Infratentorial Research Articles

Cross-Sectional Validation of an Automated Lesion Segmentation Software in Multiple Sclerosis: Comparison with Radiologist Assessments.

Magnetic Resonance Imaging is widely used to assess disease burden in multiple sclerosis (MS). This study aimed to evaluate the effectiveness of a commercially available k-nearest neighbors (k-NN) software in quantifying white matter lesion (WML) burden in MS. We compared the software's WML quantification to expert radiologists' assessments. We retrospectively reviewed brain MRI examinations of adult MS patients and of adult patients without MS and with a normal brain MRI referred from the neurology clinic. MRI images were processed using an AI-powered, cloud-based k-NN software, which generated a DICOM lesion distribution map and a report of WML count and volume in four brain regions (periventricular, deep, juxtacortical, and infratentorial white matter). Two blinded radiologists performed semi-quantitative assessments of WM lesion load and lesion segmentation accuracy. Additionally, four blinded neuroradiologists independently reviewed the data to determine if MRI findings supported an MS diagnosis. Results were considered significant when p < 0.05. The study included 32 MS patients (35.4 years ± 9.1) and 19 patients without MS (33.5 years ± 12.1). The k-NN software demonstrated 94.1% and 84.3% accuracy in differentiating MS from non-MS subjects based respectively on WML count and WML volume, compared to radiologists' accuracy of 90.2% to 94.1%. Lesion segmentation was more accurate for the deep WM and infratentorial regions than for the juxtacortical region (both p <0.001). k-NN-derived WML volume and WML count provide valuable quantitative metrics of disease burden in MS. AI-powered post-processing software may enhance the interpretation of brain MRIs in MS patientsABBREVIATIONS: MS = multiple sclerosis; k-NN＝k-Nearest Neighbors; WML＝white matter lesion; MPRAGE = Magnetization-Prepared Rapid Acquisition Gradient Echo; SPACE = Sampling Perfection with Application-optimized Contrasts using a Different Flip Angle Evolution; EDSS = Expanded Disability Status Scale.

Improved identification of multiple sclerosis brain lesions with three-dimensional double inversion recovery MRI

Background Multiple sclerosis (MS) is a chronic autoimmune disease that results in demyelination within the central nervous system, causing brain lesions and neurological dysfunction. Precise identification of these lesions is essential for proper diagnosis and management. Aim This study aims to assess the effectiveness of three-dimensional double inversion recovery (3D DIR) MRI in detecting MS brain lesions compared to two-dimensional fluid-attenuated inversion recovery (2D FLAIR) and T2-weighted imaging (T2WI). Patients and methods A cross-sectional study involving 49 MS patients (21 patients with clinically suspected MS, 28 patients with remitting relapsing MS). MRI scans were performed using a standardized protocol, including 3D DIR, 2D FLAIR, and T2WI sequences. Lesion detection rates and regional lesion visibility were analyzed and compared. Results 3D DIR MRI significantly improved lesion detection rates, identifying 19% more lesions than 2D FLAIR and 28% more than T2WI (P&lt;0.001 for both). Regionally, 3D DIR MRI showed superior detection in the periventricular (13%, 23% higher), deep white matter (8%, 14% higher), juxtacortical (10%, 27% higher), intracortical (71%, 91% higher), and infratentorial (27%, 10% higher) regions compared to 2D FLAIR and T2WI, respectively (P&lt;0.001 for all). Conclusion 3D DIR MRI is more effective in detecting MS brain lesions than 2D FLAIR and T2WI, suggesting its integration into routine MS imaging protocols for better diagnostic accuracy.

Cerebral Microbleeds and Antiplatelet Therapy in Mongolian and Han Patients with Ischemic Cerebrovascular Disease.

To analyze the differences in cerebral microbleeds (CMBs) and their correlation with intracerebral hemorrhage (ICH) in Mongolian and Han Chinese patients with ischemic cerebrovascular disease. A total of 160 patients with ischemic cerebrovascular disease who took aspirin or clopidogrel for over one year were retrospectively analyzed, including 80 Mongolian and 80han patients. The incidence, number, and distribution of CMBs were compared between groups. Logistic regression was used to identify risk factors for the occurrence of cerebral hemorrhage. The detection rate of CMBs was significantly lower in Mongolian patients compared to Han patients (P = 0.040). Mongolian patients had a higher distribution of CMBs in the deep or infratentorial regions (66.6% vs 58.1%), while Han patients had a higher lobar distribution (P = 0.007). Prolonged antiplatelet therapy (over 3 years) was a risk factor for CMB development in both groups and was also linked to an increased risk of ICH. Patients with a higher number of CMBs had a greater likelihood of experiencing ICH. Mongolian patients had a lower likelihood of developing CMBs than Han patients, but with a higher deep or infratentorial distribution. The presence of CMBs, especially with long-term antiplatelet therapy, is a significant predictor of ICH. No significant difference in ICH risk was found between ethnic groups. Close monitoring of patients with CMBs during prolonged antiplatelet therapy is crucial to reduce hemorrhagic events.

Analysis of the Molecular Profile of Melanoma Brain Metastases using Immunohistochemistry

AbstractBrain metastases from melanoma represent an advanced and aggressive form of the disease. Understanding their molecular characteristics is crucial for improving diagnosis, prognosis, and treatment. The main objectives of this study were to identify and characterize molecular alterations present in brain metastases from melanoma and to clinically correlate them. The clinical-epidemiological information was anonymously and retrospectively obtained. Brain metastases from melanoma were collected through surgical biopsies, which were processed and analyzed using immunohistochemical markers. A total of 132 samples were initially selected, resulting in 8 samples of metastatic melanoma in the brain region included in this analysis. Regarding the frequency of immunohistochemical markers in brain samples of melanoma metastasis, 75% showed positive BRAF V600R, 62% contained positive SOX10, 37% positive HMB45, and 25% positive Melan-A. Regarding the location, 4 of them were in the frontal lobe, 3 in the parietal lobe, and 1 in the cerebellum. Among the samples, 87.5% originated from the supratentorial region of the brain, while 12.5% came from the infratentorial region. At the time, half of the patients were under 65 years old, with a significant proportion of 25% of individuals being below 40 years old. Moreover, 87.5% had two or more brain lesions at the time of diagnosis. Among the 12.5% that comprised the single lesion group at the time of analysis, the frontal lobe location stands out. The findings indicate heterogeneity among tumors and metastases in different anatomical locations. Further investigations are needed to validate these findings and elucidate their clinical applicability.

Sex differences in brain MRI using deep learning toward fairer healthcare outcomes.

This study leverages deep learning to analyze sex differences in brain MRI data, aiming to further advance fairness in medical imaging. We employed 3D T1-weighted Magnetic Resonance images from four diverse datasets: Calgary-Campinas-359, OASIS-3, Alzheimer's Disease Neuroimaging Initiative, and Cambridge Center for Aging and Neuroscience, ensuring a balanced representation of sexes and a broad demographic scope. Our methodology focused on minimal preprocessing to preserve the integrity of brain structures, utilizing a Convolutional Neural Network model for sex classification. The model achieved an accuracy of 87% on the test set without employing total intracranial volume (TIV) adjustment techniques. We observed that while the model exhibited biases at extreme brain sizes, it performed with less bias when the TIV distributions overlapped more. Saliency maps were used to identify brain regions significant in sex differentiation, revealing that certain supratentorial and infratentorial regions were important for predictions. Furthermore, our interdisciplinary team, comprising machine learning specialists and a radiologist, ensured diverse perspectives in validating the results. The detailed investigation of sex differences in brain MRI in this study, highlighted by the sex differences map, offers valuable insights into sex-specific aspects of medical imaging and could aid in developing sex-based bias mitigation strategies, contributing to the future development of fair AI algorithms. Awareness of the brain's differences between sexes enables more equitable AI predictions, promoting fairness in healthcare outcomes. Our code and saliency maps are available at https://github.com/mahsadibaji/sex-differences-brain-dl.

Brachyury Expression in Atypical Teratoid/Rhabdoid Tumor.

Atypical teratoid/rhabdoid tumor (AT/RT) is a rare, highly aggressive central nervous system tumor of childhood with variable morphologic features, which is characterized by alterations in SWI/SNF complex, such as SMARCB1/INI1 or SMARCA4/BRG1 loss. Poorly differentiated chordoma is the differential diagnosis, particularly in the infratentorial region of older children and the use of brachyury in such conditions is under debate. We investigated the brachyury expression in 44 samples of AT/RT from 40 patients. All AT/RTs except 2 infratentorial tumors were negative for brachyury (2 clones: A-4 and EPR18113). Both brachyury-positive tumors (one diffuse and one patchy expression) involved the clivus of children younger than 2 years old. The DNA methylation profile of one of these tumors showed epigenetic similarity to reference examples of chordoma in 2 public unsupervised and one supervised analysis systems. The second tumor exhibited a classical epigenetic microarray pattern found in samples with degraded DNA. We revised 2 initial AT/RT diagnoses as poorly differentiated chordoma based on the morphology, brachyury expression, topographical features, and methylation profile. Differentiating poorly differentiated chordoma from AT/RT could be challenging; brachyury expression can be useful in diagnosing poorly differentiated chordoma over AT/RT in suitable clinical and radiological settings.

The MRI Features of Intracranial Lesions in MOG-IgG Associated Disease

AimTo summarize and analyze the MRI characteristics of patients with Myelin oligodendrocyte glycoprotein-IgG associated disease (MOGAD), and to enhance the accuracy of disease diagnosis and advance scientific research. Materials and methodsA retrospective collection of clinical data from 103 patients with MOGAD was conducted. The distribution and signal characteristics of intracranial lesions on MRI were analyzed. Further subgroup statistical analysis based on age was performed to explore differences in lesion locations among different subgroups. Statistical comparisons were made using the χ2 test or Fisher's exact test, with a significance level of p<0.05 considered statistically significant. ResultsMRI revealed variable lesion morphologies in patients with MOGAD. Lesions were predominantly located in the cerebral deep white matter (47.6%), subcortical white matter (38.8%) and cortex (38.8%) of supratentorial region, as well as in the brainstem (35.9%) of infratentorial region. Notably, there was a significantly higher proportion of juvenile patients with thalamic involvement compared to adult patients (p=0.013). Juvenile patients were more likely to have lesions involving both the thalamus and cerebral cortex (p=0.040), thalamus and deep white matter (p=0.026), or thalamus and brainstem (p=0.014). Conversely, lesions involving both the corpus callosum and subcortical white matter were more frequently observed in adult patients, with statistically significant differences (p=0.046). Post-contrast MRI showed mild enhancement in some lesions, with a half of cases exhibiting leptomeningeal enhancement. One rare case presented extensive thickening and enhancement of the falx cerebri. ConclusionThe distribution of intracranial lesions on MRI exhibits distinct characteristics. The differences in the spatial distribution of intracranial lesions between juvenile and adult patients suggest that MOGAD may represent a heterogeneous disease spectrum that varies with age.

Investigation of Hemorheological Parameters in Ischemic Stroke Patients.

The aim of this study is to determine whether there are any changes in hemorheological parameters, including whole blood viscosity (WBV), plasma viscosity, erythrocyte aggregation, and erythrocyte deformability, in acute ischemic stroke patients, and to establish their relationship with stroke etiology. The study also aims to observe the changes in these parameters, if any, over time and after treatment, and to assess the correlation between risk factors for ischemic stroke and neuroimaging findings. This was a prospective observational study including 70 patients diagnosed with acute ischemic stroke within the first 3 days of the onset of symptoms and 96 healthy controls. Stroke patients were categorized based on TOAST criteria, and hemorheological parameters were measured at admission and on the fifth day post-treatment. Erythrocyte aggregation and deformability were measured using a laser ektacytometer, and viscosity assessment was conducted with a rotational viscometer. Stroke patients exhibited significant differences from the control group in aggregation amplitude, aggregation index, and aggregation half-time (p = 0.001, p = 0.013, p = 0.009, respectively) and showed elevated maximum value of elongation index (p = 0.000). No significant differences in WBV and plasma viscosity were observed between the groups. Post-treatment, the small vessel occlusion subgroup demonstrated a notable reduction in WBV. Additionally, homocysteine levels showed a positive correlation with scattered white matter lesions in basal ganglia and infratentorial regions (p = 0.002, p = 0.039, respectively). Increased predisposition to erythrocyte aggregation may contribute to the occurrence of acute ischemic stroke. Moreover, gaining the ability of erythrocytes to deform and increase blood flow may serve as a compensatory mechanism in the chronic vascular disease process. The risk factors for ischemic stroke may exhibit connections to specific areas within the brain.

9375 Acth-Producing Pituitary Carcinoma in the Falx Cerebri and Other Unusual Locations

Abstract Disclosure: J.E. Esquivel Vargas: None. A. Cob Guillén: None. A.B. Santos Rojo: None. A. Hong Lo: None. ACTH-producing Pituitary Carcinoma in the Falx Cerebri and other unusual locations Background: Pituitary carcinoma is a condition defined by metastasis of a pituitary tumor to a distant location. Pituitary carcinomas are very rare and account for approximately 0.12% of adenohypophyseal tumors (1). Clinical case: We present a case of a 29-year-old female who was followed up in our Endocrinology Department. Past medical history included the diagnosis of Cushing Disease and transsphenoidal tumor resection at 12 years of age, followed by transcranial resection two years later because of persistently elevated adrenocorticotropic hormone (ACTH). Despite the surgical management, the patient persisted with increased ACTH and hypercortisolism, so bilateral adrenalectomy was performed a year later. Two years after the procedure, the patient presented with a newly diagnosed pituitary macroadenoma, so the diagnosis of Nelson Syndrome was made. Linear accelerator radiotherapy was given, which reduced the size of the tumor. Later, multiple imaging studies showed multiple lesions on the falx cerebri, posterior clinoid process, retroclival region, cerebellopontine angle, pterygopalatine fossa, infratentorial region, and posterior ethmoidal cells. Biopsy and immunohistochemistry of the falx cerebri lesions described ACTH-producing pituitary adenocarcinoma. Treatment was given with intramuscular octreotide, dabrafenib, and trametinib. Despite persistently elevated ACTH levels, the patient has since remained clinically stable, without new development or worsening of symptoms. Conclusion: There are 3 unique aspects of our case. First, we reported an unusual presentation of this disease, because the patient in our case was a female with an early age of onset, in contrast with the reported literature. Second, this is the first reported case demonstrating pituitary carcinoma in the falx cerebri. Third, the prognosis of pituitary carcinoma is very poor, and mortality is extremely high, however, the patient in our case has been followed up for 7 years since the diagnosis of the metastatic lesions and has remained clinically stable.

8386 The Evolving Face of The Great Masquerader - Panhypopituitarism Associated with Neurosyphilis

Abstract Disclosure: M. Yabe: None. Z. Lin: None. F. Joseph: None. G. Philip: None. R. Silver: None. L. Callejas: None. J. Giunta: None. Untreated syphilis is on the rise in the United States. The CDC reported syphilis cases in newborns have increased 10-fold over a decade in November 2023, signifying the emergence of untreated infections. Pituitary involvement in syphilis is a very rare phenomenon, and endocrinological investigation is often overlooked. This will be the second reported case of neurosyphilis with pituitary involvement in a non-HIV infected patient. A 45-year-old female with no significant past medical history was admitted to the hospital with progressive neurological symptoms of intermittent left-side headache, hearing loss with tinnitus, and gait instability. Postcontrast MRI Brain revealed multifocal leptomeningeal enhancement involving supratentorial and infratentorial regions. Thickening of the enhanced pituitary infundibulum was also noted. Subsequent RPR titer showed 1:16 and lumbar puncture revealed CSF-VDRL titer 1:32, with pleocytosis 83 cells/uL, CSF-glucose 19 mg/dL, and CSF-protein 370 mg/dL. The patient completed Penicillin G 4 million/units intravenous every 4 hours for 14 days, with repeat MRI showing significant interval decrease in previously described extensive supratentorial and infratentorial leptomeningeal enhancement. However, there is no record of her sexual partner receiving treatment. The patient was readmitted one year later after a fall while displaying dysmetria. MRI Brain with contrast revealed a small subacute infarct along the right frontal corona radiata and pituitary gland measuring up to 1.2 cm in craniocaudal dimension compared to 0.4 cm on the prior exam. This prompted evaluation of pituitary hormones: FSH 0.8 IU/L, LH &amp;lt;0.3 IU/L, and ACTH 6.3 pg/mL, with normal serum growth hormone and prolactin levels. Further investigation revealed morning cortisol 2.5 ug/dL, thyrotropin &amp;lt; 0.2 uIU/mL, and free thyroxine 0.63 ng/dL. Radiological evidence is suspicious of inflammatory etiology of the pituitary gland. The patient was discharged with endocrinology follow-up and plan to perform a pituitary gland biopsy as an outpatient for confirmatory result. The association of neurosyphilis and hypophysitis leading to panhypopituitarism in a non-HIV infected patient was first reported in 2015. Since then, there remains limited research regarding their association due to rarity of pituitary involvement in neurosyphilis. With untreated syphilis on the rise and increased availability of MRI, clinicians should expect to encounter more cases of neurosyphilis with pituitary involvement in the future. Early detection of panhypopituitarism in those with positive CSF-VDRL titer could lead to better clinical outcomes. It is paramount that these cases are documented to emphasize pituitary involvement in neurosyphilis and encourage a low threshold to initiate endocrinological testing in the future. Presentation: 6/3/2024

Demographic Profile and Neuro-Ophthalmic Manifestations in Primary Intracranial Tumours in Adult Patients: An Observational Study from a Tertiary Care Health Centre in Eastern India

Abstract Purpose: To evaluate the demographic profile and neuro-ophthalmic manifestations of primary brain tumours in adult patients presenting to a tertiary care centre in eastern India. Methods: A hospital-based cross-sectional study was conducted on patients with primary intracranial tumours presenting to a tertiary care multi-specialty hospital in eastern India from January 2018 to October 2021. A complete neurosurgical, endocrinal, and ophthalmological evaluation was done in each patient. Results: A total of 106 patients with primary brain tumours were included in the study. The mean age of presentation was 40.98 ± 12.85 years. There were 50 (47.17%) males and 56 (52.83%) females. The tumour location was supratentorial (ST) in 72 (67.92%) patients and infratentorial (IT) in 34 (32.08%) patients. Pituitary adenoma was the most common tumour in the present study (32.07%). The most common tumour was pituitary adenoma in the ST location and cerebellopontine angle schwannoma in the IT location. Headache and hearing loss were the most common presenting symptoms in ST and IT tumours, respectively. Blurring of vision was the most common ocular symptom in 47.16% of cases. Hemianopic field defect was the most common field defect in our study and was seen in 47.72% of cases. Conclusion: Meticulous ophthalmological examination is essential in patients with intracranial tumours. Our study emphasises the importance of a detailed ophthalmological examination and knowledge of associated neurological manifestations in ST and IT tumours.

AB055. Infantile high-grade glioma (IHG)-a case series of Hong Kong experience.

Infantile high-grade glioma (IHG) is diagnosed in patients less than 12 months of age. Studies have shown that it displays a more stable genome and is usually single mutation-driven. The most identifiable mutations are receptor tyrosine kinase (RTK) fusion, such as neurotrophic tyrosine receptor kinase (NTRK) family, reactive oxygen species (ROS1), anaplastic lymphoma kinase (ALK), and mesenchymal-epithelial transition (MET) factor. The current principal treatment remains to be surgery, but it is challenging for a complete resection due to hemispheric involvement. Use of chemotherapeutic drugs for IHG is still under debate, with targeted therapy showing efficacy in promoting tumor shrinkage. Despite being a challenging central nervous system (CNS) tumor, the overall survival of IHG is superior to other high-grade gliomas. This is a retrospective review of local IHG patients and their outcome. Up till the end of 2022, we identified eight IHG patients in our local data. Mean age of diagnosis was 3 months. There were four males and four females. Seven patients had histological diagnosis of glioblastoma and one patient had a diagnosis of anaplastic astrocytoma. One patient had her tumor located in the infratentorial region. Four patients had multilobar involvement. NTRK fusion was found in four patients (ETV6-NTRK3 fusion and TPR-NTRK1 fusion). ALK fusion was found in one patient (HMBOX1-ALK). ROS1 fusion was found in one patient (ZCCHZ8-ROS1). All patients underwent chemotherapy, with four patients switched to NTRK inhibitors and one patient to ROS1 inhibitors afterward. Surgery was performed at various time points for these patients. Two patients passed away, at 22 and 35 months of age at submission of this abstract. Infantile high-grade glioma should be regarded as a unique tumor entity and a multidisciplinary approach is paramount in improving survival for this group of patients.

Low-Field (64 mT) Portable MRI for Rapid Point-of-Care Diagnosis of Dissemination in Space in Patients Presenting with Optic Neuritis.

Low-field 64 mT portable brain MRI has recently shown diagnostic promise for MS. This study aimed to evaluate the utility of portable MRI (pMRI) in assessing dissemination in space (DIS) in patients presenting with optic neuritis and determine whether deploying pMRI in the MS clinic can shorten the time from symptom onset to MRI. Newly diagnosed patients with optic neuritis referred to a tertiary academic MS center from July 2022 to January 2024 underwent both point-of-care pMRI and subsequent 3T conventional MRI (cMRI). Images were evaluated for periventricular (PV), juxtacortical (JC), and infratentorial (IT) lesions. DIS was determined on brain MRI per 2017 McDonald criteria. Test characteristics were computed by using cMRI as the reference. Interrater and intermodality agreement between pMRI and cMRI were evaluated by using the Cohen κ. Time from symptom onset to pMRI and cMRI during the study period was compared with the preceding 1.5 years before pMRI implementation by using Kruskal-Wallis with post hoc Dunn tests. Twenty patients (median age: 32.5 years [interquartile range {IQR}, 28-40]; 80% women) were included, of whom 9 (45%) and 5 (25%) had DIS on cMRI and pMRI, respectively. Median time interval between pMRI and cMRI was 7 days (IQR, 3.5-12.5). Interrater agreement was very good for PV (95%, κ = 0.89), and good for JC and IT lesions (90%, κ = 0.69 for both). Intermodality agreement was good for PV (90%, κ = 0.80) and JC (85%, κ = 0.63), and moderate for IT lesions (75%, κ = 0.42) and DIS (80%, κ = 0.58). pMRI had a sensitivity of 56% and specificity of 100% for DIS. The median time from symptom onset to pMRI was significantly shorter (8.5 days [IQR 7-12]) compared with the interval to cMRI before pMRI deployment (21 days [IQR 8-49], n = 50) and after pMRI deployment (15 days [IQR 12-29], n = 30) (both P < .01). Time from symptom onset to cMRI in those periods was not significantly different (P = .29). In patients with optic neuritis, pMRI exhibited moderate concordance, moderate sensitivity, and high specificity for DIS compared with cMRI. Its integration into the MS clinic reduced the time from symptom onset to MRI. Further studies are warranted to evaluate the role of pMRI in expediting early MS diagnosis and as an imaging tool in resource-limited settings.

Clinico-Demographic Profile of Paediatric Patients with Primary Intracranial Tumours in South India.

The central and peripheral nervous system cancers are the second most common cancers among the paediatric population (0-14 years). The non-specific symptoms seen in its clinical presentation make the diagnosis of these tumours extremely challenging among children compared to adults. This researchstudy was therefore done to study the socio-demographic profile, clinical features, and management practices among paediatric patients with intracranial tumours. This was across-sectional medical record-based study done among 31 children aged < 18years diagnosed with primary intracranial tumours. Data were recorded using a semi-structured proforma. The median age at diagnosis of tumours among the patients was 7(4, 15) years. Majority of them were males 17(54.8%). Medulloblastoma was the common tumour 12(38.7%). 18(58.1%) tumours originated from the infratentorial region. The most common infratentorial tumour was medulloblastoma, while the most common supratentorial tumour was meningioma. The most common site of involvement was the brainstem 11(35.5%). Majority of the tumours were presently in Stage III/IV 20(64.5%). The most common symptom was vomiting 18(58.1%). 23(74.2%) patients were managed using chemotherapy and among them the most used drug was Temozolomide 7(30.4%). Medulloblastoma was present more among patients who were diagnosed at the age ≤ 5years (p = 0.022). Tumours involving the cerebellum were more among females (p = 0.0118). Headache was present more among patients who were diagnosed with tumours at theage > 5years compared to theage ≤ 5years (p = 0.0119). From the findings of this study, it can be concluded thatmajority of the patients were males. Medulloblastoma was the most common tumour. Close to two-third of the tumours were in Stage III/IV. The most common symptom reported was vomiting. The most used drug for treatment was Temozolomide.

IMG-07. PEDIATRIC CRANIAL INCIDENTALOMAS: A SYSTEMATIC REVIEW UTILIZING MACHINE LEARNING

Abstract BACKGROUND The use of diagnostic neuroimaging in the care of pediatric patients continues to increase, leading to a rise in incidentally discovered lesions. The understanding of appropriate assessment and management of these findings is critical to providing comprehensive and timely care. This review aims to analyze the current literature to characterize diagnostic and prognostic characteristics of incidentally found brain tumors in pediatric patients. METHODS Using the guidelines under the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA), Pubmed and Medline (NCBI) databases were searched using selected keyword combinations and machine learning-assisted software. Case reports, case series, and retrospective cohort studies published up to December 2023 were included. RESULTS A total of 20 studies encompassing 627 patients were included in the review. The most common indications for imaging were headache (204; 33%), seizures (96; 15%), and trauma (78; 12%). Of studies providing information regarding localization, lesions were found in the supratentorial region in 327 (52%) cases and infratentorial region in 190 (30%). Of studies reporting imaging characteristics, T1 hypointensity (183/302; 61%), T2 hyperintensity (410/497; 82%), and no contrast enhancement (58/97; 60%) were the most common findings. Four hundred and thirty (68%) patients were observed after diagnosis, 83 (13%) patients underwent surgical intervention at diagnosis, and 40 (6%) patients underwent surgery after observation. Of 91 patients with histopathology data available, pilocytic astrocytoma (19; 21%) was most commonly reported. CONCLUSION In this study, we provide a review of diagnostic characteristics of incidentally discovered brain tumors in the pediatric population. Management of such lesions has changed greatly over the past several decades, and risks of surgical intervention should be weighed against a more conservative management and interval monitoring approach. These results may help guide discussions regarding disclosure of findings and recommendations for additional clinical management.

Automatic lesion detection at Multiple Sclerosis patients – Comparison of 2D- and 3D-FLAIR-datasets

BackgroundMultiple Sclerosis (MS) is a common autoimmune inflammatory disease of the central nervous system (CNS). Magnetic Resonance Imaging (MRI) allows a sensitive assessment of the CNS and is established for diagnostic, prognostic and (therapy-) monitoring purposes. Especially lesion counting in T2- or Fluid Attenuated Inversion Recovery (FLAIR)-weighted images plays a decisive role in clinical routine. Software-packages allowing an automatic evaluation of image data are increasingly established aiming a faster and improved workflow. These programs allow e.g. the counting, spatial attribution and volumetry of MS-lesions in FLAIR-weighted images. Research has shown that 3D-FLAIR-sequences are superior to 2D-FLAIR-sequences in visual evaluation of lesion burden in MS. An influence on the automatic analysis is expectable but not yet systematically studied. This work will therefore investigate the influence of 2D- and 3D datasets on the results of an automatic assessment. Material and methodsIn this prospective study, 80 Multiple Sclerosis patients underwent a clinically indicated routine MRI examination. The clinical routine protocol already including a 3D-FLAIR sequence was adapted by an additional 2D-FLAIR sequence also conform to the 2021 MAGNIMS-CMSCNAIMS consensus recommendations. To obtain a quantitative analysis for assessment of amount, dissemination and volume of the lesions, the acquired MR images were post-processed using the CE-certified Software mdbrain (mediaire, Berlin, Germany). The resulting data were statistically analysed using the paired t-test for normally distributed data and the Wilcoxon-signed-rank-test for not normally distributed data respectively. Demographic data and data such as the subtype, duration, severity and therapy of the disease were collected, pseudonymized and evaluated. ResultsThere is a significant difference concerning the total number and lesion volume with more lesions being detected (2D: 29.7, +/- 20.22 sd; 3D: 40.1 +/- 31.67 sd; p < 0.0001) but lower total volume (2D: 6.24 +/- 6.11 sd; 3D: 5.39 +/- 6.37 sd; p < 0.0001) when using the 3D- sequence. Especially significantly more small lesions in the unspecific white matter and infratentorial region were detected by using the 3D-FLAIR sequence (p < 0.0001) compared to the 2D-FLAIR image. Main reason for the lower total volume in the 3D-FLAIR sequence was the calculated volume for periventricular lesions which was significantly beneath the calculated volume from the 2D-FLAIR sequence (p < 0.0001). ConclusionAutomatic lesion counting and volumetry is feasible with both 2D- and 3D-weightend FLAIR images. Still, it leads to partly significant differences even between two sequences that both are conform to the 2021 MAGNIMS-CMSCNAIMS consensus recommendations. This study contributes valuable insights into the impact of using different input data from the same patient for automated MS lesion evaluation.

Brain perfusion changes in beta-thalassemia

BackgroundBrain injury in hereditary hemoglobinopathies is commonly attributed to anemia-related relative hypoperfusion in terms of impaired oxygen blood supply. Supratentorial and infratentorial vascular watershed regions seem to be especially vulnerable, but data are very scarce.AimsWe investigated a large beta-thalassemia sample with arterial spin labeling in order to characterize regional perfusion changes and their correlation with phenotype and anemia severity.MethodsWe performed a multicenter single-scanner cross-sectional 3T-MRI study analyzing non-invasively the brain perfusion in 54 transfusion-dependent thalassemia (TDT), 23 non-transfusion-dependent thalassemia (NTDT) patients and 56 Healthy Controls (HC). Age, hemoglobin levels, and cognitive functioning were recorded.ResultsBoth TDT and NTDT patients showed globally increased brain perfusion values compared to healthy controls, while no difference was found between patient subgroups. Using age and sex as covariates and scaling the perfusion maps for the global cerebral blood flow, beta-thalassemia patients showed relative hyperperfusion in supratentorial/infratentorial watershed regions. Perfusion changes correlated with hemoglobin levels (p = 0.013) and were not observed in the less severely anemic patients (hemoglobin level > 9.5 g/dL). In the hyperperfused regions, white matter density was significantly decreased (p = 0.0003) in both patient subgroups vs. HC. In NTDT, white matter density changes correlated inversely with full-scale Intelligence Quotient (p = 0.007) while in TDT no correlation was found.ConclusionRelative hyperperfusion of watershed territories represents a hemodynamic hallmark of beta-thalassemia anemia challenging previous hypotheses of brain injury in hereditary anemias. A careful management of anemia severity might be crucial for preventing structural white matter changes and subsequent long-term cognitive impairment.

Radiotherapy plus temozolomide with or without anlotinib in H3K27M-mutant diffuse midline glioma: A retrospective cohort study.

Besides the hallmark of H3K27M mutation, aberrant amplifications of receptor tyrosine kinases (RTKs) are commonly observed in diffuse midline glioma (DMG), a highly malignant brain tumor with dismal prognosis. Here, we intended to evaluate the efficacy and safety of a multitarget RTK inhibitor anlotinib in patients with H3K27M-DMG. A total of 40 newly diagnosed H3K27M-DMG patients including 15 with anlotinib and 25 without anlotinib treatment were retrospectively enrolled in this cohort. Progression-free survival (PFS), overall survival (OS), and toxicities were assessed and compared. The median PFS and OS of all patients in this cohort were 8.5 months (95% CI, 6.5-11.3) and 15.5 months (95% CI, 12.6-17.1), respectively. According to the Response Assessment in Neuro-Oncology (RANO) criteria, the disease control rate in the anlotinib group [93.3%, 95% confidence interval (CI), 70.2-98.8] was significantly higher than those without anlotinib (64%, 95% CI: 40.5-79.8, p = 0.039). The median PFS of patients with and without anlotinib was 11.6 months (95% CI, 7.8-14.3) and 6.4 months (95% CI, 4.3-10.3), respectively. Both the median PFS and OS of DMG patients treated with anlotinib were longer than those without anlotinib in the infratentorial patients (PFS: 10.3 vs. 5.4 months, p = 0.006; OS: 16.6 vs. 8.7 months, p = 0.016). Multivariate analysis also indicated anlotinib (HR: 0.243, 95% CI: 0.066-0.896, p = 0.034) was an independent prognosticator for longer OS in the infratentorial subgroup. In addition, the adverse events of anlotinib administration were tolerable in the whole cohort. This study first reported that anlotinib combined with Stupp regimen is a safe and feasible regimen for newly diagnosed patients with H3K27M-DMG. Further, anlotinib showed significant efficacy for H3K27M-DMG located in the infratentorial region.

Clinical features of adult patients with positive NMDAR-IgG coexisting with MOG-IgG.

This study was designed to analyze clinical and radiographic features of adult patients coexisting with NMDAR-IgG and MOG-IgG. Eleven adult patients coexisting with NMDAR-IgG and MOG-IgG were collected from Xiangya Hospital, Central South University, between June 2017 and December 2021. Fifty-five patients with anti-NMDAR encephalitis and 49 with MOG-AD were served as controls. Onset age was 27 (IQR 20-34) years old. Seizures and psychotic symptoms were prominent symptoms. Ten of eleven patients presented abnormal T2/FLAIR hyperintensity, mainly involving the cortex, brainstem, and optic nerve. Compared with the NMDAR IgG ( +)/MOG IgG ( -) group, the NMDAR IgG ( +)/MOG IgG ( +) group showed more ataxia symptoms (27.3% vs. 3.6%, P = 0.037), while more T2/FLAIR hyperintensity lesions were found in the brainstem (54.5% vs. 7.3%, P < 0.001) and optic nerve (27.3% vs. 1.8%, P = 0.011) with more abnormal MRI patterns (90.9% vs. 41.8%, P = 0.003). In comparison with the NMDAR IgG ( -)/MOG IgG ( +) group, the NMDAR IgG ( +)/MOG IgG ( +) group had more seizures (72.7% vs. 24.5%, P = 0.007) and mental symptoms (45.5% vs. 0, P < 0.001). The NMDAR IgG ( +)/MOG IgG ( +) group tended to be treated with corticosteroids alone (63.6% vs. 20.0%, P = 0.009), more prone to recur (36.5% vs. 7.3%, P = 0.028) and lower mRS score (P = 0.036) at the last follow-up than pure anti-NMDAR encephalitis. The symptoms of the NMDAR IgG ( +)/MOG IgG ( +) group were more similar to anti-NMDAR encephalitis, while MRI patterns overlapped more with MOG-AD. Detecting both NMDAR-IgG and MOG-IgG maybe warranted in patients with atypical encephalitis symptoms and demyelinating lesions in infratentorial regions.

Infratentorial Relapsing Neuroglial Tumors in Adults: Management and Unsolved Issues-A Systematic Review.

(1) Background: Gangliogliomas are rare tumors accounting for about 0.4% of all central nervous system tumors. They are usually located in the temporal lobes of children and young adults, though such tumors in the infratentorial region and adult-age patients rarely reported. (2) Methods: A systematic review on ganglioglioma with infratentorial location in the adult population was conducted in accordance with the PRISMA guidelines. A total of 275 articles were found, and 23 were included. Demographic data, the location and histology of the lesion, pre-operative neurological status, the type of surgery, recurrence, radiotherapy/chemotherapy adjuvant treatments, neurological outcomes and follow-up information were collected. We also presented an illustrative case. (3) Results: A total of 27 patients were included. In 51%, the location was the cerebellum; in 40%, it was the fourth ventricle; in 11%, it was brainstem; and in 4%, it was the cerebellopontine angle. STR was performed in 44%, GTR in 26% and biopsy in 15% of the cases. Adjuvant radiotherapy was found in 22% of cases. Disease recurrence occurred in 15% of patients between 1 and 12 months after surgery with a diagnosis of high-grade ganglioglioma, while in six cases, no disease recurrence was documented. (4) Conclusions: Infratentorial glioneuronal tumors are rare findings in the adult population. Histopathological characterization does not seem to fully reflect their true behavior. Future studies are warranted for better characterizing histopathological findings and treatment.