Blood Pressure Management After Endovascular Therapy for Acute Ischemic Stroke: The BEST-II Randomized Clinical Trial.

Importance: The effects of moderate systolic blood pressure (SBP) lowering after successful recanalization with endovascular therapy for acute ischemic stroke are uncertain. Objective: To determine the futility of lower SBP targets after endovascular therapy (<140 mm Hg or 160 mm Hg) compared with a higher target (≤180 mm Hg). Design, Setting, and Participants: Randomized, open-label, blinded end point, phase 2, futility clinical trial that enrolled 120 patients with acute ischemic stroke who had undergone successful endovascular therapy at 3 US comprehensive stroke centers from January 2020 to March 2022 (final follow-up, June 2022). Intervention: After undergoing endovascular therapy, participants were randomized to 1 of 3 SBP targets: 40 to less than 140 mm Hg, 40 to less than 160 mm Hg, and 40 to 180 mm Hg or less (guideline recommended) group, initiated within 60 minutes of recanalization and maintained for 24 hours. Main Outcomes and Measures: Prespecified multiple primary outcomes for the primary futility analysis were follow-up infarct volume measured at 36 (±12) hours and utility-weighted modified Rankin Scale (mRS) score (range, 0 [worst] to 1 [best]) at 90 (±14) days. Linear regression models were used to test the harm-futility boundaries of a 10-mL increase (slope of 0.5) in the follow-up infarct volume or a 0.10 decrease (slope of -0.005) in the utility-weighted mRS score with each 20-mm Hg SBP target reduction after endovascular therapy (1-sided α = .05). Additional prespecified futility criterion was a less than 25% predicted probability of success for a future 2-group, superiority trial comparing SBP targets of the low- and mid-thresholds with the high-threshold (maximum sample size, 1500 with respect to the utility-weighted mRS score outcome). Results: Among 120 patients randomized (mean [SD] age, 69.6 [14.5] years; 69 females [58%]), 113 (94.2%) completed the trial. The mean follow-up infarct volume was 32.4 mL (95% CI, 18.0 to 46.7 mL) for the less than 140-mm Hg group, 50.7 mL (95% CI, 33.7 to 67.7 mL), for the less than 160-mm Hg group, and 46.4 mL (95% CI, 24.5 to 68.2 mL) for the 180-mm Hg or less group. The mean utility-weighted mRS score was 0.51 (95% CI, 0.38 to 0.63) for the less than 140-mm Hg group, 0.47 (95% CI, 0.35 to 0.60) for the less than 160-mm Hg group, and 0.58 (95% CI, 0.46 to 0.71) for the high-target group. The slope of the follow-up infarct volume for each mm Hg decrease in the SBP target, adjusted for the baseline Alberta Stroke Program Early CT score, was -0.29 (95% CI, -0.81 to ∞; futility P = .99). The slope of the utility-weighted mRS score for each mm Hg decrease in the SBP target after endovascular therapy, adjusted for baseline utility-weighted mRS score, was -0.0019 (95% CI, -∞ to 0.0017; futility P = .93). Comparing the high-target SBP group with the lower-target groups, the predicted probability of success for a future trial was 25% for the less than 140-mm Hg group and 14% for the 160-mm Hg group. Conclusions and Relevance: Among patients with acute ischemic stroke, lower SBP targets less than either 140 mm Hg or 160 mm Hg after successful endovascular therapy did not meet prespecified criteria for futility compared with an SBP target of 180 mm Hg or less. However, the findings suggested a low probability of benefit from lower SBP targets after endovascular therapy if tested in a future larger trial. Trial Registration: ClinicalTrials.gov Identifier: NCT04116112.

Decompressive hemicraniectomy for space-occupying brain infarction: Nationwide population-based registry study.

OBJECTIVE: We analyzed data from the Norwegian Stroke Registry (NSR) to study access to and outcomes of decompressive hemicraniectomy for brain infarction in a nationwide routine clinical setting. We also discretionary assessed whether the outcomes were comparable with those achieved in randomized controlled trials (RCTs), and whether the use was in accordance with guidelines. METHODS: The NSR is a nationwide (population 5.3 million) clinical quality registry. We included all stroke-cases operated in 2017 through 2019, and retrieved data on baseline characteristics, treatment and functional outcome after three months (dichotomized modified Rankin Scale score; favorable (0-3) or unfavorable (4-6)). Crude treatment rates and the expected proportion of patients transferred from a local hospital to a stroke-center for the operation were estimated, based on the total population's distribution of residency. RESULTS: The 68 cases were 17 (25%) women and 51 (75%) men with a median National Institute of Health Stroke Scale (NIHSS) score on admission of 14.0 (inter-quartile range (IQR) 11.0) and a median time from onset to hemicraniectomy of 34.3 (IQR 40.9) hours. The crude treatment rate varied between regions from 0.29 to 1.40 operations per 100,000 population per year, and the proportion transferred from a local hospital (50%) was lower than expected (68%). A favorable outcome was achieved in 20/52 (38.5%) cases. CONCLUSIONS: The findings indicate gender- and geographic-inequalities in access. Among operated cases, outcomes were comparable with those reported from RCTs, and the use in accordance with recommendations in the current guidelines from the American Stroke Association.

Editor's Choice - Relevance of Infarct Size, Timing of Surgery, and Peri-operative Management for Non-ischaemic Cerebral Complications After Carotid Endarterectomy.

OBJECTIVE: To assess the incidence of post-operative non-ischaemic cerebral complications as a pivotal outcome parameter with respect to size of cerebral infarction, timing of surgery, and peri-operative management in patients with symptomatic carotid stenosis who underwent carotid endarterectomy (CEA). METHODS: Retrospective analysis of prospectively collected single centre CEA registry data. Consecutive patients with symptomatic carotid stenosis were subjected to standard patch endarterectomy. Brain infarct size was measured from the axial slice of pre-operative computed tomography/magnetic resonance imaging demonstrating the largest infarct dimension and was categorised as large (> 4 cm2), small (≤ 4 cm2), or absent. CEA was performed early (within 14 days) or delayed (15 - 180 days) after the ischaemic event. Peri-operative antiplatelet regimen (none, single, dual) and mean arterial blood pressure during surgery and at post-operative stroke unit monitoring were registered. Non-ischaemic post-operative cerebral complications were recorded comprising haemorrhagic stroke and encephalopathy, i.e., prolonged unconsciousness, delirium, epileptic seizure, or headache. RESULTS: 646 symptomatic patients were enrolled of whom 340 (52.6%) underwent early CEA; 367 patients (56.8%) demonstrated brain infarction corresponding to stenosis induced symptoms which was small in 266 (41.2%) and large in 101 (15.6%). Post-operative non-ischaemic cerebral complications occurred in 12 patients (1.9%; 10 encephalopathies, two haemorrhagic strokes) and were independently associated with large infarcts (adjusted odds ratio [OR] 6.839; 95% confidence interval [CI] 1.699 - 27.534) and median intra-operative mean arterial blood pressure in the upper quartile, i.e., above 120 mmHg (adjusted OR 13.318; 95% CI 2.749 - 64.519). Timing of CEA after the ischaemic event, pre-operative antiplatelet regimen, and post-operative blood pressure were not associated with non-ischaemic cerebral complications. CONCLUSION: Infarct size and unintended high peri-operative blood pressure may increase the risk of non-ischaemic complications at CEA independently of whether performed early or delayed.

Effects of mechanical thrombectomy for post-stroke patients with upper limb hemiparesis: Use of Propensity Score Matching.

BACKGROUND: Mechanical Thrombectomy (MT) is a recommended approach for post-cerebral ischemia in acute settings. Although a large amount of evidence suggests the use of MT, existing evidence has primarily focused on assessing lower limb performance or gait performance as an outcome measure. METHODS: This study was to investigate whether MT would be an effective approach for improving upper limb performance in post-stroke patients.This case control was divided into two groups: 154 patients as a control group only given conventional rehabilitation; and 25 patients as an intervention group given MT and conventional rehabilitation. Outcome variables were measured by calculating the change of Fugl-Meyer Assessment score at the last intervention compared with the beginning of the intervention. RESULT: By comparing the FMA scores after, the propensity matching compared between before receiving therapy intervention and after, the intervention group showed as follows: 30.4 ± 26.4-44.3 ± 25.4, p = 0.0019, r = 0.59. The control group showed as follows: 39.9 ± 24.1-49.1 ± 21.3, p = 0.002, r = 0.69. Lastly, a comparison of the intervention group with the control group about their FMA score change indicates as follows: intervention group: 13.9 ± 19.4, control group 9.2 ± 10.0, p = 0.2967, r = 0.15. CONCLUSION: This study indicated that there was no significant difference between MT and a conventional approach for improving UE function. However, this is the first study to investigate the course of recovery of UE function in the acute phase after MT, and this finding supports the need for further research.

Comparisons of Characteristics and Outcomes after Mechanical Thrombectomy for Vertebrobasilar Occlusion with Cardioembolism or Atherosclerotic Brain Infarction: Data from the Tokyo-Tama-Registry of Acute Endovascular Thrombectomy (TREAT).

BACKGROUND: Some reports suggest the efficacy of mechanical thrombectomy (MT) for acute vertebrobasilar artery (VBA) occlusion. The major causes of VBA occlusion include cardioembolism (CE) and large-artery atherosclerosis (LAA). However, the clinical characteristics of each cause remain unclear, and they might be important for decision making related to the indications and strategy of MT. OBJECTIVE: This study aimed to compare functional outcomes and factors affecting outcomes between patients with CE and LAA with acute VBA occlusion. METHODS: This was a retrospective and prospective observational study using data from TREAT (Tokyo-Tama-Registry of Acute Endovascular Thrombectomy), a multicenter registry of MT for acute large-vessel occlusion in the Tokyo metropolitan area. Patients with VBA occlusion classified into CE and LAA groups were analyzed. The primary outcome was a modified Rankin Scale score of 0-2 at 90 days. RESULTS: Seventy-nine patients (57 with CE and 22 with LAA) were eligible from January 2015 to March 2020. Despite significantly shorter puncture-to-recanalization and onset-or-last-well-known-to-recanalization times in the CE group, the primary outcome was not significantly different between the 2 groups (CE, 31.6% vs. LAA, 45.5%; P = 0.248). In the subgroup analysis, patients with CE had worse clinical outcomes in the onset-or-last-well-known-to-door time ≥180 minutes, onset-or-last-well-known-to-door time ≥300 minutes, and low posterior circulation Alberta Stroke Program Early CT Score (≤7) subgroups. CONCLUSIONS: Functional outcomes of VBA occlusion were not significantly different between CE and LAA. Based on the subgroup analysis, patients with CE might have poorer collateral status than do patients with LAA, and earlier recanalization might therefore be desired.

Cerebellar Necrosectomy Instead of Suboccipital Decompression: A Suitable Alternative for Patients with Space-Occupying Cerebellar Infarction.

OBJECTIVE: Space-occupying cerebellar ischemic strokes (SOCSs) often lead to neurological deterioration and require surgical intervention to release pressure from the posterior fossa. Current guidelines recommend suboccipital decompressive craniectomy (SDC) with dural expansion when medical therapy is not sufficient. However, no good-quality evidence is available to support this surgical practice, and the surgical timing and technique both remain controversial. We have described an alternative to SDC, surgical evacuation of infarcted tissue (necrosectomy) and its clinical outcomes. METHODS: In the present retrospective, single-center study, 34 consecutive patients with SOCS undergoing necrosectomy via osteoplastic craniotomy were included. The patient characteristics and radiological findings were evaluated. To differentiate the effects of age on the functional outcomes, the patients were divided into 2 groups (group I, age ≤60 years; and group II, age >60 years). Functional outcomes were assessed using the Glasgow outcome scale, modified Rankin scale, and Barthel index at discharge and 30 days postoperatively. RESULTS: In our cohort, we observed overall mortality of 21%, with good functional outcomes (Glasgow outcome scale score ≥4) for 76% of the patients. No statistically significant differences in mortality or functional outcomes were observed between the 2 patient groups. Comparing our data with a recent meta-analysis of SDC, the number of adverse events and unfavorable outcome showed equipoise between the 2 treatment modalities. CONCLUSIONS: Necrosectomy appears to be a suitable alternative to SDC for SOCS, achieving comparable mortality and functional outcomes. Further trials are necessary to evaluate which surgical technique is more beneficial in the setting of SOCSs.

Choice of ANaesthesia for EndoVAScular treatment of acute ischaemic stroke at posterior circulation (CANVAS II): protocol for an exploratory randomised controlled study.

INTRODUCTION: Observational and interventional studies indicate that the type of anaesthesia may be associated with the postprocedural neurological function in patients with anterior circulation acute ischaemic stroke undergoing endovascular treatment. Patients with acute posterior circulation ischaemic stroke may experience different physiological changes and result in severe neurological outcome. However, the effect of the type of anaesthesia on postprocedure neurological function remained unclear in this population. METHODS AND ANALYSIS: This is an exploratory randomised controlled trial that will be carried out at Beijing Tiantan Hospital, Capital Medical University. Patients with acute posterior circulation ischaemic stroke and deemed suitable for emergency endovascular recanalisation will be recruited in this trial. Eighty-four patients will be randomised to receive either general anaesthesia or conscious sedation with 1:1 allocation ratio. The primary endpoint is the 90-day modified Rankin Scale. ETHICS AND DISSEMINATION: The study has been reviewed by and approved by Ethics Committee of Beijing Tiantan Hospital of Capital Medical University (KY2017-074-02). If the results are positive, the study will indicate whether the type of anaesthesia affects neurological outcome after endovascular treatment of posterior stroke. The findings of the study will be published in peer-reviewed journals and presented at national or international conferences. TRIAL REGISTRATION NUMBER: NCT03317535.

Infarct growth patterns may vary in acute stroke due to large vessel occlusion and recanalization with endovascular therapy.

OBJECTIVES: This study aimed to investigate infarct growth patterns in stroke patients with large vessel occlusion (LVO) and successful recanalization by endovascular therapy (EVT). METHODS: A total of 135 patients with LVO of the internal carotid artery or proximal segment of the middle cerebral artery admitted within 12 h after onset, having baseline National Institute of Health Stroke Scale score ≥ 5 points, and successfully recanalized by EVT were enrolled. Infarct growth pattern models were developed based on infarct volumes on diffusion-weighted imaging before and after reperfusion. Single pattern models of linear, logarithmic, and exponential shapes were initially tested. Their appropriateness was predetermined. If none of these patterns was suitable, the best pattern model, which was the most suitable pattern among the three shapes selected for each individual, was tested. Clinical correlates were explored. RESULTS: Each single pattern model was tested for their suitability. However, none of the single pattern models successfully represented infarct growth curves: Of all subjects, only 63.7%, 62.2%, and 54.1% of patients were explained by the logarithmic, linear, and exponential model, respectively. Compared with the single pattern models, the best pattern model explained 80.7% of the subjects. The linear shape fit best in 40 patients, the logarithmic in 51, and the exponential in 44. Those fit best for the logarithmic pattern showed more favorable outcomes at discharge (31.4%) than did the others (linear, 10.0%; exponential, 9.1%; p = 0.01). CONCLUSIONS: Infarct growth patterns may vary among individual patients with acute stroke due to LVO and successful treatment with EVT. KEY POINTS: • Infarct growth during the acute stage of stroke is highly dynamic and the exact shape remains unknown. • Infarct growth pattern models were developed based on infarct volumes on diffusion-weighted imaging before and after reperfusion. • Infarct growth patterns may not be singular, rather various among individual patients with acute stroke due to LVO and successful treatment with EVT.

Predictors of Good Outcomes in Patients with Failed Endovascular Thrombectomy.

OBJECTIVE: Endovascular thrombectomy (EVT) fails in approximately 20% of anterior circulation large vessel occlusion (AC-LVO). Nonetheless, the factors that affect clinical outcomes of non-recanalized AC-LVO despite EVT are less studied. The purpose of this study was to identify the factors affecting clinical outcomes in non-recanalized AC-LVO patients despite EVT. MATERIALS AND METHODS: This was a retrospective analysis of clinical and imaging data from 136 consecutive patients who demonstrated recanalization failure (modified thrombolysis in cerebral ischemia [mTICI], 0-2a) despite EVT for AC-LVO. Data were collected in prospectively maintained registries at 16 stroke centers. Collateral status was categorized into good or poor based on the CT angiogram, and the mTICI was categorized as 0-1 or 2a on the final angiogram. Patients with good (modified Rankin Scale [mRS], 0-2) and poor outcomes (mRS, 3-6) were compared in multivariate analysis to evaluate the factors associated with a good outcome. RESULTS: Thirty-five patients (25.7%) had good outcomes. The good outcome group was younger (odds ratio [OR], 0.962; 95% confidence interval [CI], 0.932-0.992; p = 0.015), had a lower incidence of hypertension (OR, 0.380; 95% CI, 0.173-0.839; p = 0.017) and distal internal carotid artery involvement (OR, 0.149; 95% CI, 0.043-0.520; p = 0.003), lower initial National Institute of Health Stroke Scale (NIHSS) (OR, 0.789; 95% CI, 0.713-0.873; p < 0.001) and good collateral status (OR, 13.818; 95% CI, 3.971-48.090; p < 0.001). In multivariate analysis, the initial NIHSS (OR, 0.760; 95% CI, 0.638-0.905; p = 0.002), good collateral status (OR, 14.130; 95% CI, 2.264-88.212; p = 0.005) and mTICI 2a recanalization (OR, 5.636; 95% CI, 1.216-26.119; p = 0.027) remained as independent factors with good outcome in non-recanalized patients. CONCLUSION: Baseline NIHSS score, good collateral status, and mTICI 2a recanalization remained independently associated with clinical outcome in non-recanalized patients. mTICI 2a recanalization would benefit patients with good collaterals in non-recanalized AC-LVO patients despite EVT.

Postpartum Headache due to Cerebellar Infarct Initially Misdiagnosed as Postdural Puncture Headache: A Case Report.

We present the case of a 39-year-old woman with postpartum cerebellar infarction (CI) following spinal anesthesia for cesarean delivery. The patient experienced mild headache after postoperative day 1 and returned on postoperative day 6 with a severe headache. For the subsequent 3 days, she underwent conservative treatment for presumed postdural puncture headache (PDPH) before neurologic decline and diagnosis of CI on postoperative day 9. She subsequently underwent craniotomy and debridement of necrotic tissues. Prolonged or position-independent postpartum headache should prompt broadening of the differential diagnosis beyond PDPH to include other more rare but serious causes of postpartum headache.

Postoperative hyperglycemia predicts symptomatic intracranial hemorrhage after endovascular treatment in patients with acute anterior circulation large artery occlusion.

INTRODUCTION: Acute phase hyperglycemia is independently associated with an increased risk of death and symptomatic intracranial hemorrhage (sICH) in stroke patients treated with intravenous thrombolysis. Whether postoperative hyperglycemia is an independent predictor of sICH after endovascular therapy remains unknown. Here, we assessed whether hyperglycemia after endovascular therapy can predict sICH. METHODS: Consecutive acute ischemic stroke patients who were treated with mechanical thrombectomy with or without subsequent stent implantation were analyzed. The primary outcome was the occurrence of sICH within the first 7 days after endovascular treatment. The second outcome was other forms of hemorrhagic transformation (HT), including parenchymal hematoma (PH) and parenchymal hematoma type 2 (PH-2). RESULTS: One hundred and fifty-six patients were included. Fifteen patients (9.62%) developed sICH after endovascular therapy. After adjusting for potential confounding factors, postoperative glucose values were independently associated with sICH after endovascular therapy. Furthermore, adding postoperative glucose values to conventional risk factors led to a substantial reclassification for sICH following endovascular therapy (net reclassification improvement = 28.1%; p = .014). Moreover, postoperative glucose values were found to be risk factors for PH-2. CONCLUSIONS: We found that postoperative glucose values might be an independent risk factor for sICH in patients with anterior circulation large vessel occlusion who are treated with mechanical thrombectomy. Adding postoperative glucose values to conventional risk factors could improve risk stratification for sICH following endovascular therapy.

New CT measurements to assess decompression after hemicraniectomy: A two-center reliability study.

OBJECTIVES: To test the reliability of three simplified measurements made after decompressive hemicraniectomy (DHC) for malignant hemispheric infarction on computed tomography (CT) scan. PATIENTS AND METHODS: We defined new simple methods to measure the thickness of the soft tissues overlying the craniectomy defect and the extent of infarction beyond the anterior and posterior craniectomy edges on post-DHC CT. Multiple raters independently made the three new CT measurements in 49 patients from two institutions. The Intraclass Correlation Coefficient (ICC) compared the raters for interrater agreements (reliability). RESULTS: Between two raters at Augusta University Medical Center, each measuring 21 CT scans, the ICC coefficient point estimates were good to excellent (0.83 - 0.92). Among four raters at University of Virginia Medical Center, with three raters measuring each of 28 CT scans, the ICC coefficient point estimates were good to excellent (0.87 - 0.95). CONCLUSIONS: The proposed simple methods to obtain three additional CT measurements after DHC in malignant hemispheric infarction have good to excellent reliability in two independent patient samples. The clinical usefulness of these measurements should be investigated.

Endovascular Vertebral Artery Transposition Using Flow Reversal Technique for Left Subclavian Artery Stump Syndrome.

BACKGROUND: Cerebellar strokes are a rare complication related to thoracic endovascular aortic repair (TEVAR). This can manifest in an indolent manner or as a neurological catastrophe. Often it is unclear when a surgical intervention would be needed. Patients at risk for this relatively rare complication are not easily identified. CASE: We describe an endovascular option with flow reversal for left vertebral artery transposition using stent grafts for relocating arterial inflow and excluding a floating thrombus at the proximal subclavian artery (SCA) related to a previous TEVAR. CONCLUSIONS: Ligation of the subclavian artery proximal to the vertebral artery should be considered when performing a carotid subclavian bypass for elective TEVAR. This case details a unique, less invasive approach for vertebral artery transposition and thrombus exclusion in a high-risk patient with previous neck dissection.

Endovascular Revascularization of Nonacute Symptomatic Proximal Extracranial Vertebral Artery Occlusion.

OBJECTIVE: To evaluate the feasibility, success rate, and safety of endovascular revascularization of nonacute symptomatic proximal extracranial vertebral artery occlusion (PEVAO). METHODS: In a retrospective, single-center study, we analyzed clinical and imaging data from consecutive patients with PEVAO who underwent endovascular revascularization from June 2011 to March 2018. RESULTS: The study enrolled 23 patients (mean age, 59 years; range, 42-77 years; 17 men). The rate of successful technical revascularization was 91.3% (n = 21), and the rate of complications was 4.3% (n = 1). At 3-month follow-up, the patients with successful endovascular revascularization of nonacute symptomatic PEVAO did not present any neurologic symptoms and computed tomography angiography did not show restenosis of the stent. CONCLUSIONS: Endovascular revascularization of nonacute symptomatic PEVAO is feasible and associated with a high rate of procedural success and low rate of procedural complications. A large, multicenter, randomized study is warranted to confirm the findings.

The Surgical Treatment of a Large Cortical Atherosclerotic Middle Cerebral Artery Aneurysm Presenting With Parietal Lobe Infarction.

Distal MCA aneurysms are rarely seen in daily neurosurgical practice and they are, more commonly associated with infectious processes. Here, the authors present a 65-year-old, patient who had an atherosclerotic M4 segment located aneurysm. It was confirmed, that the aneurysm was not related with any infectious process. The patient had, presented clinically by a parietal infarction and she had been successfully operated. The neuronavigation system for this particular case aided us for a precise localization of the aneurysm and gave a chance for a smaller craniotomy.

Anesthesia practice for endovascular therapy of acute ischemic stroke in Europe.

PURPOSE OF REVIEW: Anesthetic assistance is often required during endovascular therapy (EVT) of large vessel occlusion in patients with acute ischemic stroke. It is currently debated whether EVT should be performed under general anesthesia or conscious sedation. This review will summarize the recent literature with emphasis on the influence of anesthesia method on neurological outcome. RECENT FINDINGS: Recent randomized trials have reported no difference in outcome after EVT performed under either conscious sedation or general anesthesia. This is in contrast to a substantial number of retrospective studies, which found that EVT performed under general anesthesia was associated with a worse neurologic outcome compared with conscious sedation. Anesthetic drugs affect vessel tone and the level of blood pressure may influence outcome. The most favorable choice of anesthetic agents and ventilatory strategy is still debated. SUMMARY: The optimal anesthetic practice for EVT remains to be identified. Currently, conscious sedation is often an easy first-line strategy, but general anesthesia can be considered an equal and safe alternative to conscious sedation when there is a carefully administered anesthetic that maintains strict hemodynamic control. Attention to ventilation is advocated. The presence of a specialized neuroanesthesiologist or otherwise dedicated anesthesia personnel is highly recommended.

Genotype-Phenotype Correlation in Long-Term Cohort of Japanese Patients with Moyamoya Disease.

BACKGROUND: Homozygosity of this p.R4810K founder variant of RNF213moyamoya disease (MMD) susceptibility gene is known to influence the severity of the clinical disease phenotype at disease onset. However, the association between this genotype and long-term clinical manifestations has remained unclear. OBJECTIVES: The principal goal of this study was to investigate whether and how the p.R4810K variant of RNF213influences the long-term phenotype in Japanese patients with MMD. METHOD: This retrospective cohort study included 94 Japanese patients with MMD who underwent direct or combined bypass for revascularization with the p.R4810K genotype determined in our hospital. The following phenotypic parameters were analyzed at disease onset and over a long-term period: age and initial presentation at onset, recurrent stroke after initial revascularization, and final modified Rankin Scale. RESULTS: The p.R4810K genotype was significantly associated with the phenotype at onset, especially in younger patients. Over a median follow-up period of 100 months, recurrent stroke occurred in 6 out of 94 patients: none out of 5 patients with the homozygous variant, 5 out of 64 with the heterozygous variant, and 1 out of 25 in the wild-type group. There were no significant differences among the genotypes. In particular, recurrent cerebral hemorrhage occurred in 5 patients, all possessing the heterozygous variant. The log-rank test showed no difference between the genotypes in the stroke-free survival rate. Furthermore, the p.R4810K genotype was not associated with a poor functional condition. CONCLUSIONS: The p.R4810K founder variant of RNF213 affects the phenotype at disease onset. However, the optimal revascularization may be effective, regardless of the genotype, even for the homozygous variant, which has been thought to be the most pathogenic. This genotype may not strongly influence the long-term clinical manifestations or poor prognosis in MMD.

Status dystonicus: Diagnosis and management of a rare and challenging entity.

We report the case of a six-year-old girl with Moyamoya disease who presented with bilateral internal carotid artery malignant infarct following encephaloduroarteriosynangiosis (EDAS). During her neurorehabilitation, she developed gradually worsening dystonic spasms with opisthotonic posturing, tachycardia, tachypnea and desaturation. This rare life threatening movement disorder was diagnosed as status dystonicus based on the history and clinical presentation. Status Dystonicus occurs commonly in children and the etiology is often diverse. It occurs in patients with preexisting dystonia or following an acute central nervous system insult of varied etiology. Status dystonicus is usually precipitated by one or more triggering factors. Rarity and lack of objective criteria for diagnosis often delays the management thereby increasing the risk of mortality and morbidity. Here, we discuss the challenges faced in the diagnosis and management of a child with denovo status dystonicus.

Unexpected Intracranial Hemorrhage and Death After Cranioplasty in a Patient With Massive Hemispheric Infarction.

The benefits and common complications of cranioplasty are often mentioned, but fatal complications are rarely documented. Here, the authors report a patient of intracranial hemorrhage and death after cranioplasty and discussed the possible mechanism. A 42-year-old man was admitted with the diagnosis of massive cerebral infarction in left fronto-temporo- parietal lobe, emergency surgery for decompressive large craniotomy and Encephalo-Myo-Synangiosis were performed. One year after surgery, cranioplasty was performed using a titanium mesh plate. Intraoperative cerebrospinal fluid leakage was occurred and dura mater was repaired using pieces of silk. During the postoperative anesthesia emergence, the patient had epileptic seizures and did not wake after surgery. The authors also observed about 150 mL bloody cerebrospinal fluid (CSF) in the subcutaneous vacuum drainage system within 2 hours. Emergency computed tomography of the brain showed epidural, subdural, subarachnoid hemorrhages in the postischemic area, the middle line left, and the brain stem swelling. The patient's family refused to immediately remove the titanium mesh plate. Finally, nonoperative treatment is invalid and the patient's neurological condition did not recover and he died 3 days after the surgery. In the authors' mind, patients with previous massive cerebral infarction and Encephalo-Myo-Synangiosis undergoing cranioplasty might be at heightened risk of a fatal event than other cranioplasty. Therefore, the patients should be paid more attention to prevent and treat the fatal complications.