Another 'BEE'? - Brain-Eye-Ear (BEE) Disease Secondary to HbSC Disease Masquerading as Multiple Sclerosis.

Recurrent episodes of neurological dysfunction and white matter lesions in a young adult raise suspicion for multiple sclerosis (MS). However, occlusive retinopathy, hearing loss and absence of CSF oligoclonal bands are atypical for MS and should make the clinician consider an alternative diagnosis. We describe a man with hearing loss, visual signs and symptoms, and an accumulating burden of brain lesions, who was treated for a clinical diagnosis of MS for nearly two decades. Genetic testing revealed a unifying diagnosis.

COVID-19-associated Diffuse Leukoencephalopathy and Microhemorrhages.

Diffuse leukoencephalopathy and juxtacortical and/or callosal microhemorrhages were brain imaging features in critically ill patients with coronavirus disease 2019. Coronavirus disease 2019 (COVID-19) has been reported in association with a variety of brain imaging findings such as ischemic infarct, hemorrhage, and acute hemorrhagic necrotizing encephalopathy. Herein, the authors report brain imaging features in 11 critically ill patients with COVID-19 with persistently diminished mental status who underwent MRI between April 5 and April 25, 2020. These imaging features include (a) confluent T2 hyperintensity and mild restricted diffusion in bilateral supratentorial deep and subcortical white matter (in 10 of 11 patients) and (b) multiple punctate microhemorrhages in juxtacortical and callosal white matter (in seven of 11 patients). The authors also discuss potential pathogeneses.

A Severe Case of Streptococcus pneumoniae Meningoencephalitis in an Infant Resulting in Fatal Strokes.

Streptococcus pneumoniae (S. Pneumoniae) is a common cause of bacterial meningitis in the pediatric population, but rarely causes complications such as encephalitis, abscess, and seizures with the prompt initiation of proper antimicrobial therapy. In this report, we present a rare and severe case of S. Pneumoniae meningoencephalitis in a full term 6-month-old which progressed to multiple cerebral and cerebellar infarcts with concomitant cerebellar tonsillar herniation and hypercarbic respiratory failure requiring intubation and mechanical ventilation despite early initiation of antibiotics. Given the patient's clinical status and poor neurological prognosis, the family eventually opted for palliative extubation.

Penetrating diaphragmatic injury: accuracy of 64-section multidetector CT with trajectography.

PURPOSE: To (a) determine the diagnostic performance of 64-section multidetector computed tomography (CT) trajectography for penetrating diaphragmatic injury (PDI), (b) determine the diagnostic performance of classic signs of diaphragmatic injury at 64-section multidetector CT, and (c) compare the performance of these signs with that of trajectography. MATERIALS AND METHODS: This HIPAA-compliant retrospective study had institutional review board approval, with a waiver of the informed consent requirement. All patients who had experienced penetrating thoracoabdominal trauma, who had undergone preoperative 64-section multidetector CT of the chest and abdomen, and who had surgical confirmation of findings during a 2.5-year period were included in this study (25 male patients, two female patients; mean age, 32.6 years). After a training session, four trauma radiologists unaware of the surgical outcome independently reviewed all CT studies and scored the probability of PDI on a six-point scale. Collar sign, dependent viscera sign, herniation, contiguous injury on both sides of the diaphragm, discontinuous diaphragm sign, and transdiaphragmatic trajectory were evaluated for sensitivity, specificity, negative predictive value (NPV), and positive predictive value (PPV). Accuracies were determined and receiver operating characteristic curves were analyzed. RESULTS: Sensitivities for detection of PDI by using 64-section multidetector CT with postprocessing software ranged from 73% to 100%, specificities ranged from 50% to 92%, NPVs ranged from 71% to 100%, PPVs ranged from 68% to 92%, and accuracies ranged from 70% to 89%. Discontinuous diaphragm, herniation, collar, and dependent viscera signs were highly specific (92%-100%) but nonsensitive (0%-60%). Contiguous injury was generally more sensitive (80%-93% vs 73%-100%) but less specific (50%-67% vs 83%-92%) than transdiaphragmatic trajectory when patients with multiple entry wounds were included in the analysis. Transdiaphragmatic trajectory was a much more sensitive sign of PDI than previously reported (73%-100% vs 36%), with NPVs ranging from 71% to 100% and PPVs ranging from 85% to 92%. CONCLUSION: Sixty-four-section multidetector CT trajectography facilitates the identification of transdiaphragmatic trajectory, which accurately rules in PDI when identified. Contiguous injury remains a highly sensitive sign, even when patients with multiple injuries are considered, and is useful for excluding PDI.

Conventional and advanced MRI features of pediatric intracranial tumors: posterior fossa and suprasellar tumors.

OBJECTIVE: In this article, we review the most common posterior fossa and suprasellar intracranial neoplasms in the pediatric population. We briefly discuss basic MRI concepts used in the initial evaluation of a pediatric brain tumor and then discuss sophisticated MRI techniques that give insight into the physiology and chemical makeup of these tumors to help the radiologist make a more specific diagnosis. CONCLUSION: Diagnosis and treatment of pediatric CNS tumors necessitate a multi-disciplinary approach and require expertise and diligence of all parties involved. Imaging is an essential component has evolved greatly over the past decade. We are becoming better at making a preoperative diagnosis of that tumor type, detecting recurrence, and guiding surgical management to avoid injury to vital brain structures.

Conventional and advanced MRI features of pediatric intracranial tumors: supratentorial tumors.

OBJECTIVE. Our objective is to review the imaging characteristics and applications of conventional and advanced neuroimaging techniques of supratentorial intracranial masses in the pediatric population. Specifically, we review astrocytomas, oligodendrogliomas, primary neuroectodermal tumors, dysembryoplastic neuroepithelial tumors, gangliogliomas, arachnoid cysts, and choroid plexus and pineal region masses. CONCLUSION. Advanced imaging methods, such as MR spectroscopy, perfusion MRI, functional MRI, diffusion-tensor imaging, and tractography, help develop a more accurate differential diagnosis and aid in planning tumor treatment.

Pearls & Oy-sters: CSF1R-Related Leukoencephalopathy With Spinal Cord Lesions Mimicking Multiple Sclerosis.

CSF1R-related leukoencephalopathy is an autosomal dominant neurologic disorder causing microglial dysfunction with a wide range of neurologic complications, including motor dysfunction, dementia, and seizures. This case report highlights an unusual presentation of CSF1R-related leukoencephalopathy with radiographic spinal cord involvement initially diagnosed as multiple sclerosis. This case highlights the importance of considering adult-onset neurogenetic disorders in the setting of white matter disease. Genetic testing provides a confirmatory diagnosis for an expanding number of adult-onset leukoencephalopathies and informs therapeutic decision-making.

Highly time-resolved 4D MR angiography using golden-angle radial sparse parallel (GRASP) MRI.

Current dynamic MRA techniques are limited by temporal resolution and signal-to-noise penalties. GRASP, a fast and flexible MRI technique combining compressed-sensing, parallel imaging, and golden-angle radial sampling, acquires volumetric data continuously and can be reconstructed post hoc for user-defined applications. We describe a custom pipeline to retrospectively reconstruct ultrahigh temporal resolution, dynamic MRA from GRASP imaging obtained in the course of routine practice. GRASP scans were reconstructed using a custom implementation of the GRASP algorithm and post-processed with MeVisLab (MeVis Medical Solutions AG, Germany). Twenty consecutive examinations were scored by three neuroradiologists for angiographic quality of specific vascular segments and imaging artifacts using a 4-point scale. Unsubtracted images, baseline-subtracted images, and a temporal gradient dataset were available in 2D and 3D reconstructions. Distinct arterial and capillary phases were identified in all reconstructions, with a median of 2 frames (IQR1-3 and 2-3, respectively). Median rating for vascular segments was 3 (excellent) in all reconstructions and for nearly all segments, with excellent intraclass correlation (range 0.91-1.00). No cases were degraded by artifacts. GRASP-MRI obtained in routine practice can be seamlessly repurposed to produce high quality 4D MRA with 1-2-s resolved isotropic cerebrovascular angiography. Further exploration into diagnostic accuracy in disease-specific applications is warranted.

Impact on Participants of Family Connect, a Novel Program Linking COVID-19 Inpatients' Families With the Frontline Providers.

PURPOSE: With clinical volumes decreased, radiologists volunteered to participate virtually in daily clinical rounds and provide communication between frontline physicians and patients with coronavirus disease 2019 (COVID-19) and their families affected by restrictive hospital visitation policies. The purpose of this survey-based assessment was to demonstrate the beneficial effects of radiologist engagement during this pandemic and potentially in future crises if needed. METHODS: After the program's completion, a survey consisting of 13 multiple-choice and open-ended questions was distributed to the 69 radiologists who volunteered for a minimum of 7 days. The survey focused on how the experience would change future practice, the nature of interaction with medical students, and the motivation for volunteering. The electronic medical record system identified the patients who tested positive for or were suspected of having COVID-19 and the number of notes documenting family communication. RESULTS: In all, 69 radiologists signed or cosigned 7,027 notes. Of the 69 radiologists, 60 (87.0%) responded to the survey. All found the experience increased their understanding of COVID-19 and its effect on the health care system. Overall, 59.6% agreed that participation would result in future change in communication with patients and their families. Nearly all (98.1%) who worked with medical students agreed that their experience with medical students was rewarding. A majority (82.7%) chose to participate as a way to provide service to the patient population. CONCLUSION: This program provided support to frontline inpatient teams while also positively affecting the radiologist participants. If a similar situation arises in the future, this communication tool could be redeployed, especially with the collaboration of medical students.

Diffusion MR Imaging in Mild Traumatic Brain Injury.

Remarkable advances have been made in the last decade in the use of diffusion MR imaging to study mild traumatic brain injury (mTBI). Diffusion imaging shows differences between mTBI patients and healthy control groups in multiple different metrics using a variety of techniques, supporting the notion that there are microstructural injuries in mTBI patients that radiologists have been insensitive to. Future areas of discovery in diffusion MR imaging and mTBI include larger longitudinal studies to better understand the evolution of the injury and unravel the biophysical meaning that the detected changes in diffusion MR imaging represent.

Influence of clinical history on MRI interpretation of optic neuropathy.

BACKGROUND AND PURPOSE: Clinical history is known to influence interpretation of a wide range of radiologic examinations. We sought to evaluate the influence of the clinical history on MRI interpretation of optic neuropathy. MATERIALS AND METHODS: 107 consecutive orbital MRI scans were retrospectively reviewed by three neuroradiologists. The readers independently evaluated the coronal STIR sequence for optic nerve hyperintensity and/or atrophy (yes/no) and the coronal post-contrast T1WI for optic nerve enhancement (yes/no). Readers initially evaluated the cases blinded to the clinical history. Following a two week washout period, readers again evaluated the cases with the clinical history provided. Inter-reader and reader-clinical radiologist agreement was assessed using Cohen's simple kappa coefficient. RESULTS: Intra-reader agreement, without and with provision of clinical history, was 0.564-0.716 on STIR and 0.270-0.495 on post-contrast T1WI. Inter-reader agreement was overall fair-moderate. On post-contrast T1WI, inter-reader agreement was significantly higher when the clinical history was provided (p = 0.001). Reader-clinical radiologist agreement improved with provision of the clinical history to the readers on both the STIR and post-contrast T1WI sequences. CONCLUSIONS: In the MRI assessment of optic neuropathy, only modest levels of inter-reader agreement were achieved, even after provision of clinical history. Provision of clinical history improved inter-reader agreement, especially when assessing for optic nerve enhancement. These findings confirm the subjective nature of orbital MRI interpretation in cases of optic neuropathy, and point to the importance of an accurate clinical history. Of note, the accuracy of orbital MRI in the context of optic neuropathy was not assessed, and would require further investigation.

Ultrafast Brain MRI: Clinical Deployment and Comparison to Conventional Brain MRI at 3T.

BACKGROUND AND PURPOSE: To compare an ultrafast brain magnetic resonance imaging (MRI) protocol to the conventional protocol in motion-prone inpatient clinical settings. METHODS: This retrospective study was HIPAA compliant and approved by the Institutional Review Board with waived inform consent. Fifty-nine inpatients (30 males, 29 females; mean age 55.1, range 23-93 years)who underwent 3-Tesla brain MRI using ultrafast and conventional protocols, both including five sequences, were included in the study. The total scan time for five ultrafast sequences was 4 minutes 59 seconds. The ideal conventional acquisition time was 10 minutes 32 seconds but the actual acquisition took 15-20 minutes. The average scan times for ultrafast localizers, T1-weighted, T2-weighted, fluid-attenuated inversion recovery (FLAIR), diffusion-weighted, T2*-weighted sequences were 14, 41, 62, 96, 80, 6 seconds, respectively. Two blinded neuroradiologists independently assessed three aspects: (1) image quality, (2) gray-white matter (GM-WM) differentiation, and (3) diagnostic concordance for the detection of six clinically relevant imaging findings. Wilcoxon signed-rank test was used to compare image quality and GM-WM scores. Interobserver reproducibility was calculated. RESULTS: The ultrafast T1-weighted sequence demonstrated significantly better image quality (P = .005) and GM-WM differentiation (P < .001) compared to the conventional sequence. There was high agreement (>85%) between both protocols for the detection of mass-like lesion, hemorrhage, diffusion restriction, WM FLAIR hyperintensities, subarachnoid FLAIR hyperintensities, and hydrocephalus. CONCLUSIONS: The ultrafast protocol achieved at least comparable image quality and high diagnostic concordance compared to the conventional protocol. This fast protocol can be a viable option to replace the conventional protocol in motion-prone inpatient clinical settings.

Rosai-Dorfman disease of the central nervous system: report of 6 cases and review of the literature.

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is an uncommon benign idiopathic lymphoproliferative disorder. The histologic hallmark of RDD is the finding of emperipolesis displayed by lesional histiocytes. While RDD most commonly affects lymph nodes, extranodal involvement of multiple organs has been reported, including the central nervous system (CNS). However, CNS involvement in RDD is rare and is not well characterized. As a result, therapeutic approaches to CNS involvement in RDD are not well established. Herein we report 6 cases of RDD with isolated CNS involvement and review the literature on RDD with CNS involvement. One of the presented cases exhibited intramedullary involvement of the spinal cord--a very rare form of RDD with CNS involvement.

Prefemoral fat pad impingement syndrome: identification and diagnosis.

Fat pad impingement syndrome refers to anterior knee pain caused by hemorrhage, inflammation, fibrosis and/or degeneration of the anterior knee fat pads. Symptomatic impingement of the prefemoral fat pad can be clinically significant but easily overlooked on magnetic resonance imaging, unless looked for. It should be evaluated in patients with persistent anterior knee pain, particulary if accompanied with mechanical symptoms and lack of intra-articular pathology.

Operating room or angiography suite for hemodynamically unstable pelvic fractures?

BACKGROUND: Few patients require angiography and therapeutic embolization for bleeding pelvic fractures, but they are risk for significant morbidity and mortality. In hemodynamically unstable trauma patients with pelvic fractures, the decision to proceed to the operating room (OR) to address intraabdominal bleeding, or angiography to address pelvic bleeding (ANGIO), is rarely straightforward. This study tested the hypothesis that outcomes are similar regardless if the sequence was OR-ANGIO or ANGIO-OR. METHODS: All pelvic fractures between 1999 and 2011 were retrospectively reviewed and stratified by initial management with ANGIO or OR. RESULTS: Of 2,922 patients with pelvic fractures, only 183 (6%) required angiography for suspected bleeding. For OR-ANGIO (n = 49) versus ANGIO (n = 134), injury severity score was similar (40 ± 15 vs. 35 ± 16), but systolic blood pressure (97 ± 28 vs. 108 ± 32 mmHg, p = 0.038) and base excess were both lower (-9 ± 5 vs. -5 ± 5 mEq/L, p < 0.001). During initial resuscitation and in the first 24 hours, crystalloid, blood product usage and total fluid requirements were all increased 50% to 100% (all p < 0.001). Despite these differences, lengths of stay (32 ± 32 vs. 26 ± 28 days) and mortality (33% vs. 31%) were similar. The same trends in fluid requirements remained in the subset of patients with unstable pelvic fractures, with an increased mortality (67% vs. 20%, p = 0.011) in those requiring ANGIO-OR versus OR-ANGIO. CONCLUSION: These data support current management algorithms. In hemodynamically unstable trauma patients with pelvic fractures, those who proceeded immediately to the OR to address intraabdominal bleeding tended to be sicker but had outcomes that were the same or better compared with those who received angiography to address pelvic bleeding. LEVEL OF EVIDENCE: III, retrospective review.