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Background and purpose: We aimed to assess the course and predictors of functional outcome after single and multiple intracerebral hemorrhage (ICH) in pediatric patients with cerebral cavernous malformations (CCMs) and to conduct a risk assessment of a third bleed during the first follow-up year after second ICH. Methods: We included patients aged ≤18 years with complete baseline characteristics, a magnetic resonance imaging dataset, ≥1 CCM-related ICH and ≥1 follow-up examination, who were treated between 2003 and 2021. Neurological functional status was obtained using modified Rankin Scale scores at diagnosis, before and after each ICH, and at last follow-up. Kaplan–Meier analysis was performed to determine the cumulative 1-year risk of third ICH. Results: A total of 55 pediatric patients (median [interquartile range] age 12 [11] years) were analyzed. Univariate analysis identified brainstem cavernous malformation (BSCM; p = 0.019) as a statistically significant predictor for unfavorable outcome after second ICH. Outcome after second ICH was significantly worse in 12 patients (42.9%; p = 0.030) than after first ICH and in five patients (55.6%; p = 0.038) after a third ICH compared to a second ICH. Cumulative 12-month risk of rebleeding during the first year after a second ICH was 10.7% (95% confidence interval 2.8%–29.37%). Conclusions: Pediatric patients with a BSCM have a higher risk of worse outcome after second ICH. Functional outcome improves over time after an ICH but worsens following each ICH compared to baseline or previous ICH. Second bleed was associated with neurological deterioration compared to initial ICH, and this deteriorated further after a third ICH. in press

Background and Purpose: The purpose of this study was to investigate the natural course of cerebral cavernous malformations (CCM) in the pediatric population, with special emphasis on the risk of first and recurrent bleeding over a 5-year period. Methods: Our institutional database was screened for patients with CCM treated between 2003 and 2020. Patients ≤18 years of age with complete magnetic resonance imaging data set, clinical baseline characteristics, and ≥1 follow-up examination were included. Surgically treated individuals were censored after CCM removal. We assessed the impact of various parameters on first or recurrent intracerebral hemorrhage (ICH) at diagnosis using univariate and multivariate logistic regression adjusted for age and sex. Kaplan-Meier and Cox regression analyses were performed to determine the cumulative 5-year risk for (re)hemorrhage. Results: One hundred twenty-nine pediatric patients with CCM were analyzed. Univariate logistic regression identified brain stem CCM (odds ratio, 3.15 [95% CI, 1.15−8.63]; P =0.026) and familial history of CCM (odds ratio, 2.47 [95% CI, 1.04−5.86]; P =0.041) as statistically significant predictors of ICH at diagnosis. Multivariate logistic regression confirmed this correlation (odds ratio, 3.62 [95% CI, 1.18−8.99]; P =0.022 and odds ratio, 2.53 [95% CI, 1.07−5.98]; P =0.035, respectively). Cox regression analysis identified ICH as mode of presentation (hazard ratio, 14.01 [95% CI, 1.80−110.39]; P =0.012) as an independent predictor for rehemorrhage during the 5-year follow-up. The cumulative 5-year risk of (re)bleeding was 15.9% (95% CI, 10.2%−23.6%) for the entire cohort, 30.2% (20.2%−42.3%) for pediatric patients with ICH at diagnosis, and 29.5% (95% CI, 13.9%−51.1%) for children with brain stem CCM. Conclusions: Pediatric patients with brain stem CCM and familial history of CCM have a higher risk of ICH as mode of presentation. During untreated 5-year follow-up, they revealed a similar risk of (re)hemorrhage compared to adult patients. The probability of (re)bleeding increases over time, especially in cases with ICH at presentation or brain stem localization.

To evaluate the predictive value of intraoperative neuromonitoring (IONM) in brainstem cavernous malformation (BSCM) surgery.Surgically treated patients with BSCM were included. All patients received IONM consisting of motor-evoked potentials (MEPs) and somatosensory-evoked potentials (SSEPs). Neurologic examination was conducted preoperatively and at discharge and follow-up3 months after BSCM removal. Demographic, radiographic, and clinical features were assessed. Study end points were new motor or somatosensory deficits and functional disability.A total of 62 patients were included. MEP decrease was associated with new motor deficits at discharge (P = 0.022), and SSEP decrease was associated with new somatosensory deficits at discharge (P0.001) and follow-up (P0.001). Sensitivity and specificity values for MEPs (discharge: 31% and 93%; follow-up: 33% and 91%) and SSEPs (discharge: 82% and 80%; follow-up: 85% and 79%) were calculated, respectively. Receiver operating characteristic analyses with area under the curve (AUC) metrics revealed acceptable performance of MEPs (AUC, 0.75; P = 0.022) and SSEPs (AUC, 0.72; P = 0.004) in predicting early deficits. Intraoperative decrease of MEPs (P = 0.047) and SSEPs (P = 0.017) was associated with early functional disability. Surgery-related subdural air accumulation impaired IONM reliability in predicting early (P = 0.048) and long-term (P = 0.013) deficits.Established IONM warning criteria may be valid for BSCM removal. However, surgical approaches in the sitting position significantly limit the predictive value of IONM, to some extent because of intraoperative pneumocephalus.

Background: Previous medical history strongly contributes to the genesis of intracranial aneurysms (IA). A possible impact of regular medication on the occurrence of abdominal aortic aneurysms has been reported. Aim: To evaluate the value of regular medication on the risk of development and rupture of IA. Methods: Data on medication use and related comorbidities were obtained from the institutional IA registry. A 1:1 age- and sex-matched patient sample was collected from the population-based Heinz Nixdorf Recall Study with individuals from the same area. Results: In the analysis comparing IA cohort ( n = 1960) with the matched normal population ( n = 1960), the use of statins (adjusted odds ratio, 1.34 [95% confidence interval 1.02–1.78]), antidiabetics (1.46 [1.08–1.99]), and calcium channel blockers (1.49 [1.11–2.00]) was independently associated with higher risk of IA, whereas uricostatics (0.23 [0.14–0.38]), aspirin (0.23 [0.13–0.43]), beta-blockers (0.51 [0.40–0.66]), and angiotensin-converting enzyme inhibitors (0.38 [0.27–0.53]) were related to lower risk of IA. In the multivariable analysis within the IA cohort ( n = 2446), SAH patients showed higher drug exposure with thiazide diuretics (2.11 [1.59–2.80]), but lower prevalence of remaining antihypertensive medication—beta-blockers (0.38 [0.30–0.48]), calcium channel blockers (0.63 [0.48–0.83]), angiotensin-converting enzyme inhibitors (0.56 [0.44–0.72]), and angiotensin-1 receptor blockers (0.33 [0.24–0.45]). Patients with ruptured IA were less likely to be treated with statins (0.62 [0.47–0.81]), thyroid hormones (0.62 [0.48–0.79]), and aspirin (0.55 [0.41–0.75]). Conclusions: Regular medication might impact the risks related to the development and rupture of IA. Further clinical trials are required to clarify the effect of regular medication on IA genesis.

Background and purpose: Aneurysmal subarachnoid hemorrhage (aSAH) is characterized by high morbidity and mortality proceeding from the initial severity and following complications of aSAH. Various scores have been developed to predict these risks. We aimed to analyze the clinical value of different radiographic scores for prognostication of aSAH outcome. Methods: Initial computed tomography scans (≤48 h after ictus) of 745 aSAH cases treated between January 2003 and June 2016 were reviewed with regard to Subarachnoid Hemorrhage Early Brain Edema Score (SEBES), and Claassen, Barrow Neurological Institute (BNI), Hijdra, original Graeb and Fisher scale scores. The primary endpoints were development of delayed cerebral ischemia (DCI), in-hospital mortality and unfavorable outcome (modified Rankin Scale score >3) at 6 months after subarachnoid hemorrhage. Secondary endpoints included the different complications that can occur during aSAH. Clinically relevant cutoffs were defined using receiver-operating characteristic curves. The radiographic scores with the highest values for area under the curve (AUC) were included in the final multivariate analysis. Results: The Hijdra sum score had the most accurate predictive value and independent associations with all primary endpoints: DCI (AUC 0.678, adjusted odds ratio [aOR] 2.83; p < 0.0001); in-hospital mortality (AUC 0.704, aOR 2.83; p < 0.0001) and unfavorable outcome (AUC 0.726, aOR 2.91; p < 0.0001). Multivariate analyses confirmed the independent predictive value of the radiographic scales for risk of decompressive craniectomy (SEBES and Fisher score), cerebral vasospasm (SEBES, BNI score and Fisher score) and shunt dependency (Hijdra ventricle score and Fisher score) after aSAH. Conclusions: Initial radiographic severity of aSAH was independently associated with occurrence of different complications during aSAH and the final outcome. The Hijdra sum score showed the highest diagnostic accuracy and robust predictive value for early detection of risk of DCI, in-hospital mortality and unfavorable outcome after aSAH.

Background and purpose: According to the International Study of Unruptured Intracranial Aneurysms, small (20% of all ruptured IAs being small IAs of the aC. This contradiction was addressed in this study by analyzing the rates and risk factors for rupture of small IAs within the aC. Methods: Of the institutional observational cohort, 1676 small IAs of the aC were included. Different demographic, clinical, laboratory, and radiographic characteristics were collected. A rupture risk score was established using all independent prognostic factors. The score performance was checked using receiver operating characteristic curve analysis. Results: Of all registered small IAs of the aC, 20.1% were ruptured. The developed small IAs of the aC (SIAAC) score (range = −4 to +13 points) contained five major risk factors: IA location and size, arterial hypertension, alcohol abuse, and chronic renal failure. In addition, three putative protective factors were also included in the score: hypothyroidism, dyslipidemia, and peripheral arterial disease. Increasing rates of ruptured IA with increasing SIAAC scores were observed, from 0% (≤−1 points) through >50% (≥8 points) and up to 100% in patients scoring ≥12 points. The SIAAC score achieved excellent discrimination (area under the curveSIAAC = 0.803) and performed better than the PHASES (Population,Hypertension, Age, Size of the aneurysm, Earlier SAH from another aneurysm, Site of aneurysm) score. Conclusions: Small IAs of the aC carry a considerable rupture risk. After external validation, the proposed rupture risk score might provide a basis for better decision-making regarding the treatment of small unruptured IAs of the aC.

Objective Occurrence of brain metastases BM is associated with poor prognosis in patients with breast cancer (BC). Magnetic resonance imaging (MRI) is the standard of care in the diagnosis of BM and determines further treatment strategy. The aim of the present study was to evaluate the association between the radiographic markers of BCBM on MRI with other patients’ characteristics and overall survival (OS). Methods We included 88 female patients who underwent BCBM surgery in our institution from 2008 to 2019. Data on demographic, clinical, and histopathological characteristics of the patients and postoperative survival were collected from the electronic health records. Radiographic features of BM were assessed upon the preoperative MRI. Univariable and multivariable analyses were performed. Results The median OS was 17 months. Of all evaluated radiographic markers of BCBM, only the presence of necrosis was independently associated with OS (14.5 vs 22.5 months, p = 0.027). In turn, intra-tumoral necrosis was more often in individuals with shorter time interval between BC and BM diagnosis (p = 0.035) and preoperative leukocytosis (p = 0.022). Moreover, dural affection of BM was more common in individuals with positive human epidermal growth factor receptor 2 status (p = 0.015) and supratentorial BM location (p = 0.024). Conclusion Intra-tumoral necrosis demonstrated significant association with OS after BM surgery in patients with BC. The radiographic pattern of BM on the preoperative MRI depends on certain tumor and clinical characteristics of patients.

Hormone and human epidermal growth factor receptor 2 (HER2/neu) receptor status is prognostic and predictive in breast cancer (BC) and guides the choice of therapy. However, owing to receptor conversion, the receptor status can differ in metastases compared with that of the primary tumor. The aim of the present study was to analyze the prognostic value of receptor status, receptor conversion, and clinical parameters in patients with resected BC brain metastases (BMs).Patients with BCBMs treated at our institution from July 2007 to December 2019 were eligible for the present study. The receptor status of the BC and corresponding BMs and the occurrence of receptor conversion were separately recorded for 3 common receptors: HER2/neu, estrogen receptor, and progesterone receptor. The association between the receptor status or receptor conversion and clinical parameters was adjusted for outcome-relevant patient and tumor characteristics.The final analysis included 78 patients. HER2/neu receptor status in BMs was associated with overall survival (P = 0.033). Receptor conversion was identified in 39 patients (50.0%): HER2/neu, n = 9 (11.5%); estrogen receptor, n = 22 (28.2%); and progesterone receptor, n = 25 (32.1%). In the final multivariate Cox regression analysis, HER2/neu receptor conversion (adjusted hazard ratio [aHR], 3.58; P = 0.006), Karnofsky performance status score70% (aHR, 3.11; P = 0.048), infratentorial BM location (aHR, 2.49; P = 0.007), and age ≥55 years at BM diagnosis (aHR, 2.20; P = 0.046) were independently associated with poorer survival.Of the 3 common BC receptors, only HER2/neu receptor conversion was strongly associated with the prognosis of patients with surgically treated BCBMs. The clinical relevance of the reevaluation of receptor status in BMs favors surgical treatment of patients with noneloquent BCBMs.

Objective: Previous reports indicate a negative impact of anemia on the outcome of an aneurysmal subarachnoid hemorrhage (SAH). We aimed to identify the outcome-relevant severity of post-SAH anemia. Methods: SAH cases treated at our institution between 01/2005 and 06/2016 were included (n = 640). The onset, duration, and severity (nadir hemoglobin (nHB) level) of anemia during the initial hospital stay were recorded. Study endpoints were new cerebral infarctions, a poor outcome six months post-SAH (modified Rankin scale > 3), and in-hospital mortality. To assess independent associations with the study endpoints, different multivariable regression models were performed, adjusted for relevant patient and baseline SAH characteristics as well as anemia-associated clinical events during the SAH. Results: The rates of anemia were 83.3%, 67.7%, 40.0%, 15.9%, and 4.5% for an nHB < 11 g/dL, < 10 g/dL, < 9 g/dL, < 8 g/dL, and < 7 g/dL, respectively. The higher the anemia severity, the later was the onset (post-SAH days 2, 4, 5.4, 7.6 and 8, p < 0.0001) and the shorter the duration (8 days, 6 days, 4 days, 3 days, and 2 days, p < 0.0001) of anemia. In the final multivariable analysis, only an nHB < 9 g/dL was independently associated with all study endpoints: adjusted odds ratio 1.7/3.22/2.44 for cerebral infarctions/in-hospital mortality/poor outcome. The timing (post-SAH day 3.9 vs. 6, p = 0.001) and duration (3 vs. 5 days, p = 0.041) of anemia with an nHB < 9 g/dL showed inverse associations with the risk of in-hospital mortality, but not with other study endpoints. Conclusions: Anemia is very common in SAH patients affecting four of five individuals during their hospital stay. An nHB decline to < 9 g/dL was strongly associated with all study endpoints, independent of baseline characteristics and SAH-related clinical events. Our data encourage further prospective evaluations of the value of different transfusion strategies in the functional outcomes of SAH patients.

Introduction Brachial plexus lipomas are extremely rare benign tumors that may cause slow progression of neurological deficits leading to thoracic outlet syndrome. Up to now, surgery remains challenging. The aim of this study is to present our surgical treatment regime and long-term neurological outcome in three cases of giant brachial plexus lipomas and to show results of systematic review.Patients and Methods Retrospective analysis of our database “peripheral nerve lesion” to identify patients suffering from brachial plexus lipomas between January 1, 2012, and December 31, 2019. Systematic review was performed for literature published until March 31, 2020, analyzing PubMed, Google Scholar, Scopus, and the Cochrane Collaboration Library independently by two authors.Results Over the past years, three patients suffering from giant brachial plexus lipomas attended to our neurosurgical department. All patients underwent preoperative magnetic resonance imaging (MRI), ultrasound examinations, and electrophysiological testing. Tumors were removed microsurgically via anterior/posterior, supraclavicular/infraclavicular, and combined approaches. The patients were accessed postoperatively by MRI and clinical follow-up. Systematic review of the literature revealed 22 cases, which were analyzed in regard to demographics, surgical treatment, and neurological outcome.Conclusion Brachial plexus lipomas are an extremely rare cause for brachial plexus compression. Total microsurgical removal with intraoperative electrophysiological monitoring is the treatment of choice with excellent long-term MRI and clinical outcome.

Cerebral infarction is a major contributor to poor outcome in patients with aneurysmal subarachnoid hemorrhage (aSAH). Timing of treatment has been discussed as a possible contributor. We aimed to analyze the impact of treatment timing on the risk of cerebral infarction and poor outcome after aSAH.Consecutive cases of patients with aSAH treated at our institution between January 2003 and June 2016 were included. The cohort was divided into 2 groups, depending on the treatment during (day 4-14 after ictus) or beyond the vasospasm phase. Statistical assessment included a 1:1 propensity score matching analysis and multivariable logistic regression analysis within the whole cohort.Of 943 patients with aSAH, 111 underwent treatment in the vasospasm phase. In the propensity score matching analysis, patients treated during the vasospasm phase were at higher risk of vasospasm requiring intra-arterial spasmolysis (P0.0001), cerebral infarction distal to the treated vessel (P0.0001), and poor outcome (modified Rankin Scale score2) at 6 months follow-up (P = 0.025). In the multivariable analysis, aneurysm treatment in the vasospasm phase was independently associated with higher risk of cerebral vasospasm necessitating intra-arterial spasmolysis (P0.0001; adjusted odds ratio [aOR], 3.62), cerebral infarction distal to the treated aneurysm (P = 0.01; aOR, 2.02), and poor outcome (P = 0.03; aOR, 2.05).Our data confirm a considerable risk of cerebral infarction and poor outcome in cases of aneurysm treatment between day 4 and 14 after aSAH. A more intense surveillance and prophylactic treatment of cerebral vasospasm might be necessary in cases of aneurysm treatment in the vasospasm phase.

Purpose Breast cancer (BC) is the most frequently diagnosed tumor entity in women. Occurring at different time intervals (TI) after BC diagnosis, brain metastases (BM) are associated with poor prognosis. We aimed to identify the risk factors related to and the clinical impact of timing on overall survival (OS) after BM surgery. Methods We included 93 female patients who underwent BC BM surgery in our institution (2008–2019). Various clinical, radiographic, and histopathologic markers were analyzed with respect to TI and OS. Results The median TI was 45.0 months (range: 9–334.0 months). Fifteen individuals (16.1%) showed late occurrence of BM (TI ≥ 10 years), which was independently related to invasive lobular BC [adjusted odds ratio (aOR) 9.49, 95% confidence interval (CI) 1.47–61.39, p = 0.018] and adjuvant breast radiation (aOR 0.12, 95% CI 0.02–0.67, p = 0.016). Shorter TI ( Conclusions Several factors seem to influence the period between BC and BM. Occurrence of BM within five years is independently associated with poorer prognosis after BM surgery. Patients with invasive lobular BC and without adjuvant breast radiation are more likely to develop BM after a long progression-free survival necessitating more prolonged cancer aftercare of these individuals.

Spinal epidural lipomatosis (SEL) is a rare condition caused by hypertrophic growth of epidural fat. The prevalence of SEL in the Western world is approximately 1 in 40 patients and is likely to increase due to current medical and socio-economic developments. Rarely, SEL can lead to rapid severe neurological deterioration. The pathophysiology, optimal treatment, and outcome of these patients remain unclear. This study aims to widen current knowledge about this “SEL subform” and to improve its clinical management. A systematic literature review according to the PRISMA guidelines using PubMed, Scopus, Web of Science, and Cochrane Library was used to identify publications before 7 November 2021 reporting on acute/rapidly progressing, severe SEL. The final analysis comprised 12 patients with acute, severe SEL. The majority of the patients were male (9/12) and multimorbid (10/12). SEL mainly affected the thoracic part of the spinal cord (11/12), extending a median number of 7 spinal levels (range: 4–19). Surgery was the only chosen therapy (11/12), except for one critically ill patient. Regarding the outcome, half of the patients regained independence (6/11; = modified McCormick Scale ≤ II). Acute, severe SEL is a rare condition, mainly affecting multimorbid patients. The prognosis is poor in nearly 50% of the patients, even with maximum therapy. Further research is needed to stratify patients for conservative or surgical treatment.

Background: Brain metastases requiring surgical treatment determine the prognosis of patients with breast cancer. We aimed to develop the scores for the prediction of short (

Purpose Degenerative cervical myelopathy (DCM) is the most common non-traumatic cause of spinal cord dysfunction. Prediction of the neurological outcome after surgery is important. The aim of this study was to analyze the relationship between first symptoms of DCM and the neurological outcome after surgery. Methods A retrospective analysis over a period of 10 years was performed. First symptoms such as cervicobrachial neuralgia, sensory and motor deficits and gait disturbances were evaluated regarding the postoperative neurological outcome. The modified Japanese Orthopedic Association Score (mJOA Score) was used to evaluate neurological outcome. Results In total, 411 patients (263 males, 64%) with a median age of 62.6 ± 12.1 years were included. Cervicobrachial neuralgia was described in 40.2%, gait disturbance in 31.6%, sensory deficits in 19% and motor deficits in 9.2% as first symptom. Patients with cervicobrachial neuralgia were significantly younger (median age of 58 years, p = 0.0005) than patients with gait disturbances (median age of 68 years, p = 0.0005). Patients with gait disturbances and motor deficits as first symptom showed significantly lower mJOA Scores than other patients (p = 0.0005). Additionally, motor deficits and gait disturbance were negative predictors for postoperative outcome according to the mJOA Score. Conclusion Motor deficits and gait disturbances as the first symptom of DCM are negative predictors for postoperative neurological outcome. Nevertheless, patients with motor deficits and gait disturbance significantly profit from the surgical treatment despite poor preoperative mJOA Score.

The partial pressure of carbon dioxide (PaCO2) in the arterial blood is a strong vasomodulator affecting cerebral blood flow and the risk of cerebral edema and ischemia after acute brain injury. In turn, both complications are related to poor outcome in patients with aneurysmal subarachnoid hemorrhage (aSAH). We aimed to analyze the effect of PaCO2 levels on the course and outcome of aSAH. All patients of a single institution treated for aSAH over 13.5 years were included (n = 633). Daily PaCO2 values from arterial blood gas measurements were recorded for up to 2 weeks after ictus. The study endpoints were: delayed cerebral ischemia (DCI), need for decompressive craniectomy due to increased intracranial pressure > 20 mmHg refractory to conservative treatment and poor outcome at 6-months follow-up (modified Rankin scale > 2). By correlations with the study endpoints, clinically relevant cutoffs for the 14-days mean values for the lowest and highest daily PaCO2 levels were defined by receiver operating characteristic curve analysis. Association with the study endpoints for the identifies subgroups was analyzed using multivariate analysis. The optimal range for PaCO2 values was identified between 30 and 38 mmHg. ASAH patients with poor initial condition (WFNS 4/5) were less likely to show PaCO2 values within the range of 30–38 mmHg (p p = 0.042, aOR = 0.27), DCI occurrence (p = 0.035; aOR = 0.50), and poor patient outcome (p = 0.004; aOR = 0.42). The data from this study shows an independent positive association between low normal mean PaCO2 values during the acute phase of aSAH and patients’ outcome. This effect might be attributed to the reduction of intracranial hypertension and alterations in the cerebral blood flow.

Introduction The severity of early brain edema after aneurysm rupture was reported to be strongly associated with the risk of poor outcome after aneurysmal subarachnoid hemorrhage (SAH). Objective/Aim Using the recently developed SEBES, we analyzed the predictors of early brain edema and its impact on the complications related to intracranial pressure (ICP) increase after SAH. Methods All consecutive SAH cases treated between January 2003 and June 2016 with assessable SEBES were included (n=745). Data on demographic characteristics, previous medical history, initial severity of SAH, need for conservative ICP treatment and decompressive craniectomy, occurrence of cerebral infarcts and unfavorable outcome at 6 months (mRS>2) were collected. Univariable and multivariable analyses were performed. Results Younger age ( Conclusions The SEBES is a reliable predictor of ICP-related complications and poor outcome of SAH. Along with age- and sex-related predisposition to early brain edema, presence of certain comorbidities like hypothyroidism and renal diseases seem to play a protective role in the severity of early brain edema after SAH. References Ahn SH, Savarraj JP, Pervez M, Jones W, Park J, Jeon SB, et al. The subarachnoid hemorrhage early brain edema score predicts delayed cerebral ischemia and clinical outcomes. Neurosurgery 2018;83(1):137–45. Rass V, Helbok R. Early brain injury after poor-grade subarachnoid hemorrhage. Curr Neurol Neurosci Rep 2019;19(10):78. Eibach M, Won SY, Bruder M, Keil F, Herrmann E, Berkefeld J, et al. Age dependency and modification of the subarachnoid hemorrhage early brain edema score. J Neurosurg 2020:1–7. Disclosure Nothing to disclose

Background: The exact causes of intracranial aneurysms (IAs) are still unknown. However, certain diseases are known to be associated with IAs. Objective: To analyze the differences in IA characteristics in the general population and in individuals with sickle-cell disease (SCD). Methods: We systematically searched PubMed, Scopus, Web of Science, and Cochrane Library for Data on SCD patients with IAs. We compared IA characteristics of SCD patients with those from 2451 healthy IA carriers from our observational cohort. Results: 129 SCD patients with IAs were identified in 42 studies. The SCD patient cohort was characterized by younger age (mean 27.1 vs 54.9 years, p Conclusion: There is a distinct demographic and radiographic pattern of IA in SCD. Risk factors for IA rupture in SCD are mostly congruent with those in healthy individuals.

Simple Summary The WHO classification of tumors of the central nervous system described for the first time the extremely rare entity of rosette-forming glioneuronal tumors (RGNT, CNS WHO grade 1) in 2007. Due to the rarity of this entity in the pineal region, no specific therapy guidelines currently exist. With our large cohort of patients treated at a single center (from August 2018–June 2021) and with the already described cases in the literature, we would like to highlight possible therapy and follow-up concepts. After the main symptoms of headache or generalized epileptic seizure, cystic lesions adjacent to the pineal gland and the third ventricle were diagnosed in imaging. None of the patients underwent additional chemotherapy or radiotherapy after gross total (GTR)/subtotal resection or endoscopic biopsy. In cases where surgical resection seems feasible with a reasonable surgical risk, we advocate GTR. Long-term MRI follow-up is essential to detect a slow tumor progression. Abstract Background: Rosette-forming glioneuronal tumor (RGNT) is an extremely rare entity described for the first time in the WHO classification of tumors of the central nervous system in 2007. Predominantly, single case reports of RGNT in the pineal region have been published, and specific therapy concepts are pending. Methods: The study group comprised all patients with the RGNT (CNS WHO grade 1) in the pineal region that underwent microsurgical tumor removal in our center (August 2018–June 2021). Surgical strategy, histological findings, and clinical outcome are presented, and the results are evaluated and compared to published case reports. Results: Four male patients aged under 50 years (range between 20 and 48 years) and one female patient, 51 years old, were included in this study. Chronic headaches and generalized epileptic seizures were the main symptoms. Supra-cerebellar infratentorial gross total tumor resection (GTR) was performed in two cases, two patients underwent subtotal tumor resection, and an endoscopic biopsy was performed in case five. Conclusion: In cases where surgical resection seems feasible with a reasonable surgical risk, we advocate GTR. Regular and long-term MRI follow-up is essential to detect a slow tumor progression. The role of additional chemotherapy or radiotherapy remains unclear.

Traumatic acute subdural hematoma (aSDH) is associated with a high mortality rate caused by post-operative cerebral infarction. Recently, brain natriuretic peptide (BNP) was considered a reliable biomarker in the acute phase of traumatic brain injuries. We therefore aimed in this study to analyze BNP levels on admission, identify the predictors of their elevation, and assess the relationship between BNP and the risk of post-operative cerebral infarction. Patients with isolated, unilateral, traumatic aSDH who were admitted to our department between July 2017 and May 2020 were enrolled in this study. On admission, cranial computer tomography (CCT) and BNP sampling were simultaneously performed. Additionally, the time between head trauma and BNP sampling (TTS) was assessed. Admission radiographic variables included hematoma volumes, midline shift, and degree of brain edema. Cerebral infarction was detected on postoperative CCT. In total, 130 patients were included in this study. Surgical treatment was performed in 82.3% (

Objective Seizures at the onset (SAO) of aneurysmal subarachnoid hemorrhage (aSAH) occur in up to one of every five cases. To date, there is no consensus on causal background and clinical value of these early bleeding-related seizures. This study aimed to analyze the predictors and the impact of SAO in aSAH. Methods All aSAH patients from the institutional observational cohort (01/2003–06/2016) were retrospectively reviewed. Patients’ charts and emergency protocols from first responders were screened for the occurrence of seizures in the first 24 h after aSAH. Patients’ baseline characteristics and occurrence of post-hemorrhagic complications were analyzed. Outcome endpoints included in-hospital mortality and poor outcome at 6-month follow-up (modified Rankin Scale > 3). Results Of 984 patients included in the final analysis, SAO occurred in 93 cases (9.5%) and were independently associated with younger age (p p p = 0.037] and irregular sac [p = 0.019]) and admission body temperature > 38.3 ℃ (p = 0.008). There was an association between SAO and early complications (early infarcts [p = 0.004] and primary decompressive craniectomy [p = 0.024]). Only in the subgroup analysis restricted to the younger individuals, SAO independently predicted poor outcome of aSAH (p = 0.002). Significance Onset seizures following aSAH are rare and most likely related to the severity of early brain injury. Particularly, younger individuals are not only at higher risk for SAO, but are also prone to poor outcome in case of aSAH accompanied with SAO. Trial registration number German clinical trial registry (DRKS, unique identifier: DRKS00008749, 06/09/2015)

Objective Microsurgical resection of spinal ependymomas is associated with a considerable risk of postoperative neurological deterioration. We aimed to develop a risk score for outcome prediction after surgery for spinal ependymoma. Materials and methods All patients who underwent microsurgical resection of spinal ependymoma between 1980 and 2015 were included. Different perioperative parameters were collected for the score construction. Poor outcome was defined as the modified McCormick Scale (MMCS) >2 at 6 months after surgery. Results Of 131 patients (mean age: 45.6 ± 16.7 years; 63 females), 38 cases (29%) showed poor outcome. Based on the univariate analysis, preoperative MMCS, subtotal tumor resection, proximal tumor level on the spinal cord, tumor extension, intramedullary location, and WHO grading were included in the multivariate analysis. The final risk score consisted of the following independent predictors: preoperative MMCS > 1 (1 point), proximal tumor level at Th 10 and higher (1 point), and tumor extension ≥ 3 vertebrae (1 point). The constructed score (0–3 points; Score for OUtcome after Resection of Spinal Ependymoma [SOURSE]) showed high diagnostic accuracy (area under the curve [AUC] = 0.883), which was superior to preoperative MMCS (AUC = 0.798) and Karnofsky Performance Status (AUC = 0.794). Patients scoring 0, 1, 2, and 3 points showed poor outcome in 0%, 12.9%, 54.6%, and 76.2% of the cases respectively. Conclusion The presented SOURSE score based on preoperative neurologic condition, tumor location, and tumor extension could accurately predict the postoperative outcome in patients undergoing microsurgery of spinal ependymoma. Our data should be validated in a prospective trial.

Background and Purpose— Multiple intracranial aneurysms (MIAs) are common findings of cerebral angiographies; however, MIA prevalence varies in different patient cohorts. We sought to elucidate risk factors influencing MIA prevalence and the clinical consequences. Methods— We systematically searched PubMed, Scopus, Embase, and Cochrane Library databases for publications before January 15, 2017, reporting MIA prevalence and risk factors. We used random-effects meta-analysis and multivariate regression analysis to assess the impacts of individual, study, and population characteristics. Results— We included 174 studies reporting on MIA (mean overall prevalence, 20.1%; range, 2%–44.9%) in 134 study populations with 86 989 intracranial aneurysm (IA) patients enrolled between 1950 and 2015. Studies from Europe and North America ( P P =0.046) were independently associated with higher MIA prevalence. In meta-analysis, MIA correlated with female sex (odds ratio [OR], 1.59; 95% confidence interval [CI], 1.4–1.8), higher patient age (>40 years; OR, 1.6; 95% CI, 1.14–2.25), arterial hypertension (OR, 1.51; 95% CI, 1.17–1.94), smoking (OR, 1.89; 95% CI, 1.37–2.6) and familial IA (OR, 2.02; 95% CI, 1.47–2.77), and formation of de novo (OR, 3.92; 95% CI, 1.95–7.87) and growth of initial IA (OR, 3.47; 95% CI, 1.87–6.45). Risk of subarachnoid hemorrhage in MIA patients was higher only in longitudinal studies from Japan and Korea (OR, 2.08; 95% CI, 1.46–2.96). Conclusions— Female sex, higher age, arterial hypertension, smoking, and familial IA are major risk factors for MIA. In addition, MIA patients are at risk for enhanced IA formation. Further studies are needed to evaluate rupture risk and the role of ethnicity, especially in the context of increased MIA identification with improved neurovascular imaging.

Objective Despite recent advances in molecular biology and genetics, the development of intracranial aneurysms (IA) is still poorly understood. Elucidation of the processes occurring in the IA wall is essential for a better understanding of IA pathophysiology. We sought to analyze the current evidence from histological, molecular and genetic studies of IA. Methods We systematically searched PubMed, Scopus, Web of Science and Cochrane Library for articles published before Mar 1, 2019 reporting on different diagnostic markers in human IA specimens. Expression of the markers in IA wall (vs. healthy arterial wall) and association with the rupture status were analyzed. The quality of the included studies and the level of the evidence for the markers were incorporated into the final data assessment. Results We included 123 studies reporting on analyses of 3476 IA (median 19 IA/study) published between 1966 and 2018. Based on microscopic, biochemical, genetic and biomechanical analyses, data on 358 diagnostic targets in the IA wall were collected. We developed a scale to distribute the diagnostic markers according to their specificity for IA or healthy arterial wall, as well as for ruptured or unruptured IA. We identified different functional pathways, which might reflect the intrinsic and extrinsic processes underlying IA pathophysiology. Conclusions Multiple histological and molecular markers and the related functional pathways contributing to the development of IA might present promising targets for future therapeutic interventions. Because of small numbers of IA samples in each study, 89% of the analyzed diagnostic markers presented with the lowest level of evidence. This underlines the need for the initiation of a multi-centric prospective histological IA register for pooled data analysis.

OBJECTIVETuberous sclerosis complex (TSC) is a rare multisystem genetic disease. Arterial wall developmental disorders, such as aneurysms, in association with TSC have been well described for extracranial vasculature. The characteristics of intracranial aneurysms (IAs) in TSC have not previously been addressed in the literature. This systematic review was performed to identify and assess the distinct characteristics of IAs in patients with TSC.METHODSThe authors searched PubMed, Scopus, and Web of Science for publications describing cases of TSC and IA reported before August 7, 2018. They also report 2 cases of IAs in TSC patients treated at their own institution.RESULTSThirty-three TSC patients with a total of 42 IAs were included in this review. Three individuals presented with subarachnoid hemorrhage. The IAs were large or giant in 57.1% and fusiform in 45.2% of the cases. Most of the IAs (61.9%, 26 of 42) originated from the internal carotid artery. There was a higher prevalence of pediatric cases (66.7%) and male patients (63.6%, 21 of 32 individuals with known sex) among the collected series.CONCLUSIONSTSC patients with IAs are characterized with a higher proportion of large/giant and fusiform IAs and young age, suggesting rapid aneurysmal growth. Furthermore, there is a distinct location pattern of IAs and an inverse sex ratio than in the healthy population. Large population-based patient registers are required to improve the understanding of epidemiology and pathophysiology of IA formation in TSC.

Intraoperative aneurysm rupture (IOAR) is a common complication during intracranial aneurysm (IA) surgery. In light of the paradigm shift regarding IA selected for clipping in the post-International Subarachnoid Aneurysm Trial (ISAT) era, we aimed to evaluate the risk factors and effects of IOAR in an institutional series of clipped ruptured IA (RIA) and unruptured IA (UIA).All IAs treated with microsurgical clipping at our institution between 2003 and 2016 were eligible for this study. Demographic, clinical, and radiographic factors were correlated with occurrence of IOAR in univariate and multivariate analyses. The effect on outcome was analyzed for RIA and UIA separately.Nine hundred and three clipped IAs were included in the final analysis (538 UIA and 365 RIA). IOAR occurred in 163 cases (18.1%), mostly during clipping of RIA (37.5% vs. 4.8%) In multivariate analysis, ruptured status (adjusted odds ratio [aOR], 10.46; P0.001), sack size (aOR, 1.05 per mm increase; P = 0.038) and IA location in the anterior communicating artery (aOR, 2.31; P0.001) independently predicted IOAR. For RIA cases, IOAR was also independently predicted by rebleeding before therapy (aOR, 3.11; P = 0.033) and clinical severity of subarachnoid hemorrhage (aOR, 1.18 per WFNS grade increase; P = 0.049). IOAR independently predicted poor outcome (aOR, 1.83; P = 0.042) after RIA surgery. In turn, IOAR affected only the risk for cerebral infarct (OR, 3.75; P = 0.003) and incomplete IA occlusion (OR, 3.45; P = 0.003) for UIA cases, but not the outcome (P = 0.263).IOAR was independently predicted by the ruptured status, location, and size of IA and by initial severity of aneurysmal bleeding and pretreatment rebleeding. The influence of IOAR differed between RIA and UIA cases.