Histopathological correlation of brain tumor recurrence vs. radiation effect post-radiosurgery as detected by MRI contrast clearance analysis: a validation study.

PURPOSE: The differentiation between adverse radiation effects (ARE) and tumor recurrence or progression (TRP) is a major decision-making point in the follow-up of patients with brain tumors. The advent of immunotherapy, targeted therapy and radiosurgery has made this distinction difficult to achieve in several clinical situations. Contrast clearance analysis (CCA) is a useful technique that can inform clinical decisions but has so far only been histologically validated in the context of high-grade gliomas. METHODS: This is a series of 7 patients, treated between 2018 and 2023, for various brain pathologies including brain metastasis, atypical meningioma, and high-grade glioma. MRI with contrast clearance analysis was used to inform clinical decisions and patients underwent surgical resection as indicated. The histopathology findings were compared with the CCA findings in all cases. RESULTS: All seven patients had been treated with gamma knife radiosurgery and were followed up with periodic MR imaging. All patients underwent CCA when the necessity to distinguish tumor recurrence from radiation necrosis arose, and subsequently underwent surgery as indicated. Concordance of CCA findings with histological findings was found in all cases (100%). CONCLUSIONS: Based on prior studies on GBM and the surgical findings in our series, delayed contrast extravasation MRI findings correlate well with histopathology across a wide spectrum of brain tumor pathologies. CCA can provide a quick diagnosis and have a direct impact on patients' treatment and outcomes.

A 47-Year-Old Woman with Confusion and Weakness.

A 47-Year-Old Woman with Confusion and WeaknessA 47-year-old woman with metastatic breast cancer presents with 3 months of confusion, memory loss, and lower-extremity weakness. How do you approach the evaluation, and what is the differential diagnosis?

Spatiotemporal assessment of immunogenomic heterogeneity in multiple myeloma.

Spatial heterogeneity is a common phenomenon in metastatic solid tumors and an evolving concept in multiple myeloma (MM). The interplay between malignant plasma cells (PCs) and the microenvironment has not yet been analyzed in MM. For this purpose, we performed bone marrow aspirates and imaging-guided biopsies of corresponding lesions in newly diagnosed MM (NDMM) and relapsed/refractory MM (RRMM) patients. PCs were isolated and subjected to whole-exome sequencing (WES). Non-PCs were studied with next-generation flow (NGF) and T-cell receptor sequencing (TCRseq) to analyze the connection between malignant and nonmalignant cells in the bone marrow and in lesions. Although we observed a strong overlap from WES, NGF, and TCRseq in patients with intramedullary disease, WES revealed significant spatial heterogeneity in patients with extramedullary disease. NGF showed significant immunosuppression in RRMM compared with NDMM as indicated by fewer myeloid dendritic cells, unswitched memory B cells, Th9 cells, and CD8 effector memory T cells but more natural killer and regulatory T cells. Additionally, fewer T-cell receptor (TCR) sequences were detected in RRMM compared with NDMM and healthy individuals. After induction therapy, TCR repertoire richness increased to levels of healthy individuals, and NGF showed more regulatory T cells and myeloid-derived suppressor cells, regardless of depth of response. Clinical significance of imaging-guided biopsies of lesions was demonstrated by detection of monoclonal PCs in patients without measurable residual disease (MRD) in aspirates from the iliac crest as well as identification of secondary primary malignancies in MRD- patients. Furthermore, site-specific clones with different drug susceptibilities and genetically defined high-risk features were detected by our workflow.

Transcranial Magnetic Stimulation for Long-Term Smoking Cessation: Preliminary Examination of Delay Discounting as a Therapeutic Target and the Effects of Intensity and Duration.

Background: Repetitive transcranial magnetic stimulation (rTMS) is a novel treatment for smoking cessation and delay discounting rate is novel therapeutic target. Research to determine optimal therapeutic targets and dosing parameters for long-term smoking cessation is needed. Due to potential biases and confounds introduced by the COVID-19 pandemic, we report preliminary results from an ongoing study among participants who reached study end prior to the pandemic. Methods: In a 3 × 2 randomized factorial design, participants (n = 23) received 900 pulses of 20 Hz rTMS to the left dorsolateral prefrontal cortex (PFC) in one of three Durations (8, 12, or 16 days of stimulation) and two Intensities (1 or 2 sessions per day). We examined direction and magnitude of the effect sizes on latency to relapse, 6-month point-prevalence abstinence rates, research burden, and delay discounting rates. Results: A large effect size was found for Duration and a medium for Intensity for latency to relapse. Increasing Duration increased the odds of abstinence 7-8-fold while increasing Intensity doubled the odds of abstinence. A large effect size was found for Duration, a small for Intensity for delay discounting rate. Increasing Duration and Intensity had a small effect on participant burden. Conclusion: Findings provide preliminary support for delay discounting as a therapeutic target and for increasing Duration and Intensity to achieve larger effect sizes for long-term smoking cessation and will provide a pre-pandemic comparison for data collected during the pandemic. Clinical Trial Registration: [www.ClinicalTrials.gov], identifier [NCT03865472].

Tumor-Avid 3-(1'-Hexyloxy)ethyl-3-devinylpyrpyropheophorbide-a (HPPH)-3Gd(III)tetraxetan (DOTA) Conjugate Defines Primary Tumors and Metastases.

3-(1'-Hexyloxyethyl)-3-devinylpyropheophorbide-a (HPPH or Photochlor), a tumor-avid chlorophyll a derivative currently undergoing human clinical trials, was conjugated with mono-, di-, and tri-Gd(III)tetraxetan (DOTA) moieties. The T1/T2 relaxivity and in vitro PDT efficacy of these conjugates were determined. The tumor specificity of the most promising conjugate was also investigated at various time points in mice and rats bearing colon tumors, as well as rabbits bearing widespread metastases from VX2 systemic arterial disseminated metastases. All the conjugates showed significant T1 and T2 relaxivities. However, the conjugate containing 3-Gd(III)-aminoethylamido-DOTA at position 17 of HPPH demonstrated great potential for tumor imaging by both MR and fluorescence while maintaining its PDT efficacy. At an MR imaging dose (10 µmol/kg), HPPH-3Gd(III)DOTA did not cause any significant organ toxicity in mice, indicating its potential as a cancer imaging (MR and fluorescence) agent with an option to treat cancer by photodynamic therapy (PDT).

Deciphering spatial genomic heterogeneity at a single cell resolution in multiple myeloma.

Osteolytic lesions (OL) characterize symptomatic multiple myeloma. The mechanisms of how malignant plasma cells (PC) cause OL in one region while others show no signs of bone destruction despite subtotal infiltration remain unknown. We report on a single-cell RNA sequencing (scRNA-seq) study of PC obtained prospectively from random bone marrow aspirates (BM) and paired imaging-guided biopsies of OL. We analyze 148,630 PC from 24 different locations in 10 patients and observe vast inter- and intra-patient heterogeneity based on scRNA-seq analyses. Beyond the limited evidence for spatial heterogeneity from whole-exome sequencing, we find an additional layer of complexity by integrated analysis of anchored scRNA-seq datasets from the BM and OL. PC from OL are characterized by differentially expressed genes compared to PC from BM, including upregulation of genes associated with myeloma bone disease like DKK1, HGF and TIMP-1 as well as recurrent downregulation of JUN/FOS, DUSP1 and HBB. Assessment of PC from longitudinally collected samples reveals transcriptional changes after induction therapy. Our study contributes to the understanding of destructive myeloma bone disease.

Increasing Physical Activity among Breast Cancer Survivors by Modulating Temporal Orientation with rTMS: Feasibility and Potential Efficacy.

Maintaining adequate amounts of physical activity is a critical component of survivorship care for women with breast cancer. Increased physical activity is associated with increases in well-being, quality of life, and longevity, but women with cancer face unique, cancer-related factors that might affect physical activity. Consistent with the Competing Neurobehavioral Decision Systems model of decision making, we proposed to decrease delay discounting and increase physical activity by stimulating the executive function system via high-frequency repetitive transcranial magnetic stimulation (HF rTMS) of the left dorsolateral prefrontal cortex (LDLPFC). This randomized, sham-controlled, double-blinded trial examined the feasibility and potential efficacy of this approach to increase physical activity in breast cancer survivors. We hypothesized that active rTMS would significantly increase the mean number of steps per day and decrease delay discounting. Participants (n = 30) were primarily middle-aged (M = 53.7, SD = 7.9) and white with a mean BMI and body mass indices below 40. Indicators of feasibility and limited efficacy testing were positive. Although repeated-measures ANOVA revealed no significant changes in delay discounting, generalized estimating equations (GEE) found that participants in the active condition increased their mean daily steps by 400 steps per day, while those in the sham condition decreased this by nearly 600 steps per day. These findings indicate that the continued investigation of HF rTMS for increasing physical activity among women with breast cancer is justified.

Dosing parameters for the effects of high-frequency transcranial magnetic stimulation on smoking cessation: study protocol for a randomized factorial sham-controlled clinical trial.

BACKGROUND: Despite the considerable success of comprehensive tobacco control efforts, tobacco use remains one of the greatest preventable causes of death and disease today. Over half of all smokers in the US make quit attempts every year, but over 90% relapse within 12 months, choosing the immediate reinforcement of smoking over the long-term benefits of quitting. Conceptual and empirical evidence supports continued investigation of high frequency repetitive transcranial magnetic stimulation (rTMS) of the left dorsolateral prefrontal cortex in reducing relapse and decreasing cigarette consumption. While this evidence is compelling, an optimal dosing strategy must be determined before a long-term efficacy trial can be conducted. The goal of this study is to determine a dosing strategy for 20 Hz rTMS that will produce the best long-term abstinence outcomes with the fewest undesirable effects. METHODS: This is a fully crossed, double-blinded, sham-controlled, 3x2x2 randomized factorial study. The three factors are duration (stimulation days: 8, 12, and 16); intensity (900 or 1800 pulses per day); and sham control. Participants (n = 258) will consist of adults (18-65) who are motivated to quit smoking cigarettes and who will be followed for 6 months post-quit. Outcomes include latency to relapse, point prevalence abstinence rates, delay discounting rates, cognitive-behavioral skills acquisition, and multiple measures of potential undesirable effects that impact participant compliance. DISCUSSION: This study integrates existing theoretical concepts and methodologies from neuropsychology, behavioral economics, brain stimulation, clinical psychology, and the evidence-based treatment of tobacco dependence in the development of a promising and innovative approach to treat tobacco dependence. This study will establish an optimal dosing regimen for efficacy testing. Findings are expected to have a significant influence on advancing this approach as well as informing future research on clinical approaches that combine rTMS with other evidence-based treatments for tobacco dependence and perhaps other addictions. TRIAL REGISTRATION: Clinical Trials NCT03865472 (retrospectively registered). The first participant was fully enrolled on November 26, 2018. Registration was posted on March 7, 2019.

Pediatric neuroimaging.

This article provides clinical neurologists with an overview of pediatric neuroimaging. Pediatric neuroimaging is a broad subject, and its details are beyond the scope of any short review article. First this article briefly highlights different stages of brain development and explains how these stages correlate with various congenital brain anomalies. It then focuses on the safety of pediatric neuroimaging, discussing important issues in pediatric sedation and hazards of exposure of ionizing radiation. Last, it describes the advent of modern neuroimaging tools, such as diffusion tensor imaging and MR spectroscopy, and their emerging role in evaluating multiple pediatric brain disorders.

Radiofrequency Ablation and Radiation Therapy Improve Local Control in Spinal Metastases Compared to Radiofrequency Ablation Alone.

PURPOSE:: The spinal column is the most common location for osseous metastases and is associated with pain and decreased quality of life. This study evaluated combined radiofrequency ablation (RFA) with radiation therapy (RT) compared to RFA alone for improving pain and local control. METHODS:: This was a single-institution retrospective review of patients who underwent RFA of spinal metastases between 2016 and 2017, with or without RT to the same vertebral level. Pain was measured with visual analog scale at initial presentation and at 3 and 12 weeks of follow-up. Local failure (LF), distant failure, and overall survival (OS) were compared and Kaplan-Meier statistics were calculated. RESULTS:: Twenty-six patients with 28 spinal metastases were treated with RFA. Ten patients with 11 metastases were treated with RFA + RT. More patients with lung primaries were treated with RFA alone and more patients with breast primaries were treated with combination RFA+RT. There was no significant difference in pain scores between groups ( P = .96). At a median follow-up of 8.2 months, LF was noted in 8 of 17 metastases treated with RFA alone compared to 1 of 11 metastases treated with RFA+RT ( P = .049). There was a significant benefit in time to LF favoring RFA+RT ( P = .02) and a significant benefit in OS ( P = .0045). CONCLUSION:: This study demonstrates a benefit in local control with RFA+RT versus RFA alone. Palliation of pain was effective using both regimens. This study was limited by a nearly unequal distribution of primary tumor histologies between groups. Literature regarding combined treatment of RFA and RT for spinal metastases is scarce and prospective protocols are warranted.

The role of cement augmentation with percutaneous vertebroplasty and balloon kyphoplasty for the treatment of vertebral compression fractures in multiple myeloma: a consensus statement from the International Myeloma Working Group (IMWG).

Multiple myeloma (MM) represents approximately 15% of haematological malignancies and most of the patients present with bone involvement. Focal or diffuse spinal osteolysis may result in significant morbidity by causing painful progressive vertebral compression fractures (VCFs) and deformities. Advances in the systemic treatment of myeloma have achieved high response rates and prolonged the survival significantly. Early diagnosis and management of skeletal events contribute to improving the prognosis and quality of life of MM patients. The management of patients with significant pain due to VCFs in the acute phase is not standardised. While some patients are successfully treated conservatively, and pain relief is achieved within a few weeks, a large percentage has disabling pain and morbidity and hence they are considered for surgical intervention. Balloon kyphoplasty and percutaneous vertebroplasty are minimally invasive procedures which have been shown to relieve pain and restore function. Despite increasing positive evidence for the use of these procedures, the indications, timing, efficacy, safety and their role in the treatment algorithm of myeloma spinal disease are yet to be elucidated. This paper reports an update of the consensus statement from the International Myeloma Working Group on the role of cement augmentation in myeloma patients with VCFs.

Stereotactic gamma knife surgery for trigeminal neuralgia: detailed analysis of treatment response.

OBJECT: The purpose of this study was to assess the durability and completeness of pain relief in patients treated using stereotactic gamma knife surgery (GKS) for trigeminal neuralgia (TN). METHODS: Thirty-eight patients with refractory TN were treated with stereotactic GKS. All patients received a prescription radiation dose of 35, 40, or 45 Gy to the 50% isodose surface through a 4-mm collimator helmet. The group was assessed regularly based on physician-directed interviews for a median follow up of 24 months (range 6-27 months). Pain relief was classified as excellent (no pain without medication), good (well-controlled pain with continued medication), fair (decreased but residual pain with continued medication), or poor (unimproved or increased pain with the same or increased medication). Three months after treatment, pain relief was good or excellent in 71% of patients. By 24 months post-GKS, 50% of the original cohort had poor pain relief, 21% continued to have either excellent or good relief, 3% had fair relief, and 26% had not reached the 24-month follow up. Based on their status at the last follow up, 29% of patients had excellent and 16% had good pain relief. Thirty-seven percent experienced facial numbness, which was dose related. In addition, there was a significantly higher rate of complete pain relief in patients who had facial numbness following treatment (p = 0.003). CONCLUSIONS: Stereotactic GKS is an effective treatment in patients with TN; however, the durability of pain relief and the time to treatment response are limiting factors. As with other types of ablative treatment, facial numbness is strongly associated with better treatment response.

Gadolinium-enhanced computed tomographic angiography: current status.

This article reviews the research to date, as well as our clinical experience from two institutions, on gadolinium-enhanced computed tomographic angiography (gCTA) for imaging the body. gCTA may be an appropriate examination for the small percentage of patients who would benefit from noninvasive vascular imaging, but who have contraindications to both iodinated contrast and magnetic resonance imaging. gCTA is more expensive than CTA with iodinated contrast, due to the dose of gadolinium administered, and gCTA has limitations compared with CTA with iodinated contrast, in that parenchymal organs are not optimally enhanced at doses of 0.5 mmol/kg or lower. However, in our experience, gCTA has been a very useful problem-solving examination in carefully selected patients. With the advent of 16-64 detector CT, in combination with bolus tracking, we believe that the overall dose of gadolinium needed for diagnostic CTA examinations, while relatively high, can be safely administered.

In vivo model of intracranial stent implantation: a pilot study to examine the histological response of cerebral vessels after randomized implantation of heparin-coated and uncoated endoluminal stents in a blinded fashion.

OBJECT: No animal model currently exists for the examination of time-dependent histological changes occurring in intracranial vessels after endoluminal stent placement. The authors' goal was to develop a reproducible in vivo model of stent implantation in intracranial vessels in dogs that was capable of demonstrating stent-related vascular changes after the implantation of coated and uncoated devices. METHODS: The authors implanted heparin-coated or uncoated stents in the basilar arteries (BAs) of 11 mongrel dogs. In a 12th animal, one coated stent was implanted in the BA and a second uncoated one was implanted in the distalanterior spinal artery. All the devices were oversized to induce intimal injury. Surviving animals were observed for 12 weeks, after which they underwent repeated angiography before planned death and removal of the brain. Histological studies and computer-assisted morphometric analyses were conducted on stent-treated and untreated sections of the BAs to assess the percentage of stenosis, neointimal proliferation, vessel injury, and inflammation. Perforating vessels partially covered by stent struts ("jailing") were studied for evidence of stenosis or occlusion. The pathologist, interventionists, histopathologist, histopathology technicians, and radiologist were blinded to the stent type. Seven stents (three uncoated and four coated) were removed from the six animals that were observed during the follow-up period. The mean neointimal proliferation was 0.42 mm2 in the group treated with uncoated stents and 0.18 mm2 in the group treated with heparin-coated devices (p = 0.04). Neointimal thickness was significantly increased in the group with uncoated stents (p = 0.04). The mean percentage of occlusion was less (12%) in the group with heparin-coated stents, compared with 22% in the group with uncoated devices (p = 0.07). When comparing results between the heparin-coated and uncoated devices implanted in the five animals that received a single stent only, greater differences (indicating a benefit from heparin-coated stents) were observed in neointimal area (p = 0.009), neointima/media ratio (p = 0.001), neointimal thickness (p = 0.002), and percentage of occlusion (p = 0.009). All brainstem perforating vessels covered by stent struts remained patent. CONCLUSIONS: This in vivo intracranial stent model was developed to assess proliferative and inflammatory responses to endoluminal stent implantation in the cerebrovasculature. The results indicate that a lower percentage of occlusion occurs 12 weeks after implantation of heparin-coated compared with uncoated stents.

Paradoxical brain embolism from thrombus associated with vena caval filter in a patient with cancer.

A 71-year-old man experienced sudden onset of hemiparesis and aphasia. He had a 4-month history of gallbladder cholangiocarcinoma, complicated with a postoperative deep-vein thrombosis (DVT) that necessitated a vena caval filter placement. Diffusion-weighted magnetic resonance imaging of the brain showed multiple hyperintense foci. Magnetic resonance spectroscopy was compatible with cerebral infarction. Abdominal computed tomography showed a thrombus in the inferior vena cava extending through the filters. A transcranial Doppler bubble study revealed the presence of a right-to-left shunt. Paradoxical cerebral embolism must be considered in patients with DVT who have new onset neurologic deficits even in the presence of a caval filter.

Bronchogenic cyst presenting as a symptomatic neck mass in an adult: case report and review of the literature.

We report the unusual clinical manifestation and subsequent management of a symptomatic congenital bronchogenic cyst that connected to the trachea and presented in the neck of an adult. The embryology, clinical presentation, diagnostic evaluation, and management options of this rare aberration are discussed.

Imaging in head and neck oncology.

Evaluation of head and neck cancer with imaging is a topic that is far more extensive than can be covered in this article. The main reason for head and neck imaging is to evaluate the true extent of disease to best determine surgical and therapeutic options. This process includes evaluation of the size, location, and extent of tumor infiltration into surrounding vascular and visceral structures. Important anatomic variants must be pointed out so the surgeon can avoid potential intraoperative complications. These variant scan be evaluated with the appropriate multiplanar and three-dimensional images to provide as much information as possible to the surgeon preoperatively. Second, nodal staging should be assessed in an effort to increase the number of abnormal nodes detected by physical examination and, more important, to precisely define their location by a standard classification system that can be understood and consistently applied by the radiologist, surgeon, radiation oncologist, and pathologist. Although secondary to the previously described tasks, imaging frequently enables a limitation of the diagnostic and histologic possibilities based on lesion location and signal-attenuation characteristics, which may lead the clinical investigation along a different path. saving the patient unnecessary risk and shortening the time to diagnosis and ultimate treatment. This article has attempted to detail the current state of the controversy between CT, MRI, and other modalities, and has emphasized the constant evolution of this controversy because of the evolving imaging technology. Although CT and MRI are both well suited to evaluation of the deep spaces and submucosal spaces of the head and neck, each has some limitations.MRI has the advantages of higher soft tissue contrast resolution, the lack of iodine-based contrast agents, and high sensitivity for perineural and intracranial disease. The disadvantages of MRI include lower patient tolerance, contraindications in pacemakers and certain other implanted metallic devices, and artifacts related to multiple causes, not the least of which is motion. CT is fast, well tolerated, and readily available but has lower contrast resolution and requires iodinated contrast and ionizing radiation. The current authors' practice is heavily centered on CT for initial evaluation, preoperative planning, biopsy targeting, and postoperative follow-up. They reserve MRI for tumors that are suspicious for perineural,cartilaginous, or bony invasion on CT, or for tumors such as adenoid cystic carcinoma that are highly likely to spread by way of these routes. For patients who have head and neck cancer, a radiologist who is educated in the treatment options, patterns of tumor growth, and important surgical landmarks, and who has a well-established pattern of communication with the head and neck clinical services, including surgery, radiation oncology,and pathology, is key in providing accurate and useful image interpretation.

Laser-interstitial thermal therapy for refractory cerebral edema from post-radiosurgery metastasis.

BACKGROUND: Stereotactic radiosurgery is often an effective tool for the treatment of brain metastases. A complication of radiosurgical treatment for brain metastasis can be persistent cerebral edema. Treatments of this refractory cerebral edema include observation, corticosteroids, and surgical resection of the edema-inducing mass. Laser-interstitial thermal therapy is a minimally invasive technique for ablating intracranial lesions. It may provide a treatment option for metastases after radiosurgery causing refractory cerebral edema. CASE DESCRIPTION: We report the case of a 64-year-old man with lung adenocarcinoma presenting to our department with left hemiparesis. Brain magnetic resonance imaging showed an 18-mm enhancing lesion in the right external capsule with significant surrounding edema. The lesion was treated by radiosurgery. There was persistent edema after radiosurgery. The patient required continued corticosteroid therapy to maintain his ability to ambulate. He developed refractory hyperglycemia, weight gain, and bilateral proximal muscle weakness secondary to this therapy. Fourteen weeks after radiosurgery, he underwent laser-interstitial thermal therapy for lesion ablation. He was weaned off corticosteroids during 2 weeks and maintained his strength during the following month. CONCLUSIONS: Laser-interstitial thermal therapy may be a treatment option for refractory cerebral edema after stereotactic radiosurgery to a metastasis. This therapy may be of particular use in deep-seated lesions refractory to corticosteroid therapy.

Patterns of magnetic resonance imaging abnormalities in symptomatic patients with Krabbe disease correspond to phenotype.

BACKGROUND: Initial magnetic resonance imaging studies of individuals with Krabbe disease were analyzed to determine whether the pattern of abnormalities corresponded to the phenotype. METHODS: This was a retrospective, nonblinded study. Families/patients diagnosed with Krabbe disease submitted medical records and magnetic resonance imaging discs for central review. Institutional review board approval/informed consents were obtained. Sixty-four magnetic resonance imaging scans were reviewed by two neuroradiologists and a child neurologist according to phenotype: early infantile (onset 0-6 months) = 39 patients; late infantile (onset 7-12 months) = 10 patients; later onset (onset 13 months-10 years) = 11 patients; adolescent (onset 11-20 years) = one patient; and adult (21 years or greater) = three patients. Local interpretations were compared with central review. RESULTS: Magnetic resonance imaging abnormalities differed among phenotypes. Early infantile patients had a predominance of increased intensity in the dentate/cerebellar white matter as well as changes in the deep cerebral white matter. Later onset patients did not demonstrate involvement in the dentate/cerebellar white matter but had extensive involvement of the deep cerebral white matter, parieto-occipital region, and posterior corpus callosum. Late infantile patients exhibited a mixed pattern; 40% had dentate/cerebellar white matter involvement while all had involvement of the deep cerebral white matter. Adolescent/adult patients demonstrated isolated corticospinal tract involvement. Local and central reviews primarily differed in interpretation of the early infantile phenotype. CONCLUSION: Analysis of magnetic resonance imaging in a large cohort of symptomatic patients with Krabbe disease demonstrated imaging abnormalities correspond to specific phenotypes. Knowledge of these patterns along with typical clinical signs/symptoms should promote earlier diagnosis and facilitate treatment.