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BackgroundCOVID-19 mediated immune dysregulation and cytokine storm can precipitate and aggravate Moyamoya angiopathy (MMA), influencing its disease course. This index study was undertaken to prospectively evaluate the status of neurological symptoms of MMA in relation to COVID-19 affection.Methodology and resultsFollow-up MMA patients of institute’s Stroke-clinic were telephonically interview from 24th March to 30th September, 2020. The first call familiarized them with COVID-19 symptoms and neurological manifestations of MMA, followed by monthly-calls with predesigned questionnaire. Patients with suggestion of COVID-19 underwent nasopharyngeal-swab-testing for COVID-19 Reverse transcription-polymerase chain reaction (RT-PCR) positive cases were subjected to antibody levels for COVID-19 Enzyme-linked immunoassay (ELISA) 8–12 weeks after recovery. During symptomatic phase till 14 days of asymptomatic, they were contacted daily/alternate day. Any new onset/worsening of neurological symptoms were noted. The baseline clinico-radiological details were obtained from stroke-clinic registery. Subsequently, all data were analyzed and compared using descriptive statistics. Seventy four of 104 MMA patients could be contacted and enrolled. The mean age, time since last follow-up and compliance to previously prescribed medication were 23.5 ± 16.1 years, 9.2 ± 1.7 months and 90.5% (n = 67), respectively. Aggravation/new onset neurological symptom were seen in 64.3% (n = 9) of COVID-19 positive MMA (n = 14), of which 8 were seen among the 11 pediatric COVID-19 positive MMA [(Transient ischemic attacks) TIA-4, TIA with headache-1, seizure-2, stroke causing mortality-1].ConclusionCOVID-19 infection can potentiate MMA causing significant morbidity and mortality, especially in children. Providing optimal care for severe diseases (such as MMA) in developing countries during pandemic remains a challenge.

Limb shaking TIA(LS-TIA) is an rare manifestation seen in high grade steno-occlusive carotid disease. LS-TIA is rarely recognized as a presenting manifestation of MMA and has never been previously studied prospectively in MMA cohort. Of 104 cases of MMA collected in our Indian center in last 4 years, 5 new patients had LS-TIA. MMA was diagnosed by MR angiography and LS-TIA was confirmed by three different neurologist by detailed scrutiny of history, neurological examination and EEG-recording (to rule out seizure). Seven previously reported cases of LS-TIA in MMA were retrieved by literature search in PubMed using keywords “Moyamoya” AND “Limb shaking TIA”, “Moyamoya” AND “Limb shaking”. Subsequently all data were analyzed and compared using descriptive statistics. Analysis of our 5 cases and those 7 found in the literature showed mean age at the time of diagnosis of MMA was 12.2 ± 8.9(Mean ± S.D.) years in our cases and 32.7 ± 13.6 years in previous cases. The commonest precipitating factors included prolonged crying and hyperventilation(3 of 5 our cases each) and exercise (3 of 7 previous cases). CT-perfusion demonstrated frontal white matter hypoperfusion in 2 of our 5 cases, corroborative with fronto-parietal hypoperfusion in previous cases. Medical management showed decreased symptoms in 4 of our 5 cases in a follow-up of 15.8 ± 11.6 months, while all previous patients had underwent revascularization surgery with resolution of symptoms. LS-TIA in MMA may not be so uncommon, especially in children. Early recognition is imperative to avoid future catastrophe. Steno-occlusive posterior circulation lesions might be an independent predictor of development of LS-TIA in MMA. In a resource-limited setting, careful avoidance of triggers and anti-platelet therapy can lead to decreased symptomatology of LS-TIA in MMA. •Limb shaking TIA in MMA may not be a rare entity, particularly in children.•Careful elicitation of history especially the precipitating factors helps in timely diagnosis and management.•Steno-occlusive lesions of posterior circulation might be an independent predictor of development of limb shaking TIA in MMA.•In resource poor setting, careful avoidance of triggers and anti-platelet therapy can circumvent attacks of limb shaking TIA.

Background Moyamoya angiopathy (MMA) is a rare, progressive cerebrovascular disorder characterized by stenosis or occlusion of the terminal internal carotid arteries, leading to the development of fragile collateral vessels. Headache is a common but understudied symptom of MMA, reported in up to 75% of patients. The headache phenotype often mimics migraine or tension‐type headache, although cluster headache‐like episodes have also been described. Aims to summarize current evidence on the clinical characteristics, underlying mechanisms, and treatment strategies for headache in MMA. Materials and Methods A narrative review of the literature was conducted, focusing on the prevalence, phenotype, pathophysiological mechanisms, and therapeutic options for headache in MMA. Results The pathogenesis of headache in MMA remains unclear but is likely multifactorial, involving impaired cerebrovascular autoregulation, microvascular ischemia, and collateral vessel development. No standardized treatment exists for MMA‐related headache. Antiplatelet therapy, particularly aspirin, may offer some benefit, whereas NSAIDs and triptans require caution due to cerebrovascular risks. Emerging therapies such as calcitonin gene‐related peptide (CGRP) inhibitors and Lasmiditan show potential but lack specific data in MMA patients. Surgical revascularization, mainly through direct or combined bypass, is an established intervention for stroke prevention and may also reduce headache burden. However, postoperative outcomes are heterogeneous, with reports of both headache improvement and new‐onset headache. Discussion and Conclusion Headache is a frequent and clinically relevant manifestation of MMA that significantly impacts quality of life. Evidence on optimal management remains scarce, and current strategies are largely empirical. Further studies are needed to clarify pathogenic mechanisms, refine patient selection for surgical interventions, and evaluate pharmacological treatments, including novel agents, to improve clinical outcomes. This article explores headache as a common yet underrecognized symptom of Moyamoya angiopathy (MMA), affecting up to 75% of patients. It reviews the potential pathophysiological mechanisms, including impaired autoregulation and microvascular ischemia, and discusses current and emerging treatment strategies, such as revascularization surgery and CGRP inhibitors. Recognizing and managing headache in MMA is essential to improve patients' daily functioning and overall quality of life.

Background and purposeLittle is known about the angiographic presentation of Moyamoya angiopathy (MMA) in non-Asian patients.MethodsConventional cerebral angiograms from 155 Caucasian patients diagnosed as MMA were analyzed with respect to extracranial champagne bottle neck sign, Suzuki stages, collateral status, as well as presence of aneurysms and posterior cerebral artery stenosis.ResultsIn 84 of 155 angiograms, the extracranial carotid artery was visualized, in 65 of them (77.4%), a champagne bottle neck sign was noted. Of the 278 analyzable hemispheres, 13.7%,11.2%, 37.8%, 27.3%, 8.6%, and 1.4% were classified as Suzuki stage I, stage II, stage III, stage IV, stage V, and stage VI, respectively. Among 280 hemispheres, in 53 hemispheres (18.9%) isolated basal collaterals (pathway I) and in 104 hemispheres (37.1%) choroidal and pericallosal collaterals (including basal collaterals, pathway II) were found. In 74 hemispheres (26.4%) ethmoidal collaterals (pathways III), and in 17 hemispheres (6.1%) vault collaterals were visualized. Patients with higher Suzuki stages IV–VI (p = 0.008) and ethmoidal collaterals (p < 0.001) suffered more often from cerebral hemorrhage. Transient ischemic attacks occurred more frequently in patients with Suzuki stage I to III (p < 0.001). In 10 of 155 patients (6.5%), the angiogram revealed a cerebral aneurysm. In 13 patients (8.4%), a stenotic P1 segment of the posterior cerebral artery was found.ConclusionsThis is so far the largest observational study about angiography in Caucasian European MMA patients. A comparison with Asian data indicates similarity of disease in Caucasian and Asian patients.

IntroductionMoyamoya angiopathy (MMA) is a rare vasopathy, especially among European Caucasians. Data about demographics, clinical presentation, comorbid conditions, radiological findings as well as laboratory and cerebral spinal fluid (CSF) data are sparse.MethodsPatients with MMA treated in the Alfried Krupp Hospital, Essen, Germany, between 2010 and 2017 with focus on demographic, clinical, radiological and laboratory as well as CSF data were evaluated retrospectively. Patients with non-Caucasian family background were excluded from this study.ResultsAltogether 200 European Caucasian patients with MMA were identified. There was a female predominance of 3.2:1. The mean age at first presentation was 32.9 years and the mean age of diagnosis was 36.0 years. Eleven of 194 index patients (5.7%) showed a familial presentation. In 11.6% posterior cerebral artery was additionally involved, in 4% additionally cerebral aneurysm and in 2.5% dysgenesis of corpus callosum was found. Most patients suffered from transient ischemic attacks (71.5%) and stroke (82%). Cerebral hemorrhage was found in 9.5%. Livedo racemosa was an associated symptom in 12.8% of patients and thyroid diseases were found in 23.8%.ConclusionsCompared with Asian data, cerebral hemorrhages are infrequent and female predominance is accentuated among European Caucasians. Some former unknown rare features like associated livedo racemosa, dysgenesis of corpus callosum and associated syncope have been discovered systematically for the first time in this huge European Caucasian cohort.

Introduction Moyamoya angiopathy (MMA) has been known to manifest with myriad of neurological manifestations, often in association with various precipitating factors. This is the first study to systematically analyze the precipitating triggers to neurological symptoms done on the largest cohort of MMA in India. Methods A single-centered, cross-sectional observational study, recruiting 160 patients with consecutive angiographically proven MMA over a period of 5 years (2016–2021), was undertaken to evaluate the profile of immediate precipitating factors in temporal association to the neurological symptoms, along with their clinical and radiological characteristics. SPSS 25 was used for statistical analysis. Results Among the 160 patients (Adult-85, children-75), precipitating factors were seen in 41.3%, significantly higher in children (52%) than adults (31.8%) ( p value: 0.011). The commonest triggers included fever (18.8%), emotional stress (8.1%), heavy exercise and diarrhea (6.3% each). Cold bath triggered MMA symptoms in 1.3%. Fever ( p value: 0.008) and persistent crying ( p value: 0.010) triggered neurological symptoms more commonly in children than in adults. Amongst MMA patients with precipitating factors, the commonest MMA presentation included cerebral infarction type (37.9%) and TIA (31.8%). The majority of precipitating factors that preceded an ischemic event were BP-lowing ones (54.7%). Conclusion Neurological symptoms of MMA are commonly associated with several precipitating factors, including the lesser known triggers like cold bath. The frequency and profile precipitating factors varies with the age of presentation and type of MMA. It can serve as an early clue to the diagnosis of MMA and its careful avoidance can be largely beneficial in limiting the distressing transient neurological symptoms.

Moyamoya angiopathy (MMA) is a cerebrovascular disease determining chronic progressive steno-occlusion of the supraclinoid internal carotid arteries and their main branches. The pathogenesis of MMA remains largely unknown. Multiple sclerosis (MS) is a chronic, inflammatory, demyelinating disease of the central nervous system characterized by the progressive accumulation of focal demyelinating lesions, whose pathophysiology has been theorized but still incompletely understood. Beyond misdiagnoses due to mimicking features among the two disorders, MS coexisting with MMA have been previously, rarely, reported. Herein, we present two other cases of patients with MMA with a concomitant, previously missed, diagnosis of MS and discuss their overlapping features as a hint for a potentially shared pathophysiology. The finding of typical angiographic features enables MMA diagnosis, yet it does not allow us to rule out other potentially concomitant disorders affecting the CNS. The association may be easily missed if the clinical/neuroradiological picture is not carefully assessed. Cerebral spinal fluid analysis and spine neuroimaging should be suggested in all MMA patients with atypical MRI lesions.

Despite intensive investigations, about 30% of stroke cases remains of undetermined origin. After exclusion of common causes of stroke, there is a number of rare heritable and non-heritable conditions, which often remain misdiagnosed, that should be additionally considered in the diagnosis of cryptogenic stroke. The identification of these diseases requires a complex work up including detailed clinical evaluation for the detection of systemic symptoms and signs, an adequate neuroimaging assessment and a careful family history collection. The task becomes more complicated by phenotype heterogeneity since stroke could be the primary or unique manifestation of a syndrome or represent just a manifestation (sometimes minor) of a multisystem disorder. The aim of this review paper is to provide clinicians with an update on clinical and neuroradiological features and a set of practical suggestions for the diagnostic work up and management of these uncommon causes of stroke. The identification of these stroke causes is important to avoid inappropriate and expensive diagnostic tests, to establish appropriate management measures, including presymptomatic testing, genetic counseling, and, if available, therapy. Therefore, physicians should become familiar with these diseases to provide future risk assessment and family counseling.

Background Moyamoya angiopathy (MMA) is a rare cause of stroke in Caucasians, but it is much more frequent in East Asia. Since 2021, diagnostic criteria not only comprise bilateral, but also unilateral MMA. Hitherto, progression of unilateral MMA has predominantly been described in East Asians. Our study aimed to analyze the occurrence and characteristics of progression of initially unilateral MMA in Caucasian Europeans. Methods By retrospective analysis of medical records of 200 European Caucasians with MMA, admitted to our German center between 2010 and 2022, cases of unilateral MMA and its progression, i.e. progressive ipsi- or novel contralateral arterial stenosis, during follow-up were identified. Kruskal Wallis Test and Fisher’s Exact Test were used to identify statistically significant differences between progressive and stable patients concerning demographic, clinical, laboratory, and radiographic features. Results Our cohort comprised 63 patients with initially unilateral MMA. Fourteen (22.2%) had an ipsi- ( n  = 3, 21.4%) or contralateral ( n  = 11, 78.6%) progression. Mean age of patients with progressive MMA at symptom onset was 32 ± 14.1 years. The ratio of women to men in this subgroup was 2.5:1. Mean follow-up period was 5.4 ± 3.7 years, mean age at progression was 39.9 ± 12.7 years. Mean time interval between penultimate follow-up and progression was 4.8 ± 4.5 years. Patients with progression showed affection of the posterior cerebral artery ( p  = 0.009) and suffered from vertigo ( p  = 0.009) significantly more often. Conclusion Unilateral MMA progresses in a substantial proportion in European Caucasians. Long-term follow-up is required due to potential late progression with consecutive symptoms and the need for bypass surgery.

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 ( k w = 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.