High-Grade Astrocytoma with Piloid Features: A Dual Institutional Review of Imaging Findings of a Novel Entity.

High-grade astrocytoma with piloid features (HGAP) is a recently identified brain tumor characterized by a distinct DNA methylation profile. Predominantly located in the posterior fossa of adults, HGAP is notably prevalent in individuals with neurofibromatosis type 1. We present an image-centric review of HGAP and explore the association between HGAP and neurofibromatosis type 1. Data were collected from 8 HGAP patients treated at two tertiary care institutions between January 2020 and October 2023. Demographic details, clinical records, management, and tumor molecular profiles were analyzed. Tumor characteristics, including location and imaging features on MR imaging, were reviewed. Clinical or imaging features suggestive of neurofibromatosis 1 or the presence of NF1 gene alteration were documented. The mean age at presentation was 45.5 years (male/female = 5:3). Tumors were midline, localized in the posterior fossa (n = 4), diencephalic/thalamic (n = 2), and spinal cord (n = 2). HGAP lesions were T1 hypointense, T2-hyperintense, mostly without diffusion restriction, predominantly peripheral irregular enhancement with central necrosis (n = 3) followed by mixed heterogeneous enhancement (n = 2). Two NF1 mutation carriers showed signs of neurofibromatosis type 1 before HGAP diagnosis, with one diagnosed during HGAP evaluation, strengthening the HGAP-NF1 link, particularly in patients with posterior fossa masses. All tumors were IDH1 wild-type, often with ATRX, CDKN2A/B, and NF1 gene alteration. Six patients underwent surgical resection followed by adjuvant chemoradiation. Six patients were alive, and two died during the last follow-up. Histone H3 mutations were not detected in our cohort, such as the common H3K27M typically seen in diffuse midline gliomas, linked to aggressive clinical behavior and poor prognosis. HGAP lesions may involve the brain or spine and tend to be midline or paramedian in location. Underlying neurofibromatosis type 1 diagnosis or imaging findings are important diagnostic cues.

Evaluation of MR Elastography as a Noninvasive Diagnostic Test for Spontaneous Intracranial Hypotension.

BACKGROUND AND PURPOSE: Spontaneous intracranial hypotension is a condition resulting from a leak of CSF from the spinal canal arising independent of a medical procedure. Spontaneous intracranial hypotension can present with normal brain MR imaging findings and nonspecific symptoms, leading to the underdiagnosis in some patients and unnecessary invasive myelography in others who are found not to have the condition. Given the likelihood that spontaneous intracranial hypotension alters intracranial biomechanics, the goal of this study was to evaluate MR elastography as a potential noninvasive test to diagnose the condition. MATERIALS AND METHODS: We performed MR elastography in 15 patients with confirmed spontaneous intracranial hypotension from September 2022 to April 2023. Age, sex, symptom duration, and brain MR imaging Bern score were collected. MR elastography data were used to compute stiffness and damping ratio maps, and voxelwise modeling was performed to detect clusters of significant differences in mechanical properties between patients with spontaneous intracranial hypotension and healthy control participants. To evaluate diagnostic accuracy, we summarized each examination by 2 spatial pattern scores (one each for stiffness and damping ratio) and evaluated group-wise discrimination by receiver operating characteristic curve analysis. RESULTS: Patients with spontaneous intracranial hypotension exhibited significant differences in both stiffness and damping ratio (false discovery rate-corrected, Q < 0.05). Pattern analysis discriminated patients with spontaneous intracranial hypotension from healthy controls with an area under the curve of 0.97 overall, and the area under the curve was 0.97 in those without MR imaging findings of spontaneous intracranial hypotension. CONCLUSIONS: Results from this pilot study demonstrate MR elastography as a potential imaging biomarker and a noninvasive method for diagnosing spontaneous intracranial hypotension, including patients with normal brain MR imaging findings.

Radiologic Classification of Hippocampal Sclerosis in Epilepsy.

Temporal lobe epilepsy is a common form of epilepsy that is often associated with hippocampal sclerosis (HS). Although HS is commonly considered a binary assessment in radiological evaluation, it is known that histopathological changes occur in distinct clusters. Some subtypes of HS only affect certain subfields, resulting in minimal changes to the overall volume of the hippocampus. This is likely a major reason why whole hippocampal volumetrics have underperformed versus expert readers. With recent advancements in MRI technology, it is now possible to characterize the substructure of the hippocampus more accurately. However, this is not consistently addressed in radiographic evaluations. The histological subtype of HS is critical for prognosis and treatment decision making, necessitating improved radiological classification of HS. The International League Against Epilepsy (ILAE) has issued a consensus classification scheme for subtyping HS histopathological changes. This review aims to explore how the ILAE subtypes of HS correlate with radiographic findings, introduce a grading system that integrates radiological and pathological reporting in HS, and outline an approach to detecting HS subtypes using MRI. This framework will not only benefit current clinical evaluations, but also enhance future studies involving high-resolution MRI in temporal lobe epilepsy.ABBREVIATIONS: CA = cornu ammonis; DG = dentate gyrus; HS = hippocampal sclerosis; ILAE = International League Against Epilepsy; SRLM = strata radiatum, lacunosum, and moleculare layers; TLE = temporal lobe epilepsy.

Magnetic Resonance Imaging Artifact Associated With Transcutaneous Bone Conduction Implants: Cholesteatoma and Vestibular Schwannoma Surveillance.

OBJECTIVE: To evaluate the magnetic resonance (MR) image artifact and image distortion associated with the two transcutaneous bone conduction implants currently available in the United States. STUDY DESIGN: Cadaveric study. METHODS: Two cadaveric head specimens (1 male, 1 female) were unilaterally implanted according to manufacturer guidelines and underwent MR imaging (General Electric and Siemens 1.5 T scanners) under the following device conditions: (1) no device, (2) Cochlear Osia with magnet and headwrap, (3) Cochlear Osia without magnet, and (4) MED-EL Bonebridge with magnet. Maximum metal mitigation techniques were employed in all conditions, and identical sequences were obtained. Blinded image scoring (diagnostic vs nondiagnostic image) was performed by experienced neuroradiologists according to anatomical subsites. RESULTS: All device conditions produced artifact and image distortion. The Osia with magnet produced diagnostic T1- and T2-weighted images of the ipsilateral temporal bone, however, non-echo planar imaging diffusion-weighted imaging (DWI) was nondiagnostic. The Osia without magnet scanned on the Siemens MR imaging demonstrated the least amount of artifact and was the only condition that allowed for diagnostic imaging of the ipsilateral temporal bone on DWI. The Bonebridge produced a large area of artifact and distortion with the involvement of the ipsilateral and contralateral temporal bones. CONCLUSION: In summary, of the three device conditions (Osia with magnet, Osia without magnet, and Bonebridge), Osia without magnet offered the least amount of artifact and distortion and was the only condition in which diagnostic DWI was available for the middle ear and mastoid regions on the Siemens MR imaging scanner.

Direct pseudomeningocele contrast injection for spinal CSF leak localization.

CT myelography has been traditionally used to evaluate post-operative paraspinal fluid collections to discern CSF leaking into a pseudomeningocele versus a contained seroma. Rather than performing a lumbar puncture and injecting intrathecal contrast for myelography, we present the first report of direct contrast injection into a post-operative paraspinal pseudomeningocele for CSF leak confirmation and localization. This is a simple procedure that has several advantages over a conventional CT myelogram for the evaluation of post-operative paraspinal fluid collections.

Detection and Mitigation of Neurovascular Uncoupling in Brain Gliomas.

Functional magnetic resonance imaging (fMRI) with blood oxygen level-dependent (BOLD) technique is useful for preoperative mapping of brain functional networks in tumor patients, providing reliable in vivo detection of eloquent cortex to help reduce the risk of postsurgical morbidity. BOLD task-based fMRI (tb-fMRI) is the most often used noninvasive method that can reliably map cortical networks, including those associated with sensorimotor, language, and visual functions. BOLD resting-state fMRI (rs-fMRI) is emerging as a promising ancillary tool for visualization of diverse functional networks. Although fMRI is a powerful tool that can be used as an adjunct for brain tumor surgery planning, it has some constraints that should be taken into consideration for proper clinical interpretation. BOLD fMRI interpretation may be limited by neurovascular uncoupling (NVU) induced by brain tumors. Cerebrovascular reactivity (CVR) mapping obtained using breath-hold methods is an effective method for evaluating NVU potential.

Impact of e-ASPECTS software on the performance of physicians compared to a consensus ground truth: a multi-reader, multi-case study.

Background: The Alberta Stroke Program Early CT Score (ASPECTS) is used to quantify the extent of injury to the brain following acute ischemic stroke (AIS) and to inform treatment decisions. The e-ASPECTS software uses artificial intelligence methods to automatically process non-contrast CT (NCCT) brain scans from patients with AIS affecting the middle cerebral artery (MCA) territory and generate an ASPECTS. This study aimed to evaluate the impact of e-ASPECTS (Brainomix, Oxford, UK) on the performance of US physicians compared to a consensus ground truth. Methods: The study used a multi-reader, multi-case design. A total of 10 US board-certified physicians (neurologists and neuroradiologists) scored 54 NCCT brain scans of patients with AIS affecting the MCA territory. Each reader scored each scan on two occasions: once with and once without reference to the e-ASPECTS software, in random order. Agreement with a reference standard (expert consensus read with reference to follow-up imaging) was evaluated with and without software support. Results: A comparison of the area under the curve (AUC) for each reader showed a significant improvement from 0.81 to 0.83 (p = 0.028) with the support of the e-ASPECTS tool. The agreement of reader ASPECTS scoring with the reference standard was improved with e-ASPECTS compared to unassisted reading of scans: Cohen's kappa improved from 0.60 to 0.65, and the case-based weighted Kappa improved from 0.70 to 0.81. Conclusion: Decision support with the e-ASPECTS software significantly improves the accuracy of ASPECTS scoring, even by expert US neurologists and neuroradiologists.

Anti-LGI1 Autoimmune Epilepsy.

ABSTRACT: Leucine-rich glioma inactivated 1 autoimmune encephalitis is a treatable cause of autoimmune epilepsy associated with faciobrachial dystonic seizures-a rare form of epilepsy with frequent brief seizures primarily affecting the arm and face. We report a case with characteristic imaging findings. 18 F-FDG PET/CT demonstrated severe hypometabolism in the left basal ganglia, a regional abnormality associated with leucine-rich glioma inactivated 1 encephalitis.

Automated sleep classification with chronic neural implants in freely behaving canines.

Objective.Long-term intracranial electroencephalography (iEEG) in freely behaving animals provides valuable electrophysiological information and when correlated with animal behavior is useful for investigating brain function.Approach.Here we develop and validate an automated iEEG-based sleep-wake classifier for canines using expert sleep labels derived from simultaneous video, accelerometry, scalp electroencephalography (EEG) and iEEG monitoring. The video, scalp EEG, and accelerometry recordings were manually scored by a board-certified sleep expert into sleep-wake state categories: awake, rapid-eye-movement (REM) sleep, and three non-REM sleep categories (NREM1, 2, 3). The expert labels were used to train, validate, and test a fully automated iEEG sleep-wake classifier in freely behaving canines.Main results. The iEEG-based classifier achieved an overall classification accuracy of 0.878 ± 0.055 and a Cohen's Kappa score of 0.786 ± 0.090. Subsequently, we used the automated iEEG-based classifier to investigate sleep over multiple weeks in freely behaving canines. The results show that the dogs spend a significant amount of the day sleeping, but the characteristics of daytime nap sleep differ from night-time sleep in three key characteristics: during the day, there are fewer NREM sleep cycles (10.81 ± 2.34 cycles per day vs. 22.39 ± 3.88 cycles per night;p< 0.001), shorter NREM cycle durations (13.83 ± 8.50 min per day vs. 15.09 ± 8.55 min per night;p< 0.001), and dogs spend a greater proportion of sleep time in NREM sleep and less time in REM sleep compared to night-time sleep (NREM 0.88 ± 0.09, REM 0.12 ± 0.09 per day vs. NREM 0.80 ± 0.08, REM 0.20 ± 0.08 per night;p< 0.001).Significance.These results support the feasibility and accuracy of automated iEEG sleep-wake classifiers for canine behavior investigations.

PET/MRI Applications in Pediatric Epilepsy.

Epilepsy neuroimaging assessment requires exceptional anatomic detail, physiologic and metabolic information. Magnetic resonance (MR) protocols are often time-consuming necessitating sedation and positron emission tomography (PET)/computed tomography (CT) comes with a significant radiation dose. Hybrid PET/MRI protocols allow for exquisite assessment of brain anatomy and structural abnormalities, in addition to metabolic information in a single, convenient imaging session, which limits radiation dose, sedation time, and sedation events. Brain PET/MRI has proven especially useful for accurate localization of epileptogenic zones in pediatric seizure cases, providing critical additional information and guiding surgical decision making in medically refractory cases. Accurate localization of seizure focus is necessary to limit the extent of the surgical resection, preserve healthy brain tissue, and achieve seizure control. This review provides a systematic overview with illustrative examples demonstrating the applications and diagnostic utility of PET/MRI in pediatric epilepsy.

Field strength difference in extent of artifacts induced by CERTAS Plus valves in patients with idiopathic normal pressure hydrocephalus.

BACKGROUND AND PURPOSE: : Post-shunt MRI is usually performed at 1.5T under the general assumption that shunt-related susceptibility artifacts would be greater at higher field strengths. PURPOSE: The purpose is to show that imaging post-shunt idiopathic normal pressure hydrocephalus (iNPH) patients at 3T is feasible and with reduced artifacts as compared to 1.5T. METHODS: We manually measured transverse dimensions of artifact at the levels of lateral ventricles, cerebral aqueduct, and cerebellar hemisphere. Areas/volumes of artifacts were calculated assuming an elliptic/ellipsoid shape. Relative extent of shunt-related artifact between field strengths was rated by 3 readers on a 5-point Likert scale. A Wilcoxon Signed Rank Test was used to compare artifact at 1.5T vs 3T for each sequence, with a significance level set at p < 0.05. RESULTS: Artifact areas were calculated in 22 iNPH patients; artifacts were on average smaller at 3T vs 1.5T on MPRAGE, DWI, and GRE sequences. On T2 FLAIR and T2 FSE, artifacts at 3T were larger than 1.5T. On the qualitative analysis, artifact effects were less at 3T vs 1.5T on DWI, greater at 3T on T2 FSE, and had mixed results on GRE. CONCLUSION: Our results indicate feasibility of post-shunt imaging with the CERTAS Plus valve at 3T based on shunt-related artifact that is less than or equal in extent to that on 1.5T on most standard clinical imaging sequences. Our findings, corroborated by the qualitative image review, suggest that dedicated clinical imaging sequences for devices may allow for reduction in artifact extent at both 1.5T and 3T.

Post-COVID-19 syndrome: persistent neuroimaging changes and symptoms 9 months after initial infection.

A previously healthy and active middle-aged woman acquired COVID-19 as an occupational exposure with subsequent persistent post-COVID-19 symptoms including headache, dyspnoea on exertion, chest pressure, tachycardia, anosmia, parosmia, persistent myalgia, vertigo, cognitive decline and fatigue. She presented to a tertiary medical centre for further evaluation after 9 months of persistent symptoms and had a largely unremarkable workup with the exception of a persistently elevated monocyte chemoattractant protein 1, blunted cardiovagal response and non-specific scattered areas of low-level hypometabolism at the bilateral frontal, left precuneus, occipital and parietal regions on PET scan.

Brain structural differences in temporal lobe and frontal lobe epilepsy patients: A voxel-based morphometry and vertex-based surface analysis.

PURPOSE: Exploration of the effect of chronic recurrent seizures in focal epilepsy on brain volumes has produced many conflicting reports. To determine differences in brain structure in temporal lobe epilepsy (TLE) and extratemporal epilepsy (using frontal lobe epilepsy (FLE) a surrogate) further, we performed a retrospective analysis of a large cohort of patients with seizure-onset zone proven by intracranial monitoring. METHODS: A total of 120 TLE patients, 86 FLE patients, and 54 healthy controls were enrolled in this study. An analysis of variance of voxel-based morphometry (VBM) was used to seek morphometric brain differences among TLE patients, FLE patients, and healthy controls. Additionally, a vertex-based surface analysis was utilized to analyze the hippocampus and thalamus. Significant side-specific differences in hippocampal gray matter volume were present between the left TLE (LTLE), right TLE RTLE (RTLE), and control groups (p<0.05, family-wise error (FWE) corrected). RESULTS: Vertex analyses revealed significant volume reduction in inferior parts of the left hippocampus in the LTLE group and lateral parts of the right hippocampus in the RTLE group compared to controls (p<0.05, FWE corrected). Significant differences were also detected between the LTLE and control group in the bilateral medial and inferior thalamus (p<0.05, FWE corrected). FLE patients did not exhibit focal atrophy of gray matter across the brain. CONCLUSION: Our results highlight the variation in morphometric lateralized changes in the brain between different epilepsy onset zones, providing critical insight into the natural history of people with drug-resistant focal epilepsies.

Anterior nucleus of the thalamus seizure detection in ambulatory humans.

There is a paucity of data to guide anterior nucleus of the thalamus (ANT) deep brain stimulation (DBS) with brain sensing. The clinical Medtronic Percept DBS device provides constrained brain sensing power within a frequency band (power-in-band [PIB]), recorded in 10-min averaged increments. Here, four patients with temporal lobe epilepsy were implanted with an investigational device providing full bandwidth chronic intracranial electroencephalogram (cEEG) from bilateral ANT and hippocampus (Hc). ANT PIB-based seizure detection was assessed. Detection parameters were cEEG PIB center frequency, bandwidth, and epoch duration. Performance was evaluated against epileptologist-confirmed Hc seizures, and assessed by area under the precision-recall curve (PR-AUC). Data included 99 days of cEEG, and 20, 278, 3, and 18 Hc seizures for Subjects 1-4. The best detector had 7-Hz center frequency, 5-Hz band width, and 10-s epoch duration (group PR-AUC = .90), with 75% sensitivity and .38 false alarms per day for Subject 1, and 100% and .0 for Subjects 3 and 4. Hc seizures in Subject 2 did not propagate to ANT. The relative change of ANT PIB was maximal ipsilateral to seizure onset for all detected seizures. Chronic ANT and Hc recordings provide direct guidance for ANT DBS with brain sensing.

Comparison of MRI Lesion Evolution in Different Central Nervous System Demyelinating Disorders.

BACKGROUND AND OBJECTIVE: There are few studies comparing lesion evolution across different CNS demyelinating diseases, yet knowledge of this may be important for diagnosis and understanding differences in disease pathogenesis. We sought to compare MRI T2 lesion evolution in myelin oligodendrocyte glycoprotein immunoglobulin G (IgG)-associated disorder (MOGAD), aquaporin 4 IgG-positive neuromyelitis optica spectrum disorder (AQP4-IgG-NMOSD), and multiple sclerosis (MS). METHODS: In this descriptive study, we retrospectively identified Mayo Clinic patients with MOGAD, AQP4-IgG-NMOSD, or MS and (1) brain or myelitis attack; (2) available attack MRI within 6 weeks; and (3) follow-up MRI beyond 6 months without interval relapses in that region. Two neurologists identified the symptomatic or largest T2 lesion for each patient (index lesion). MRIs were then independently reviewed by 2 neuroradiologists blinded to diagnosis to determine resolution of T2 lesions by consensus. The index T2 lesion area was manually outlined acutely and at follow-up to assess variation in size. RESULTS: We included 156 patients (MOGAD, 38; AQP4-IgG-NMOSD, 51; MS, 67) with 172 attacks (brain, 81; myelitis, 91). The age (median [range]) differed between MOGAD (25 [2-74]), AQP4-IgG-NMOSD (53 [10-78]), and MS (37 [16-61]) (p < 0.01) and female sex predominated in the AQP4-IgG-NMOSD (41/51 [80%]) and MS (51/67 [76%]) groups but not among those with MOGAD (17/38 [45%]). Complete resolution of the index T2 lesion was more frequent in MOGAD (brain, 13/18 [72%]; spine, 22/28 [79%]) than AQP4-IgG-NMOSD (brain, 3/21 [14%]; spine, 0/34 [0%]) and MS (brain, 7/42 [17%]; spine, 0/29 [0%]) (p < 0.001). Resolution of all T2 lesions occurred most often in MOGAD (brain, 7/18 [39%]; spine, 22/28 [79%]) than AQP4-IgG-NMOSD (brain, 2/21 [10%]; spine, 0/34 [0%]) and MS (brain, 2/42 [5%]; spine, 0/29 [0%]) (p < 0.01). There was a larger median (range) reduction in T2 lesion area in mm2 on follow-up axial brain MRI with MOGAD (213 [55-873]) than AQP4-IgG-NMOSD (104 [0.7-597]) (p = 0.02) and MS (36 [0-506]) (p < 0.001) and the reductions in size on sagittal spine MRI follow-up in MOGAD (262 [0-888]) and AQP4-IgG-NMOSD (309 [0-1885]) were similar (p = 0.4) and greater than in MS (23 [0-152]) (p < 0.001). DISCUSSION: The MRI T2 lesions in MOGAD resolve completely more often than in AQP4-IgG-NMOSD and MS. This has implications for diagnosis, monitoring disease activity, and clinical trial design, while also providing insight into pathogenesis of CNS demyelinating diseases.

e-ASPECTS software improves interobserver agreement and accuracy of interpretation of aspects score.

INTRODUCTION: There is increased interest in the use of artificial intelligence-based (AI) software packages in the evaluation of neuroimaging studies for acute ischemic stroke. We studied whether, compared to standard image interpretation without AI, Brainomix e-ASPECTS software improved interobserver agreement and accuracy in detecting ASPECTS regions affected in anterior circulation LVO. METHODS: We included 60 consecutive patients with anterior circulation LVO who had TICI 3 revascularization within 60 minutes of their baseline CT. A total of 16 readers, including senior neuroradiologists, junior neuroradiologists and vascular neurologists participated. Readers interpreted CT scans on independent workstations and assessed final ASPECTS and evaluated whether each individual ASPECTS region was affected. Two months later, readers again evaluated the CT scans, but with assistance of e-ASPECTS software. We assessed interclass correlation coefficient for total ASPECTS and interobserver agreement with Fleiss' Kappa for each ASPECTS region with and without assistance of the e-ASPECTS. We also assessed accuracy for the readers with and without e-ASPECTS assistance. In our assessment of accuracy, ground truth was the 24 hour CT in this cohort of patients who had prompt and complete revascularization. RESULTS: Interclass correlation coefficient for total ASPECTS without e-ASPECTS assistance was 0.395, indicating fair agreement compared, to 0.574 with e-ASPECTS assistance, indicating good agreement (P < 0.01). There was significant improvement in inter-rater agreement with e-ASPECTS assistance for each individual region with the exception of M6 and caudate. The e-ASPECTS software had higher accuracy than the overall cohort of readers (with and without e-ASPECTS assistance) for every region except the caudate. CONCLUSIONS: Use of Brainomix e-ASPECTS software resulted in significant improvements in inter-rater agreement and accuracy of ASPECTS score evaluation in a large group of neuroradiologists and neurologists. e-ASPECTS software was more predictive of final infarct/ASPECTS than the overall group interpreting the CT scans with and without e-ASPECTS assistance.

Progenitor cell therapy for acquired pediatric nervous system injury: Traumatic brain injury and acquired sensorineural hearing loss.

While cell therapies hold remarkable promise for replacing injured cells and repairing damaged tissues, cell replacement is not the only means by which these therapies can achieve therapeutic effect. For example, recent publications show that treatment with varieties of adult, multipotent stem cells can improve outcomes in patients with neurological conditions such as traumatic brain injury and hearing loss without directly replacing damaged or lost cells. As the immune system plays a central role in injury response and tissue repair, we here suggest that multipotent stem cell therapies achieve therapeutic effect by altering the immune response to injury, thereby limiting damage due to inflammation and possibly promoting repair. These findings argue for a broader understanding of the mechanisms by which cell therapies can benefit patients.

Brainstem and cerebellar involvement in MOG-IgG-associated disorder versus aquaporin-4-IgG and MS.

OBJECTIVE: To determine the frequency and characteristics of brainstem or cerebellar involvement in myelin-oligodendrocyte-glycoprotein-antibody-associated-disorder (MOGAD) versus aquaporin-4-IgG-seropositive-neuromyelitis optica spectrum disorder (AQP4-IgG-NMOSD) and multiple sclerosis (MS). METHODS: In this observational study, we retrospectively identified 185 Mayo Clinic MOGAD patients with: (1) characteristic MOGAD phenotype, (2) MOG-IgG seropositivity by live cell-based assay and (3) MRI lesion(s) of brainstem, cerebellum or both. We compared the symptomatic attacks to AQP4-IgG-NMOSD (n=30) and MS (n=30). RESULTS: Brainstem or cerebellar involvement occurred in 62/185 (34%) MOGAD patients of which 39/62 (63%) were symptomatic. Ataxia (45%) and diplopia (26%) were common manifestations. The median age in years (range) in MOGAD of 24 (2-65) was younger than MS at 36 (16-65; p=0.046) and AQP4-IgG-NMOSD at 45 (6-72; p=0.006). Isolated attacks involving the brainstem, cerebellum or both were less frequent in MOGAD (9/39 (23%)) than MS (22/30 (73%); p<0.001) but not significantly different from AQP4-IgG-NMOSD (14/30 (47%); p=0.07). Diffuse middle cerebellar peduncle MRI-lesions favoured MOGAD (17/37 (46%)) over MS (3/30 (10%); p=0.001) and AQP4-IgG-NMOSD (3/30 (10%); p=0.001). Diffuse medulla, pons or midbrain MRI lesions occasionally occurred in MOGAD and AQP4-IgG-NMOSD but never in MS. Cerebrospinal fluid (CSF) oligoclonal bands were rare in MOGAD (5/30 (17%)) and AQP4-IgG-NMOSD (2/22 (9%); p=0.68) but common in MS (18/22 (82%); p<0.001). Disability at nadir or recovery did not differ between the groups. CONCLUSION: Involvement of the brainstem, cerebellum or both is common in MOGAD but usually occurs as a component of a multifocal central nervous system attack rather than in isolation. We identified clinical, CSF and MRI attributes that can help discriminate MOGAD from AQP4-IgG-NMOSD and MS.