Evaluation of the contribution of individual arteries to the cerebral blood supply in patients with Moyamoya angiopathy: comparison of vessel-encoded arterial spin labeling and digital subtraction angiography.

PURPOSE: Vessel-encoded arterial spin labeling (VE-ASL) is able to provide noninvasive information about the contribution of individual arteries to the cerebral perfusion. The aim of this study was to compare VE-ASL to the diagnostic standard digital subtraction angiography (DSA) with respect to its ability to visualize vascular territories. METHODS: In total, 20 VE-ASL and DSA data sets of 17 patients with Moyamoya angiopathy with and without revascularization surgery were retrospectively analyzed. Two neuroradiologists independently assessed the agreement between VE-ASL and DSA using a 4-point Likert scale (no- very high agreement). Additionally, grading of the vascular supply of subterritories (A1-A2, M1-M6) on the VE-ASL images and angiograms was performed. The intermodal agreement was calculated for all subterritories in total and for the subdivision into without and after revascularization (direct or indirect bypass). RESULTS: There was a very high agreement between the VE-ASL and the DSA data sets (median = 1, modus = 1) with a substantial inter-rater agreement (kw = 0.762 (95% CI 0.561-0.963)). The inter-modality agreement between VE-ASL and DSA in vascular subterritories was almost perfect for all subterritories (k = 0.899 (0.865-0.945)), in the subgroup of direct revascularized subterritories (k = 0.827 (0.738-0.915)), in the subgroup of indirect revascularized subterritories (k = 0.843 (0.683-1.003)), and in the subgroup of never revascularized subterritories (k = 0.958 (0.899-1.017)). CONCLUSION: Vessel-encoded ASL seems to be a promising non-invasive method to depict the contributions of individual arteries to the cerebral perfusion before and after revascularization surgery.

3D exoscopic versus microscopic superficial temporal artery to middle cerebral artery bypass surgery for moyamoya disease - a comparative series.

PURPOSE: Superficial temporal artery to middle cerebral artery (STA-MCA) direct bypass surgery is the most common surgical procedure to treat moyamoya disease (MMD). Here, we aim to compare the performance of the 3D exoscope in bypass surgery with the gold standard operative microscope. METHODS: All direct STA-MCA bypass procedures performed at a single university hospital for MMD between 2015 and 2023 were considered for inclusion. Data were retrospectively collected from patient files and surgical video material. From 2020 onwards, bypass procedures were exclusively performed using a digital three-dimensional exoscope as visualization device. Results were compared with a microsurgical bypass control group (2015-2019). The primary endpoint was defined as total duration of surgery, duration of completing the vascular anastomosis (ischemia time), bypass patency, number of stiches to perform the anastomosis, added stiches after leakage testing of the anastomosis and the Glasgow outcome scale (GOS) at last follow-up as secondary outcome parameter. RESULTS: A total of 16 consecutive moyamoya patients underwent 21 STA-MCA bypass procedures. Thereof, six patients were operated using a microscope and ten patients using an exoscope (ORBEYE® n = 1; AEOS® n = 9). Total duration of surgery was comparable between devices (microscope: 313 min. ± 116 vs. exoscope: 279 min. ± 42; p = 0.647). Ischemia time also proved similar between groups (microscope: 43 min. ± 19 vs. exoscope: 41 min. ± 7; p = 0.701). No differences were noted in bypass patency rates. The number of stiches per anastomosis was similar between visualization devices (microscope: 17 ± 4 vs. exoscope: 17 ± 2; p = 0.887). In contrast, more additional stiches were needed in microscopic anastomoses after leakage testing the bypass (p = 0.035). CONCLUSION: Taking into account the small sample size, end-to-side bypass surgery for moyamoya disease using a foot switch-operated 3D exoscope was not associated with more complications and led to comparable clinical and radiological results as microscopic bypass surgery.

Risk Factors for Cerebrovascular Events in Moyamoya Angiopathy Using 4D Flow MRI: A Pilot Study.

BACKGROUND: Little is known about internal carotid artery (ICA) hemodynamics in patients with moyamoya angiopathy (MMA) and its role in cerebrovascular events. PURPOSE: To characterize ICA hemodynamics in MMA patients by 4D flow MRI and investigate its relationship with cerebrovascular events. STUDY TYPE: Prospective. SUBJECTS: Seventy MMA patients (50 years old ± 9, 30 males). FIELD STRENGTH/SEQUENCE: Time-resolved three-directional velocity encoded fast field echo sequence (4D flow) MRI, T1-weighted fast field echo sequence, T2 weighted turbo spin echo sequence, diffusion weighted echo planar imaging; T2-weighted fluid-attenuated inversion recovery turbo spin echo sequence, susceptibility weighted fast field echo sequence, and time-of-flight MR angiography fast field echo sequence at 3.0T. ASSESSMENT: ICA hemodynamics (maximum and average velocity [Vmax , Vavg ], average blood flow [Flowavg ], and wall shear stress) were analyzed based on 4D flow data. Cerebral infarction, defined as the occurrence of events, in 124 brain hemispheres was determined according to clinical symptoms and conventional brain MR imaging. STATISTICAL TESTS: The independent-samples T test was used to evaluate differences in ICA hemodynamics between infarcted and non-infarcted hemispheres. Binary logistic regression was performed to investigate the relationship between ICA hemodynamics and events. A P value < 0.05 was considered statistically significant. RESULTS: Sixty-one infarcted hemispheres (eight hemispheres with acute ischemic damage, 30 with chronic ischemic damage, and 23 with chronic hemorrhagic damage) had cerebrovascular events and 63 non-infarcted hemispheres did not. The hemodynamic parameters in the infarcted hemispheres (Vmax : P < 0.001; Vavg : P = 0.003; and Flowavg : P = 0.004) were significantly lower than those in the non-infarcted hemispheres. However, Vmax (P = 0.052), Vavg (P = 0.107), and Flowavg (P = 0.074) were not significantly different among hemispheres with acute ischemic lesions, chronic ischemic lesions and chronic hemorrhagic lesions. Vmax (odds ratio 3.033, 95% CI: 1.075-8.562) was independently associated with cerebrovascular events. DATA CONCLUSION: Vmax maybe a higher risk factor of cerebrovascular events in MMA patients. EVIDENCE LEVEL: 2 TECHNICAL EFFICACY STAGE: 3.

Evaluation of the cerebrovascular reactivity in patients with Moyamoya Angiopathy by use of breath-hold fMRI: investigation of voxel-wise hemodynamic delay correction in comparison to [15O]water PET.

PURPOSE: Patients with Moyamoya Angiopathy (MMA) require hemodynamic assessment to evaluate the risk of stroke. Hemodynamic evaluation by use of breath-hold-triggered fMRI (bh-fMRI) was proposed as a readily available alternative to the diagnostic standard [15O]water PET. Recent studies suggest voxel-wise hemodynamic delay correction in hypercapnia-triggered fMRI. The aim of this study was to evaluate the effect of delay correction of bh-fMRI in patients with MMA and to compare the results with [15O]water PET. METHODS: bh-fMRI data sets of 22 patients with MMA were evaluated without and with voxel-wise delay correction within different shift ranges and compared to the corresponding [15O]water PET data sets. The effects were evaluated combined and in subgroups of data sets with most severely impaired CVR (apparent steal phenomenon), data sets with territorial time delay, and data sets with neither steal phenomenon nor delay between vascular territories. RESULTS: The study revealed a high mean cross-correlation (r = 0.79, p < 0.001) between bh-fMRI and [15O]water PET. The correlation was strongly dependent on the choice of the shift range. Overall, no shift range revealed a significantly improved correlation between bh-fMRI and [15O]water PET compared to the correlation without delay correction. Delay correction within shift ranges with positive high high cutoff revealed a lower agreement between bh-fMRI and PET overall and in all subgroups. CONCLUSION: Voxel-wise delay correction, in particular with shift ranges with high cutoff, should be used critically as it can lead to false-negative results in regions with impaired CVR and a lower correlation to the diagnostic standard [15O]water PET.

How to reduce the complication rate of multiple burr holes surgery in moyamoya angiopathy.

PURPOSE: This study is aimed at analyzing clinical outcome, absence of stroke recurrence, revascularization, and complications and long-term follow-up in the surgical treatment of moyamoya angiopathy (MMA) using the multiple burr holes (MBH) technique with dura opening and arachnoid preservation as a single procedure. To the best of our knowledge, this is the first to describe an MBH technique with arachnoid preservation. METHOD: We retrospectively reviewed all patients operated from June 2001 to March 2021, for a symptomatic and progressive MMA operated with opening of the dura but arachnoid preservation. Clinical examinations were obtained in all patients, and radiological monitoring was performed by cerebral 3D-magnetic resonance angiography (MRA) with perfusion or single-photon emission computed tomography (SPECT) with acetazolamide. RESULTS: In total, 21 consecutive patients (6 children and 15 adults) were included with a mean age of 7.4 years in the pediatric group and 36.9 years in the adult group. Initial presentation was permanent ischemic stroke in 15 cases, transient ischemic attack (TIA) in 5 cases, and cerebral hemorrhage in one case. The MBH with dura opening and arachnoid preservation was performed bilaterally in 9 cases (43%) and unilaterally in 12 cases (57%). One patient died due to intraoperative bilateral ischemic stroke. Of the 20 other patients, 30% demonstrated clinical stability and 70% showed partial or complete recovery. Although one patient experienced a perioperative stroke, we did not observe any pseudomeningocele or postoperative ischemic stroke (IS) recurrence in all surviving cases during the average follow-up period of 55.5 months (range: 1-195). These outcomes emphasize the importance of preoperative monitoring to ensure the effectiveness and safety of the intervention. Postoperative angiography studies showed revascularization in 96.3% of treated hemispheres (100% in the adult group vs 80% in the pediatric group). CONCLUSIONS: Our results on this small cohort suggest that the MBH technique with opening of the dura and arachnoids preservation can prevent recurrent strokes and reduce the risk of pseudomeningocele.

Evaluation and treatment of headache associated with moyamoya disease - a narrative review.

BACKGROUND: Headache in patients with moyamoya disease is an under-addressed topic in the medical literature. Delay in the diagnosis of moyamoya disease or inappropriate treatment of headache could lead to devastating cerebrovascular outcome. With the evolving understanding of moyamoya disease, migraine pathophysiology, and various migraine-specific medications that have become available, it is crucial to provide an updated overview on this topic. METHODS: We searched PubMed for keywords including moyamoya disease, moyamoya syndrome, headache in moyamoya, surgical revascularization, surgical bypass, migraine and moyamoya, and calcitonin gene-related peptide (CGRP). We summarized the literature and provide a comprehensive review of the headache presentation, possible mechanisms, the impact of various surgical revascularizations on headache in patients with moyamoya disease, and the medical management of headache incorporating novel migraine-specific treatments.Results and conclusion: The most common headache phenotype is migraine; tension-type headache, hemiplegic migraine, and cluster headache have also been reported. Most patients experience improvement of headache after surgical revascularization, though some patients report worsening, or new-onset headache after surgery. Given the complexity of moyamoya disease, careful consideration of different types of medical therapy for headache is necessary to improve the quality of life while not increasing the risk of adverse cerebrovascular events. More prospective studies are warranted to better understand and manage headache in patients with moyamoya disease.

Hemodynamic evaluation of patients with Moyamoya Angiopathy: comparison of resting-state fMRI to breath-hold fMRI and [15O]water PET.

PURPOSE: Patients with Moyamoya Angiopathy (MMA) require hemodynamic evaluation to assess the risk of stroke. Assessment of cerebral blood flow with [15O]water PET and acetazolamide challenge is the diagnostic standard for the evaluation of the cerebral perfusion reserve (CPR). Estimation of the cerebrovascular reactivity (CVR) by use of breath-hold-triggered fMRI (bh-fMRI) as an index of CPR has been proposed as a reliable and more readily available approach. Recent findings suggest the use of resting-state fMRI (rs-fMRI) which requires minimum patient compliance. The aim of this study was to compare rs-fMRI to bh-fMRI and [15O]water PET in patients with MMA. METHODS: Patients with MMA underwent rs-fMRI and bh-fMRI in the same MRI session. Maps of the CVR gained by both modalities were compared retrospectively by calculating the correlation between the mean CVR of 12 volumes of interest. Additionally, the rs-maps of a subgroup of patients were compared to CPR-maps gained by [15O]water PET. RESULTS: The comparison of the rs-maps and the bh-maps of 24 patients revealed a good correlation (Pearson's r = 0.71 ± 0.13; preoperative patients: Pearson's r = 0.71 ± 0.17; postoperative patients: Pearson's r = 0.71 ± 0.11). The comparison of 7 rs-fMRI data sets to the corresponding [15O]water PET data sets also revealed a high level of agreement (Pearson's r = 0.80 ± 0.19). CONCLUSION: The present analysis indicates that rs-fMRI might be a promising non-invasive method with almost no patient cooperation needed to evaluate the CVR. Further prospective studies are required.

Moyamoya angiopathy in a case of Klinefelter syndrome.

Moyamoya angiopathy, a rare cerebrovascular condition, can be primary (moyamoya disease) or secondary (moyamoya syndrome). Genetic factors, such as the ring finger protein 213 (RNF213), have been associated with moyamoya disease. However, X-linked moyamoya angiopathy/moyamoya syndrome and hypergonadotropic hypogonadism associated with moyamoya syndrome are rare. We report a case of a 14-year-old boy who presented with transient bilateral hemiparesis, recurrent seizures and cognitive decline. He previously had surgery for left-sided cryptorchidism and had been diagnosed with "epileptic attacks" or "functional movement disorders" in previous hospital admissions. Magnetic resonance angiography of the brain showed narrowing of supraclinoid portion of internal carotid arteries, as well as of middle and anterior cerebral arteries, and the presence of multiple collaterals. These findings were suggestive of moyamoya angiopathy. Laboratory investigations and karyotyping revealed a diagnosis of Klinefelter syndrome. This case presents a unique association of moyamoya angiopathy and Klinefelter syndrome in a boy from a poor socio-economic background, where the diagnosis and adequate treatment were delayed due to a lack of awareness and expertise.

"Asymptomatic" Moyamoya Angiopathy: Is it Truly Asymptomatic?

INTRODUCTION: An ambiguous definition of "asymptomatic" Moyamoya Angiopathy(aMMA) of absence of ischemic/hemorrhagic episodes in MMA patients, has led to its variable adaptation in the limited past-studies. OBJECTIVE: To observe the clinic-radiological characteristics and prospective follow-up of apparently "asymptomatic" MMA, and to determine if it is truly asymptomatic or not. MATERIALS AND METHODS: An observation, cohort study of 122 angiographically proven MMA over 6 years was undertaken from a single, tertiary-care-center to observe the clinico-radiological characteristics, prospective follow-up of apparently aMMA. Amongst them, 6 had an initial diagnosis of aMMA following evaluation by atleast one post-graduate doctor, which were further scrutinized by 3 different neurologists for epidemiological, clinical, radiological characteristics and subsequent follow-up. Data were analyzed using descriptive statistics. RESULTS: Mean age was 23.7 ± 13.14 years. 3 of 6 underwent brain-imaging for evaluation of non-migraine-like headache, 1 for dizziness, 2 as part of familial screening for MMA. 4 of 6 patients had specific-triggers for aggravation of symptoms. Brain-imaging revealed old vascular insults and ivy sign in 5 of 6 each (83.3%), mean suzuki staging was 3.6±0.82. 4 of 6 underwent cerebral perfusion study, all had hypoperfusion. Revascularization surgery was done in 2 of 6, rest were managed conservatively. None had any new-onset neurological deficit or radiological progression over a mean follow-up period of 22.3 ± 20.22 months. CONCLUSIONS: Apparently aMMA may not be truly asymptomatic and often have subtle "paroxysmal events" precipitated by specific-triggers, indicative of transient ischemic symptoms. Thus, warrants for a more precise definition to avoid misclassification of aMMA.

Capillary microscopy in Europeans with idiopathic Moyamoya angiopathy.

OBJECTIVE: In Europe, MMA is a very rare non-inflammatory vasculopathy. MMA is an important differential diagnosis of cerebral vasculitis. Systemic manifestations, such as livedo racemosa or renal artery stenosis, associated with Moyamoya variants suggest the involvement also of non-cerebral vessels. Hypothetically, capillary microscopy could be a promising non-invasive screening method to visualize microcirculation, for example prior to cerebral angiography. METHODS: Standardized capillary microscopic images were taken in European patients with MMA and subsequently evaluated in a blinded analysis, using data obtained from a large NP cohort and a large SLE cohort by the same blinded Investigator as controls. RESULTS: Twenty-four European MMD patients and 14 healthy accompanying controls were included in this study. The results were compared to 116 SLE patients and 754 NP subjects. In MMD patients, no capillary morphological differences were found in comparison with NP, in particular no density reduction or increased neoangiogenesis. The pattern observed in the SLE cohort was clearly distinct from NP and MMD with regard to vascular density, vascular damage, and neoangiogenesis. CONCLUSIONS: MMD is not associated with microvascular changes of the nailfold capillaries. In this respect, it is clearly distinct from SLE.

The pleiotropy associated with de novo variants in CHD4, CNOT3, and SETD5 extends to moyamoya angiopathy.

PURPOSE: Moyamoya angiopathy (MMA) is a cerebrovascular disease characterized by occlusion of large arteries, which leads to strokes starting in childhood. Twelve altered genes predispose to MMA but the majority of cases of European descent do not have an identified genetic trigger. METHODS: Exome sequencing from 39 trios were analyzed. RESULTS: We identified four de novo variants in three genes not previously associated with MMA: CHD4, CNOT3, and SETD5. Identification of additional rare variants in these genes in 158 unrelated MMA probands provided further support that rare pathogenic variants in CHD4 and CNOT3 predispose to MMA. Previous studies identified de novo variants in these genes in children with developmental disorders (DD), intellectual disability, and congenital heart disease. CONCLUSION: These genes encode proteins involved in chromatin remodeling, and taken together with previously reported genes leading to MMA-like cerebrovascular occlusive disease (YY1AP1, SMARCAL1), implicate disrupted chromatin remodeling as a molecular pathway predisposing to early onset, large artery occlusive cerebrovascular disease. Furthermore, these data expand the spectrum of phenotypic pleiotropy due to alterations of CHD4, CNOT3, and SETD5 beyond DD to later onset disease in the cerebrovascular arteries and emphasize the need to assess clinical complications into adulthood for genes associated with DD.

Decreased frontal white-matter diffusion and improved cognitive flexibility after burr-hole surgery in moyamoya angiopathy.

BACKGROUND: In Moyamoya Angioplasty (MMA), increased apparent diffusion coefficient (ADC) in frontal white matter (WM) with a normal appearance has been associated with frontal hypoperfusion and executive dysfunction. Multiple burr-hole surgery enables the revascularization of large frontal areas. GOAL: To assess the effect of multiple burr-hole surgery on the ADC and cognitive functions in adults with MMA. METHODS: ADC was measured in 26 brain hemispheres of 14 consecutive adults with MMA (9 women, mean age ± SD: 38.1 ± 10.7 years) prior to and 6 months after burr-hole surgery. ADC was obtained from regions of interest located in frontal and posterior (temporo-occipital) normal-appearing WM. Ten patients had neuropsychological assessment that focused on executive and attentional functions before and after surgery. RESULTS: Anterior and posterior ADC values did not differ before surgery (815.8 ± 60.1 vs. 812.1 ± 35.3 mm2/s, p = 0.88). After surgery, frontal ADC was lower than prior to surgery (789.9 ± 64.5 vs. 815.8 ± 60.1 mm2/s; p <0.001) whereas no change occurred in posterior ADC (p = 0.31). Trail-making test part B median z-score increased from - 1.47 to - 0.21 (p = 0.018), suggesting improved cognitive flexibility. CONCLUSION: In adults with MMA, indirect revascularization with burr-hole is followed by a decrease of ADC in normal-appearing frontal WM and may have improved some executive functions in the flexibility process. Change in ADC may reflect the improvement in cerebral perfusion after surgery. The measuring of ADC may be a promising tool in exploring potentially reversible microstructural WM damage related to hypoperfusion and cognitive change in MMA.

The disease presentation of Moyamoya angiopathy in Eastern India.

INTRODUCTION: Clinical spectrum of Moyamoya angiopathy (MMA) differs across populations with different ethnicity. This study, the largest one done among Indian population was undertaken to assess clinico-radiological profile of MMA patients in eastern India. METHODS: A single centre cross-sectional study was undertaken among 76 MMA cases. Each patient was evaluated for epidemiological, clinical and radiological characteristics. SPSS 25 was used for statistical analysis. P < 0.05 was taken as statistically significant. RESULTS: 36 (47.4%) were children without gender preponderance. There were female predominance among adults (male:female = 1:2.33). Mean age at onset of first neurological symptoms for children was 4.2 ± 2.0years, followed by 34.9 ± 58.2months of latency with final diagnosis at the mean age of 7.4 ± 3.5years. For adults, mean age of onset of first neurological symptoms was 31.5 ± 12.3years, followed by 14.7 ± 41.7months time gap and diagnosed at the mean age of 33.5 ± 12.5years. There was a statistically significant difference between child and adult regarding the diagnostic latency (p = 0.035). Fixed motor weakness (FMW) was the predominant symptom across the whole disease course. Among children predominant first neurological symptom was fixed motor weakness (FMW) (52.8%), followed by seizures (22.2%). FMW was predominant (55%) first neurological complaint, followed by headache (22.5%) among adults. Seizure was more prevalent among children both as first (p = 0.002) and presenting symptom at the time of diagnosis (p = 0.048). Over the course of the disease seizure was more common among children (p = 0.001), while headache was more common among adults (p = 0.017). Recurrence of symptoms was more common among children (p = 0.059). Infarcts were more common among children (91.7%) than adults (72.5%), while hemorrhage was seen only among adults (25%) (p = 0.004). Isolated cerebral cortex was involved more commonly among children (59.4%) than adults (36.1%), while isolated subcortical involvement was seen only among adults (19.4%) (p = 0.016). Majority of the MMA cases were of Suzuki stage 4 (39.5%) and 5 (27.6%). Brain atrophy was associated with diagnostic latency (p = 0.009). CONCLUSION: Indian Moyamoya presents similar to disease presentation in Caucasian and Japanese patients. It is a frequently overlooked cause of stroke in young, often with various non-motor presentations, failure to recognize which leads to delay in diagnosis. Radiological burden disproportionate to number of acute vascular events, with subtle neurological manifestations like headache or seizure, often with cognitive decline, should raise suspicion of MMA.

Vascular Remodeling in Moyamoya Angiopathy: From Peripheral Blood Mononuclear Cells to Endothelial Cells.

The pathophysiological mechanisms of Moyamoya angiopathy (MA), which is a rare cerebrovascular condition characterized by recurrent ischemic/hemorrhagic strokes, are still largely unknown. An imbalance of vasculogenic/angiogenic mechanisms has been proposed as one possible disease aspect. Circulating endothelial progenitor cells (cEPCs) have been hypothesized to contribute to vascular remodeling of MA, but it remains unclear whether they might be considered a disease effect or have a role in disease pathogenesis. The aim of the present study was to provide a morphological, phenotypical, and functional characterization of the cEPCs from MA patients to uncover their role in the disease pathophysiology. cEPCs were identified from whole blood as CD45dimCD34+CD133+ mononuclear cells. Morphological, biochemical, and functional assays were performed to characterize cEPCs. A significant reduced level of cEPCs was found in blood samples collected from a homogeneous group of adult (mean age 46.86 ± 11.7; 86.36% females), Caucasian, non-operated MA patients with respect to healthy donors (HD; p = 0.032). Since no difference in cEPC characteristics and functionality was observed between MA patients and HD, a defective recruitment mechanism could be involved in the disease pathophysiology. Collectively, our results suggest that cEPC level more than endothelial progenitor cell (EPC) functionality seems to be a potential marker of MA. The validation of our results on a larger population and the correlation with clinical data as well as the use of more complex cellular model could help our understanding of EPC role in MA pathophysiology.

Moyamoya angiopathy in PHACE syndrome not associated with RNF213 variants.

Moyamoya angiopathy is a rare vasculopathy with stenosis and/or occlusion of bilateral intracranial parts of internal carotid arteries and/or proximal parts of middle and anterior cerebral arteries. PHACE syndrome is characterized by large segmental hemangiomas in the cervical-facial region. Both conditions are known to be associated in rare cases. Recently, it was discussed in the literature that RNF213 variants could be etiologically involved in this association. Here, we describe a childhood case with this rare co-occurrence in which we did not identify any rare RNF213 variant. The clinical and genetic backgrounds are discussed.

Headache in Caucasian patients with Moyamoya angiopathy - a systematic cohort study.

Background Headache is common in patients with Moyanoya angiopathy (MMA), but usually underestimated in its management and not well characterized. Methods A validated self-administered headache screening questionnaire and a telephone interview were used in order to investigate headache characteristics, frequency and pain intensity in a large cohort of 55 German patients with MMA. Results Thirty-seven patients (67.3%) had suffered from headache in the past year. Headache intensity was rated 3.2 ± 1.3 on a verbal rating scale from 0 to 10. Seventeen patients (47.9%) reported migraine-like headache, 10 patients (27.0%) reported tension type-like headache and 10 patients (27.0%) had a combination of both. The majority of patients with migraine-like headache ( n = 10, 58.8%) described migrainous aura. Headache frequency and intensity improved significantly after revascularization surgery; however, nine patients developed new-onset headache postoperatively. Conclusion Headache is very common in MMA, often with a migraine-like phenotype. Tension type-like headache was also found in 27% of patients, which is a new finding that has not been reported before.

[Moyamoya disease in adults: treatment methods in modern era]. / Sovremennyi podkhod k lecheniyu angiopatii moyamoya u vzroslykh.

Moyamoya angiopathy is a chronic progressive cerebrovascular disease characterized by stenosis and occlusion of the distal segments of the internal carotid arteries and/or proximal segments of the middle and anterior cerebral arteries, with a gradual compensatory restructuring of the cerebral circulation to the system of the external carotid arteries. Today, the main treatment method for Moyamoya angiopathy is surgical revascularization of the brain. A search and analysis of publications on the treatment of adult patients with Moyamoya angiopathy was carried out in the PubMed and Medscape databases over the past 10 years. We present a case of an adult female patient with a hemorrhagic form of Moyamoya angiopathy stage IV according to J. Suzuki, who underwent staged combined revascularization of both cerebral hemispheres. Surgical revascularization included the creation of a low-flow extra-intracranial shunt combined with a combination of indirect synangiosis. The combination of direct and indirect methods of surgical revascularization enables to achieve the development of an extensive network of collaterals and fully compensate for cerebral circulatory disorders both in the early and late postoperative periods, which is confirmed by instrumental diagnostic data. Combined revascularization is the most effective modern method of treating patients with Moyamoya angiopathy due to the complementary influence of direct and indirect components of revascularization.

Low Incidence of Neurological Adverse Events Among Pediatric Patients With Moyamoya Undergoing General Anesthesia for NonRevascularization Procedures.

BACKGROUND: General anesthesia poses a significant risk for patients with MMA, yet its risk in the setting of nonrevascularization procedures remains unclear. Data regarding the risk of general anesthesia for nonrevascularization procedures are scarce. We therefore aimed to assess the incidence of neurological adverse events (NAEs) among pediatric patients with MMA undergoing general anesthesia for nonrevascularization procedures. METHODS: We conducted a retrospective cohort study at a tertiary referral pediatric center of patients with MMA aged ≤18 years, who underwent general anesthesia for nonrevascularization procedures between January 2014 and July 2023. Postanesthesia NAEs were defined as occurrence of transient ischemic attacks, seizures, altered mental status, severe headache, or evidence of arterial ischemic stroke (AIS) in the 30 days postprocedure. RESULTS: Among 149 procedures on 38 patients (median age [interquartile range]: 8.3 [5.0, 12.7] years; 57% female), 124 (83.2%) procedures were imaging studies and angiographies and 25 (16.8%) were surgical procedures. Preprocedural hyperhydration treatment was administered before most (111, 74.5%) procedures per our institutional protocol. The incidence of postanesthesia NAEs was 0.67% (one of 149), as a result of acute AIS following a ventriculoperitoneal shunt revision surgery, in a patient with postradiation MMA, panhypopituitarism, and uncontrolled diabetes insipidus, despite preprocedural hyperhydration treatment. There were no NAEs after imaging studies performed under general anesthesia. CONCLUSIONS: Our results suggest that general anesthesia for nonrevascularization procedures in pediatric patients with MMA prepared with hyperhydration is safe. As neuroradiological follow-up is central in children with MMA, this information can be valuable for reassuring patients and their families.

MRI hypoperfusion as a determinant of cognitive impairment in adults with Moyamoya angiopathy.

INTRODUCTION: In Moyamoya angiopathy (MMA), mechanisms underlying cognitive impairment remain debated. We aimed to assess the association of cognitive impairment with the degree and the topography of cerebral hypoperfusion in MMA. METHODS: A retrospective analysis of neuropsychological and perfusion MRI data from adults with MMA was performed. Ischemic and haemorrhagic lesion masks were created to account for cerebral lesions in the analysis of cerebral perfusion. Whole brain volume of hypoperfused parenchyma was outlined on perfusion maps using different Tmax thresholds from 4 to 12 s. Regional analysis produced mean Tmax values at different regions of interest. Analyses compared perfusion ratios in patients with and without cognitive impairment, with multivariable logistic regression analysis to identify predictive factors. RESULTS: Cognitive impairment was found in 20/48 (41.7%) patients. Attention/processing speed and memory were equally impaired (24%) followed by executive domain (23%). After adjustment, especially for lesion volume, hypoperfused parenchyma volume outlined by Tmax > 4 s or Tmax > 5 s thresholds was an independent factor of cognitive impairment (OR for Tmax > 4 s = 1.06 [CI 95% 1.008-1.123]) as well as attention/processing speed (OR for Tmax > 4 s = 1.07 [CI 95% 1.003-1.133]) and executive domains (OR for Tmax > 5 s = 1.08 [CI 95% 1.004-1.158]). Regarding cognitive functions, patients with processing speed and flexibility impairment had higher frontal Tmax compared to other ROIs and to patients with normal test scores. DISCUSSION: Cerebral hypoperfusion emerged as an independent factor of cognitive impairment in MMA particularly in attention/processing speed and executive domains, with a strong contribution of frontal areas. CONCLUSION: Considering this association, revascularization surgery could improve cognitive impairment.