Klijn, Catharina J M; van der Zwan, Albert; Regli, Luca (2015). "STA-MCA bypass with encephalo-duro-myo-synangiosis combined with bifrontal encephalo-duro-periosteal-synangiosis" as a one-staged revascularization strategy for pediatric moyamoya vasculopathy. Child's Nervous System, 31 (5) hypoperfusion. The middle cerebral artery (MCA) territory and the bifrontal areas are the regions most frequently affected. Although most techniques aim to only revascularize the MCA territory, augmentation of blood flow of the bifrontal areas is of importance in the pediatric moyamoya population since these regions play an important role in cognition, intellectual development, and in lower extremity and sphincter function. We recently described a one-staged surgical procedure combining revascularization of three regions, the MCA territory unilaterally and the frontal areas bilaterally. The purpose of this article is to report our surgical experience in e...

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Moyamoya disease: functional and neurocognitive outcomes in the pediatric and adult populations

Cited by 64 publications

References 57 publications

Different aspects of dysexecutive syndrome in patients with moyamoya disease and its clinical subtypes

Different aspects of dysexecutive syndrome in patients with moyamoya disease and its clinical subtypes

Combined Bypass Technique for Contemporary Revascularization of Unilateral MCA and Bilateral Frontal Territories in Moyamoya Vasculopathy

“STA-MCA bypass with encephalo-duro-myo-synangiosis combined with bifrontal encephalo-duro-periosteal-synangiosis” as a one-staged revascularization strategy for pediatric moyamoya vasculopathy

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