Extra-axial cranial nerve enhancement: a pattern-based approach.

Cranial nerve enhancement is a common and challenging MRI finding that requires a meticulous and systematic evaluation to identify the correct diagnosis. Literature mainly describes the various pathologies with the associated clinic-radiological characteristics, while the radiologist often needs a reverse approach that starts from the radiological findings to reach the diagnosis. Therefore, our aim is to provide a new and practical pattern-based approach to cranial nerve enhancement, which starts from the radiological findings and follows pattern-driven pipelines to navigate through multiple differential diagnoses, guiding the radiologist to reach the proper diagnosis. Firstly, we reviewed the literature and identified four patterns to categorize the main pathologies presenting with cranial nerve enhancement: unilateral linear pattern, bilateral linear pattern, unilateral thickened pattern, and bilateral thickened pattern. For each pattern, we describe the underlying pathogenic origin, and the main radiological features are displayed through high-quality MRI images and illustrative panels. A suggested MRI protocol for studying cranial nerve enhancement is also provided. In conclusion, our approach for cranial nerve enhancement aims to be an easy tool immediately applicable to clinical practice for converting challenging findings into specific pathological patterns.

Neurologic outcome following liver transplantation for methylmalonic aciduria.

Liver and liver/kidney transplantation are increasingly used in methylmalonic aciduria, but little is known on their impact on CNS. The effect of transplantation on neurological outcome was prospectively assessed in six patients pre- and post-transplant by clinical evaluation and by measuring disease biomarkers in plasma and CSF, in combination with psychometric tests and brain MRI studies. Primary (methylmalonic- and methylcitric acid) and secondary biomarkers (glycine and glutamine) significantly improved in plasma, while they remained unchanged in CSF. Differently, biomarkers of mitochondrial dysfunction (lactate, alanine, and related ratios) significantly decreased in CSF. Neurocognitive evaluation documented significant higher post-transplant developmental/cognitive scores and maturation of executive functions corresponding to improvement of brain atrophy, cortical thickness, and white matter maturation indexes at MRI. Three patients presented post-transplantation reversible neurological events, which were differentiated, by means of biochemical and neuroradiological evaluations, into calcineurin inhibitor-induced neurotoxicity and metabolic stroke-like episode. Our study shows that transplantation has a beneficial impact on neurological outcome in methylmalonic aciduria. Early transplantation is recommended due to the high risk of long-term complications, high disease burden, and low quality of life.

Single brain metastasis versus glioblastoma multiforme: a VOI-based multiparametric analysis for differential diagnosis.

PURPOSE: The authors' purpose was to create a valid multiparametric MRI model for the differential diagnosis between glioblastoma and solitary brain metastasis. MATERIALS AND METHODS: Forty-one patients (twenty glioblastomas and twenty-one brain metastases) were retrospectively evaluated. MRIs were analyzed with Olea Sphere® 3.0. Lesions' volumes of interest (VOIs) were drawn on enhanced 3D T1 MP-RAGE and projected on ADC and rCBV co-registered maps. Another two VOIs were drawn in the region of hyperintense cerebral edema, surrounding the lesion, respectively, within 5 mm around the enhancing tumor and into residual edema. Perfusion curves were obtained, and the value of signal recovery (SR) was reported. A two-sample T test was obtained to compare all parameters of GB and BM groups. Receiver operating characteristics (ROC) analysis was performed. RESULTS: According to ROC analysis, the area under the curve was 88%, 78% and 74%, respectively, for mean ADC VOI values of the solid component, the mean and max rCBV values in the perilesional edema and the PSR. The cumulative ROC curve of these parameters reached an area under the curve of 95%. Using perilesional max rCBV > 1.37, PSR > 75% and mean lesional ADC < 1 × 10-3 mm2 s-1 GB could be differentiated from solitary BM (sensitivity and specificity of 95% and 86%). CONCLUSION: Lower values of ADC in the enhancing tumor, a higher percentage of SR in perfusion curves and higher values of rCBV in the peritumoral edema closed to the lesion are strongly indicative of GB than solitary BM.

Early alterations of cortical thickness and gyrification in migraine without aura: a retrospective MRI study in pediatric patients.

BACKGROUND: Migraine is the most common neurological disease, with high social-economical burden. Although there is growing evidence of brain structural and functional abnormalities in patients with migraine, few studies have been conducted on children and no studies investigating cortical gyrification have been conducted on pediatric patients affected by migraine without aura. METHODS: Seventy-two pediatric patients affected by migraine without aura and eighty-two controls aged between 6 and 18 were retrospectively recruited with the following inclusion criteria: MRI exam showing no morphological or signal abnormalities, no systemic comorbidities, no abnormal neurological examination. Cortical thickness (CT) and local gyrification index (LGI) were obtained through a dedicated algorithm, consisting of a combination of voxel-based and surface-based morphometric techniques. The statistical analysis was performed separately on CT and LGI between: patients and controls; subgroups of controls and subgroups of patients. RESULTS: Patients showed a decreased LGI in the left superior parietal lobule and in the supramarginal gyrus, compared to controls. Female patients presented a decreased LGI in the right superior, middle and transverse temporal gyri, right postcentral gyrus and supramarginal gyrus compared to male patients. Compared to migraine patients younger than 12 years, the ≥ 12-year-old subjects showed a decreased CT in the superior and middle frontal gyri, pre- and post-central cortex, paracentral lobule, superior and transverse temporal gyri, supramarginal gyrus and posterior insula. Migraine patients experiencing nausea and/or vomiting during headache attacks presented an increased CT in the pars opercularis of the left inferior frontal gyrus. CONCLUSIONS: Differences in CT and LGI in patients affected by migraine without aura may suggest the presence of congenital and acquired abnormalities in migraine and that migraine might represent a vast spectrum of different entities. In particular, ≥ 12-year-old pediatric patients showed a decreased CT in areas related to the executive function and nociceptive networks compared to younger patients, while female patients compared to males showed a decreased CT of the auditory cortex compared to males. Therefore, early and tailored therapies are paramount to obtain migraine control, prevent cerebral reduction of cortical thickness and preserve executive function and nociception networks to ensure a high quality of life.

Correction to: Spinal cord involvement in Kearns-Sayre syndrome: a neuroimaging study.

The original article was published with an incorrect presentation of all author names.

Decoding the puzzle: A multidisciplinary systematic review of adult brainstem glioma.

Adult brainstem gliomas (BSGs) are a group of rare central nervous system tumors with varying prognoses and controversial standard treatment strategies. To provide an overview of current trends, a systematic review using the PRISMA guidelines, Class of evidence (CE) and strength of recommendation (SR), was conducted. The review identified 27 studies. Surgery was found to have a positive impact on survival, particularly for focal lesions with CE II SR C. Stereotactic image-guided biopsy was recommended when resective surgery was not feasible with CE II and SR B. The role of systemic treatments remains unclear. Eight studies provided molecular biology data. This review gathers crucial literature on diagnosis and management of adult BSGs. It provides evidence-based guidance with updated recommendations for diagnosing and treating, taking into account recent molecular and genetic advancements. The importance of brain biopsy is emphasized to optimize treatment using emerging genetic-molecular findings and explore potential targeted therapies.

Congenital Malformations of the Eye: A Pictorial Review and Clinico-Radiological Correlations.

Congenital malformations of the eye represent a wide and heterogeneous spectrum of abnormalities that may be part of a complex syndrome or be isolated. Ocular malformation severity depends on the timing of the causative event during eye formation, ranging from the complete absence of the eye if injury occurs during the first weeks of gestation, to subtle abnormalities if the cause occurs later on. Knowledge of ocular malformations is crucial to performing a tailored imaging protocol and correctly reporting imaging findings. Together with the ophthalmologic evaluation, imaging may help frame ocular malformations and identify underlying genetic conditions. The purpose of this pictorial review is to describe the imaging features of the main ocular malformations and the related ophthalmologic findings in order to provide a clinico-radiological overview of these abnormalities to the clinical radiologist. Sight is a crucial sense for children to explore the world and relate with their parents from birth. Vision impairment or even blindness secondary to ocular malformations deeply affects children's growth and quality of life.

The Role of Advanced MRI Sequences in the Diagnosis and Follow-Up of Adult Brainstem Gliomas: A Neuroradiological Review.

The 2021 WHO (World Health Organization) classification of brain tumors incorporated the rapid advances in the molecular, genetic, and pathogenesis understanding of brain tumor pathogenesis, behavior, and treatment response. It revolutionized brain tumor classification by placing great emphasis on molecular types and completely splitting adult-type and pediatric-type diffuse gliomas. Brainstem gliomas (BSGs) are the leading primary tumors of the brainstem, although they are quite uncommon in adults compared with the pediatric population, representing less than 2% of adult gliomas. Surgery is not always the treatment of choice since resection is rarely feasible and does not improve overall survival, and biopsies are not generally performed since the location is treacherous. Therefore, MRI (Magnetic Resonance Imaging) without and with gadolinium administration represents the optimal noninvasive radiological technique to suggest brainstem gliomas diagnosis, plan a multidisciplinary treatment and for follow-up evaluations. The MRI protocol encompasses morphological sequences as well as functional and advanced sequences, such as DWI/ADC (Diffusion-Weighted Imaging/Apparent Diffusion Coefficient), DTI (Diffusion Tensor Imaging), PWI (Perfusion-Weighted Imaging), and MRS (Magnetic Resonance Spectroscopy), which improve the accuracy of the diagnosis of BSGs by adding substantial information regarding the cellularity, the infiltrative behavior toward the v fiber tracts, the vascularity, and the molecular changes. Brainstem gliomas have been divided into four categories on the basis of their MRI radiological appearance, including diffuse intrinsic low-grade gliomas, enhancing malignant gliomas, localized tectal gliomas, and other forms. The aim of our review is to provide insight into the role of advanced MRI sequences in the diagnosis and follow-up of adult brainstem gliomas.

Dural Tail Sign and Middle Meningeal Artery Hypertrophy in Glioblastoma: A Rarity?

BACKGROUND: Dural tail sign and increased caliber of branches of the external carotid artery (ECA) are common findings in meningioma and they have been rarely reported in intra-axial lesions. Anyway, some cases of glioblastoma (GBM) are reported in the literature, mostly superficially localized, characterized by these 2 findings and therefore, misdiagnosed with meningioma. The aim of this study is to verify the prevalence of dural tail sign and hypertrophy of middle meningeal artery (MMA) in a large cohort of GBMs. METHODS: 180 GBM patients were retrospectively evaluated. Deep or superficial localization of GBM was established and the presence of dural tail sign and hypertrophy of the ipsilateral MMA were assessed. The rate of tumor necrosis and the incidence of dural metastases during the radiological follow-up were also evaluated. Inter-rater reliability was calculated using Cohen's K-test. RESULTS: Dural tail sign and enlarged MMA were evident in 30% and 19% of 96 superficial GBM, respectively. Deep GBM did not present those signs. Only one patient developed dural metastasis at follow-up and no differences in terms of tumor necrosis and hypoxic biomarkers expression were evident among GBMs with and without dural and vessel signs. CONCLUSIONS: Dural tail sign and hypertrophy of the MMA in superficial GBM are more common than expected. They probably represent reactive rather than a neoplastic infiltration. Knowing these radiological signs may be important in terms of neurosurgery planning and avoiding excessive bleeding. Anyway, this hypothesis should be confirmed by a prospective neurosurgery studio.

A New Pattern of Brain and Cord Gadolinium Enhancement in Molybdenum Cofactor Deficiency: A Case Report.

Molybdenum cofactor deficiency (MoCD) is a rare and severe autosomal recessive in-born error of metabolism caused by the mutation in MOCS1, MOCS2, MOCS3 or GEPH genes, with an incidence ranging between 1 in 100,000 and 200,000 live births. The clinical presentation with seizures, lethargy and neurologic deficits reflects the neurotoxicity mediated via sulphite accumulation, and it occurs within the first hours or days after birth, often leading to severe neurodegeneration and the patient's death within days or months. The Imaging of Choice is a brain-specific MRI technique, which is usually performed without contrast and shows typical radiological findings in the early phase, such as diffuse cerebral oedema and infarction affecting the cortex and the basal ganglia and the white matter, as well as in the late phase, such as multicystic encephalomalacia. Our case report represents a novelty in the field, since the patient underwent a contrast-enhanced MRI to exclude a concomitant infectious disease. In the frame of the clinical presentation and laboratory data, we describe the MoCD Imaging findings for MRI morphological and advanced sequences, presenting a new contrast-enhanced MRI pattern characterized by the diffuse and linear leptomeningeal enhancement of brain, cord and spinal roots. The early identification of molybdenum cofactor deficiency is crucial because it may lead to the best multidisciplinary therapy for the patient, which is focused on the prompt and optimal management of the complications.

Predictive Value of MRI in Hypoxic-Ischemic Encephalopathy Treated with Therapeutic Hypothermia.

BACKGROUND: Hypoxic-ischemic encephalopathy (HIE) is a severe pathology, and no unique predictive biomarker has been identified. Our aims are to identify associations of perinatal and outcome parameters with morphological anomalies and ADC values from MRI. The secondary aims are to define a predictive ADC threshold value and detect ADC value fluctuations between MRIs acquired within 7 days (MR0) and at 1 year (MR1) of birth in relation to perinatal and outcome parameters. METHODS: Fifty-one term children affected by moderate HIE treated with hypothermia and undergoing MRI0 and MRI1 were recruited. Brain MRIs were evaluated through the van Rooij score, while ADC maps were co-registered on a standardized cerebral surface, on which 29 ROIs were drawn. Statistical analysis was performed in Matlab, with the statistical significance value at 0.05. RESULTS: ADC0 < ADC1 in the left and right thalami, left and right frontal white matter, right visual cortex, and the left dentate nucleus of children showing abnormal perinatal and neurodevelopmental parameters. At ROC analysis, the best prognostic ADC cut-off value was 1.535 mm2/s × 10-6 (sensitivity 80%, specificity 86%) in the right frontal white matter. ADC1 > ADC0 in the right visual cortex and left dentate nucleus, positively correlated with multiple abnormal perinatal and neurodevelopmental parameters. The van Rooij score was significantly higher in children presenting with sleep disorders. CONCLUSIONS: ADC values could be used as prognostic biomarkers to predict children's neurodevelopmental outcomes. Further studies are needed to address these crucial topics and validate our results. Early and multidisciplinary perinatal evaluation and the subsequent re-assessment of children are pivotal to identify physical and neuropsychological disorders to guarantee early and tailored therapy.

Radiosurgery for Brain Metastases: Challenges in Imaging Interpretation after Treatment.

Stereotactic radiosurgery (SRS) has transformed the management of brain metastases by achieving local tumor control, reducing toxicity, and minimizing the need for whole-brain radiation therapy (WBRT). This review specifically investigates radiation-induced changes in patients treated for metastasis, highlighting the crucial role of magnetic resonance imaging (MRI) in the evaluation of treatment response, both at very early and late stages. The primary objective of the review is to evaluate the most effective imaging techniques for assessing radiation-induced changes and distinguishing them from tumor growth. The limitations of conventional imaging methods, which rely on size measurements, dimensional criteria, and contrast enhancement patterns, are critically evaluated. In addition, it has been investigated the potential of advanced imaging modalities to offer a more precise and comprehensive evaluation of treatment response. Finally, an overview of the relevant literature concerning the interpretation of brain changes in patients undergoing immunotherapies is provided.

COVID-19 Pneumonia and Lung Cancer: A Challenge for the RadiologistReview of the Main Radiological Features, Differential Diagnosis and Overlapping Pathologies.

The COVID-19 pneumonia pandemic represents the most severe health emergency of the 21st century and has been monopolizing health systems' economic and human resources world-wide. Cancer patients have been suffering from the health systems' COVID-19 priority management with evidence of late diagnosis leading to patients' poor prognosis and late medical treatment. The radiologist plays a pivotal role as CT represents a non-invasive radiological technique which may help to identify possible overlap and differential diagnosis between COVID-19 pneumonia and lung cancer, which represents the most frequent cancer histology in COVID-19 patients. Our aims are: to present the main CT features of COVID-19 pneumonia; to provide the main differential diagnosis with lung cancer, chemotherapy-, immunotherapy-, and radiotherapy-induced lung disease; and to suggest practical tips and key radiological elements to identify possible overlap between COVID-19 pneumonia and lung cancer. Despite similarities or overlapping findings, the combination of clinics and some specific radiological findings, which are also identified by comparison with previous and follow-up CT scans, may guide differential diagnosis. It is crucial to search for typical COVID-19 pneumonia phase progression and typical radiological features on HRTC. The evidence of atypical findings such as lymphadenopathies and mediastinal and vessel invasion, as well as the absence of response to therapy, should arouse the suspicion of lung cancer and require contrast administration. Ground-glass areas and/or consolidations bound to radiotherapy fields or pneumonitis arising during and after oncological therapy should always arouse the suspicion of radiation-induced lung disease and chemo/immunotherapy-induced lung disease. The radiological elements we suggest for COVID-19 and lung cancer differential diagnosis may be used to develop AI protocols to guarantee an early and proper diagnosis and treatment to improve patients' quality of life and life expectancy.

Dermoid Cysts of the Asterion: An Unusual Location for Unusual Dermoids, Radiological Findings and Neurosurgical Implications.

Asterion is an uncommon site for lesions, especially dermoid cysts. We report a case series of three asterional intracranial dermoid cysts, which, to the best of our knowledge, have never been described before. Patients presented with non-specific symptoms and underwent surgical excision of the lesions. It is crucial to correctly diagnose intracranial masses and to identify their relationships with surrounding anatomical structures, especially if the location is unusual as the asterion, to plan surgery. The challenge of this tumor location is to preserve the venous drainage system during surgical procedures, because of the contiguity between the asterion and the transverse-sigmoid junction. Rupturing or damaging of the venous drainage system have been proven to be catastrophic because they lengthen surgical time and present dire consequences for patients. In conclusion, it is crucial to familiarize with atypical dermoid presentation to ensure proper diagnoses and to perform adequate imaging for optimal surgical planning.

From Fetal to Neonatal Neuroimaging in TORCH Infections: A Pictorial Review.

Congenital infections represent a challenging and varied clinical scenario in which the brain is frequently involved. Therefore, fetal and neonatal neuro-imaging plays a pivotal role in reaching an accurate diagnosis and in predicting the clinical outcome. Congenital brain infections are characterized by various clinical manifestations, ranging from nearly asymptomatic diseases to syndromic disorders, often associated with severe neurological symptoms. Brain damage results from the complex interaction among the infectious agent, its specific cellular tropism, and the stage of development of the central nervous system at the time of the maternal infection. Therefore, neuroradiological findings vary widely and are the result of complex events. An early detection is essential to establishing a proper diagnosis and prognosis, and to guarantee an optimal and prompt therapeutic perinatal management. Recently, emerging infective agents (i.e., Zika virus and SARS-CoV2) have been related to possible pre- and perinatal brain damage, thus expanding the spectrum of congenital brain infections. The purpose of this pictorial review is to provide an overview of the current knowledge on fetal and neonatal brain neuroimaging patterns in congenital brain infections used in clinical practice.

Idiopathic Interstitial Pneumonias and COVID-19 Pneumonia: Review of the Main Radiological Features and Differential Diagnosis.

COVID-19 pneumonia represents a challenging health emergency, due to the disproportion between the high transmissibility, morbidity, and mortality of the virus and healthcare systems possibilities. Literature has mainly focused on COVID-19 pneumonia clinical-radiological diagnosis and therapy, and on the most common differential diagnoses, while few papers investigated rare COVID-19 pneumonia differential diagnoses or the overlapping of COVID-19 pneumonia on pre-existing lung pathologies. This article presents the main radiological characteristics of COVID-19 pneumonia and Idiopathic Interstitial Pneumonias (IIPs) to identify key radiological features for a differential diagnosis among IIPs, and between IIPs and COVID-19 pneumonia. COVID-19 pneumonia differential diagnosis with IIPs is challenging, since these entities may share common radiological findings as ground glass opacities, crazy paving patterns, and consolidations. Multidisciplinary discussion is crucial to reach a final and correct diagnosis. Radiologists have a pivotal role in identifying COVID-19 pneumonia patterns, reporting possible overlapping with long-lasting lung diseases, and suggesting potential differential diagnoses. An optimal evaluation of HRTC may help in containing the disease, in promoting better treatment for patients, and in providing an efficient allocation of human and economic resources.

Differential diagnoses of COVID-19 pneumonia: the current challenge for the radiologist-a pictorial essay.

BACKGROUND: COVID-19 pneumonia represents the most severe pandemic of the twenty-first century and has crucial clinical, social and economical implications. The scientific community has focused attention and resources on clinical and radiological features of COVID-19 pneumonia. Few papers analysing the vast spectrum of differential diagnoses have been published. MAIN BODY: Complexity of differential diagnosis lays in the evidence of similar radiological findings as ground-glass opacities, crazy paving pattern and consolidations in COVID-19 pneumonia and a multitude of other lung diseases. Differential diagnosis is and will be extremely important during and after the pandemic peak, when there are fewer COVID-19 pneumonia cases. The aim of our pictorial essay is to schematically present COVID-19 pneumonia most frequent differential diagnoses to help the radiologist face the current COVID-19 pneumonia challenge. CONCLUSIONS: Clinical data, laboratory tests and imaging are pillars of a trident, which allows to reach a correct diagnosis in order to grant an excellent allocation of human and economical resources. The radiologist has a pivotal role in the early diagnosis of COVID-19 pneumonia because he may raise suspicion of the pathology and help to avoid COVID-19 virus spread.