Bilateral Hunter's bow syndrome: a rare case diagnosed by dynamic digital subtraction angiography.

A 27-year-old female patient suffered from recurrent episodes of dizziness, visual rotation, and intermittent right-hand numbness over one month. Symptoms persisted and were triggered by rotating the head to the right or left for more than 10 seconds. Neurological examination showed that the symptoms were most pronounced when the head was rotated over 45 degrees to the right. Dynamic digital subtraction angiography (dDSA) was performed to confirm the diagnosis. Leftward head rotation caused occlusion of the right vertebral artery(VA) . However, the symptoms were mild, owing to sufficient compensation by the right posterior communicating artery (PCA) . Rightward head rotation exceeding 45 degrees resulted in occlusion of the left VA. The resultant symptoms were pronounced due to inadequate compensation of the left PCA. CT angiographic reconstruction showed bilateral vertebral arteries with tortuous loops of vessels at the level of the C2 vertebrae . CT images showed no cleavage between the left VA and the anterior surface of the left C2 transverse foramen. Conservative treatment was recommended considering the patient's young age and limited severity of her symptoms. Bow Hunter's syndrome is a rare neurovascular disorder characterized by dynamic occlusion of the VAs during head rotation, leading to inadequate blood flow to the posterior cerebral circulation. Bow hunter syndrome, where bilateral dynamic occlusion occurs without a discernible dominant side of the VA, is uncommon. The medical community must acknowledge cervical vertigo as a distinct disorder. dDSA remains the gold standard for its diagnosis.

Efficacy of Early Intensive Blood Pressure Management After Thrombectomy: Protocol for a Randomized Controlled Clinical Trial (IDENTIFY).

BACKGROUND: The optimal strategy for blood pressure management after thrombectomy remains unknown. The primary objective of The Early Intensive Blood Pressure Management after Endovascular Thrombectomy (IDENTIFY) study is to explore the efficacy and safety of early intensive blood pressure management strategies after thrombectomy compared with that of standard management. METHODS: The IDENTIFY study is a prospective, randomized, open-label, assessor-blinded multicenter clinical trial. Patients with acute anterior circulation ischaemic stroke who underwent endovascular thrombectomy within 6 h of stroke onset, achieved successful recanalization, and had two consecutive blood pressure readings > 130 mm Hg during the first 6 h after thrombectomy will be enrolled and centrally randomized into intensive or standard management groups in a 1:1 ratio. Continuous blood pressure monitoring will be initiated at the end of thrombectomy, and patients with high blood pressure during the transfer to the wards will also be enrolled. For patients in the intensive management group, the target blood pressure will be < 130 mm Hg, and the use of antihypertensive drugs will be discontinued if systolic blood pressure goes below 110 mm Hg. The target blood pressure for the standard management group will be < 180 mm Hg, and if systolic blood pressure decreases below 140 mm Hg, the use of antihypertensive drugs will be stepwise decreased until the systolic blood pressure reaches 140 mm Hg again or the infusion is discontinued. Patients will have their blood pressure reduced to the target range within 1 h from randomization and maintained until 24 h after thrombectomy with intravenous hypertensive drugs. A sample size of 600 was predicted. The primary outcome will be the rate of dependency (modified Rankin Scale scores 3-6) at 90 days. Secondary outcomes will include intracerebral hemorrhage (either symptomatic or asymptomatic) within 24 h and 7 days, malignant brain oedema, all-cause death, death and severe disability at 90 days, and quality of life at 90 days, which will be measured using the EuroQol-5 Dimensions-5 Level (EQ-5D-5L) and the 36-Item Short Form Health Survey (SF-36). Safety outcomes will include stroke recurrence within 24 h, early neurological deterioration, hypotension within 24 h, death within 7 days after endovascular thrombectomy, and all-cause acute kidney injury. Trial registration chictr.org.cn (identifier: ChiCTR2200057770). Registered March 17, 2022, http://www.chictr.org.cn/edit.aspx?pid=162575&htm=4.

Early Prediction of Malignant Edema After Successful Recanalization in Patients with Acute Ischemic Stroke.

BACKGROUND: Postinterventional cerebral hyperdensities are common on non-contrast-enhanced computed tomography (CT) after endovascular thrombectomy in patients with acute ischemic stroke, which may reflect blood-brain barrier damage. The disruption of the blood-brain barrier may lead to malignant brain edema. The relationship between the extent of postinterventional cerebral hyperdensities and malignant brain edema is unclear. METHODS: Patients with middle cerebral artery territory infarction and successful recanalization were consecutively enrolled. Postinterventional non-contrast-enhanced CT was performed to evaluate postinterventional cerebral hyperdensities within 24 h after endovascular thrombectomy. On the basis of the areas of the Alberta Stroke Program Early CT Score, we devised the Hyperdensity on CT Score to evaluate the extent of postinterventional cerebral hyperdensities. The primary outcome was malignant brain edema, defined as the development of clinical signs of herniation (including a decrease in consciousness and/or anisocoria), accompanied by imaging evidence of brain swelling. The component of postinterventional cerebral hyperdensities was divided into contrast staining and hemorrhage on the basis of persistency. RESULTS: Three hundred sixty patients were included (50.6% male, mean age 67.9 years), of whom 247 (68.6%) developed postinterventional cerebral hyperdensities and 66 (18.3%) developed malignant brain edema. After adjustment for confounders, including the component of postinterventional cerebral hyperdensities, the extent of postinterventional cerebral hyperdensities assessed by the Hyperdensity on CT Score was significantly associated with malignant brain edema (odds ratio 1.46, 95% confidence interval 1.20-1.77, p < 0.001). A Hyperdensity on CT Score greater than 3 had a sensitivity of 0.73 and a specificity of 0.87 for predicting malignant brain edema. CONCLUSIONS: The extent of postinterventional cerebral hyperdensities on postinterventional non-contrast-enhanced CT was associated with malignant brain edema. The Hyperdensity on CT Score could be used to predict malignant brain edema regardless of the component of postinterventional cerebral hyperdensities.

Decreased serum ferritin may be associated with increased restless legs syndrome in Parkinson's disease (PD): a meta-analysis for the diagnosis of RLS in PD patients.

Objective: Restless legs syndrome (RLS) is one of the most common non-motor symptoms of Parkinson's disease (PD), but its pathogenesis in a PD background is unclear. Abnormal iron metabolism may be involved, in which case it may be a marker of RLS risk. Here, the literature was systematically searched and meta-analyzed to compare iron metabolism markers between PD patients with or without RLS. Method: The databases PubMed, Embase, Chinese National Knowledge Infrastructure, Wanfang, Web of Science, and SinoMed were searched for case-control and observational studies examining RLS-related changes in iron metabolism in PD, in terms of serum iron, serum ferritin and hemoglobin. Eligible studies were meta-analyzed using Stata 12.0. Results: Meta-analysis of 11 case-control studies showed that serum ferritin concentration was lower in PD patients with RLS than in those without RLS. (95%CI -0.32 to -0.03, p = 0.018). In contrast, levels of serum iron or hemoglobin did not differ significantly between PD patients with or without RLS. Conclusion: This meta-analysis may provide the first reliable pooled estimate of the correlation between abnormal iron metabolism and RLS in PD. The available evidence indicates that levels of ferritin, but not of serum iron or hemoglobin, correlate significantly with RLS in PD, with lower ferritin levels correlating to greater prevalence of RLS.

Is Artery Web Seen in Vertebral Artery?

Carotid artery web has been frequently reported. However, the vertebral artery web has been less reported. It is difficult and seldom to diagnose vertebral artery web with noninvasive examinations. Here, we present a case of asymptomatic vertebral artery web diagnosed by ultrasound and confirmed by digital subtraction angiography.

Prevalence of restless legs syndrome in individuals with migraine: a systematic review and meta-analysis of observational studies.

OBJECTIVE: Recent studies have shown an association between migraine and restless legs syndrome (RLS), but RLS prevalence among individuals with migraine differs substantially across studies. The present work aimed to comprehensively assess available evidence to estimate RLS prevalence among individuals with migraine and non-migraine controls. METHOD: Web of Science, PubMed, Embase, Chinese National Knowledge Infrastructure, Wanfang, and SinoMed databases were searched for observational and case-control studies of RLS prevalence among individuals with migraine. Eligible studies were meta-analyzed using Stata 12.0 software. RESULTS: Pooled RLS prevalence in migraine was 19%, and the prevalence was lower in Asia (16%) than outside Asia (21%). Pooled RLS prevalence was 18.8% among individuals with migraine with aura, and 18.5% among individuals with migraine without aura; the RLS prevalence in migraine with aura (MA) was higher than that of migraine without aura (MO) (OR 1.17, 95%CI 1.01-1.34; p = 0.037). Pooled RLS prevalence in a case-control study was significantly higher among individuals with migraine (17.9%) than among non-migraine controls (7.1%) (OR 2.65, 95%CI 2.26-3.10; p < 0.001). CONCLUSION: Our meta-analysis provides the first reliable pooled estimate of RLS prevalence among individuals with migraine, and it provides strong evidence that RLS risk is higher among individuals with migraine than among controls.

Meta-analysis of the association between ZNF512B polymorphism rs2275294 and risk of amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS), the most common motor neuron disease, appears to result from the combination of genetic and environmental factors. Whether the rs2275294 polymorphism in the ZNF512B gene influences ALS risk is controversial. We meta-analysed the association between rs2275294 and ALS risk based on evidence published in the PubMed database. Five case-control studies involving 2559 patients with sporadic ALS and 5740 controls were analysed. Based on random-effects meta-analysis, the polymorphism rs2275294 was associated with increased risk of ALS disease in an allele model (C vs. T: OR 1.222, 95%CI 1.057 to 1.414, p = 0.007). The available evidence suggests that the ZNF512B polymorphism rs2275294 is associated with ALS risk. These results should be validated in large, well-designed studies, especially in non-Asian populations.

Genetic predictors of Stevens-Johnson syndrome and toxic epidermal necrolysis induced by aromatic antiepileptic drugs among the Chinese Han population.

BACKGROUND: Previous studies suggested that one or more HLA alleles participate in the pathogenesis of AED-induced SJS/TEN, but most of these studies focused only on the HLA-B alleles. PURPOSE: The aim of this study was to investigate the pathogenesis of AED-induced SJS/TEN across a broader spectrum of HLA alleles, including the HLA-A, -B and -DRB1 alleles, to further explore the association between each HLA allele and SJS/TEN induced by aromatic AEDs. METHODS: A total of 27 patients exhibiting AED-induced SJS/TEN (16 CBZ-SJS/TEN, seven LTG-SJS/TEN, two PHT-SJS/TEN, and two PB-SJS/TEN patients) and 64 patients who exhibited tolerance to AEDs were recruited. High-resolution HLA genotyping was performed to estimate the prevalence of the HLA-A, -B and -DRB1 alleles for each subject. RESULTS: Fifteen subjects in the SJS/TEN group (12 exhibiting CBZ-SJS/TEN, two exhibiting LTG-SJS, and one exhibiting PB-SJS) carried the HLA-B*15:02 allele, whereas only 4/64 subjects in the AED-tolerant group carried this allele; the carrier rate of HLA-B*15:02 was significantly different between the groups (P<0.001). Nine patients in the SJS/TEN group carried the HLA-DRB1*15:01 allele, while 12/64 subjects in the tolerant group carried this allele; considering that two patients in the SJS/TEN group (one exhibiting LTG-SJS and one exhibiting PB-SJS) were homozygous for this allele, the prevalence of HLA-DRB1*15:01 expression between the two groups was significantly different (P=0.041). Furthermore, the carrier rates of HLA-A*33:03, HLA-B*58:01, and HLA-DRB1*03:01 were lower in the SJS/TEN group compared with the AED-tolerant group. The carrier rates of these alleles between the two groups were significantly different (P=0.009, 0.016, and 0.009, respectively). CONCLUSIONS: The HLA-DRB1*15:01 allele may represent a risk factor for AED-induced SJS/TEN among Han Chinese. The HLA-A*33:03, HLA-B*58:01, and HLA-DRB1*03:01 alleles may be "protectors" against AED-induced SJS/TEN, especially CBZ-SJS/TEN.

Intraoperative decision-making in thrombectomy for bilateral cerebral embolism: a mimic of thrombus migration.

This study thoroughly examines the challenges in managing bilateral embolization, emphasizing intraoperative judgment. It aims to underscore the necessity of accurate etiological judgments and the limitations of cross-circulation thrombectomy. A 59-year-old male with hypertension and atrial fibrillation developed sudden limb twitching, progressing to limb paralysis and speech impairment. Neurological assessments identified multiple symptoms, while a head CT scan excluded intracranial hemorrhage. A mechanical thrombectomy revealed occlusions in the right internal cerebral artery (ICA) and left middle cerebral artery (MCA). Thrombus migration from the ICA terminus was misinterpreted as an escape from the anterior communicating artery, leading to unsuccessful thrombus retrieval. This study highlights the infrequency of bilateral embolic infarctions and intraoperative misjudgments arising from thrombus migration during thrombectomy. It underscores the limitations of cross-circulation thrombectomy and emphasizes a comprehensive risk-benefit analysis. The insights provided deepen the comprehension of thrombus migration patterns and inform strategies for bilateral embolism, focusing on optimal revascularization techniques.

Pathological migrating intramural hematoma in stenting for carotid artery dissection: A ten-year consecutive cohort study.

OBJECTIVE: To study the phenomenon, incidence and management of pathological migrating intramural hematoma in stenting for carotid artery dissection. METHODS: We consecutively enrolled CAD patients with stenting treatment over 10-year period, and retrospectively analyzed the pathological migrating intramural hematoma (PMIH) incidence of these CAD patients. Besides, we also explored the related factors with PMIH and provided an appropriate management strategy. RESULTS: A total of 67 CAD underwent stenting. PMIH occurred in 7 cases (10.4%). The median time from onset of symptoms to stenting was 5 days (3 to 11 days). There were 4 cases of PMIH in the proximal segment of stent and 3 cases of PMIH in the distal segment of stent. All the patients presented with new stenosis and no patient presented with dissecting aneurysm. Through proper management, none of the patients had occurred clinical complications. CONCLUSION: Pathological migrating intramural hematoma phenomenon exists in the stenting for carotid artery dissection, rescue angioplasty or stenting is needed for early treatment of moderate and severe stenosis due to migrating intramural hematoma on preventing further ischemic events.

Genetic and phenotypic analyses of PRRT2 positive and negative paroxysmal kinesigenic dyskinesia.

Background: Paroxysmal kinesigenic dyskinesia (PKD) is a rare neurological disorder, characterized by attacks of involuntary movements triggered by sudden action. Variants in proline-rich transmembrane protein 2 (PRRT2) are the most common genetic cause of PKD. Objective: The objective was to investigate the clinical and genetic characteristics of PKD and to establish genotype-phenotype correlations. Methods: We enrolled 219 PKD patients, documented their clinical information and performed PRRT2 screening using Sanger sequencing. Whole exome sequencing was performed on 49 PKD probands without PRRT2 variants. Genotype-phenotype correlation analyses were conducted on the probands. Results: Among 219 PKD patients (99 cases from 39 families and 120 sporadic cases), 16 PRRT2 variants were identified. Nine variants (c.879+4A>G, c.879+5G>A, c.856G>A, c.955G>T, c.884G>C, c.649C>T, c.649dupC, c.649delC and c.696_697delCA) were previously known, while seven were novel (c.367_403del, c.347_348delAA, c.835C>T, c.116dupC, c.837_838insC, c.916_937del and c.902G>A). The mean interval from onset to diagnosis was 7.94 years. Compared to patients without PRRT2 variants, patients with the variants were more likely to have a positive family history, an earlier age of onset and a higher prevalence of falls during pre-treatment attacks (27.14% versus 8.99%, respectively). Patients with truncated PRRT2 variants tend to have bilateral attacks. We identified two transmembrane protein 151A (TMEM151A) variants including a novel variant (c.368G>C) and a reported variant (c.203C>T) in two PRRT2-negative probands with PKD. Conclusion: These findings provide insights on the clinical characteristics, diagnostic timeline and treatment response of PKD patients. PKD patients with truncated PRRT2 variants may tend to have more severe paroxysmal symptoms. This study expands the spectrum of PRRT2 and TMEM151A variants. Carbamazepine and oxcarbazepine are both used as a first-line treatment choice for PKD patients.

Time-dependent systolic blood pressure within 72 h after endovascular treatment in large vessel occlusion stroke.

BACKGROUND: The association of systolic blood pressure (SBP) and ischemic stroke outcome has recently been proved to be varied at different time points within 72 h after acute ischemic stroke onset; however, the specific status of how SBP affects prognosis at different time points within 72 h after endovascular treatment (EVT) among patients with large vessel occlusion (LVO) remains unclear. METHODS: Consecutive LVO patients treated with EVT were enrolled in our study. BP data were collected at eight time points (1, 2, 4, 8, 16, 24, 48, and 72 h post-EVT). Outcome measure of interest was functional dependence, which was defined as mRS >2 at 90 days. RESULTS: A total of 406 LVO patients treated with EVT from 2016 to 2022 were included. At 16 h after EVT, the relationship between SBP and functional dependence showed a nonlinear association. At other time points after EVT, SBP had linear relationships with functional dependence. Furthermore, higher SBP, as either a linear or quadratic term, had an adverse effect on functional outcome. In addition, three SBP trajectories were observed, and the high-to-low group was independently associated with functional dependence. CONCLUSION: Taken together, higher SBP within the first 72 h after EVT has a time-dependent association with adverse clinical outcomes. Optimal blood pressure management during the first 72 h after EVT may be important to improve clinical outcome.

Genome-Wide Association Study Identifies IFIH1 and HLA-DQB1*05:02 Loci Associated With Anti-NMDAR Encephalitis.

BACKGROUND AND OBJECTIVES: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a rare autoimmune neurologic disorder, the genetic etiology of which remains poorly understood. Our study aims to investigate the genetic basis of this disease in the Chinese Han population. METHODS: We performed a genome-wide association study and fine-mapping study within the major histocompatibility complex (MHC) region of 413 Chinese patients with anti-NMDAR encephalitis recruited from 6 large tertiary hospitals and 7,127 healthy controls. RESULTS: Our genome-wide association analysis identified a strong association at the IFIH1 locus on chromosome 2q24.2 (rs3747517, p = 1.06 × 10-8, OR = 1.55, 95% CI, 1.34-1.80), outside of the human leukocyte antigen (HLA) region. Furthermore, through a fine-mapping study of the MHC region, we discovered associations for 3 specific HLA class I and II alleles. Notably, HLA-DQB1*05:02 (p = 1.43 × 10-12; OR, 2.10; 95% CI 1.70-2.59) demonstrates the strongest association among classical HLA alleles, closely followed by HLA-A*11:01 (p = 4.36 × 10-7; OR, 1.52; 95% CI 1.29-1.79) and HLA-A*02:07 (p = 1.28 × 10-8; OR, 1.87; 95% CI 1.50-2.31). In addition, we uncovered 2 main HLA amino acid variation associated with anti-NMDAR encephalitis including HLA-DQß1-126H (p = 1.43 × 10-12; OR, 2.10; 95% CI 1.70-2.59), exhibiting a predisposing effect, and HLA-B-97R (p = 3.40 × 10-8; OR, 0.63; 95% CI 0.53-0.74), conferring a protective effect. Computational docking analysis suggested a close relationship between the NR1 subunit of NMDAR and DQB1*05:02. DISCUSSION: Our findings indicate that genetic variation in IFIH1, involved in the type I interferon signaling pathway and innate immunity, along with variations in the HLA class I and class II genes, has substantial implications for the susceptibility to anti-NMDAR encephalitis in the Chinese Han population.

Association Between Systemic Immune-Inflammation Index and Symptomatic Intracranial Hemorrhage in Acute Ischemic Stroke Patients Undergoing Endovascular Treatment.

BACKGROUND/OBJECTIVE: Systemic immune-inflammation index (SII) is a novel inflammatory factor, which may be involved in the destruction of the blood-brain barrier (BBB) after acute ischemic stroke (AIS); however, the association between SII and symptomatic intracranial hemorrhage (sICH) in AIS patients undergoing endovascular treatment (EVT) remains unclear. METHODS: Patients with acute ischemic stroke due to large vessel occlusion (AIS-LVO) who underwent EVT were consecutively enrolled. Blood samples were collected in the emergency room and SII was calculated by neutrophils × platelets/lymphocytes. Participants were categorized into tertiles according to admission SII. The main outcome was defined as the occurrence of sICH, following the European Cooperative Acute Stroke Study III (ECASS-III) criteria. RESULTS: A total of 379 AIS-LVO patients with EVT were enrolled (median age = 71 years, 52.5% males). The median baseline National Institutes of Health Stroke Scale (NIHSS) score was 15 (IQR, 12-18). The median of SII was 820.9 × 109/L (IQR, 473.1-1345.2). Forty-three (11.3%) patients developed sICH. SII was found to be independently associated with sICH after EVT (adjusted odd ratio (OR) = 1.005 (per 10 units increase); 95% confidence interval (CI): 1.002-1.008; p = 0.002). Compared to patients in the lowest SII tertile, patients in the highest tertile had a higher risk of sICH (adj-OR 3.379; 95% CI 1.302-8.768; p = 0.012). The risk of sICH increased with the increase of SII in a dose-dependent manner (p for trend = 0.004). There was no interaction between potential modifiers and SII on sICH. CONCLUSION: Admission SII is positively associated with sICH in AIS-LVO patients treated with EVT. These results need to be confirmed in future studies.

Choroidal Changes in Carotid Stenosis Patients After Stenting Detected by Swept-source Optical Coherence Tomography Angiography.

BACKGROUND: Carotid artery stenosis (CAS) patients show reduced blood flow in the ophthalmic artery. This study aimed to assess the changes in the choriocapillaris and choroidal thickness in patients with unilateral carotid artery stenosis after carotid stenting using swept-source optical coherence tomography (SS-OCT)/swept-source optical coherence tomography angiography (SSOCTA). METHODS: Fifty-three mild to moderate CAS patients and 40 controls were enrolled in this study. All participants underwent digital subtraction angiography (DSA) and SS-OCT/SS-OCTAA imaging before and 4 days after carotid artery stenting. SS-OCTA was used to image and measure the perfusion of the choriocapillaris (mm2), while SS-OCT was used to image and measure the choroidal thickness (µm). The stenosed side was described as the ipsilateral eye, while the other side was the contralateral eye. RESULTS: Choroidal thickness was significantly thinner (P = 0.024) in CAS when compared with controls. Ipsilateral eyes of CAS patients showed significantly thinner (P = 0.008) choroidal thickness when compared with contralateral eyes. Ipsilateral eyes of CAS patients showed thicker (P = 0.027) choroidal thickness after carotid artery stenting, while contralateral eyes showed thinner choroidal thickness (P = 0.039). CONCLUSION: Our report suggests that in vivo quantification of the choroid with the SS-OCT/SSOCTA may allow monitoring of CAS and enable the assessment of purported treatments.

Association of Blood Pressure Within 6 h After Endovascular Thrombectomy and Functional Outcomes in Ischemic Stroke Patients With Successful Recanalization.

Background and Purpose: Blood pressure in the days following endovascular thrombectomy (EVT) can influence functional outcomes of patients who have suffered an acute ischemic stroke, but whether the same is true of blood pressure during the first few hours after EVT is unclear. Methods: Several blood pressure parameters were retrospectively analyzed in acute ischemic stroke patients who underwent EVT at West China Hospital from March 2016 to December 2019. Baseline blood pressure, speed of blood pressure reduction, postoperative blood pressure, degree of blood pressure reduction, and quality of blood pressure management were evaluated during the first 24 h after EVT. We explored whether these parameters during different time windows correlated significantly with patients' modified Rankin Scale (mRS) score at 90 days. Results: Analysis of 163 patients showed that poor functional outcome (mRS scores 3-6) correlated significantly with higher postoperative blood pressure and worse blood pressure management during the first 6 h after EVT. Postoperative systolic blood pressure at 37 min after EVT was significantly higher in patients with poor outcome (141 mmHg) than in those with good outcome (mRS scores 0-2; 122 mmHg, p = 0.006), and systolic pressure >136 mmHg at this time point was associated with a significantly higher risk of poor outcome, before and after adjusting for other risk factors (adjusted OR 0.395, 95% CI 0.20-0.79). Conclusions: Among acute ischemic patients who successfully undergo recanalization, adequate blood pressure management during the first 30-40 min after EVT may be important for ensuring good 90-day functional outcomes.