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This paper presents an automated approach to perform multiclass classification of four majorly diagnosed Central nervous system brain tumors. The Astrocytoma, Glioblastoma multiforme, Meningioma and Oligodendroglioma are the four types of central nervous system brain tumors types, whose classification is being performed with the aid of this proposed approach. In addition, this proposed approach also used to perform binary classification of Glioma brain tumors into low grade and high grade Glioma tumor. The proposed automated approach for multiclass and binary class classification is based on the threshold segmentation of fused Magnetic Resonance Imaging sequences, proposed hybrid feature extraction methods along with shape based features and ensemble learning classifier. The two hybrid feature extraction methods are proposed in this paper, one based on the Discrete wavelet transform + Gradient Grey level co-occurrences matrix and second one based on the Discrete wavelet transform + Local binary pattern + Grey level run length matrix. The extracted texture features along with the shape based features are further reduced employing Principal component analysis. The resulted selected features are finally used to train the majority voting based ensemble classifier model with the aid of Central nervous system local dataset, Brain Tumor Segmentation 2013 and 2015 global dataset. The proposed automated system delivers an accuracies of 99.12, 95.24, 97.62 and 97.62 for the correct classification of Astrocytoma, Glioblastoma multiforme, Meningioma and Oligodendroglioma over the Central nervous system local dataset. Whereas delivers an accuracy of 100 and 99.52 for the binary classification of Glioma on the Brain Tumor Segmentation 2013 and 2015 global datasets employing 10-fold cross validation. [ABSTRACT FROM AUTHOR]

Brain metastasis (BM) is one of the main complications of many cancers, and the most frequent malignancy of the central nervous system. Imaging studies of BMs are routinely used for diagnosis of disease, treatment planning and follow-up. Artificial Intelligence (AI) has great potential to provide automated tools to assist in the management of disease. However, AI methods require large datasets for training and validation, and to date there have been just one publicly available imaging dataset of 156 BMs. This paper publishes 637 high-resolution imaging studies of 75 patients harboring 260 BM lesions, and their respective clinical data. It also includes semi-automatic segmentations of 593 BMs, including pre- and post-treatment T1-weighted cases, and a set of morphological and radiomic features for the cases segmented. This data-sharing initiative is expected to enable research into and performance evaluation of automatic BM detection, lesion segmentation, disease status evaluation and treatment planning methods for BMs, as well as the development and validation of predictive and prognostic tools with clinical applicability. [ABSTRACT FROM AUTHOR]

The use of magnetic resonance imaging (MRI) in the evaluation of the central extracranial nervous system, namely the brachial and lumbosacral plexuses, is well established and has been performed for many years. Only recently after numerous advances in MRI, has image quality been sufficient to properly visualize small structures, such as nerves in the extremities. Despite the advances, peripheral MR Neurography remains a complex and difficult examination to perform, especially in the pediatric patient population, in which the risk for motion artifact and compliance is always of concern. Thus, technical aspects of the MR imaging protocol must be flexible but robust, to balance image quality with scan time, in a patient population of varying sizes. An additional important step for reliably performing a successful MR Neurography examination is the non-technical pre-imaging preparation, which includes patient/family education and open communication with referring teams. This paper will discuss in detail the individual technical and non-technical/operational aspects of peripheral MR Neurography, to help guide in building a successful program in the pediatric population. [ABSTRACT FROM AUTHOR]

Fetal ventriculomegaly is the most common central nervous system abnormality detected by prenatal imaging. It has a high association with other anomalies. Etiologies and prognoses for fetal ventriculomegaly range from normal outcomes to significant neurodevelopmental sequelae. In this paper, we review the development, terminology, pathogenesis, imaging and prognosis of fetal ventriculomegaly. [ABSTRACT FROM AUTHOR]

Objectives: To perform a systematic review regarding the developments in the field of radiomics in lymphoma. To evaluate the quality of included articles by the Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2), the phases classification criteria for image mining studies, and the radiomics quality scoring (RQS) tool. Methods: We searched for eligible articles in the MEDLINE/PubMed and EMBASE databases using the terms "radiomics", "texture" and "lymphoma". The included studies were divided into two categories: diagnosis-, therapy response- and outcome-related studies. The diagnosis-related studies were evaluated using the QUADAS-2; all studies were evaluated using the phases classification criteria for image mining studies and the RQS tool by two reviewers. Results: Forty-five studies were included; thirteen papers (28.9%) focused on the differential diagnosis of primary central nervous system lymphoma (PCNSL) and glioblastoma (GBM). Thirty-two (71.1%) studies were classified as discovery science according to the phase classification criteria for image mining studies. The mean RQS score of all studies was 14.2% (ranging from 0.0 to 40.3%), and 23 studies (51.1%) were given a score of < 10%. Conclusion: The radiomics features could serve as diagnostic and prognostic indicators in lymphoma. However, the current conclusions should be interpreted with caution due to the suboptimal quality of the studies. In order to introduce radiomics into lymphoma clinical settings, the lesion segmentation and selection, the influence of the pathological pattern and the extraction of multiple modalities and multiple time points features need to be further studied. Key Points: • The radiomics approach may provide useful information for diagnosis, prediction of the therapy response, and outcome of lymphoma. • The quality of published radiomics studies in lymphoma has been suboptimal to date. • More studies are needed to examine lesion selection and segmentation, the influence of pathological patterns, and the extraction of multiple modalities and multiple time point features. [ABSTRACT FROM AUTHOR]

Chronic pain surrounding the temporomandibular joints and masticatory muscles is often the primary chief complaint of patients with temporomandibular disorders (TMD) seeking treatment. Yet, the neuro-pathophysiological basis underlying it remains to be clarified. Neuroimaging techniques have provided a deeper understanding of what happens to brain structure and function in TMD patients with chronic pain. Therefore, we performed a systematic review of magnetic resonance imaging (MRI) studies investigating structural and functional brain alterations in TMD patients to further unravel the neurobiological underpinnings of TMD-related pain. Online databases (PubMed, EMBASE, and Web of Science) were searched up to August 3, 2019, as complemented by a hand search in reference lists. A total of 622 papers were initially identified after duplicates removed and 25 studies met inclusion criteria for this review. Notably, the variations of MRI techniques used and study design among included studies preclude a meta-analysis and we discussed the findings qualitatively according to the specific neural system or network the brain regions were involved in. Brain changes were found in pathways responsible for abnormal pain perception, including the classic trigemino-thalamo-cortical system and the lateral and medial pain systems. Dysfunction and maladaptive changes were also identified in the default mode network, the top-down antinociceptive periaqueductal gray-raphe magnus pathway, as well as the motor system. TMD patients displayed altered brain activations in response to both innocuous and painful stimuli compared with healthy controls. Additionally, evidence indicates that splint therapy can alleviate TMD-related symptoms by inducing functional brain changes. In summary, MRI research provides important novel insights into the altered neural manifestations underlying chronic pain in TMD. [ABSTRACT FROM AUTHOR]

Background and aims: Metronidazole, a commonly used antibiotic drug, can cause adverse effects in the central nervous system termed metronidazole-induced encephalopathy, leading to diagnostic challenges. The condition is rare and a detailed description of its phenotype is lacking. In this systematic review we investigated the clinical features of metronidazole-induced encephalopathy to promote recognition and elaborate the description. Methods: We performed a systematic literature search using PubMed.gov and hand searched the reference lists of included articles and other publications of interest. We included case series and single reports describing individual patients developing symptoms from the central nervous system in relation to metronidazole treatment. Data were extracted and analyzed descriptively. Results: We identified 779 publications of which 112 papers comprising 136 patients were included. Typical findings were dysarthria, gait instability, limb dyscoordination and altered mental status. Frequently, patients concomitantly presented with metronidazole-induced polyneuropathy. Liver disease was the most common pre-existing condition. MRI showed a characteristic pattern of reversible symmetrical hyperintense lesions on T2/FLAIR of the dentate nuclei in 90% of patients. Most patients improved significantly after discontinuation of metronidazole. Poor outcome was associated with severe comorbidity. Conclusion: Metronidazole-induced encephalopathy should be considered in patients presenting with neurological symptoms in relation to newly initiated or prolonged metronidazole treatment. MRI changes are highly characteristic and specific. Patients with liver disease are at increased risk. Prognosis is good if recognized early. [ABSTRACT FROM AUTHOR]

Affections of the central nervous system (CNS) rarely occur in Lyme neuroborreliosis (LNB). CNS manifestations can have residual neurological symptoms despite antibiotic treatment. We explored the spectrum of CNS affections in patients with LNB in a tertiary care center in a region endemic for Lyme borreliosis. We retrospectively included patients treated at a tertiary care center from January 2020–December 2021 fulfilling the case criteria for LNB as stated in the current German guideline on LNB. Clinical data, cerebrospinal fluid (CSF) findings and MRI imaging were collected. We included 35 patients with LNB, 24 with early manifestations and 11 with CNS-LNB. CNS-LNB patients had encephalomyelitis (n = 6) or cerebral vasculitis (n = 5). Patients with early LNB and CNS-LNB differed regarding albumin CSF/serum quotient and total protein in CSF. Duration from onset of symptoms until diagnosis was statistically significantly longer in patients with encephalomyelitis. MRI findings were heterogeneous and showed longitudinal extensive myelitis, perimedullar leptomeningeal enhancement, pontomesencephalic lesions or cerebral vasculitis. CNS-LNB can present with a variety of clinical syndromes and MRI changes. No clear pattern of MRI findings in CNS-LNB could be identified. The role of MRI consists in ruling out other causes of neurological symptoms. [ABSTRACT FROM AUTHOR]

Purpose: This paper aims to analyze the contribution of mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) in the detection of microstructural abnormalities in amyotrophic lateral sclerosis (ALS) and to evaluate the degree of agreement between structural and functional changes through concomitant diffusion tensor imaging (DTI), transcranial magnetic stimulation (TMS), and clinical assessment.Methods: Fourteen patients with ALS and 11 healthy, age- and gender-matched controls were included. All participants underwent magnetic resonance imaging including DTI. TMS was additionally performed in ALS patients. Differences in the distribution of DTI-derived measures were assessed using tract-based spatial statistical (TBSS) and volume of interest (VOI) analyses. Correlations between clinical, imaging, and neurophysiological findings were also assessed through TBSS.Results: ALS patients showed a significant increase in AD and MD involving the corticospinal tract (CST) and the pre-frontal white matter in the right posterior limb of the internal capsule (p < 0.05) when compared to the control group using TBSS, confirmed by VOI analyses. VOI analyses also showed increased AD in the corpus callosum (p < 0.05) in ALS patients. Fractional anisotropy (FA) in the right CST correlated significantly with upper motor neuron (UMN) score (r = − 0.79, p < 0.05), and right abductor digiti minimi central motor conduction time was highly correlated with RD in the left posterior internal capsule (r = − 0.81, p < 0.05). No other significant correlation was found.Conclusion: MD, AD, and RD, besides FA, are able to further detect and characterize neurodegeneration in ALS. Furthermore, TMS and DTI appear to have a role as complementary diagnostic biomarkers of UMN dysfunction. [ABSTRACT FROM AUTHOR]

Congenital central nervous system (CNS) infections are a cause of significant morbidity and mortality. The recent Zika virus outbreak raised awareness of congenital CNS infections. Imaging can be effective in diagnosing the presence and severity of infection. In this paper we review the clinical presentations and imaging characteristics of several common and less common congenital CNS infections. [ABSTRACT FROM AUTHOR]

Introduction: Apert syndrome is one of the most common craniosynostosis syndrome caused by mutations in genes encoding fibroblast growth factor receptor 2 (FGFR2). It is characterized by multisuture craniosynostosis, midfacial hypoplasia, abnormal skull base development and syndactyly of all extremities. Apert syndrome is associated with a wide array of central nervous system (CNS) anomalies, possibly the cause of the common occurrence of mental deficiency in patients with Apert syndrome. These CNS anomalies can be broadly classified into two groups; (1) those that are primary malformations and (2) those that occur secondary to osseous deformity/malformation. Conclusion: Familiarity with CNS anomalies associated with Apert syndrome is important to both clinicians and radiologist as it impacts on management and prognostication. Cognitive development of patients has been linked to associated CNS anomalies, timing of surgery and social aspects. These associated anomalies can be broadly classified into (1) those that are primary malformations and (2) those that occur secondary to osseous deformity/malformation, as illustrated in our review paper. [ABSTRACT FROM AUTHOR]

Background: This study investigates the functional brain connectivity in patients with anterior knee pain (AKP). While biomechanical models are frequently employed to investigate AKP, it is important to recognize that pain can manifest even in the absence of structural abnormalities. Leveraging the capabilities of functional magnetic resonance imaging (fMRI), this research aims to investigate the brain mechanisms present in AKP patients. Methods: Forty-five female subjects (24 AKP patients, 21 controls) underwent resting-state fMRI and T1-weighted structural MRI. Functional brain connectivity patterns were analyzed, focusing on pain network areas, and the influence of catastrophizing thoughts was evaluated. Results: Comparing patients and controls, several findings emerged. First, patients with AKP exhibited increased correlation between the right supplementary motor area and cerebellum I, as well as decreased correlation between the right insula and the left rostral prefrontal cortex and superior frontal gyrus. Second, in AKP patients with catastrophizing thoughts, there was increased correlation of the left lateral parietal cortex with two regions of the right cerebellum (II and VII) and the right pallidum, and decreased correlation between the left medial frontal gyrus and the right thalamus. Furthermore, the correlation between these regions showed promising results for discriminating AKP patients from controls, achieving a cross-validation accuracy of 80.5%. Conclusions: Resting-state fMRI revealed correlation differences in AKP patients compared to controls and based on catastrophizing thoughts levels. These findings shed light on neural correlates of chronic pain in AKP, suggesting that functional brain connectivity alterations may be linked to pain experience in this population. Relevance statement: Etiopathogenesis of pain in anterior knee pain patients might not be limited to the knee, but also to underlying alterations in the central nervous system: cortical changes might lead a perpetuation of pain. Key points: • Anterior knee pain patients exhibit distinct functional brain connectivity compared to controls, and among catastrophizing subgroups. • Resting-state fMRI reveals potential for discriminating anterior knee pain patients with 80.5% accuracy. • Functional brain connectivity differences improve understanding of pain pathogenesis and objective anterior knee pain identification. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the multiparametric diagnostic performance with non-enhancing tumor volume, apparent diffusion coefficient (ADC), and arterial spin labeling (ASL) to differentiate between atypical primary central nervous system lymphoma (PCNSL) and glioblastoma (GBM). Methods: One hundred and fifty-eight patients with pathologically confirmed typical PCNSL (n = 59), atypical PCNSL (hemorrhage, necrosis, or heterogeneous contrast enhancement, n = 29), and GBM (n = 70) were selected. Relative minimum ADC (rADCmin), mean (rADCmean), maximum (rADCmax), and rADCmax-min (rADCdif) were obtained by standardization of the contralateral white matter. Maximum cerebral blood flow (CBFmax) was obtained according to the ASL-CBF map. The regions of interests (ROIs) were manually delineated on the inner side of the tumor to further generate a 3D-ROI and obtain the non-enhancing tumor (nET) volume. The area under the curve (AUC) was used to evaluate the diagnostic performance. Results: Atypical PCNSLs showed significantly lower rADCmax, rADCmean, and rADCdif than that of GBMs. GBMs showed significantly higher CBFmax and nET volume ratios than that of atypical PCNSLs. Combined three-variable models with rADCmean, CBFmax, and nET volume ratio were superior to one- and two-variable models. The AUC of the three-variable model was 0.96, and the sensitivity and specificity were 90% and 96.55%, respectively. Conclusion: The combined evaluation of rADCmean, CBFmax, and nET volume allowed for reliable differentiation between atypical PCNSL and GBM. Key Points: • Atypical PCNSL is easily misdiagnosed as glioblastoma, which leads to unnecessary surgical resection. • The nET volume, ADC, and ASL-derived parameter (CBF) were lower for atypical PCNSL than that for glioblastoma. • The combination of multiple parameters performed well (AUC = 0.96) in the discrimination between atypical PCNSL and glioblastoma. [ABSTRACT FROM AUTHOR]

Patients with malignant central nervous system (CNS) involvement of lymphoma have a poor prognosis with intrathecal chemotherapy and radiation. In this paper, we report the results we obtained in such patients by intravenous chemotherapy with high-dose methotrexate and ifosfamide (HDMTX/IFO). The study involved a review of all patients who received HDMTX/IFO for CNS involvement of malignant lymphoma at our hospital. Therapy consisted of 4 g/m(2) of MTX (4 h infusion on day 1) and 1.5-2 g/m(2)/day of IFO (3 h infusion on days 3-5). The study included 20 patients with a median age of 65 years (range, 30-83) and CNS relapse of a malignant lymphoma. Seventeen patients had been pretreated with up to two chemotherapy regimens. The objective response rate was 90% with 12 complete or unconfirmed complete (CR and CRu) and six partial remissions. All patients had at least stabilization of their neurological symptoms. Myelosuppression was the most common toxicity. The median follow-up time was 14.9 months. The median time to neurological progression was 8.9 months. Twelve patients received subsequent therapy, including high-dose chemotherapy with autologous stem cell transplantation in five cases. The median overall survival was not reached. Systemic chemotherapy with HDMTX/IFO is a feasible and promising treatment modality for CNS relapse of a malignant lymphoma. [ABSTRACT FROM AUTHOR]

The purpose of this paper is to describe brain pathology in neonates after major traffic trauma in utero during the third trimester. Our patient cohort consisted of four neonates born by emergency cesarean section after car accident in the third trimester of pregnancy. The median gestational age ( n=4) was 36 weeks (range: 30-38). Immediate post-natal and follow-up brain imaging consisted of cranial ultrasound ( n=4), computed tomography (CT) ( n=1) and post-mortem magnetic resonance imaging (MRI) ( n=1). Pathology findings were correlated with the imaging findings ( n=3). Cranial ultrasound demonstrated a huge subarachnoidal hemorrhage ( n=1), subdural hematoma ( n=1), brain edema with inversion of the diastolic flow ( n=1) and severe ischemic changes ( n=1). In one case, CT demonstrated the presence and extension of the subarachnoidal hemorrhage, a parietal fracture and a limited intraventricular hemorrhage. Cerebellar hemorrhage and a small cerebral frontal contusion were seen on post-mortem MRI in a child with a major subarachnoidal hemorrhage on ultrasound. None of these four children survived (three children died within 2 days and one child died after 1 month). Blunt abdominal trauma during pregnancy can cause fetal cranial injury. In our cases, skull fracture, intracranial hemorrhage and hypoxic-ischemic encephalopathy were encountered. [ABSTRACT FROM AUTHOR]

Summary The aim of this paper is to demonstrate the unusual MR features of thoracic syringomyelia following TB meningitis and to discuss the neurosurgical aspect of the treatment of this rare entity. Four years after a TB meningitis episode, a 30 year-old female patient developed a progressive spastic paraparesis. MR studies revealed multiloculated syrinxes throughout the thoracic cord. She had a syringosubarachnoid shunt with a silastic “T” tube inserted. On the first postoperative day, she showed a dramatic neurological improvement, but unfortunately her paraparesis progressed to the preoperative level within a month despite diminished size of the syrinxes on the control MRI examination. Two and a half years after the operation the patient complained of having a burning type of central pain, and further deterioration in neurological function. Thoracic spinal MRI examination demonstrated enlarged syringomyelic cavities. At the second operation syringo-peritoneal shunt insertion was performed via right T10–11 hemilaminectomy using a “T” tube. At present, 4 months after the second operation, the patient's neurological examination demonstrated decreased spasticity, and improved strength in the legs compared to the preoperative level. MRI is the first choice of investigation in detecting TB related myelopathy as it provides a greater detail of pathological changes within and around the spinal cord such as syrinx formation and arachnoiditis. The MR findings are also helpful in deciding the management and predicting the outcome. Presence of multifocal loculations and arachnoid adhesions is the likely cause of treatment failures and poor prognosis. [ABSTRACT FROM AUTHOR]

Multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) are autoimmune inflammatory disorders of the central nervous system (CNS) with similar characteristics. The differential diagnosis between MS and NMOSD is critical for initiating early effective therapy. In this study, we developed a deep learning model to differentiate between multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) using brain magnetic resonance imaging (MRI) data. The model was based on a modified ResNet18 convolution neural network trained with 5-channel images created by selecting five 2D slices of 3D FLAIR images. The accuracy of the model was 76.1%, with a sensitivity of 77.3% and a specificity of 74.8%. Positive and negative predictive values were 76.9% and 78.6%, respectively, with an area under the curve of 0.85. Application of Grad-CAM to the model revealed that white matter lesions were the major classifier. This compact model may aid in the differential diagnosis of MS and NMOSD in clinical practice. [ABSTRACT FROM AUTHOR]

Objective: To explore the value of magnetic resonance imaging (MRI) in fetuses with periventricular pseudocysts (PVPC) and the neurodevelopmental outcomes of these fetuses via meta-analysis. Methods: MEDLINE and EMBASE database were searched for studies reporting on the MRI assessment of fetuses diagnosed with PVPC on neurosonography. The neurosonography was conducted according to the International Society of Ultrasound in Obstetrics and Gynecology (ISUOG) guidelines or standard axial, coronal and sagittal planes for advanced central nervous system (CNS) assessment. Single-shot fast spin-echo T2-weighted sequences of MRI technique in three orthogonal planes were necessarily performed. The pooled proportion of CNS anomalies missed on neurosonography and detected only at prenatal MRI was calculated. Subanalysis was performed according to the types of intracranial anomalies. The pregnancy outcomes (including normal, abnormal, termination of pregnancy, and perinatal death) of PVPC fetuses were also analyzed. Results: Five studies comprising 136 fetuses were included in this meta-analysis. Mean gestational age was 29.8 weeks (16–38 weeks) at ultrasonography and 31.5 weeks (25–37 weeks) at MRI. Overall, MRI detected exclusively CNS anomalies in 25.2% (95% CI 15.9–35.8%) of cases. Among them, the highest incidence was white matter abnormalities with the pooled proportion of 16.3% (95% CI 9.7–24.2%). When getting rid of white matter abnormalities, the risk of associated CNS anomalies only detected on MRI was reduced to 9.1% (95% CI 1.8–21.4%). Meanwhile, 130 cases were studied to assess the pregnancy outcomes with the scope of 1 month to 10 years. The pooled proportion of normal outcomes in isolated PVPC fetuses was as high as 95.0% (95% CI 83.9–99.8%). When analyzing the neurodevelopmental outcomes in non-isolated PVPC fetuses, the incidence of normal neurodevelopmental outcomes was about 22.1% (95% CI 5.6–45.5%) with mild and single additional abnormalities, the rate of abnormal neurodevelopmental outcomes was 19.5% (95% CI 11.0–29.7%) with apparent and/or multiple abnormalities. Besides, 53.6% (95% CI 35.4–71.3%) of non-isolated PVPC cases were terminated mainly due to infections, genetic anomalies, metabolic disorders and hemorrhage. Conclusions: MRI assessment of PVPC fetuses is recommended to detect associated intracranial anomalies that may be missed on dedicated neurosonography. White matter abnormalities on MRI account for the majority of additional anomalies, which might to be the clue of CMV infection, aminoacidopathy or white matter disease. Moreover, the neurodevelopmental outcome of isolated PVPC fetuses remains favorable, while the neurodevelopmental outcomes of non-isolated PVPC fetuses depend on the accompanying anomaly. [ABSTRACT FROM AUTHOR]

Background: Ependymoma is a rare adult tumor that originates from ependymal cells of the ventricles and the spinal cord. The diagnosis and management can be very challenging. This study aims to bring out the hypothalamus as an atypical location of ependymoma and to underline the consequences of treatment delay in anaplastic ependymomas through a concrete case. Case presentation: We present a case of 20 years with no previous medical history, suffering from progressive intracranial hypertension syndrome, tetraparesis, cerebellar ataxia, and a weight loss of 3 kg in 4 months. Cerebral and medullar Magnetic Resonance Imaging showed multiple processes including the temporal region, the cerebellum, the 4th ventricular floor, the interpeduncular cistern, the hypothalamus, and almost the entire spinal cord, in addition to a posterior intradural lesion. The hypothalamic localization was very suggestive of neurohistiocytosis. A stereotactic biopsy and immunohistochemical study confirmed the diagnosis of anaplastic ependymoma. Total resection was impossible and radiotherapy was delayed by inconclusive dosimetric scans. The patient's outcome was unfortunately quickly fatal. Conclusions: Ependymomas should be evoked whatever the location in the central nervous system. Radiotherapy must be quickly discussed and started when the gross total resection is impossible. The locations, as well as the doses of irradiation, are now well-established and any delay in initiating radiotherapy only darkens the prognosis. [ABSTRACT FROM AUTHOR]

Background and objective: Central nervous system (CNS) infiltration commonly occurs in children with acute lymphoblastic leukemia (ALL). Nevertheless, CNS infiltration is rarely detected at the initial diagnosis. The glymphatic system, which regulates cerebrospinal fluid (CSF) and interstitial fluid transport, is considered one of the possible routes of CNS infiltration by leukemia cells. In this study, we used diffusion tensor image analysis along the perivascular space (DTI-ALPS) method to investigate glymphatic system function and obtained CSF volume using synthetic magnetic resonance imaging (SyMRI) in pediatric ALL without clinically diagnosed CNS infiltration. Materials and methods: Twenty-nine ALL and 29 typically developing (TD) children were prospectively recruited (age 4–16 years) in the present study. Group differences in brain volumetric parameters, brain water diffusivities, and the ALPS index were evaluated after controlling for age, gender, and handedness. Furthermore, significant group-different parameters were correlated with clinical information using partial correlations analysis. Results: Lower Dxassoc and ALPS index, and increased CSF volume were found in pediatric ALL (all pFDR-corrected < 0.05). Moreover, the ALPS index was negatively associated with the risk classification (r = − 0.59, pFDR-corrected = 0.04) in pediatric ALL. Conclusions: Dysfunction of the glymphatic system and accumulation of CSF were presented in pediatric ALL without clinically diagnosed CNS infiltration. These novel findings suggested that the glymphatic system might be essential in the early-stage process of ALL CNS infiltration, which provides a new direction for exploring underlying mechanisms and early detection of pediatric ALL CNS infiltration. Key Points: • Lower Dxassoc and ALPS index, and increased CSF volume were found in pediatric ALL (all pFDR-corrected < 0.05). • The ALPS index was negatively associated with the risk classification (r = −0.59, pFDR-corrected = 0.04) in pediatric ALL. • Dysfunction of the glymphatic system and accumulation of CSF were presented in pediatric ALL without clinically diagnosed CNS infiltration, which suggested that the ALPS index and CSF volume might be promising imaging markers for early detection of pediatric ALL CNS infiltration. [ABSTRACT FROM AUTHOR]

Neurodegenerative diseases are caused by progressive degeneration of the central nervous system (CNS)'s neuronal structure. Well-known diseases in this category include Alzheimer's disease (AD), Parkinson's disease (PD), and multiple sclerosis (MS), which are also addressed in this study. The CNS appears to be a complex dynamic system, whose parameters change during the disease due to neuronal damage, resulting in various symptoms. Since the change in dynamic behavior is due to the neurons' death and change in neurons' connectivity, brain images of the affected areas appear to provide a good understanding of this change. This work attempts to focus on brain magnetic resonance images (MRI) and examine the effect of neuronal loss on the images. To this end, the complex features of these images, including 2D and Higuchi's fractal dimensions (HFD), correlation dimension (CD), largest Lyapunov exponent (LLE), and approximate entropy (ApEn) were calculated. Despite small differences in numerical values (0.01–0.35), these values differ significantly. This shows that the brain dynamic system behaves and functions differently in the disease state, which is clear in the behavior of global features. These three diseases have the same functional pattern, and this study seems to have captured the roots of these seemingly variant diseases. [ABSTRACT FROM AUTHOR]

Background: Long-term sequelae of COVID-19 can result in reduced functionality of the central nervous system and substandard quality of life. Gaining insight into the recovery trajectory of admitted COVID-19 patients on their cognitive performance and global structural brain connectivity may allow a better understanding of the diseases' relevance. Objectives: To assess whole-brain structural connectivity in former non-intensive-care unit (ICU)- and ICU-admitted COVID-19 survivors over 2 months following hospital discharge and correlate structural connectivity measures to cognitive performance. Methods: Participants underwent Magnetic Resonance Imaging brain scans and a cognitive test battery after hospital discharge to evaluate structural connectivity and cognitive performance. Multilevel models were constructed for each graph measure and cognitive test, assessing the groups' influence, time since discharge, and interactions. Linear regression models estimated whether the graph measurements affected cognitive measures and whether they differed between ICU and non-ICU patients. Results: Six former ICU and six non-ICU patients completed the study. Across the various graph measures, the characteristic path length decreased over time (β = 0.97, p = 0.006). We detected no group-level effects (β = 1.07, p = 0.442) nor interaction effects (β = 1.02, p = 0.220). Cognitive performance improved for both non-ICU and ICU COVID-19 survivors on four out of seven cognitive tests 2 months later (p < 0.05). Conclusion: Adverse effects of COVID-19 on brain functioning and structure abate over time. These results should be supported by future research including larger sample sizes, matched control groups of healthy non-infected individuals, and more extended follow-up periods. [ABSTRACT FROM AUTHOR]

The indications for fetal body MRI are amplifying because of the expanding possibilities of fetal and perinatal therapy. However, huge heterogeneity regarding the indications for fetal body MRI is seen among different European countries that is mostly related to local use of US, but also to local fetal MRI expertise and legislation on pregnancy termination. The purpose of this article is to summarize the precise indications for fetal MRI, excluding the central nervous system. MRI indications arise from the sonographic findings, based on the operator's experience and the various practices in the countries and institutions represented on the European Society of Paediatric Radiology Fetal Task Force. We also highlight the strengths and weaknesses of fetal US and MRI of the fetal body. [ABSTRACT FROM AUTHOR]

We report a rare case of a cerebral infection with Taenia crassiceps tapeworm larvae in an immunocompetent 71-year-old German male. Initially, an intracerebral malignoma was suspected after the patient experienced stroke-like symptoms. After surgery, helminth larvae, later identified as T. crassiceps, were detected. Identification on the species level was possible by specific PCR and sequencing. After complete surgical removal, the patient was treated with albendazole and dexamethasone for two weeks. No residual symptoms were reported up to date. [ABSTRACT FROM AUTHOR]

Fetal central nervous system MRI is a well-established method to complement a high-quality fetal ultrasound and to clarify sonographically detected abnormalities in complex pregnancies. However, there is still worldwide heterogeneity and confusion regarding the indications of fetal central nervous system MRI, which has roots in differences among countries regarding the performance of ultrasound examinations and legislation on pregnancy termination. The purpose of this article is to clarify the indications for fetal central nervous system MRI by focusing on the ultrasound findings that guide further investigation with MRI and highlight the strengths and the weaknesses of each modality on imaging the fetal central nervous system. [ABSTRACT FROM AUTHOR]

Some patients with scoliosis have a relatively short vertebral canal. This poses the question of whether a short spinal cord may sometimes cause scoliosis. The present paper presents two observations that may support this concept. It presents a scoliosis model demonstrating what effect a short, unforgiving spinal cord might have on the spinal column. The model uses two flexible parallel tubes with the facility to tighten one. It demonstrates that a short, unforgiving spinal cord could produce the abnormal rotatory anatomy observed at the apex in scoliosis, with first lordosis, then lateral deviation and finally a rotation of the vertebral column, with the rotation occurring between the canal and the vertebral body, around the axis of the cord. The anatomy of the apical vertebra is described from two museum specimens, a computed tomography (CT) myelogram and seven magnetic resonance imaging (MRI) studies. The study confirms that the vertebral canal and the intervertebral foraminae retain their original orientation. The spinal cord is eccentric in the canal towards the concavity of the curve; the major component of rotation occurs anterior to the vertebral canal and the axis of this rotation seems to be at the site of the spinal cord. These observations do not establish that a short spinal cord will result in scoliosis, but the results are compatible with this hypothesis, and that impairment of spinal cord growth factors may sometimes be responsible for scoliosis. [ABSTRACT FROM AUTHOR]

Purpose: Developmental venous anomalies (DVAs) have been found to be more prevalent in patients with multiple sclerosis (MS). The aim of the study was to compare the prevalence of DVAs in a large population of patients with MS compared with controls and to investigate the correlation of 3D Fluid Attenuated Inversion Recovery (FLAIR) hyperintense signal abnormalities adjacent to DVAs between MS patients and controls having DVAs, as well as DVA potential role in differential diagnosis.Methods: Between January 2001 and December 2019, 349 patients who met the McDonald criteria for MS diagnosis (249 females, 100 males, age range 18-70 years) were retrospectively included in the study. All patients and 340 age-matched healthy controls had brain MRIs performed on a 1.5 Tesla MR system. Two radiologists reviewed all images to identify DVAs; their presence was compared between the MS and control groups. Among the subjects having DVAs, age, gender, adjacent FLAIR anomalies, and DVA location were compared between the two groups.Results: Fifty (14.3%) out 349 patients presented 51 DVAs (35 supratentorial and 16 infratentorial), in comparison to 21/340 (6.2%) controls (P = 0.0005). One patient showed 2 simultaneous DVAs, while 3 patients had coexisting pontine capillary telangiectasias. FLAIR white matter changes adjacent to DVAs were found in 46.2% of patients and in 28.1% of controls (P = 0.0001).Conclusions: DVAs demonstrated a higher prevalence in the MS group in comparison to controls. We confirmed the association between DVAs and FLAIR anomalies in MS patients. However, currently there are no evidences that the presence of DVAs may be used in MS differential diagnosis. [ABSTRACT FROM AUTHOR]

Purpose: With therapeutic advances, central nervous system (CNS) involvement in leukemia has become more common. Leukemic optic disc infiltration, often a clinical diagnosis, can present as an isolated finding in primary or relapsed CNS disease and therefore requires early recognition. Not previously well appreciated, we report here signs of intraocular inflammation accompanying leukemic optic disc infiltration, suggesting infectious or non-infectious uveitis as an alternative diagnosis. We describe a novel optical coherence tomography (OCT) sign favoring leukemic infiltration. Methods: Retrospective consecutive case series of all leukemic patients with disc edema (5 patients, 6 eyes) presenting to the University of Michigan's Ocular Oncology Clinic between October 2019 and March 2020. Results: We report five leukemic patients (6 eyes) who were evaluated for disc edema and vitritis and eventually diagnosed with leukemic papillopathy. All five patients initially had a bland lumbar puncture (LP), and all four patients who underwent magnetic resonance imaging (MRI) had no retrobulbar nerve involvement. Clinical findings included preserved visual acuity (n = 5 eyes, 83%), anterior chamber (AC) cell (n = 3 eyes, 50%), vitreous cell (n = 6 eyes, 100%), and retinal whitening (n = 4 eyes, 66%). In five eyes (83%), a diagnosis of infectious or non-infectious uveitis was initially considered. The OCT finding of inner retinal thickening and loss of inner retinal lamination with largely preserved outer retinal architecture helped point towards a leukemic infiltrative process emanating from the disc and spreading retrograde through the nerve fiber layer. Conclusions: These cases highlight the difficulty of distinguishing intraocular inflammation associated with leukemic papillopathy from infectious or non-infectious uveitis, especially considering bland LP and negative retrobulbar MRI signal in all our patients. We propose juxtapapillary inner retinal infiltration with the loss of inner retinal lamination and relative preservation of outer retinal architecture on OCT imaging as a finding that supports the diagnosis of leukemic papillopathy. [ABSTRACT FROM AUTHOR]

Objective: White matter lesions (WMLs) are the most common central nervous system changes observed during cochlear implant evaluation. However, its clinical significance in cochlear implantation (CI) remains unclear. The purpose of this study is to explore the effects of WMLs on hearing and speech rehabilitation of prelingually deaf children after CI. Methods: The data of forty-five children with WMLs who received CI from 2011 to 2014 were retrospectively reviewed. All patients underwent magnetic resonance imaging examination preoperatively. The categories of auditory performance (CAP) and speech intelligibility rating (SIR) scales were used to evaluate changes in the auditory and speech abilities of the patients, and the Fazekas scale was adopted to assess the extent of WMLs. The degree of WMLs was divided into four grades (none, mild, moderate, severe). We assessed hearing and speech abilities at the following time points: 6, 12, 24, 36, 48 and 60-months post-operation. Results: No significant differences in CAP scores were observed between WMLs groups and the control group at 12 months post-CI (p = 0.099), but marked between-group differences were found at 6, 24, 48- and 60-months post-CI. (p < 0.05). Similarly, no significant differences in the SIR scores were observed at 6 months post-CI (p = 0.087), but marked between-group differences were found at 12, 24, 48- and 60- months post-CI. (p < 0.05). Analysis of stratified group results revealed improvements in hearing and speech development for all the subgroups, including the severe WMLs subgroup following CI. However, hearing and speech ability of the severe WMLs subgroup was much slower than that of other groups. Conclusions: The auditory and speech abilities of prelingually deaf children with WMLs and those without WMLs can improve after CI. Therefore, WMLs should not be considered a contraindication for CI. However, the decision to perform CI in such patients needs a comprehensive evaluation because the post-surgery effects on children with severe WMLs are not ideal. [ABSTRACT FROM AUTHOR]

Pompe disease (PD) is caused by lysosomal glycogen accumulation in tissues, including muscles and the central nervous system (CNS). The intravenous infusion of recombinant human acid alpha-glucosidase (rhGAA) rescues the muscle pathologies in PD but does not treat the CNS because rhGAA does not cross the blood–brain barrier (BBB). To understand the CNS pathologies in PD, control and PD mice were followed and analyzed at 9 and 18 months with brain structural and ultrastructural studies. T2-weighted brain magnetic resonance imaging studies revealed the progressive dilatation of the lateral ventricles and thinning of the corpus callosum in PD mice. Electron microscopy (EM) studies at the genu of the corpus callosum revealed glycogen accumulation, an increase in nerve fiber size variation, a decrease in the g-ratio (axon diameter/total fiber diameter), and myelin sheath decompaction. The morphology of oligodendrocytes was normal. Diffusion tensor imaging (DTI) studies at the corpus callosum revealed an increase in axial diffusivity (AD) and mean diffusivity (MD) more significantly in 9-month-old PD mice. The current study suggests that axon degeneration and axon loss occur in aged PD mice and are probably caused by glycogen accumulation in neurons. A drug crossing the BBB or a treatment for directly targeting the brain might be necessary in PD. [ABSTRACT FROM AUTHOR]

Background: Primary angiitis of the CNS (PACNS) is a process causing variously combined neurological disturbances. Its rarity and kaleidoscopic presentation make it difficult to diagnose and even to suspect. Objective: (1) To provide an up-to-date review on PACNS and (2) to create a preliminary screening algorithm based on clinical and radiological first-level data, useful to suspect PACNS and guide further investigations. Methods: Review of PUBMED case series on PACNS, published from 2002 to 2017, collection of frequencies of clinical and neuroimaging features and calculation of median values. Classification of features as "major" or "minor" if frequency was higher or lower than median value. Combination of features in sets of criteria represented by all possible combinations of major and minor clinical and neuroradiological features. Application of criteria to published PACNS case reports and selection of the ones best identifying patients with definite PACNS. Results: We reviewed 24 case series. "Major" clinical features were headache, stroke, cognitive impairment, focal neurological deficits; "minor" were seizures, altered consciousness, psychiatric disorders. "Major" neuroradiological features were multiple parenchymal lesions, parenchymal/meningeal contrast enhancement, magnetic resonance angiography vessel abnormalities, vessel wall enhancement; "minor" were parenchymal/subarachnoid hemorrhage, single parenchymal lesion. The selected sets of criteria able to identify all PACNS patients were (1) one clinical (major/minor) + one major neuroradiological feature; and (2) Two clinical (≥ 1 major) + one minor neuroradiological feature. Conclusion: Our review provides a detailed clinical/neuroradiological picture of PACNS. The proposed algorithm should be regarded as a preliminary screening tool to move the first steps towards PACNS diagnosis that needs validation. [ABSTRACT FROM AUTHOR]

Introduction: Tuberous sclerosis complex (TSC) is a rare autosomal dominant disorder affecting multiple systems, due to inactivating mutations of TSC1 or TSC2 mTOR pathway genes. Neurological manifestations are observed in about 95% cases, representing the most frequent cause of morbidity and one of the most common causes of mortality. Background: Neuroimaging is crucial for early diagnosis, monitoring, and management of these patients. While computed tomography is generally used as first-line investigation at emergency department, magnetic resonance imaging is the reference method to define central nervous system involvement and investigate subtle pathophysiological alterations in TSC patients. Purpose: Here, we review the state-of-the-art knowledge in TSC brain imaging, describing conventional findings and depicting the role of advanced techniques in providing new insights on the disease, also offering an overview on future perspectives of neuroimaging applications for a better understanding of disease pathophysiology. [ABSTRACT FROM AUTHOR]

Dear Sirs, We read with great interest the article by Takai et al. [[1]] reporting seven cases of superficial siderosis (SS) associated with spontaneous cerebrospinal fluid (CSF) leak due to a ventral dural defect. Ventral epidural CSF collections (C, arrow in D, E) presuming CSF leakage are depicted, including on the CT-myelography (F), but distinct ventral dural defect with contrast leakage is only seen on dynamic myelography (arrow in G). [Extracted from the article]

Objectives: To determine whether water kurtosis and diffusional metrics derived from diffusional kurtosis imaging (DKI) within primary central nervous system lymphomas (PCNSLs) and high-grade gliomas (HGGs) correlate with cellularity and/or nuclear-to-cytoplasmic (N/C) ratio.Methods: Forty-four and 43 pathologically confirmed high-grade glioma and primary central nervous system lymphoma specimens between May 2013 and November 2016 were retrospectively reviewed. Diffusional metrics, kurtosis metrics, cellularity, and N/C ratios in PCNSLs and HGGs were compared using the Mann-Whitney U test (significant level, p < 0.007 [0.05/7]); Bonferroni correction).Results: Mean kurtosis (MK), axial kurtosis (K//), and radial kurtosis (K⊥) in PCNSLs were 0.857 (0.693-0.924), 0.837 (0.660-0.941), and 0.834 (0.685-0.937), respectively; and 0.629 (0.524-0.716), 0.575 (0.511-0.689), and 0.675 (0.532-0.766), respectively, in HGGs (all p < 0.001). No significant differences in fractional anisotropy (FA), mean diffusion (MD), axial diffusion (λ//), and radial diffusion (λ⊥) were found between HGGs and PCNSLs. Cellularity was higher in PCNSLs than in HGGs (p = 0.125); whereas, the N/C ratio in PCNSLs was significantly higher than that in HGGs (p < 0.001). All DKI metrics (FA, MD, λ//, λ⊥, MK, K//, and K⊥) were significantly correlated with N/C ratio in PCNSLs with correlation coefficients being rho = 0.418, - 0.722, - 0.525, - 0.768, 0.704, 0.579, and 0.686, respectively. Cellularity in PCNSLs and HGGs did not correlate with any kurtosis or diffusional metrics.Conclusions: Difference of kurtosis parameters between PCNSLs and HGGs is correlated with their diverse N/C ratio.Key Points: • DKI has considerable value in differentiating between PCNSLs and HGGs. • DKI can provide important information on nuclear-to-cytoplasmic ratio. • Difference of kurtosis parameters between PCNSLs and HGGs correlated well with their diverse N/C ratios. [ABSTRACT FROM AUTHOR]

Background/aims: Nausea is a major complaint of gastroparesis (GP), and the pathophysiology of this condition is poorly understood. Therefore, this study utilized fMRI to investigate the possible central nervous system (CNS) mechanisms of nausea in 10 GP patients versus 8 healthy controls (HCs).Methods: Nausea severity was assessed on a 0-10 scale and presented as mean ± SD. Nausea was increased from baseline utilizing up to 30 min of visual stimulation (VS). Functional network connectivity was measured with fMRI at baseline and after 30 min of VS. fMRI data were preprocessed using statistical parametric mapping software. Thirty-four independent components were identified as meaningful resting-state networks (RSNs) by group independent component analysis. The Functional Network Connectivity (FNC) among 5 RSNs considered important in CNS nausea mechanisms was calculated as the Pearson's pairwise correlation.Results: Baseline nausea score in GP patients was 2.7 ± 2.0 and increased to 7.0 ± 1.5 after stimulation (P < 0.01). In HCs nausea scores did not increase from baseline after stimulus (0.3 ± 0.5). When comparing GP patients to HCs after VS, a significant reduction (P < 0.001) in bilateral insula network connectivity compared to the right insula network was detected. No significant differences in connectivity were noted among the other RSNs. Additionally, the average gray matter volume was non-significantly reduced in the insula in GP patients compared to HC.Conclusions: The insula connectivity network is impaired in nauseated GP patients. This phenomenon could explain the susceptibility of GP patients to nausea or may have resulted from a state of chronic nausea. [ABSTRACT FROM AUTHOR]

Purpose: We evaluated myelin changes throughout the central nervous system in Multiple Sclerosis (MS) patients by using hybrid [18F]florbetapir PET-MR imaging. Methods: We included 18 relapsing-remitting MS patients and 12 healthy controls. Each subject performed a hybrid [18F]florbetapir PET-MR and both a clinical and cognitive assessment. [18F]florbetapir binding was measured as distribution volume ratio (DVR), through the Logan graphical reference method and the supervised cluster analysis to extract a reference region, and standard uptake value (SUV) in the 70–90 min interval after injection. The two quantification approaches were compared. We also evaluated changes in the measures derived from diffusion tensor imaging and arterial spin labeling. Results: [18F]florbetapir DVRs decreased from normal-appearing white matter to the centre of T2 lesion (P < 0.001), correlated with fractional anisotropy and with mean, axial and radial diffusivity within T2 lesions (coeff. = −0.15, P < 0.001, coeff. = −0.12, P < 0.001 and coeff. = −0.16, P < 0.001, respectively). Cerebral blood flow was reduced in white matter damaged areas compared to white matter in healthy controls (−10.9%, P = 0.005). SUV70–90 and DVR are equally able to discriminate between intact and damaged myelin (area under the curve 0.76 and 0.66, respectively; P = 0.26). Conclusion: Our findings demonstrate that [18F]florbetapir PET imaging can measure in-vivo myelin damage in patients with MS. Demyelination in MS is not restricted to lesions detected through conventional MRI but also involves the normal appearing white matter. Although longitudinal studies are needed, [18F]florbetapir PET imaging may have a role in clinical settings in the management of MS patients. [ABSTRACT FROM AUTHOR]

Objectives: To test if adding permeability measurement to perfusion obtained from dynamic susceptibility contrast MRI (DSC-MRI) improves diagnostic performance in the differentiation of primary central nervous system lymphoma (PCNSL) from glioblastoma.Materials and Methods: DSC-MRI was acquired in 145 patients with pathologically proven glioblastoma (n = 89) or PCNSL (n = 56). The permeability metrics of contrast agent extraction fraction (Ex), apparent permeability (Ka), and leakage-corrected perfusion of normalized cerebral blood volume (nCBVres) and cerebral blood flow (nCBFres) were derived from a tissue residue function. For comparison purposes, the leakage-corrected normalized CBV (nCBV) and relative permeability constant (K2) were also obtained using the established Weisskoff-Boxerman leakage correction method. The area under the receiver operating characteristics curve (AUC) and cross-validation were used to compare the diagnostic performance of the single DSC-MRI parameters with the performance obtained with the addition of permeability metrics.Results: PCNSL demonstrated significantly higher permeability (Ex, p < .001) and lower perfusion (nCBVres, nCBFres, and nCBV, all p < .001) than glioblastoma. The combination of Ex and nCBVres showed the highest performance (AUC, 0.96; 95% confidence interval, 0.92-0.99) for differentiating PCNSL from glioblastoma, which was a significant improvement over the single perfusion (nCBV: AUC, 0.84; nCBVres: AUC, 0.84; nCBFres: AUC, 0.82; all p < .001) or Ex (AUC, 0.80; p < .001) parameters.Conclusions: Analysis of the combined permeability and perfusion metrics obtained from a single DSC-MRI acquisition improves the diagnostic value for differentiating PCNSL from glioblastoma in comparison with single-parameter nCBV analysis.Key Points: • Permeability measurement can be calculated from DSC-MRI with a tissue residue function-based leakage correction. • Adding Exto CBV aids in the differentiation of PCNSL from glioblastoma. • CBV and Exmeasurements from DSC-MRI were highly reproducible. [ABSTRACT FROM AUTHOR]

Background and purpose: Although advances in imaging have allowed earlier and more accurate diagnosis of various fetal anomalies, Dandy–Walker malformation (DWM) remains one of the more challenging central nervous system anomalies to diagnose accurately before birth. Basilar invagination (BI), which is a dislocation of the dens in an upward direction, is occasionally accompanied by Klippel–Feil syndrome (KFS). We report a pediatric case of BI caused by atlanto-occipital subluxation (AOS) in KFS, suspected of having DWM prenatally but head magnetic resonance images (MRI) showed no evidence of that at 7 months of age. Case: At 28 weeks of gestation, fetal MRI study revealed a small cerebellar vermis, leading us to suspect a DWM. The patient was born at 40 weeks of gestation. Head CT showed inferior vermian hypoplasia without findings of hydrocephalus. Cervicothoracic CT showed cervical lamina assimilations, thoracic hemivertebrae, and cervicothoracic scoliosis. He was diagnosed with Dandy–Walker variant and KFS. At 7 months of age, head MRI showed near normal cerebellum and vermis and there was no evidence of the DWM. He did not have intellectual or developmental delay and imaging studies were performed periodically. At 9 years of age, an already existing cough headache deteriorated. Three-dimensional reconstructed images from CT scan showed C1 hypoplasia, fusion of C1 and C2, BI, and AOS. Sagittal T2-weighted MRI showed protrusion of cerebellar tonsils inferiorly to the level of the posterior arch of C2. Serum calcium, phosphate, and parathyroid hormone levels were normal. The diagnosis was tonsillar herniation related to BI following AOS in KFS. Posterior occipitocervical fixation was performed under traction. Conclusions: We found out two important clinical issues: DWM findings after birth can be disappearing and BI can present sequential deterioration because of AOS in KFS. Our observation indicated the possible prognosis of pediatric BI with long follow-up and can help us decide on its surgical treatment timing when associated with AOS. [ABSTRACT FROM AUTHOR]

Objectives: Bing-Neel syndrome (BNS) is a rare neurological complication of Waldenström's macroglobulinemia. The aim of this study is to describe the spectrum of radiological manifestations of this syndrome and their prevalence in order to facilitate its early diagnosis.Methods: Twenty-four patients with BNS were diagnosed between 1994 and 2016 in eight centres in France. We retrospectively examined the medical records of these patients as well as the corresponding literature, focusing on imaging studies. Recorded data were statistically analysed and radiological findings described.Results: The mean age of our patients was 62.4 years (35-80 years). The vast majority of patients were men, with a male to female ratio of 9:1. Findings included parenchymal or meningeal involvement or both. The most common finding was leptomeningeal infiltration, either intracranial or spinal, with a prevalence reaching 70.8%. Dural involvement was present in 37.5% of patients. In 41.7% (10/24) of patients, there was parenchymal involvement with a higher prevalence of brain comparing to medullar involvement (33.3% and 23.1% respectively). High T2 signal of the parenchyma was identified in 41.7% of patients and high signal in diffusion was evident in 25% of them. Intraorbital or periorbital involvement was also detected in four cases. A proposition regarding the appropriate imaging protocol completed our study.Conclusion: BNS's diagnosis remains challenging. Central nervous system MRI findings in the setting of known or suspected Waldenström's macroglobulinemia appear to be highly suggestive of BNS and appropriate imaging protocols should be implemented for their depiction.Key Points: • Diagnosis of Bing-Neel syndrome (BNS) remains challenging and recent expert recommendations include MRI in the diagnostic criteria for the syndrome. • The most common radiological manifestations of BNS are leptomeningeal/dural infiltration or parenchymal involvement of brain or spinal cord, but many atypical forms may exist with various presentations. • Appropriate imaging protocol for BNS should include enhanced MRI studies of both brain and spine. [ABSTRACT FROM AUTHOR]

Traumatic brain injury (TBI) as a major public health problem may lead to olfactory dysfunction. However, little is known about brain responses to odors in TBI olfactory loss patients. Nineteen healthy controls and forty TBI olfactory dysfunctional patients (19 with hyposmia and 21 with anosmia) underwent a functional magnetic resonance imaging scan when two odors (peach and coffee) were presented intranasally using a computerized olfactometer. Olfactory performance was measured using the “Sniffin’ Sticks” test. TBI patients with hyposmia or anosmia showed decreased odor-induced brain activations in the primary olfactory area and insular cortex as compared to healthy controls (FWE-corrected peak p < 0.05). In addition, negative correlations were found between the time since injury (in month) and odor-induced brain responses in the right primary olfactory area for patients with hyposmia. Similar correlations were seen in the insula and orbitofrontal cortex for patients with anosmia. In conclusion, results from the current study are evidences for the impairment of central nervous processing of odor perception at all levels of the olfactory system among TBI patients with olfactory loss. In addition, the duration after the initial injury may have an impact on the severity of olfactory dysfunction. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this prospective study is to investigate the central nervous system involvement in Crimean-Congo haemorrhagic fever (CCHF) with magnetic resonance imaging (MRI) in conjunction with clinical and laboratory findings.Methods: Between July 2015 and August 2016, 36 patients with CCHF were undergone brain MRI including SWI. Two MRIs, one at the time of admission and the second in the convalescent period, were performed for each patient in order to see if there is any sign of central nervous system (CNS) involvement, especially in terms of intracranial haemorrhage or viral encephalitis. Clinical severity scoring was also done and laboratory findings were noted in order to correlate with clinical and imaging findings.Results: None of the 36 patients showed any MRI findings of an acute intracranial event during the course of the disease. There was a significant difference between mild cases and moderate cases in terms of some laboratory parameters (p < 0.05).Conclusions: Although CCHF is a highly lethal disease which involves multiple organs and systems, CNS involvement seems to be extremely rare in mild and moderate cases.Key Points: • MRI is the imaging method of choice to diagnose microbleeds and encephalitis • Although CCHF causes multisystem bleeding, intracranial haemorrhage seems to be very rare • CNS complications are uncommon, even in the setting of suggestive symptoms • Death usually results from extracranial bleeding and multiorgan failure • Severity scoring is associated with some laboratory abnormalities in CCHF. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the ability of the initial area under the curve (IAUC) derived from dynamic contrast-enhanced MR imaging (DCE-MRI) and apparent diffusion coefficient (ADC) in differentiating between primary central nervous system lymphoma (PCNSL) and atypical glioblastoma (GBM).Methods: We retrospectively identified 19 patients with atypical GBM (less than 13 % necrosis of the enhancing tumour), and 23 patients with PCNSL. The histogram parameters of IAUC at 30, 60, 90 s (IAUC30, IAUC60, and IAUC90), and ADC were compared between PCNSL and GBM. The diagnostic performances and added values of the IAUC and ADC for differentiating between PCNSL and GBM were evaluated. Interobserver agreement was assessed via intraclass correlation coefficient (ICC).Results: The IAUC and ADC parameters were higher in GBM than in PCNSL. The 90th percentile (p90) of IAUC30 and 10th percentile (p10) of ADC showed the best diagnostic performance. Adding p90 of IAUC30 to p10 of ADC improved the differentiation between PCNSL and GBM (area under the ROC curve [AUC] = 0.886), compared to IAUC30 or ADC alone (AUC = 0.789 and 0.744; P < 0.05 for all). The ICC was 0.96 for p90 of IAUC30.Conclusions: The IAUC may be a useful parameter together with ADC for differentiating between PCNSL and atypical GBM.Key Points: • High reproducibility is essential for practical implementation of advanced MRI parameters. • IAUC and ADC are highly reproducible parameters. • IAUC values were higher in atypical GBM than in PCNSL. • Adding IAUC to ADC improved the differentiation between PCNSL and GBM. • IAUC with ADC are useful for differentiating PCNSL from GBM. [ABSTRACT FROM AUTHOR]

Unlabelled: Imaging plays an increasingly important role in the early diagnosis, prognosis prediction and therapy response evaluation of central nervous system (CNS) diseases. The newly emerging hybrid positron emission tomography/magnetic resonance imaging (PET/MRI) can perform "one-stop-shop" evaluation, including anatomic, functional, biochemical and metabolic information, even at the molecular level, for personalised diagnoses and treatments of CNS diseases. However, there are still several problems to be resolved, such as appropriate PET detectors, attenuation correction and so on. This review will introduce the basic physical principles of PET/MRI and its potential clinical applications in the CNS. We also provide the future perspectives for this field.Key Points: • PET/MRI can simultaneously provide anatomic, functional, biochemical and metabolic information. • PET/MRI has promising potential in various central nervous system diseases. • Research on the future implementation of PET/MRI is challenging and encouraging. [ABSTRACT FROM AUTHOR]

Purpose: Narrow cervical canal (NCC) has been a suspected risk factor for later development of cervical myelopathy. However, few studies have evaluated the prevalence in asymptomatic subjects. The purpose of this study was to investigate the prevalence of NCC in a large cohort of asymptomatic volunteers.Methods: This study was a cross-sectional study of 1211 asymptomatic volunteers. Approximately 100 men and 100 women representing each decade of life from the 20s to the 70s were included in this study. Cervical canal anteroposterior diameters at C5 midvertebral level on X-rays, and the prevalence of spinal cord compression (SCC) and increased signal intensity (ISI) changes on MRI were evaluated. Receiver operating characteristic analysis was performed to determine the cut-off value of the severity of canal stenosis resulting in SCC.Results: NCC (<14 mm) was observed in 123 (10.2 %) subjects. SCC and ISI were found in 64 (5.3 %) and 28 (2.3 %) subjects, respectively. The prevalence of NCC was significantly higher in females and older subjects, but the occurrence of severe NCC (<12 mm) did not increase with age. The canal size in subjects with SCC or ISI was significantly smaller than in those without SCC (p < 0.0001). The cut-off values of cervical canal stenosis resulting in SCC were 14.8 and 13.9 mm in males and females, respectively.Conclusions: The prevalence of NCC was considerably lower among asymptomatic healthy volunteers; the cervical canal diameter in subjects with SCC or ISI was significantly smaller than in asymptomatic subjects; NCC is a risk factor for SCC. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the impact of symmetric and asymmetric isolated mild ventriculomegaly (IMVM, atrial width 10-15 mm) on apparent diffusion coefficient (ADC) values in fetal brain areas.Methods: Sixty-seven sequential fetal head magnetic resonance imaging scans (feMRI) of VM cases performed between 2009 and 2014 were compared to 38 normal feMRI scans matched for gestational age (controls). Ultrasound- and MRI-proven IMVM cases were divided into asymmetrical (AVM, ≥2 mm difference in atrial width), symmetrical (SVM, <2 mm difference in atrial width), and asymmetrical IMVM with one normal-sized ventricle (AV1norm).Results: ADC values were significantly elevated in the basal ganglia (BG) of the SVM and AV1norm groups compared to controls (p < 0.004 and p < 0.013, respectively). High diffusivity was constantly detected in the BG ipsilateral to the enlarged atria relative to the normal-sized atria in the AV1norm group (p < 0.03). Frontal lobe ADC values were significantly reduced in the AVM and SVM groups (p < 0.003 and p < 0.003 vs. controls). Temporal lobe ADC values were significantly reduced in the AVM group (p < 0.001 vs. controls).Conclusion: Isolated mild ventriculomegaly is associated with distinct ADC value changes in different brain regions. This phenomenon could reflect the pathophysiology associated with different IMVM patterns.Key Points: Various ventriculomegaly patterns are associated with distinct diffusional changes. Frontal and temporal lobe ADC values are altered bilaterally, even in asymmetric ventriculomegaly. Basal ganglia ADC values are elevated ipsilateral to the enlarged ventricle. [ABSTRACT FROM AUTHOR]

Purpose: This study was conducted to characterize topographic variations of the optic chiasm (OC) and the pituitary stalk (PS) in situ based on MR images. Methods: Normal T2-weighted midsagittal MR images were obtained in 157 children and 323 adults. The height of OC (PC) and the inclination angle of PS (PS-AP angle) were measured in each case. All chiasms were classified into prefixed, normal and postfixed types, and low, medium and high types, respectively. All stalks were performed classification (prefixed, centered and postfixed PS) and grading (Grade 1-3 according to the relationship between PS and the dorsum sellae), respectively. Results: PC averaged 4.33 ± 1.59 mm. Adults had a greater PC ( P = 0.023) than children. There were 80 (16.7 %) prefixed, 354 (73.8 %) normal and 46 (9.6 %) postfixed chiasms, and 112 (23.3 %), 295 (61.5 %) and 73 (15.2 %) cases with low, medium and high OC, respectively. High OC was more frequent in adults than in children ( P = 0.001). The mean PS-AP angle of the whole group was 59.47 ± 8.62º. Children had a greater PS-AP angle than adults ( P = 0.012). Adults had a greater percentage of postfixed ( P = 0.000) and grade 3 ( P = 0.000) PS than children, whereas centered ( P = 0.002) and grade 1 ( P = 0.001) PS were more prevalent in children than in adults. Conclusions: The classifications of OC and PS based on MR images reflect the topographic variations of their real position in vivo. It would be helpful to identify and characterize the anomalies involving OC and PS and understand the anatomical and physiological influence in pathological situations. [ABSTRACT FROM AUTHOR]

Background: Cavernous malformations (CMs) of the central nervous system (CNS) are angiographically occult vascular lesions that affect approximately 0.5 % of the general population, and one quarter of all CMs occurs in children. Methods: We retrospectively analyzed demographic, clinical, radiological, management, and follow-up data of 36 pediatric patients with CMs from a single institution. Results: The mean age of the children at first presentation and at operation was 8.7 and 9.6 years, respectively. However, a bimodal age distribution was found with peak under 4 years and above 12 years. Seizure was the most common single presenting symptom (38.9 %), and 61.1 % of patients had at least one seizure before the admission. Focal neurological deficits (410.7 %), intracranial hypertension (27.8 %), and headache (2.8 %) were the other manifestations. Acute/subacute hemorrhage was evident at presentation in 63.9 %. The patients under 6 years of age were found to have significantly more giant cavernomas (69 vs 20 %; p = 0.011), and more overt hemorrhages (81 vs 47 %; p = 0.065) at diagnosis than those patients above 12 years. Surgery was performed in 31 patients (32 CMs), with 26 total and 6 incomplete resections. Mean follow-up duration was 6.9 ± 4.1 years. Of all patients, 63.8 % had excellent and 30.5 % had good clinical outcomes, and also 90.9 % of the epileptic patients were seizure-free (Engel Class I) at the last follow-up. Conclusions: Younger children tend to harbor larger CMs and present with hemorrhage more frequently than older ones. Microsurgical resection should be the treatment of choice in symptomatic and accessible CMs. [ABSTRACT FROM AUTHOR]

Background: Melanotic ependymoma (ME) is an extremely rare subset of ependymoma characterized by cytoplasmic and extracellular deposition of melanin, with no case series having been reported to date. The aim of this study was to analyze the clinical manifestations, pathological and radiological features, treatment, and follow-up data, and to discuss the diagnosis, management and prognosis of ME. Methods: We retrospectively reviewed the clinical and imaging data of six patients with primary ME. The diagnosis was based on radiological and pathological criteria. All the patients were treated surgically with gross total resection. The preoperative and postoperative neurological states of the patients were analyzed. All the follow-up data were obtained from office visits, and the long-term outcomes are presented and discussed. Results: Patients presented with nonspecific symptoms. On magnetic resonance imaging (MRI), although a characteristic signal variation could be found reflecting the melanin component, individual appearances varied widely and were related to the content and distribution of melanin in the ME lesions. The characteristic ependymoma-related pathological appearance and specific immunostaining for glial fibrillary acidic protein and antimelanoma monoclonal antibody were revealed. During a mean follow-up period of 46.8 months, no local recurrences or distant metastases were observed. Conclusions: The accurate diagnosis of ME depends on pathology. The histological grading used for ependymoma can be applied. Most MEs have a relatively benign course. Complete surgical resection alone is the treatment of choice for low-grade MEs, and the outcome may be favorable. Postoperative adjuvant radiotherapy can be considered for lesions with histologically anaplastic features. [ABSTRACT FROM AUTHOR]

Diabetic 'peripheral' neuropathy (DPN) is one of the common sequelae to the development of both type-1 and type-2 diabetes mellitus. Neuropathy has a major negative impact on quality of life. Abnormalities in both peripheral vasculature and nerve function are well documented and, in addition, evidence is emerging regarding changes within the central nervous system (CNS) that are concomitant with the presence of DPN. The often-resistant nature of DPN to medical treatment highlights the need to understand the role of the CNS in neuropathic symptomatology and progression, as this may modulate therapeutic approaches. Advanced neuroimaging techniques, especially those that can provide quantitative measures of structure and function, can provide objective markers of CNS status. With that comes great potential for not only furthering our understanding of involvement of the CNS in neuropathic etiology but also most importantly aiding the development of new and more effective, targeted, analgesic interventions. [ABSTRACT FROM AUTHOR]

The connection between cholinergic transmission and cognitive performance has been established in behavioural studies. The specific contribution of the muscarinic receptor system on cognitive performance and brain activation, however, has not been evaluated satisfyingly. To investigate the specific contribution of the muscarinic transmission on neural correlates of working memory, we examined the effects of scopolamine, an antagonist of the muscarinic receptors, using functional magnetic resonance imaging (fMRI). Fifteen healthy male, non-smoking subjects performed a fMRI scanning session following the application of scopolamine (0.4 mg, i.v.) or saline in a placebo-controlled, repeated measure, pseudo-randomized, single-blind design. Working memory was probed using an n-back task. Compared to placebo, challenging the cholinergic transmission with scopolamine resulted in hypoactivations in parietal, occipital and cerebellar areas and hyperactivations in frontal and prefrontal areas. These alterations are interpreted as compensatory strategies used to account for downregulation due to muscarinic acetylcholine blockade in parietal and cerebral storage systems by increased activation in frontal and prefrontal areas related to working memory rehearsal. Our results further underline the importance of cholinergic transmission to working memory performance and determine the specific contribution of muscarinic transmission on cerebral activation associated with executive functioning. [ABSTRACT FROM AUTHOR]