Diagnostic and therapeutic dilemmas of asymptomatic intracranial AVMs: A case report and evidence-based review.

First described by Virchow in the 19th century, intracranial arteriovenous malformations (AVMs) are complex, tangle-shaped vascular lesions with a number of associated neuroparenchymal, hemodynamic, and angio-architectural changes. However, the clinical description of extracranial AVMs dates back to the Ebers Papyrus (c. 1500 BC), with a still unknown definitive underlying etiology thus far. AVMs are rare lesions, with approximately 0.15% incidence and 0.001-0.5% prevalence, but of high importance as they tend to affect young patients who are frequently otherwise healthy. In the majority of cases, AVMs present as sudden intracranial hemorrhages that require immediate intervention, but incidentally-detected unruptured AVMs are only found in ∼15% of cases, leaving a confusing dilemma regarding the appropriate next step, particularly given the several therapeutic interventions available and clinical trials that were vulnerable to follow-up criticism. Herein, we present a case of an incidentally detected asymptomatic AVM in a 15-year-old boy via advanced imaging techniques that was initially misinterpreted as a post-traumatic subarachnoid hemorrhage on routine imaging studies. In providing a comprehensive overview of pathological classification schemes and the currently available diagnostic options for these silent dilemmatic AVMs, we highlight three management techniques: microsurgical resection, endovascular embolization, and stereotactic surgery, with the best option depends mostly on addressing lesion resection properly with minimal associated mortality and morbidity.

Usefulness of the Leipzig Score in the Diagnosis of Wilson's Disease - A Diagnostically Challenging Case Report.

Wilson's disease (WD) is a genetic disorder of copper metabolism that is inherited as an autosomal recessive (AR) due to mutations in the ATP7B gene, which is involved in intracellular copper transport. Approximately 40% to 50% of the patients present with neurological symptoms as their first symptom. The most common neurological symptoms are dysarthria, gait abnormalities, ataxia, dystonia, tremor, parkinsonism, and drooling. This case report aims to present a diagnostically challenging case of WD presenting with neurological symptoms. The 38-year-old male patient was admitted with complaints of imbalance, gait disturbance, weakness in the legs, speech impairment, tremors in the hands, syncope, and drooling. The MRI primarily revealed FLAIR, T1, and T hyperintensities in the bilateral globus pallidus of the basal ganglias. At first, the patient was evaluated according to the Leipzig scoring and received one point from the serum ceruloplasmin level and two points from the neurological symptoms and was evaluated as "possible WD" with a total of three points. 24-hour urine copper was collected during and after the D-Penicillamine challenge. After the test, there was an increase of more than 5 times the upper limit. The Leipzig score was recalculated, and a diagnosis of WD was made with a score of five. Even cases without important diagnostic findings such as Kayser-Fleischer ring or high 24-hour urine copper should be evaluated according to the Leipzig score. It is vital to distinguish WD in patients with young-onset movement disorder and neurological symptoms.

Review of neuroimaging findings of intracranial angioinvasive fungal infections.

With increasing use of broad-spectrum antibiotics, advances in organ and stem-cell transplant therapy, and the continued diabetes mellitus II epidemic, as well as other risk factors, reports of fungal infections of the CNS have been increasing. The most lethal subset is the angioinvasive fungal infection. Aspergillus fumigatus, Mucor, and Fusarium tend to affect immunocompromised individuals depending on their risk factors. Exserohilum rostratum and Cladophialaphora species tend to infect immunocompetent individuals. Early diagnosis and treatment are imperative for improved outcomes and reduced morbidity and mortality. Clinical presentation is often nonspecific, while neuroimaging can be helpful for accurate diagnosis. CT of the head and/or the maxillofacial structures is the primary imaging modality. Once the infection begins to proliferate, areas of vasogenic and cytotoxic edema, with regional mass effect and shift of the midline structures may be seen. These findings, however, are often nonspecific and may also be seen in underlying neoplasm, inflammatory processes, and other intracranial infections. Characteristic findings on T1, T2, diffusion-weighted imaging (DWI), and gradient echo sequences (GRE) may help to further narrow the differential diagnoses. We present a review of neuroimaging findings that will aid the neuroradiologist in distinguishing intracranial angioinvasive fungal infections and lead to improved patient outcomes.

Comparative Evaluation of AI Models Such as ChatGPT 3.5, ChatGPT 4.0, and Google Gemini in Neuroradiology Diagnostics.

AIMS AND OBJECTIVE: Advances in artificial intelligence (AI), particularly in large language models (LLMs) like ChatGPT (versions 3.5 and 4.0) and Google Gemini, are transforming healthcare. This study explores the performance of these AI models in solving diagnostic quizzes from "Neuroradiology: A Core Review" to evaluate their potential as diagnostic tools in radiology. MATERIALS AND METHODS: We assessed the accuracy of ChatGPT 3.5, ChatGPT 4.0, and Google Gemini using 262 multiple-choice questions covering brain, head and neck, spine, and non-interpretive skills. Each AI tool provided answers and explanations, which were compared to textbook answers. The analysis followed the STARD (Standards for Reporting of Diagnostic Accuracy Studies) guidelines, and accuracy was calculated for each AI tool and subgroup. RESULTS: ChatGPT 4.0 achieved the highest overall accuracy at 64.89%, outperforming ChatGPT 3.5 (62.60%) and Google Gemini (55.73%). ChatGPT 4.0 excelled in brain, head, and neck diagnostics, while Google Gemini performed best in head and neck but lagged in other areas. ChatGPT 3.5 showed consistent performance across all subgroups. CONCLUSION: This study found that advanced AI models, including ChatGPT 4.0 and Google Gemini, vary in diagnostic accuracy, with ChatGPT 4.0 leading at 64.89% overall. While these tools are promising in improving diagnostics and medical education, their effectiveness varies by area, and Google Gemini performs unevenly across different categories. The study underscores the need for ongoing improvements and broader evaluation to address ethical concerns and optimize AI use in patient care.

Central nervous system pediatric multi-disciplinary tumor board: a single center experience.

BACKGROUND: The Multidisciplinary Tumor Board (MTB) is a collaborative platform involving specialists in oncology, surgery, radiology, pathology, and radiotherapy, and aims to optimize diagnostics and treatments. Despite MTB's widespread benefits, limited literature addresses its application in pediatric neuro-oncology. After a literature revision on pediatric neuro-oncology MTB, our study describes our institute's pediatric neuro-oncology MTB, focuses on evaluating its impact and the neuroradiologist's role in patient-centric approaches, considering recent genetic insights into pediatric brain tumors. MATERIALS AND METHODS: Literature Review concerning pediatric neuro-oncology MTB from January 2002 to June 2024. CLINICAL DATA: retrospective study of all patient files presented in the pediatric neuro-oncology MTB (pnMTB) between 2019 and 2022. Statistical analysis was mainly carried out by directly comparing the absolute or relative values of the respective parameters examined; qualitative variables compared mainly with the chi-square test, quantitative variables mainly with the t-test. RESULTS: Literature Review: 7 papers encompass a multidisciplinary approach for the pediatric CNS tumors. CLINICAL DATA: A total of 236 discussions were analyzed representing 107 patients. Median age was 14,3 years (range: 6 months - 17 years). The requests for case evaluations primarily came from the pediatric oncologists (83%) and neurosurgeons (14.8%), and they were mainly addressed to the neuroradiologists (70.3%). Proposals during pnMTB mainly involved imaging follow-up (47.8%) and management with chemotherapy (34.7%). Changes in patient treatment (CPT) occurred in 115 cases, and pediatric neuroradiologist intervention contributed to 72.4% of these changes. CONCLUSION: Thanks to their multidisciplinarity, high number of cases discussed, and usual respect for their proposals, the pnMTB has made it possible to improve the coordination among specialties involved in patient management, to apply the recent protocols, and to exchange knowledge among teams managing pediatric CNS tumors.

Progressive Multifocal Leukoencephalopathy in a Patient With Cirrhosis and Hepatocellular Carcinoma.

The following case describes a constellation of progressive cognitive and motor deficits in a 73-year-old man with cirrhosis and history of early-stage hepatocellular carcinoma confined to his liver. He had deficits in calculation, language, and writing, as well as subtle right-sided weakness. Magnetic resonance imaging (MRI) of the brain demonstrated non-enhancing white matter lesions without mass effect in the bilateral parietal and left occipitotemporal regions, correlating with neurologic exam findings. The patient's basic blood and cerebrospinal fluid (CSF) studies were within normal limits. Our differential included inflammatory and demyelinating conditions, hepatic encephalopathy, posterior reversible encephalopathy syndrome, progressive multifocal leukoencephalopathy (PML), and central nervous system (CNS) tumors. He did not improve with an empiric course of high-dose steroids or adequate hepatic encephalopathy treatment. A repeat lumbar puncture sent for additional CSF studies revealed a positive John Cunningham (JC) virus PCR test, confirming diagnosis of PML. Although the patient did not have any known overt immunosuppressive condition or treatment, the patient's cirrhosis and age placed him at higher risk for developing JC virus CNS reactivation. In a published case series of patients with PML and no classic immunosuppressive condition that includes several patients with concomitant cirrhosis, prognosis is much worse compared to those with known, reversible causes of immunosuppression.

Artificial intelligence and advanced MRI techniques: A comprehensive analysis of diffuse gliomas.

INTRODUCTION: The complexity of diffuse gliomas relies on advanced imaging techniques like MRI to understand their heterogeneity. Utilizing the UCSF-PDGM dataset, this study harnesses MRI techniques, radiomics, and AI to analyze diffuse gliomas for optimizing patient outcomes. METHODS: The research utilized the dataset of 501 subjects with diffuse gliomas through a comprehensive MRI protocol. After performing intricate tumor segmentation, 82.800 radiomic features were extracted for each patient from nine segmentations across eight MRI sequences. These features informed neural network and XGBoost model training to predict patient outcomes and tumor grades, supplemented by SHAP analysis to pinpoint influential radiomic features. RESULTS: In our analysis of the UCSF-PDGM dataset, we observed a diverse range of WHO tumor grades and patient outcomes, discarding one corrupt MRI scan. Our segmentation method showed high accuracy when comparing automated and manual techniques. The neural network excelled in prediction of WHO tumor grades with an accuracy of 0.9500 for the necrotic tumor label. The SHAP-analysis highlighted the 3D First Order mean as one of the most influential radiomic features, with features like Original Shape Sphericity and Original Shape Elongation were notably prominent. CONCLUSION: A study using the UCSF-PDGM dataset highlighted AI and radiomics' profound impact on neuroradiology by demonstrating reliable tumor segmentation and identifying key radiomic features, despite challenges in predicting patient survival. The research emphasizes both the potential of AI in this field and the need for broader datasets of diverse MRI sequences to enhance patient outcomes. IMPLICATION FOR PRACTICE: The study underline the significant role of radiomics in improving the accuracy of tumor identification through radiomic features.

Diagnostic reference levels in interventional neuroradiology procedures - a systematic review.

INTRODUCTION: The establishment of diagnostic reference levels (DRLs) is challenge for interventional neuroradiology (INR) due to the complexity and variability of its procedures. OBJECTIVE: The main objective of this systematic review is to analyse and compare DRLs in fluoroscopy-guided procedures in INR. METHODS: An observational study reporting DRLs in INR procedures, specifically cerebral arteriography, cerebral aneurysm embolisation, cerebral thrombectomy, embolisation of arteriovenous malformations (AVM), arteriovenous fistulas (AVF), retinoblastoma embolisation, and spinal cord arteriography. Comprehensive literature searches for relevant studies published between 2017 and 2023 were conducted using the Scopus, PubMed, and Web of Science databases. RESULTS: A total of 303 articles were identified through an extensive literature search, with 159 removed due to duplication. The title and abstract of 144 studies were assessed and excluded if they did not meet the inclusion criteria. Thirty-one out of the 144 articles were selected for a thorough full-text screening. Twenty-one articles were included in the review after the complete text screening. CONCLUSION: The different conditions of patients undergoing INR procedures pose a barrier to the standardization of DRLs; nevertheless, they are extremely important for monitoring and optimising radiological practices.

Brain microbleeds resulting from presumed extensive fat emboli in a patient with bone marrow necrosis following a sickle cell disease vaso-occlusive crisis.

Acute manifestations of sickle cell disease (SCD) are numerous and multisystemic. Cerebral fat embolism (CFE) is a rare but serious complication of SCD caused by bone marrow necrosis (BMN) during vaso-occlusive crises (VOC). We present the case of a 41-year-old man with SCD who developed severe VOC and multi-organ dysfunction. He subsequently experienced neurological deterioration with decreased consciousness and diffuse encephalopathy on serial electroencephalograms. Bone marrow aspiration confirmed BMN. Brain MRI revealed extensive diffuse leukoencephalopathy, vasogenic and cytotoxic edema in the white matter, patchy edema in the cranial vault bone marrow on fat-suppressed FLAIR sequence (a finding consistent with the confirmed BMN), and multiple cerebral microbleeds on susceptibility-weighted imaging consistent with CFE. The management of acute neurological complications of SCD varies depending on the specific complication. Brain MRI plays a crucial role in the accurate diagnosis of these complications to guide appropriate treatment.

IgG4-related disease in the nervous system.

IgG4-related disease (IgG4-RD) is a recently described multisystemic disorder with a spectrum of manifestations that continue to be described. Nonetheless, there are recognised distinct patterns of disease. Neurological involvement is rare, particularly in isolation, but IgG4-RD may present with orbital disease, hypophysitis or pachymeningitis. Typically, it is highly responsive to treatment. This review highlights neurological manifestations of IgG4-RD and emphasises the importance of a high index of clinical suspicion to facilitate investigation and appropriate management, avoiding irreversible tissue damage and neurological dysfunction. We present a treatment algorithm for suggested management of IgG4-RD affecting the nervous system.

StentRetriever- aspiration (SRa) vs direct aspiration (DA) in the treatment of acute M2 segment occlusion of middle cerebral artery: A single center randomized prospective study.

Decision making and patient selection for isolated occlusion of the M2 segment of the middle cerebral artery is challenging, with the efficacy of treatment for such distal occlusions still controversial in recent studies, as occlusion in such small and thin vessels tends to manifest with less severe symptoms initially and hold higher surgical risks. It is even less clear which endovascular technique is more effective for this type of occlusion, with the choice usually left to the radiologist's preference. We conducted a controlled prospective study of consecutive patients presenting to Novara Hospital with an acute M2 occlusion diagnosed at the AngioCt and, following a predetermined randomization, we divided them into two treatment arms with either stent retriever aspiration (SRa) or direct aspiration (DA). All patients were examined on admission, at discharge and after 3 months, and clinical data as well as mRS and NIHSS scale scores were recorded. Our primary aim was to evaluate the difference in recanalization rate in the two groups by comparing the angiographic eTICI obtained with the two techniques. We then investigated whether there were differences in clinical outcomes and complications. Our data confirm a good recanalization rate with an eTICI ≥ 2b in 76.19% of patients in the cohort. An overall good outcome was achieved in 57% of patients as ∆NIHSS and in 50.79% of patients considering mRS. We found no statistically significant difference in recanalization rate nor higher complication rate in either the SRa or DA group. Both techniques are safe, effective and can be considered equally.

Neuroimaging in Nonsyndromic Craniosynostosis: Key Concepts to Unlock Innovation.

Craniosynostoses (CRS) are caused by the premature fusion of one or more cranial sutures, with isolated nonsyndromic CRS accounting for most of the clinical manifestations. Such premature suture fusion impacts both skull and brain morphology and involves regions far beyond the immediate area of fusion. The combined use of different neuroimaging tools allows for an accurate depiction of the most prominent clinical-radiological features in nonsyndromic CRS but can also contribute to a deeper investigation of more subtle alterations in the underlying nervous tissue organization that may impact normal brain development. This review paper aims to provide a comprehensive framework for a better understanding of the present and future potential applications of neuroimaging techniques for evaluating nonsyndromic CRS, highlighting strategies for optimizing their use in clinical practice and offering an overview of the most relevant technological advancements in terms of diagnostic performance, radiation exposure, and cost-effectiveness.

Utility of MR spectroscopy and MR perfusion in characterizing intracranial pathology in Erdheim-Chester disease: A case report.

This case report describes the potential utility of MR spectroscopy and MR perfusion imaging in a patient with central nervous system involvement of Erdheim-Chester disease (ECD). A 57-year-old male presented with a variety of neurological symptoms, and conventional MRI of the brain showed multiple supratentorial and infratentorial findings that generated a wide differential diagnosis. Advanced MRI sequences along with subsequent CT imaging of the abdomen and ultimately a renal biopsy helped narrow the differential and confirm the diagnosis of ECD. Case reports of ECD are sporadic, and the role of advanced neuroimaging in diagnosing this disease has not been fully elucidated.

Vestibular schwannoma causing normal pressure hydrocephalus.

Vestibular schwannoma is a common benign tumour that may cause local complications. However, vestibular schwannoma has a known association with communicating hydrocephalus presenting with symptoms of normal pressure hydrocephalus and requiring treatment by ventricular shunting or tumour resection. We report a 79-year-old woman who presented with subacute gait apraxia, cognitive impairment and urinary incontinence. CT and MR imaging identified a 20 mm vestibular schwannoma and communicating hydrocephalus; her cerebrospinal fluid (CSF) protein was elevated. Her symptoms improved following ventriculoperitoneal shunt insertion. The mechanism by which non-obstructing vestibular schwannoma causes hydrocephalus is unclear, but hyperproteinorrachia is probably important, likely by impeding CSF resorption.

An interdisciplinary integrated specialized one-stop outpatient clinic for idiopathic intracranial hypertension-a comprehensive assessment of clinical outcome.

BACKGROUND AND PURPOSE: Management of idiopathic intracranial hypertension (IIH) is complex requiring multiple specialized disciplines. In practice, this creates considerable organizational and communicational challenges for healthcare professionals and patients. Thus, an interdisciplinary integrated outpatient clinic for IIH (comprising neurology, neuroophthalmology, neuroradiology, neurosurgery and endocrinology) was established with central coordination and a one-stop concept. Here, the aim was to evaluate the effects of this one-stop concept on objective clinical outcome. METHODS: In a retrospective cohort study, the one-stop era with integrated care (IC) (1 July 2021 to 31 December 2022) was compared to a reference group receiving standard care (SC) (1 July 2018 to 31 December 2019) regarding visual impairment/worsening and headache improvement/freedom 6 months after diagnosis. Multivariate binary logistic regression models were used to adjust for confounders. RESULTS: Baseline characteristics of the IC group (n = 85) and SC group (n = 81) were comparable (female 90.6% vs. 90.1%; mean age 33.6 vs. 32.8 years; median body mass index 31.8 vs. 33.0; median cerebrospinal fluid opening pressure 32 vs. 34 cmH2O; at diagnosis, visual impairment was present in 71.8% vs. 69.1% and chronic headache in 55.3% vs. 56.8% in IC vs. SC). IC was associated with a higher likelihood of achieving both headache improvement (odds ratio [OR] 2.24, 95% confidence interval [CI] 1.52-4.33, p < 0.001) and headache freedom (OR 1.75, 95% CI 1.11-3.09, p = 0.031). Regarding the risk of visual impairment and visual worsening IC was superior numerically but not statistically significantly (OR 0.87, 95% CI 0.69-1.16, p = 0.231, and OR 0.67, 95% CI 0.41-1.25, p = 0.354). CONCLUSIONS: Interdisciplinary integrated care of IIH is favourably associated with headache outcomes and potentially also visual outcomes.

Glycaemia and ischaemia-reperfusion brain injury in patients with ischaemic stroke treated with mechanical thrombectomy (GLIAS-MT): an observational, unicentric, prospective study protocol.

INTRODUCTION: Poststroke hyperglycaemia is an independent risk factor for poorer outcomes in patients treated with mechanical thrombectomy (MT) and is associated with a lower probability of functional recovery and higher mortality at 3 months. This study aims to evaluate the association between glucose levels during cerebral reperfusion with MT and functional recovery at 3 months, measured by subcutaneous continuous glucose monitoring (CGM) devices. METHODS: This prospective observational study aims to recruit 100 patients with ischaemic stroke and large anterior circulation vessel occlusion, in whom MT is indicated. CGM will be performed using a Freestyle Libre ProIQ device (FSL-CGM, Abbott Diabetes Care, Alameda, California, USA), which will be implanted on admission to the emergency department, to monitor glucose levels before, during and after reperfusion. The study's primary endpoint will be the functional status at 3 months, as measured by the dichotomised modified Rankin Scale (0-2 indicating good recovery and 3-6 indicating dependency or death). We will analyse expression profiles of microRNA (miRNA) at the time of reperfusion and 24 hours later, as potential biomarkers of ischaemic-reperfusion injury. The most promising miRNAs include miR-100, miR-29b, miR-339, miR-15a and miR-424. All patients will undergo treatment according to current international recommendations and local protocols for the treatment of stroke, including intravenous thrombolysis if indicated. ETHICS AND DISSEMINATION: This study (protocol V.1.1, dated 29 October 2021, code 6017) has been approved by the Clinical Research Ethics Committee of La Paz University Hospital (Madrid, Spain) and has been registered in ClinicalTrials.gov (NCT05871502). Study results will be disseminated through peer-reviewed publications in Open Access format and at conference presentations. TRIAL REGISTRATION NUMBER: NCT05871502.

Diffuse scalp neurofibromas: Case series and Clinicoradiological characteristics of a rare vascular lesion.

Diffuse neurofibroma of the head and neck are rare tumours which have unique clinical and radiological findings. Presurgical diagnosis is important as these lesions are usually highly vascular and pre-operative embolisation can reduce the risk of intra-operative haemorrhage. In this article we describe four cases; two which underwent pre-operative embolisation, which should aid the reader in successfully diagnosing this entity before biopsy/surgery.

Using The Comaneci temporary assist device to expand a partially expanded flow diverting stent.

The article describes a case of successful treatment of cerebral aneurysm with a flow-diverting stent using the temporary assist device Comaneci (Rapid Medical, Yokneam, Israel). Clinical symptoms and methods of instrumental diagnostic for cerebral aneurysm are presented. Endovascular management technique is described in detail. Flow diversion has become a widely accepted treatment option for intracranial aneurysms, especially those with a wide neck. However, a complication of this procedure is incomplete stent expansion. This clinical case describes resolving the incomplete flow divertor expansion using the controllable temporary assistance device Comaneci.