Anesthesia Depresses Cerebrovascular Reactivity to Acetazolamide in Pediatric Moyamoya Vasculopathy

Measurements of cerebrovascular reactivity (CVR) are essential for treatment decisions in moyamoya vasculopathy (MMV). Since MMV patients are often young or cognitively impaired, anesthesia is commonly used to limit motion artifacts. Our aim was to investigate the effect of anesthesia on the CVR in pediatric MMV. We compared the CVR with multidelay-ASL and BOLD MRI, using acetazolamide as a vascular stimulus, in all awake and anesthesia pediatric MMV scans at our institution. Since a heterogeneity in disease and treatment influences the CVR, we focused on the (unaffected) cerebellum. Ten awake and nine anesthetized patients were included. The post-acetazolamide CBF and ASL-CVR were significantly lower in anesthesia patients (47.1 ± 15.4 vs. 61.4 ± 12.1, p = 0.04; 12.3 ± 8.4 vs. 23.7 ± 12.2 mL/100 g/min, p = 0.03, respectively). The final BOLD-CVR increase (0.39 ± 0.58 vs. 3.6 ± 1.2% BOLD-change (mean/SD), p < 0.0001), maximum slope of increase (0.0050 ± 0.0040%/s vs. 0.017 ± 0.0059%, p < 0.0001), and time to maximum BOLD-increase (~463 ± 136 and ~697 ± 144 s, p = 0.0028) were all significantly lower in the anesthesia group. We conclude that the response to acetazolamide is distinctively different between awake and anesthetized MMV patients, and we hypothesize that these findings can also apply to other diseases and methods of measuring CVR under anesthesia. Considering that treatment decisions heavily depend on CVR status, caution is warranted when assessing CVR under anesthesia

Cognitive functions in children and adults with Moyamoya Vasculopathy: A systematic review and meta-analysis

Background and Purpose Patients with moyamoya vasculopathy (MMV) may experience cognitive impairment, but its reported frequency, severity, and nature vary. In a systematic review and meta-analysis, we aimed to assess the presence, severity, and nature of cognitive impairments in children and adults with MMV. Methods We followed the MOOSE guidelines for meta-analysis and systematic reviews of observational studies. We searched Ovid Medline and Embase for studies published between January 1, 1969 and October 4, 2016. Independent reviewers extracted data for mean intelligence quotient (IQ) and standardized z-scores for cognitive tests, and determined percentages of children and adults with cognitive deficits, before and after conservative or surgical treatment. We explored associations between summary measures of study characteristics and cognitive impairments by linear regression analysis. Results We included 17 studies (11 studies reporting on 281 children, six on 153 adults). In children, the median percentage with impaired cognition was 30% (range, 13% to 67%); median IQ was 98 (range, 71 to 107). Median z-score was –0.39 for memory, and –0.43 for processing speed. In adults, the median percentage with impaired cognition was 31% (range, 0% to 69%); median IQ was 95 (range, 94 to 99). Median z-scores of cognitive domains were between –0.9 and –0.4, with multiple domains being affected. We could not identify determinants of cognitive impairment. Conclusions A large proportion of children and adults with MMV have cognitive impairment, with modest to large deficits across various cognitive domains. Further studies should investigate determinants of cognitive deficits and deterioration, and the influence of revascularization treatment on cognitive functioning

Locus coeruleus syndrome as a complication of tectal surgery

We describe a case of a 48-year-old woman who underwent a resection of a tectal pilocytic astrocytoma complicated by a sequence of fluctuating consciousness, psychosis with complex hallucinations and lasting sleeping disturbances in which she vividly acts out her dreams. Based on the clinical and anatomical evidence of this case, we propose the term locus coeruleus syndrome to describe this association of iatrogenic symptoms. Along with those of the locus coeruleus, lesions of the dorsal raphe nucleus, ventral tegmentum, substantia nigra pars compacta, the superior colliculus and other peduncular lesions (such as peduncular hallucinosis) are involved in the regulation of sleepwake/ arousal, behaviour, sleeping disorders and rapid eye movement atonia. However, iatrogenic lesion of the locus coeruleus could explain the complications on all levels in our patient

Patients with Moyamoya Vasculopathy Evaluated at a Single-Center in The Netherlands; Clinical Presentation and Outcome

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Contralateral improvement of cerebrovascular reactivity and TIA frequency after unilateral revascularization surgery in moyamoya vasculopathy

OBJECTIVE: Moyamoya vasculopathy is a rare, often bilateral disease characterized by progressive stenosis and occlusion of the distal internal carotid artery, leading to a progressive deterioration of cerebrovascular reactivity (CVR) and increased risk of transient ischemic attacks (TIAs), infarction and hemorrhage. Surgical revascularization is a widely accepted symptomatic treatment, often performed bilaterally in one or two stages. To possibly further optimize treatment strategy, we investigated the effect of unilateral revascularization surgery on the CVR of, and TIA frequency originating from, the contralateral hemisphere. METHODS: From our database of 143 moyamoya vasculopathy patients we selected those with bilateral disease, who underwent hemodynamic imaging ([15O]H2O positron emission tomography (PET)-CT with acetazolamide challenge) before and 14 months (median) after unilateral revascularization. We evaluated CVR in three regions per hemisphere, and averaged these per hemisphere for statistical comparison. Conservatively treated patients were showed as a comparison group. To examine TIA frequency, we selected patients who presented with TIAs that (also) originated from the contralateral - not to be operated - hemisphere. We scored changes in CVR and TIA frequency of the ipsilateral and contralateral hemisphere over time. RESULTS: Seven surgical and seven conservative patients were included for CVR comparison. Of the 20 scored contralateral regions in the surgical group, 15 showed improved CVR after unilateral revascularization, while 5 remained stable. The averaged scores improved significantly for both hemispheres. In conservatively treated patients, however, only 3 of the 20 scored regions improved in the least-affected (contralateral) hemispheres, and 9 deteriorated. From the 6 patients with contralateral TIAs at presentation, 4 had a decreased TIA frequency originating from the contralateral hemisphere after unilateral surgery, while 2 patients remained stable. CONCLUSION: Both CVR and TIA frequency in the contralateral hemisphere can improve after unilateral revascularization surgery in bilateral MMV

"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

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

Clinical Outcome, Cognition, and Cerebrovascular Reactivity after Surgical Treatment for Moyamoya Vasculopathy: A Dutch Prospective, Single-Center Cohort Study

Background: It remains unclear whether revascularization of moyamoya vasculopathy (MMV) has a positive effect on cognitive function. In this prospective, single-center study, we investigated the effect of revascularization on cognitive function in patients with MMV. We report clinical and radiological outcome parameters and the associations between clinical determinants and change in neurocognitive functioning. Methods: We consecutively included all MMV patients at a Dutch tertiary referral hospital who underwent pre- and postoperative standardized neuropsychological evaluation, [15O]H2O-PET (including cerebrovascular reactivity (CVR)), MRI, cerebral angiography, and completed standardized questionnaires on clinical outcome and quality of life (QOL). To explore the association between patient characteristics, imaging findings, and change in the z-scores of the cognitive domains, we used multivariable linear- and Bayesian regression analysis. Results: We included 40 patients of whom 35 (27 females, 21 children) were treated surgically. One patient died after surgery, and two withdrew from the study. TIA- and headache frequency and modified Rankin scale (mRS) improved (resp. p = 0.001, 0.019, 0.039). Eleven patients (seven children) developed a new infarct during follow-up (31%), five of which were symptomatic. CVR-scores improved significantly (p < 0.0005). The language domain improved (p = 0.029); other domains remained stable. In adults, there was an improvement in QOL. We could not find an association between change in imaging and cognitive scores. Conclusion: In this cohort of Western MMV patients, TIA frequency, headache, CVR, and mRS improved significantly after revascularization. The language domain significantly improved, while others remained stable. We could not find an association between changes in CVR and cognitive scores

Clinical Outcome, Cognition, and Cerebrovascular Reactivity after Surgical Treatment for Moyamoya Vasculopathy: A Dutch Prospective, Single-Center Cohort Study

Background: It remains unclear whether revascularization of moyamoya vasculopathy (MMV) has a positive effect on cognitive function. In this prospective, single-center study, we investigated the effect of revascularization on cognitive function in patients with MMV. We report clinical and radiological outcome parameters and the associations between clinical determinants and change in neurocognitive functioning. Methods: We consecutively included all MMV patients at a Dutch tertiary referral hospital who underwent pre- and postoperative standardized neuropsychological evaluation, [15O]H2O-PET (including cerebrovascular reactivity (CVR)), MRI, cerebral angiography, and completed standardized questionnaires on clinical outcome and quality of life (QOL). To explore the association between patient characteristics, imaging findings, and change in the z-scores of the cognitive domains, we used multivariable linear- and Bayesian regression analysis. Results: We included 40 patients of whom 35 (27 females, 21 children) were treated surgically. One patient died after surgery, and two withdrew from the study. TIA- and headache frequency and modified Rankin scale (mRS) improved (resp. p = 0.001, 0.019, 0.039). Eleven patients (seven children) developed a new infarct during follow-up (31%), five of which were symptomatic. CVR-scores improved significantly (p &lt; 0.0005). The language domain improved (p = 0.029); other domains remained stable. In adults, there was an improvement in QOL. We could not find an association between change in imaging and cognitive scores. Conclusion: In this cohort of Western MMV patients, TIA frequency, headache, CVR, and mRS improved significantly after revascularization. The language domain significantly improved, while others remained stable. We could not find an association between changes in CVR and cognitive scores