Sporadic multiple intracranial meningioma does not infer worse patient outcomes: results from a case control study.

BACKGROUND: Sporadic multiple meningioma are uncommon. Population-based data suggests that these patients have a reduced overall survival when compared to patients with solitary meningioma. The aim of this study was to investigate the clinical outcomes in multiple and solitary meningioma. METHODS: A single-center matched cohort study (2008-2018) was performed. Patients with synchronous multiple meningioma at presentation, with no history of prior intracranial radiation, concurrent hormone replacement therapy or features of NF2-schwannomatosis were included. Eligible patients were matched 1:1 to patients with solitary meningioma. Outcomes of interest were occurrence of an intervention, recurrence, new meningioma development and mortality. RESULTS: Thirty-four patients harboring 76 meningioma at presentation were included. Mean age was 59.3 years (SD = 13.5). Thirty-one (91.2%) were female. The median number of meningioma per patient was 2 (range 2-6). Eighteen patients (52.9%) were symptomatic at presentation. Median overall follow-up was 80.6 months (IQR 44.1-99.6). Compared to patients with a sporadic meningioma, there was no difference in intervention rates (67.6% vs 70.6%, P = 0.792). Eight patients (34.8%) with a multiple meningioma had a WHO grade 2 meningioma compared to 7 (29.2%) with a solitary meningioma (P = 0.679). Median recurrence-free survival was 89 months (95% CI 76-104) with no difference between the two groups (P = 0.209). Mean overall survival was 132 months (95% CI 127-138) with no difference between the two groups (P = 0.860). One patient with multiple meningioma developed two further new meningioma 36 months following diagnosis. CONCLUSION: Sporadic multiple meningioma may not have worse clinical outcomes. Management of patients with sporadic multiple meningioma should be tailored towards the symptomatic meningioma or high-risk asymptomatic meningioma.

Quality of life outcomes in incidental and operated meningiomas (QUALMS): a cross-sectional cohort study.

INTRODUCTION: Few studies have evaluated meningioma patients' longer-term health-related quality of life (HRQoL) following diagnosis and treatment, particularly in those with incidental, actively monitored tumours. METHODS: A single-center, cross-sectional study was completed. Adult patients with surgically managed or actively monitored meningioma with more than five years of follow-up were included. The patient-reported outcome measures RAND SF-36, EORTC QLQ-C30 and QLQ-BN20 were used to evaluate HRQoL. HRQoL scores were compared to normative population data. Outcome determinants were evaluated using multivariate linear regression analysis. RESULTS: 243 patient responses were analyzed, and the mean time from diagnosis was 9.8 years (range 5.0-40.3 years). Clinically relevant, statistically significant HRQoL impairments were identified across several SF-36 and QLQ-C30 domains. Increasing education level (ß = 2.9, 95% CI 0.9 to 4.9), P = .004), employment (ß = 7.7, 95% CI 2.2 to 13.1, P = .006) and absence of postoperative complications (ß=-6.7, 95% CI -13.2 to (-)0.3, P = .041) were associated with a better QLQ-C30 summary score. Other tumour and treatment variables were not. CONCLUSION: This study highlights the longer-term disease burden of patients with meningioma nearly one decade after diagnosis or surgery. Patients with actively monitored meningioma have similar HRQoL to operated meningioma patients. Healthcare professionals should be mindful of HRQoL impairments and direct patients to sources of support as needed.

Tumour treating fields in glioblastoma: is the treatment tolerable, effective, and practical in UK patients?

BACKGROUND: Tumour Treating Fields (TTF) in combination with standard therapy, prolongs survival in patients with glioblastoma (GBM). The aim of the current study was to assess the feasibility of integrating TTF into a standard UK neuro-oncology service with a focus on patient tolerability, compliance, and treatment delivery. METHODS: A prospective study was performed of UK patients with IDH 1 Wild Type, MGMT Unmethylated GBM treated with TTF, in conjunction with conventional therapy. Patient compliance data, device-specific tolerability questions, and an evaluation of disease progression and survival were collected. Monthly quality of life (QoL) questionnaires (EORTC QLQ-C30 with BN-20) examined the trend of global health, psychosocial function, and symptom progression. RESULTS: Nine patients were enrolled with a median age of 47 (seven males; two females). Overall, compliance with TTF was 89% (range 16-97%). Only one patient failed to comply with treatment. Patients tolerated the device with minimal side effects. Eight patients described mild to moderate skin irritation, whilst all patients were keen to recommend the device to other patients (100%). Most patients found the weight and size of the device to be its biggest drawback (72%). Progression-free survival was 5.5 months and median overall survival was 14.9 months. CONCLUSIONS: TTF was well-tolerated amongst a small cohort of UK patients, who were able to comply with treatment without any significant complication. QoL questionnaires showed no sustained deterioration in global health, physical and emotional function until the final months of life when the disease burden was greatest.

The growth rate and clinical outcomes of radiation induced meningioma undergoing treatment or active monitoring.

INTRODUCTION: Radiation induced meningioma (RIM) incidence is increasing in line with improved childhood cancer survival. No optimal management strategy consensus exists. This study aimed to delineate meningioma growth rates from tumor discovery and correlate with clinical outcomes. METHODS: Retrospective study of patients with a RIM, managed at a specialist tertiary neuroscience center (2007-2019). Tumor volume was measured from diagnosis and at subsequent interval scans. Meningioma growth rate was determined using a linear mixed-effects model. Clinical outcomes were correlated with growth rates accounting for imaging and clinical prognostic factors. RESULTS: Fifty-four patients (110 meningiomas) were included. Median duration of follow-up was 74 months (interquartile range [IQR], 41-102 months). Mean radiation dose was 41 Gy (standard deviation [SD] = 14.9) with a latency period of 34.4 years (SD = 13.7). Median absolute growth rate was 0.62 cm3/year and the median relative growth rate was 72%/year. Forty meningiomas (between 27 patients) underwent surgical intervention after a median follow-up duration of 4 months (IQR 2-35). Operated RIMs were clinically aggressive, likely to be WHO grade 2 at first resection (43.6%) and to progress after surgery (41%). Median time to progression was 28 months (IQR 13-60.5). A larger meningioma at discovery was associated with growth (HR 1.2 [95% CI 1.0-1.5], P = 0.039) but not progression after surgery (HR 2.2 [95% CI 0.7-6.6], P = 0.181). Twenty-seven (50%) patients had multiple meningiomas by the end of the study. CONCLUSION: RIMs exhibit high absolute and relative growth rates after discovery. Surgery is recommended for symptomatic or rapidly growing meningiomas only. Recurrence risk after surgery is high.

Management of pineal and colloid cysts.

The widespread use of MRI has led to the increasingly frequent diagnosis of pineal and colloid cysts. While most are small and incidental, do not require long-term monitoring and will never need treatment, they are a cause of patient anxiety and clinician uncertainty regarding the optimal management-particularly for larger cysts or those with an atypical appearance. Occasionally pineal cysts, and more commonly colloid cysts, cause hydrocephalus that requires urgent neurosurgical treatment. More recently the non-hydrocephalic symptomatic pineal cyst has been described in the neurosurgical literature but there is controversy over this entity and its management. This review addresses the difficulties in managing pineal and colloid cysts and provides a pragmatic framework for the practising clinician.

Ketogenic diets as an adjuvant therapy for glioblastoma (KEATING): a randomized, mixed methods, feasibility study.

PURPOSE: We conducted a feasibility study to investigate the use of ketogenic diets (KDs) as an adjuvant therapy for patients with glioblastoma (GBM), investigating (i) trial feasibility; (ii) potential impacts of the trial on patients' quality of life and health; (iii) patients' perspectives of their decision-making when invited to participate in the trial and (iv) recommending improvements to optimize future phase III trials. METHODS: A single-center, prospective, randomized, pilot study (KEATING), with an embedded qualitative design. Twelve newly diagnosed patients with GBM were randomized 1:1 to modified ketogenic diet (MKD) or medium chain triglyceride ketogenic diet (MCTKD). Primary outcome was retention at three months. Semi-structured interviews were conducted with a purposive sample of patients and caregivers (n = 15). Descriptive statistics were used for quantitative outcomes and qualitative data were analyzed thematically aided by NVivo. RESULTS: KEATING achieved recruitment targets, but the recruitment rate was low (28.6%). Retention was poor; only four of 12 patients completed the three-month diet (MCTKD n = 3; MKD n = 1). Participants' decisions were intuitive and emotional; caregivers supported diet implementation and influenced the patients' decision to participate. Those who declined made a deliberative and considered decision factoring diet burden and quality of life. A three-month diet was undesirable to patients who declined and withdrew. CONCLUSION: Recruitment to a KD trial for patients with GBM is possible. A six-week intervention period is proposed for a phase III trial. The role of caregivers should not be underestimated. Future trials should optimize and adequately support the decision-making of patients.

Correction to: Incidental intracranial meningiomas: a systematic review and meta-analysis of prognostic factors and outcomes.

Issues with data analysis have recently been highlighted by a reader of our article. These have been addressed with changes to Tables 2&4, as shown below, and Online Resources 5-7. T2 and peritumoral signal are no longer prognostic factors on simple pooled (Online Resource 5) and IPD (Table 4) analyses respectively. In Table 5, the number of patients which informed the outcomes symptom development and intervention were 575 and 947 respectively; 69 developed symptoms (pooled proportion %8.4 [95% CI 2.8-16.7], I2 = 88.9%). These included motor and cognitive deficits (n = 1). We apologise to the readership of the Journal of Neuro-Oncology for these errors and thank the reader for helping us identify them.

Incidental intracranial meningiomas: a systematic review and meta-analysis of prognostic factors and outcomes.

BACKGROUND: Incidental discovery accounts for 30% of newly-diagnosed intracranial meningiomas. There is no consensus on their optimal management. This review aimed to evaluate the outcomes of different management strategies for these tumors. METHODS: Using established systematic review methods, six databases were scanned up to September 2017. Pooled event proportions were estimated using a random effects model. Meta-regression of prognostic factors was performed using individual patient data. RESULTS: Twenty studies (2130 patients) were included. Initial management strategies at diagnosis were: surgery (27.3%), stereotactic radiosurgery (22.0%) and active monitoring (50.7%) with a weighted mean follow-up of 49.5 months (SD = 29.3). The definition of meningioma growth and monitoring regimens varied widely impeding relevant meta-analysis. The pooled risk of symptom development in patients actively monitored was 8.1% (95% CI 2.7-16.1). Associated factors were peritumoral edema (OR 8.72 [95% CI 0.35-14.90]) and meningioma diameter ≥ 3 cm (OR 34.90 [95% CI 5.17-160.40]). The pooled proportion of intervention after a duration of active monitoring was 24.8% (95% CI 7.5-48.0). Weighted mean time-to-intervention was 24.8 months (SD = 18.2). The pooled risks of morbidity following surgery and radiosurgery, accounting for cross-over, were 11.8% (95% CI 3.7-23.5) and 32.0% (95% CI 10.6-70.5) respectively. The pooled proportion of operated meningioma being WHO grade I was 94.0% (95% CI 88.2-97.9). CONCLUSION: The management of incidental meningioma varies widely. Most patients who clinically or radiologically progressed did so within 5 years of diagnosis. Intervention at diagnosis may lead to unnecessary overtreatment. Prospective data is needed to develop a risk calculator to better inform management strategies.

Postoperative seizures in meningioma patients: improving patient selection for antiepileptic drug therapy.

BACKGROUND: Epilepsy is a major cause of morbidity and mortality in meningioma patients. The aims of this study were to determine which factors predispose meningioma patients to developing perioperative seizures and to understand the impact of antiepileptic drugs. METHODS: Patients treated for a histologically-confirmed intracranial meningioma at the authors' institution between 2010 and 2015 were retrospectively examined. Clinical and imaging data were assessed. Multivariate analysis was performed using binary logistic regression. The effect of antiepileptic treatment was assessed using survival analysis. RESULTS: Two hundred and eighty-three patients met the selection criteria; seizures were present in 68 preoperatively (24%) and in 48 patients (17%) following surgery. Of the 68 with preoperative seizures, 19 continued to have them, whereas de-novo seizures arose postoperatively in 29 seizure-naïve patients. Risk factors of postoperative seizures were convexity location (OR 2.05 [95% CI 1.07-3.98], p = 0.030), fronto-parietal location (OR 4.42 [95% CI 1.49-13.16], p = 0.007) and preoperative seizures (OR 2.65 [95% CI 1.37-5.24], p = 0.005). The two locations, in addition to the presence of midline shift on preoperative imaging (OR 4.15 [95% CI 1.54-11.24], p = 0.005), were significantly correlated with postoperative seizures in seizure-naïve patients. Antiepileptic treatment in patients with those risk factors reduced the possibility of seizures at any time point within the 1st year postoperatively by approximately 40%, although this did not meet statistical significance. CONCLUSION: Prophylactic antiepileptic treatment might be warranted in seizure-naïve meningioma patients with ≥ 1 risk factor. High-quality randomised controlled trials are required to verify those factors and to define the role of antiepileptics in meningioma practice.

Surgical management and long-term outcome of intracranial subependymoma.

BACKGROUND: Intracranial subependymomas account for 0.2-0.7% of central nervous system tumours and are classified as World Health Organization (WHO) grade 1 tumours. They are typically located within the ventricular system and are detected incidentally or with symptoms of hydrocephalus. Due to paucity of studies exploring this tumour type, the objective was to determine the medium- to long-term outcome of intracranial subependymoma treated by surgical resection. METHODS: Retrospective case note review of adults with intracranial WHO grade 1 subependymoma diagnosed between 1990 and 2015 at the Walton Centre NHS Foundation Trust was undertaken. Tumour location, extent of resection (defined as gross total resection (GTR), sub-total resection (STR) or biopsy) and the WHO performance status at presentation and through follow-up were recorded. RESULTS: Thirteen patients (7 males; 6 females) with a mean age of 47.6 years (range 33-58 years) and a median follow-up of 46 months (range 25-220 months) were studied. Eight patients had symptomatic tumours (headache, visual disturbance); five had incidental finding. Tumours were most commonly located in the fourth ventricle (n = 8). The performance status scores at diagnosis were 0 (n = 8) and 1 (n = 5). The early post-operative performance status scores at 6 months were 0 (n = 5) and 1 (n = 8) and at last follow-up were 0 (n = 11) and 1 (n = 2). There was no evidence of tumour re-growth following GTR or STR. The commonest complication was hydrocephalus (n = 3). CONCLUSION: Subependymoma are indolent tumours. No patients exhibited a worsening of performance status at medium- to long-term follow-up and there were no tumour recurrence suggesting a shorter follow-up time may be sufficient. Surgical resection is indicated for symptomatic tumours or those without a clear imaging diagnosis. Incidental intraventricular subependymoma can be managed conservatively through MRI surveillance.

Multiple synchronous sites of origin of vestibular schwannomas in neurofibromatosis Type 2.

BACKGROUND: Neurofibromatosis Type 2 (NF2) is a dominantly inherited tumour syndrome with a phenotype which includes bilateral vestibular (eighth cranial nerve) schwannomas. Conventional thinking suggests that these tumours originate at a single point along the superior division of the eighth nerve. METHODS: High resolution MRI was performed in children genetically proven to have NF2. The superior vestibular nerve (SVN) and inferior vestibular nerve (IVN) were visualised along their course with points of tumour origin calculated as a percentage relative to the length of the nerve. RESULTS: Out of 41 patients assessed, 7 patients had no identifiable eighth cranial nerve disease. In 16 patients there was complete filling of the internal auditory meatus by a tumour mass such that its specific neural origin could not be determined. In the remaining 18 cases, 86 discrete separate foci of tumour origin on the SVN or IVN could be identified including 23 tumours on the right SVN, 26 tumours on the right IVN, 18 tumours on the left SVN and 19 tumours on the left IVN. DISCUSSION: This study, examining the origins of vestibular schwannomas in NF2, refutes their origin as being from a single site on the transition zone of the superior division of the vestibular nerve. We hypothesise a relationship between the number of tumour foci, tumour biology and aggressiveness of disease. The development of targeted drug therapies in addition to bevacizumab are therefore essential to improve prognosis and quality of life in patients with NF2 given the shortcomings of surgery and radiation treatments when dealing with the multifocality of the disease.

Progress of hearing loss in neurofibromatosis type 2: implications for future management.

The objective of this study was to describe changes in hearing over time in patients with neurofibromatosis type 2 (NF2) treated conservatively. A retrospective case review was conducted in a tertiary referral centre. Pure tone audiometry, speech discrimination scores, serviceable hearing (American Academy of Otolaryngology class A or B) and measurement of vestibular schwannoma (VS) size on magnetic resonance imaging were evaluated in 56 patients (89 ears) with NF2 with at least one conservatively managed VS. Over a mean follow-up period of 7 years (range 0.8-21 years) pure tone average thresholds increased gradually with a mean annual rate of 1.3 dB for the right ear (p = 0.0003) and 2 dB for the left ear (p = 0.0009). Speech discrimination scores dropped with an average annual rate of 1.3 and 0.34% in the right and left ear, respectively. Patients maintained serviceable hearing for an average of 7.6 years (range 2.7-19.3 years). The average annual VS growth was 0.4 mm without any correlation with hearing loss. There was a correlation between patients' age and pure tone threshold increase (p < 0.05 for both ears). In this selected population of patients with NF2, hearing threshold increases were very slow. In NF2 patients with indolently behaving tumours, serviceable hearing can be maintained for a significant length of time, making conservative management an attractive option.

Noninvasive tumor hypoxia measurement using magnetic resonance imaging in murine U87 glioma xenografts and in patients with glioblastoma.

PURPOSE: There is a clinical need for noninvasive, nonionizing imaging biomarkers of tumor hypoxia and oxygenation. We evaluated the relationship of T1 -weighted oxygen-enhanced magnetic resonance imaging (OE-MRI) measurements to histopathology measurements of tumor hypoxia in a murine glioma xenograft and demonstrated technique translation in human glioblastoma multiforme. METHODS: Preclinical evaluation was performed in a subcutaneous murine human glioma xenograft (U87MG). Animals underwent OE-MRI followed by dynamic contrast-enhanced MRI (DCE-MRI) and histological measurement including reduced pimonidazole adducts and CD31 staining. Area under the curve (AUC) was measured for the R1 curve for OE-MRI and the gadolinium concentration curve for DCE-MRI. Clinical evaluation in five patients used analogous imaging protocols and analyses. RESULTS: Changes in AUC of OE-MRI (AUCOE ) signal were regionally heterogeneous across all U87MG tumors. Tumor regions with negative AUCOE typically had low DCE-MRI perfusion, had positive correlation with hypoxic area (P = 0.029), and had negative correlation with vessel density (P = 0.004). DCE-MRI measurements did not relate to either hypoxia or vessel density in U87MG tumors. Clinical data confirmed comparable signal changes in patients with glioblastoma. CONCLUSION: These data support further investigation of T1 -weighted OE-MRI to identify regional tumor hypoxia. The quantification of AUCOE has translational potential as a clinical biomarker of hypoxia.

Epithelioid Hemangioendothelioma as a Dangerous, Easy to Miss, and Nearly Impossible to Clinically Diagnose Condition: Case Report.

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with metastatic potential. EHE can have single- or multiorgan involvement, with presentations ranging from asymptomatic disease to pain and systemic symptoms. The extremely heterogeneous clinical presentation and disease progression complicates EHE diagnosis and management. We present the case of a 24-year-old woman with two periauricular erythematous papules, leading to the discovery of metastatic EHE through routine biopsy, despite a noncontributory medical history. Histology revealed the dermal proliferation of epithelioid cells and vacuoles containing red blood cells. Immunohistochemistry markers consistent with EHE solidified the diagnosis. Although extremely rare, prompt diagnosis of EHE is essential for informed decision-making and favorable outcomes. Key clinical and histopathological findings are highlighted to aid dermatologists in diagnosing and managing this uncommon condition.

A semi-automatic segmentation method for meningioma developed using a variational approach model.

BACKGROUND: Meningioma is the commonest primary brain tumour. Volumetric post-contrast magnetic resonance imaging (MRI) is recognised as gold standard for delineation of meningioma volume but is hindered by manual processing times. We aimed to investigate the utility of a model-based variational approach in segmenting meningioma. METHODS: A database of patients with a meningioma (2007-2015) was queried for patients with a contrast-enhanced volumetric MRI, who had consented to a research tissue biobank. Manual segmentation by a neuroradiologist was performed and results were compared to the mathematical model, using a battery of tests including the Sørensen-Dice coefficient (DICE) and JACCARD index. A publicly available meningioma dataset (708 segmented T1 contrast-enhanced slices) was also used to test the reliability of the model. RESULTS: 49 meningioma cases were included. The most common meningioma location was convexity (n = 15, 30.6%). The mathematical model segmented all but one incidental meningioma, which failed due to the lack of contrast uptake. The median meningioma volume by manual segmentation was 19.0 cm3 (IQR 4.9-31.2). The median meningioma volume using the mathematical model was 16.9 cm3 (IQR 4.6-28.34). The mean DICE score was 0.90 (SD = 0.04). The mean JACCARD index was 0.82 (SD = 0.07). For the publicly available dataset, the mean DICE and JACCARD scores were 0.90 (SD = 0.06) and 0.82 (SD = 0.10), respectively. CONCLUSIONS: Segmentation of meningioma volume using the proposed mathematical model was possible with accurate results. Application of this model on contrast-enhanced volumetric imaging may help reduce work burden on neuroradiologists with the increasing number in meningioma diagnoses.

Baseline total brain volume predicts changes in quality of life and overall survival after cranial radiotherapy in older patients with glioblastoma: Results from the prospective BRITER study.

Background: Short-course partial brain radiotherapy ± chemotherapy for older patients with GBM extends survival but there is no validated evidence for prediction of individual risk of acute radiotherapy-related side effects. Methods: This prospective multicentre observational trial recruited patients with newly diagnosed GBM aged ≥65 planned for cranial radiotherapy. Baseline MRI scans were analyzed for markers of brain resilience including relative total brain volume (ratio of cerebrospinal fluid (CSF) volume to total intracranial volume (TIV)) and their relationship to change in quality of life (QoL). Results: 126 patients enrolled: mean age 72 years (range 65-83). 77% had debulking surgery. 79% received radiotherapy with concurrent TMZ, and 21% received palliative radiotherapy alone. The median OS was 10.7 months. After accounting for age, sex, treatment, and baseline MoCA score, there was a relationship between baseline CSF:TIV and change in QoL score at 8 weeks post treatment. For each unit point of increase in CSF:TIV, there was a corresponding decrease in QoL score of 1.72 (95% CI -3.24 to -0.19 P = .027). 35 participants were too unwell to complete questionnaires or had died by the 8 week follow-up visit. In this subgroup, post hoc logistic regression showed baseline CSF:TIV was related to the risk of non-attendance (OR 1.35, 95% CI 1.01 to 1.80, P = .042). Cox regression models showed baseline CSF:TIV was associated with worsened OS (HR 1.41, 95% CI 1.19 to 1.66, P < .001). Conclusions: This study provides evidence to support the use of an imaging biomarker to help assess the risk:benefit ratio for radiotherapy.

Tetrachloroethylene exposure and neurobehavioral performance among children living near multiple contamination sites.

BACKGROUND: Tetrachloroethylene (PCE) is a known neurotoxicant mainly observed in populations with high level occupational exposure, but health effects of low-level community exposure are poorly understood. This study evaluated PCE exposure and neurobehavioral performance in 6- to 11-year-old children living in a community with multiple PCE contamination sites. METHODS: This cross-sectional study recruited eighty-nine children who attended public schools in Martinsville, Indiana. PCE was measured in exhaled breath using proton transfer reaction mass spectrometry, children were classified into three exposure groups 'Level 1' (≤ 0.75 µg/m3), 'Level 2' (0.75-1.90 µg/m3), and 'Level 3' (>1.90 µg/m3). A battery of tests assessed children's neurobehavioral performance related to their cognition, behavior, and academic achievement and a questionnaire collected demographic and exposure characteristics. Adjusted regression models estimated associations of PCE exposure with neurobehavioral outcomes. RESULTS: Seventy-three participants completed data collection. The average scores for all participants on the Fluid, Total, Early Childhood Composites, Behavioral Symptoms Index, and Math Computation were significantly lower than standard scores of normative sample, with 30 % of participants having one or more scores two standard deviations worse than the standard scores. Compared to children having the lowest PCE exposure, significant inverse associations were identified for PCE exposure with Fluid Composite for Level 2 (ß = -12.0 (95 % CI = -23.4, -0.6)) and Level 3 (-12.2 (-23.4, -1.0)) exposure groups, Total Composite for Level 2 (-10.1 (-19.2, -1.1)) and Level 3 (-11.8 (-20.4, -3.2)) exposure groups, and Early Childhood Composite for Level 3 exposure group (-11.0 (-18.7, -3.4)). CONCLUSIONS: This study identified associations of PCE in exhaled breath with lower cognitive functioning, problem-solving abilities, and adaptive functioning in children. Mainly, working memory, episodic memory, processing speed, and executive function-cognitive flexibility were affected by PCE exposure. These results underscore the need for further investigations, considering the impact of low-level environmental exposure on children's neurobehavioral outcomes.

Tectal Plate Glioma: A Clinical and Radiologic Analysis of Progression and Management in Adults.

BACKGROUND: Tectal plate gliomas (TPGs) are a heterogeneous group of uncommon brain tumors. TPGs are considered indolent and are usually managed conservatively but they have the potential to transform into higher-grade tumors. The aims of this study were to investigate the natural history of adult TPG, treatment outcomes, and overall survival. METHODS: A retrospective cohort analysis was performed of adult patients with TPG between 1993 and 2021. Baseline clinical, radiologic, and management characteristics were collected. The primary outcome was tumor progression, defined as increasing size on radiologic assessment or new gadolinium contrast enhancement. Secondary outcomes included management and mortality. RESULTS: Thirty-nine patients were included, of whom 23 (52.2%) were men. Median age at diagnosis was 35 years (interquartile range, 27-53). Radiologic tumor progression was observed in 8 patients (20.5%). The 10-year progression-free survival was 72.6% (95% confidence interval [CI], 0.58-0.91). The 10-year overall survival was 86.5% (95% confidence interval, 0.75-1.0). Cerebrospinal fluid diversion procedures were used in 62% of the cohort (n = 24). Seventeen patients (43.6%) underwent at least 1 endoscopic third ventriculostomy, whereas only 6 patients (15.4%) underwent at least 1 ventriculoperitoneal shunt. CONCLUSIONS: TPG has an overall favorable clinical prognosis, although progression occurs in 1 in 5 patients. Showing accurate factors by which patients with TPG may be risk stratified should be a key area of further research. A follow-up duration of 10 years would be a reasonable window based on the radiologic progression rates in this study; however, larger cohort studies are needed to answer both questions definitively.

Sporadic Creutzfeldt-Jakob disease presenting as progressive nonfluent aphasia with speech apraxia.

Progressive non-fluent aphasia (PNFA) is typically associated with pathological changes consistent with frontotemporal lobar degeneration. A 65-year-old male presented with effortful speech, markedly impaired naming and features of speech apraxia, consistent with PNFA. Perceptuospatial function, calculation and executive function were intact. Brain SPECT showed left perisylvian hypoperfusion. He deteriorated profoundly over the subsequent eight months, with appearances on diffusion-weighted magnetic resonance imaging typical of sporadic Creutzfeldt-Jakob disease, which was confirmed pathologically at postmortem examination. While the presence of PNFA with speech apraxia is thought to predict underlying tauopathy, sporadic Creutzfeldt-Jakob disease may mimic this presentation and present in a highly circumscribed form not previously described.