Bathla, Girish

Alcohol use disorder is linked to a multitude of diseases and disorders, many of which can be diagnosed with imaging techniques such as US, CT, and MRI, enabling early detection and potentially preventing further damage and improving outcomes.

Despite their similar nomenclature, Neurofibromatosis type 1 (NF1) and “Neurofibromatosis type 2” are discrete and clinically distinguishable entities. The name of “neurofibromatosis type 2” has been changed to NF2-related schwannomatosis, to reflect the fact that neurofibromas do not occur in this syndrome and therefore the name “Neurofibromatosis” is factually incorrect. Furthermore, multiple schwannomas, a hallmark feature of NF2, can also occur in patients with mutations in genes including SMARCB1 and LZTR1, all exhibiting overlapping clinical features. Current understanding suggests that schwannomatosis (SWN) encompasses a range of clinical presentations consisting of clearly defined, separate subtypes which share a common phenotype of schwannomas. Recognizing these newly emerging subtypes, the International Consensus Group on Neurofibromatosis Diagnostic Criteria (I-NF-DC) proposed a revised nomenclature for NF2 and related disorders in 2022. This review article focuses on this critical update in diagnostic terminology, highlighting the key gene-related SWN subtypes relevant to neuroradiologists. By emphasizing molecular testing alongside clinical features, the revised system facilitates a more precise diagnosis, potentially paving the way for personalized treatment strategies. Additionally, the flexible structure accommodates future discoveries of genes associated with SWN.

Objective To analyze cases of medial migration of vestibular schwannomas to propose an underlying mechanism. Study Design Retrospective chart review. Patients Ten patients from one institution with sporadic vestibular schwannomas that demonstrated medial migration toward the cerebellopontine angle on serial imaging were reviewed. Interventions Among the 10 patients studied, 8 patients underwent stereotactic radiosurgery and 2 were managed with wait-and-scan. All serial imaging was analyzed throughout follow-up. Main Outcome Measures Volumetric and linear measurements were used to assess vestibular schwannoma growth. The fundal fluid cap length was measured from the most lateral end of the neoplasm within the internal auditory canal to the medial end of the cochlea. Pure tone averages of 0.5, 1, 2, and 3 kHz, word recognition scores, and AAO-HNS hearing class were used to assess levels of hearing loss per patient. Results Tumor growth exhibited a positive correlation with medial migration. Of the 10 patients studied, 9 exhibited tumor volume growth from initial to most recent imaging. Median changes in tumor volumes and fundal fluid cap lengths from initial diagnosis to the most recent imaging regardless of intervention were 130 mm3 (range 1,400–2,970) and 2.8 mm (range 1.8–6.0), respectively. Migration was noted in 4 patients prior to any intervention, in 4 patients postradiosurgery, and in 2 patients before and after radiosurgery. Therefore, half of the tumors studied migrated before an intervention and half migrated postradiosurgery. No correlation of migration and hearing decline was observed. Conclusion Medial migration of vestibular schwannoma is associated with an increase in tumor volume, supporting a “tumor squeeze” mechanism within a conical internal auditory canal toward the wider porus acusticus and cerebellopontine angle.

Abstract Objectives There are an increasing number of centres performing research on high-resolution vessel wall magnetic resonance imaging (VW-MRI) in GCA. However, harmonized approaches to VW-MRI in GCA are lacking and are essential to performing multicentre studies. Using a data-driven, consensus-based approach, an international expert group developed a standardized MRI protocol and scoring system to advance multi-centred research in cranial GCA. Methods A targeted literature review of VW-MRI in cranial GCA was conducted. A working group comprised of radiologists, rheumatologists and ophthalmologists with expertise in VW-MRI and GCA reviewed the results of the literature search, presented relevant data and images from their respective centres, and then reached consensus on recommendations related to key MRI structures, MRI sequences, scoring system and other important considerations. Results A total of 21 relevant articles were identified and reviewed. Based on published literature, structures to be evaluated on MRI were categorized based on anatomic location (extradural cranial, intradural cranial and orbits) and prioritization (core vs elective). Essential and elective sequences to comprehensively image cranial and orbital structures while minimizing scan time were determined along with scoring systems to grade contrast enhancement. Conclusion This report describes a standardized approach to facilitate research of VW-MRI in cranial GCA that is the result of a multidisciplinary, international collaboration of experts in VW-MRI and/or GCA.

Diseases of the carotid arteries can be classified into different categories based on their origin. Atherosclerotic carotid disease remains the most encountered arterial wall pathology. However, other less-common non-atherosclerotic diseases can have detrimental clinical consequences if not appropriately recognized. The underlying histological features of each disease process may result in imaging findings that possess features that are obvious of the disease. However, some carotid disease processes may have histological characteristics that manifest as non-specific radiologic findings. The purpose of this manuscript is to review various non-atherosclerotic causes of carotid artery disease as well as their histologic-radiologic characteristics to aid in the appropriate recognition of these less-commonly encountered pathologies.

Abstract Photon-counting CT (PCCT) uses a novel X-ray detection mechanism that confers many advantages over that used in traditional energy integrating CT. As PCCT becomes more available, it is important to thoroughly understand its benefits and highest yield areas for improvements in diagnosis of various diseases. Based on our early experience, we have identified several areas of neurovascular imaging in which PCCT shows promise. Here, we describe the benefits in diagnosing arterial and venous diseases in the head, neck, and spine. Specifically, we focus on applications in head and neck CT angiography (CTA), spinal CT angiography, and CT myelography for detection of CSF-venous fistulas. Each of these applications highlights the technological advantages of PCCT in neurovascular imaging. Further understanding of these applications will not only benefit institutions incorporating PCCT into their practices but will also help guide future directions for implementation of PCCT for diagnosing other pathologies in neuroimaging.

Cauda Equina Neuroendocrine Tumors (CE-NET), previously referred to as paragangliomas are a rare subset of spinal tumors, with limited data on imaging. Herein, we present a retrospective review of clinical and imaging findings of CE-NETs in ten patients who were evaluated at our institution over the past two decades. All patients had well-defined intradural lesions in the lumbar spine which demonstrated slow growth. A review of imaging findings revealed the presence of an eccentric vascular pedicle along the dorsal aspect of the tumor in 8 of the 10 patients (eccentric vessel sign), a distinctive finding that has not previously been reported with this tumor and may help improve the accuracy of imaging-based diagnosis. In all cases, a gross-total resection was performed, with resolution of symptoms in most of the cases.

BACKGROUND AND OBJECTIVES: Meningeal solitary fibrous tumors (SFTs) comprise 0.4% of primary central nervous system neoplasms and carry metastatic potential. Disease course and optimal management are largely unknown, and there is currently no literature rigorously describing neurological outcomes in surgically managed SFTs. We present one of the largest craniospinal SFT series, analyze patient outcomes, and extensively review the associated literature. METHODS: All surgically managed SFTs at our institution between January 2005 and March 2023 were retrospectively reviewed. Patient demographics, tumor and radiographic features, treatment, and clinical outcomes were collected. Neurological function was quantified using Frankel grade and Neurologic Assessment in Neuro-Oncology scores. Descriptive statistics, multivariate analysis, log-rank test, and Kaplan–Meier survival analysis were performed. RESULTS: Twenty-one patients satisfied inclusion criteria. Tumor locations included 15 supratentorial, three infratentorial, and three spinal. All patients underwent surgical resection, and 16 (76.2%) underwent radiation. Six (28.6%) patients had tumor recurrence, and three (14.3%) developed metastasis. Younger age and higher postoperative Frankel grade were significantly associated with increased overall survival (OS) ( P = .011, P = .002, respectively). All patients symptomatically improved or stabilized after surgery, and Neurologic Assessment in Neuro-Oncology score ( P = .001) and functional status significantly improved postoperatively (Karnofsky Performance Status: 65.2 ± 25.2 vs 91.4 ± 13.5, P = .001). Sex, adjuvant radiation, and extent of resection were not significantly associated with OS. CONCLUSION: SFT of the central nervous system is a rare entity with a variable clinical course. Surgical resection was associated with improved postoperative functional and neurological status. Higher postoperative neurological function was significantly associated with OS. Further studies are warranted to validate a standardized treatment algorithm and investigate the efficacy of adjuvant radiation in SFT.

Abstract Background Neurofibromatosis type 2 (NF2)-related schwannomatosis is an autosomal dominant tumor-predisposition syndrome characterized by bilateral vestibular schwannomas (VS). In patients with VS associated with NF2, vascular endothelial growth factor A inhibitor, bevacizumab, is a systemic treatment option. The aim of this study is to retrospectively evaluate NF2 patient responses to bevacizumab on VS growth and symptom progression. Methods This is a retrospective analysis of patients seen at the Mayo Clinic Rochester Multidisciplinary NF2 Clinic. Results Out of 76 patients with NF2 evaluated between 2020 and 2022, we identified 19 that received treatment with bevacizumab. Thirteen of these patients discontinued bevacizumab after median treatment duration of 12.2 months. The remaining 6 patients are currently receiving bevacizumab treatment for a median duration of 9.4 months as of March, 2023. Fifteen patients had evaluable brain MRI data, which demonstrated partial responses in 5 patients, stable disease in 8, and progression in 2. Within 6 months of bevacizumab discontinuation, 5 patients had rebound growth of their VS greater than 20% from their previous tumor volume, while 3 did not. Three patients with rebound growth went on to have surgery or irradiation for VS management. Conclusions Our single-institution experience confirms prior studies that bevacizumab can control progression of VS and symptoms associated with VS growth. However, we note that there is the potential for rapid VS growth following bevacizumab discontinuation, for which we propose heightened surveillance imaging and symptom monitoring for at least 6 months upon stopping anti-VEGF therapy.