Deep Gray Matter Lesions Research Articles

Pooled analysis of multiple sclerosis findings on multisite 7 Tesla MRI: Protocol and initial observations.

Although 7 T MRI research has contributed much to our understanding of multiple sclerosis (MS) pathology, most prior data has come from small, single-center studies with varying methods. In order to truly know if such findings have widespread applicability, multicenter methods and studies are needed. To address this, members of the North American Imaging in MS (NAIMS) Cooperative worked together to create a multicenter collaborative study of 7 T MRI in MS. In this manuscript, we describe the methods we have developed for the purpose of pooling together a large, retrospective dataset of 7 T MRIs acquired in multiple MS studies at five institutions. To date, this group has contributed five-hundred and twenty-eight 7 T MRI scans from 350 individuals with MS to a common data repository, with plans to continue to increase this sample size in the coming years. We have developed unified methods for image processing for data harmonization and lesion identification/segmentation. We report here our initial observations on intersite differences in acquisition, which includes site/device differences in brain coverage and image quality. We also report on the development of our methods and training of image evaluators, which resulted in median Dice Similarity Coefficients for trained raters' annotation of cortical and deep gray matter lesions, paramagnetic rim lesions, and meningeal enhancement between 0.73 and 0.82 compared to final consensus masks. We expect this publication to act as a resource for other investigators aiming to combine multicenter 7 T MRI datasets for the study of MS, in addition to providing a methodological reference for all future analysis projects to stem from the development of this dataset.

Development of statistical auto-segmentation method for diffusion restriction gray matter lesions in patients with newly diagnosed sporadic Creutzfeldt–Jakob disease

Quantification of diffusion restriction lesions in sporadic Creutzfeldt-Jakob disease (sCJD) may provide information of the disease burden. We aim to develop an automatic segmentation model for sCJD and to evaluate the volume of disease extent as a prognostic marker for overall survival. Fifty-six patients (mean age ± SD, 61.2 ± 9.9 years) were included from February 2000 to July 2020. A threshold-based segmentation was used to obtain abnormal signal intensity masks. Segmented volumes were compared with the visual grade. The Dice similarity coefficient was calculated to measure the similarity between the automatic vs. manual segmentation. Cox proportional hazards regression analysis was performed to evaluate the volume of disease extent as a prognostic marker. The automatic segmentation showed good correlation with the visual grading. The cortical lesion volumes significantly increased as the visual grade aggravated (extensive: 112.9 ± 73.2; moderate: 45.4 ± 30.4; minimal involvement: 29.6 ± 18.1 mm3) (P < 0.001). The deep gray matter lesion volumes were significantly higher for positive than for negative involvement of the deep gray matter (5.6 ± 4.6 mm3 vs. 1.0 ± 1.3 mm3, P < 0.001). The mean Dice similarity coefficients were 0.90 and 0.94 for cortical and deep gray matter lesions, respectively. However, the volume of disease extent was not associated with worse overall survival (cortical extent: P = 0.07; deep gray matter extent: P = 0.12).

Low-frequency MR elastography reveals altered deep gray matter viscoelasticity in multiple sclerosis

IntroductionBrain viscoelasticity as assessed by magnetic resonance elastography (MRE) has been discussed as a promising surrogate of microstructural alterations due to neurodegenerative processes. Existing studies indicate that multiple sclerosis (MS) is associated with a global reduction in brain stiffness. However, no study to date systematically investigated the MS-related characteristics of brain viscoelasticity separately in normal-appearing white matter (NAWM), deep gray matter (DGM) and T2-hyperintense white matter (WM) lesions. Methods70 MS patients and 42 healthy volunteers underwent whole-cerebral MRE using a stimulated echo sequence (DENSE) with a low-frequency mechanical excitation at 20 Hertz. The magnitude |G∗| (Pa) and phase angle φ (rad) of the complex shear modulus G∗ were reconstructed by multifrequency dual elasto-visco (MDEV) inversion and related to structural imaging and clinical parameters. ResultsWe observed φ in the thalamus to be higher by 4.3 % in patients relative to healthy controls (1.11 ± 0.07 vs. 1.06 ± 0.07, p < 0.0001). Higher Expanded Disability Status Scale (EDSS) scores were negatively associated with φ in the basal ganglia (p = 0.01). We measured φ to be lower in MS lesions compared to surrounding NAWM (p = 0.001), which was most prominent for lesions in the temporal lobe (1.01 ± 0.22 vs. 1.06 ± 0.19, p = 0.003). Age was associated with lower values of |G∗| (p = 0.04) and φ (p = 0.004) in the thalamus of patients. No alteration in NAWM stiffness relative to WM in healthy controls was observed. ConclusionLow-frequency elastography in MS patients reveals age-independent alterations in the viscoelasticity of deep gray matter at early stages of disease.

Paediatric gliomatosis cerebri presenting with deep gray matter lesions

Gliomatosis cerebri (GC) is a rare extensively infiltrating growth pattern of diffuse glioma with wide clinical heterogeneity, often mimicking other disorders. GC usually affects the white matter and involvement predominantly of the deep gray matter at onset is uncommon. We describe a teenager who presented with bi-thalamic and left hippocampal-parahippocampal lesions at first presentation with a 1-month history of right-sided motor hemiparesis and hemiataxia. Over a 1-year period the lesion spread to the bi-temporal, right insular cortex, and bi-frontal cerebral hemisphere associated with multiple cranial palsies and progressive dementia. Histological examination revealed a high- grade anaplastic astrocytoma consistent with GC. Our case widens the phenotypic spectrum of GC. Clinicians need to consider GC in children with lesions in the deep gray matter in combination with pyramidal, cranial nerve palsies and extrapyramidal signs.

Cerebral Attacks in MOG Antibody-Associated Disease: Clinical Spectrum and Fulfillment of ADEM criteria (S40.007)

Cerebral Attacks in MOG Antibody-Associated Disease: Clinical Spectrum and Fulfillment of ADEM criteria (S40.007)

Percentage brain volume change in multiple sclerosis mainly reflects white matter and cortical volume.

Brain atrophy in multiple sclerosis (MS), as measured by percentage brain volume change (PBVC) from brain magnetic resonance imaging (MRI), has been established as an outcome parameter in clinical trials. It is unknown to what extent volume changes within different brain tissue compartments contribute to PBVC. We analyzed pairs of MRI scans (at least 6 months apart) in 600 patients with relapsing-remitting MS. Multiple regression revealed that PBVC mainly reflects volume loss of white and cortical gray matter, while deep gray matter and white matter lesions were less represented. Our findings aid the interpretation of PBVC changes in MS.

PMON118 Cerebral Palsy Resulting in Hypothalamus Volume Loss and Temperature Dysregulation

Abstract Background Cerebral palsy is a syndrome caused by a non-progressive brain injury or malformation during early brain development, which can lead to severe physical disability. However, there is limited data on the consequences of cerebral palsy on other crucial brain functions such as hypothalamus and pituitary gland. We report a case of hypothalamic-pituitary involvement in a cerebral palsy patient. Clinical case A 28-year-old man with history of quadriplegic cerebral palsy, severe cognitive delay and seizure disorder presented to our clinic with temperature dysregulation for 2 years. He is wheel-chair-bound and non-verbal. His core temperature generally ranges from 96.8o to 97.2oF but occasionally he has had fluctuations as high as 102oF and as low as 88oF associated with extremes in surrounding temperature. At the time of initial evaluation, his temperature was 96.8oF (36°C) and his skin was cool and mottled. He was on levothyroxine replacement for hypothyroidism and thyroid hormone levels were within the normal range (Free T4 of 1.33 ng/dL and Total T3 of 153 ng/dL). Head CT revealed extensive encephalomalacia involving the frontal and temporal lobes, diffuse thinning of the parenchyma of the parietal and occipital lobes with secondary lateral ventricle enlargement, and volume loss in the deep gray nuclei including the hypothalamus. Further evaluation revealed a borderline response to Cortrosyn (16.9 ug/dL at 60 min) but associated with an adequate baseline cortisol of 13.7 ug/dL. IGF-1 and prolactin levels were normal (IGF-1 of 117 ng/mL and prolactin of 7.9 ng/mL), but testosterone was 175 ng/dL associated with an LH of 3.1 mIU/mL and FSH of 10.7 mIU/mL consistent with central hypogonadism. The family was advised to carefully regulate and monitor ambient temperature, and maintain dress appropriate for the surrounding temperature. Conclusion Although periventricular white matter lesions are most commonly associated with cerebral palsy resulting in motor dysfunction, deep gray matter lesions are also occasionally documented. These lesions should be recognized, as they could be detrimental to the body. The hypothalamus, with the preoptic area as its main thermoregulatory locus, plays an important role in temperature regulation. It integrates all information from the surface thermoreceptors and local brain temperature then initiates heat production or heat loss mechanisms to help maintain homeostasis. Therefore, volume loss of the hypothalamus can lead to serious consequences such as poikilothermia that can lead to life-threatening hypothermia or hyperthermia. Keywords: Cerebral palsy, hypothalamus, temperature dysregulation, poikilothermia Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Remyelination in humans due to a retinoid-X receptor agonist is age-dependent.

Remyelination efficiency declines with advancing age in animal models, but this has been harder to demonstrate in people with multiple sclerosis. We show that bexarotene, a putatively remyelinating retinoid‐X receptor agonist, shortened the visual evoked potential latency in patients with chronic optic neuropathy aged under 42 years only (with the effect diminishing by 0.45 ms per year of age); and increased the magnetization transfer ratio of deep gray matter lesions in those under 43 years only. Addressing this age‐related decline in human remyelination capacity will be an important step in the development of remyelinating therapies that work across the lifespan.

Vascular Lesions and Brain Atrophy in Alzheimer's, Vascular and Mixed Dementia: An Optimized 3T MRI Protocol Reveals Distinctive Radiological Profiles.

Vascular lesions may be a common finding also in Alzheimer's dementia, but their role on cognitive status is uncertain. The study aims to investigate their distribution in patients with Alzheimer's, vascular or mixed dementia and detect any distinctive neuroradiological profiles. Seventy-six subjects received a diagnosis of Alzheimer's (AD=32), vascular (VD=26) and mixed (MD=18) dementia. Three independent raters assessed the brain images acquired with an optimized 3T MRI protocol (including (3D FLAIR, T1, SWI, and 2D coronal T2 sequences) using semiquantitative scales for vascular lesions (periventricular lesions (PVL), deep white matter lesions (DWML), deep grey matter lesions (DGML), enlarged perivascular spaces (PVS), and microbleeds (MB)) and brain atrophy (medial temporal atrophy (MTA), posterior atrophy (PA), global cortical atrophy- frontal (GCA-F) and Evans' index). Raters reached a good-to-excellent agreement for all scales (ICC ranging from 0.78-0.96). A greater number of PVL (p<0.001), DWML (p<0.001), DGML (p=0.010), and PVS (p=0.001) was observed in VD compared to AD, while MD showed a significant greater number of PVL (p=0.001), DWML (p=0.002), DGML (p=0.018), and deep and juxtacortical MB (p=0.006 and p<0.001, respectively). Comparing VD and MD, VD showed a higher number of PVS in basal ganglia and centrum semiovale (p=0.040), while MD showed more deep and juxtacortical MB (p=0.042 and p=0.022, respectively). No significant difference was observed in scores of cortical atrophy scales and Evans' index among the three groups. The proposed MRI protocol represents a useful advancement in the diagnostic assessment of patients with cognitive impairment by more accurately detecting vascular lesions, mainly microbleeds, without a significant increase in time and resource expenditure. Our findings confirm that white and grey matter lesions predominate in vascular and mixed dementia, whereas deep and juxtacortical microbleeds predominate in mixed dementia, suggesting that cerebral amyloid angiopathy could be the main underlying pathology.

Diagnostic accuracy of double inversion recovery (DIR) in detection of cortical gray matter lesions in patients with MS

BackgroundConventional imaging techniques have a low sensitivity for detection of cortical and deep grey matter lesions in MS which hinder accurate assessment of the total lesion burden. Aim of this work was to assess the diagnostic accuracy of double inversion recovery (DIR) sequence in the detection of cortical grey matter lesions in MS patients.ResultsForty MS patients were prospectively included in this study. Imaging was performed using Philips Ingenia 1.5 T device. The sensitivity, specificity, PPV, NPV and accuracy of DIR sequence in detection of cortical grey matter lesions were 60%, 100%, 100%, 55.6% and 73.3%, respectively. The sensitivity, specificity, positive and negative predictive values as well as accuracy of Flair sequence were 50%, 100%, 100%, 50% and 66.7%, respectively. The sensitivity, specificity, positive and negative predictive values as well as accuracy of T2 sequence in the detection of cortical grey matter lesions were 22.5%, 100%, 100%, 39.2% and 48.3%, respectively.ConclusionsDetection rate of cortical gray matter lesions was significantly higher on DIR sequence than on T2 and Flair sequences.

Clinical Manifestations and Pathogenesis of Acute Necrotizing Encephalopathy: The Interface Between Systemic Infection and Neurologic Injury.

Acute necrotizing encephalopathy (ANE) is a devastating neurologic condition that can arise following a variety of systemic infections, including influenza and SARS-CoV-2. Affected individuals typically present with rapid changes in consciousness, focal neurological deficits, and seizures. Neuroimaging reveals symmetric, bilateral deep gray matter lesions, often involving the thalami, with evidence of necrosis and/or hemorrhage. The clinical and radiologic picture must be distinguished from direct infection of the central nervous system by some viruses, and from metabolic and mitochondrial disorders. Outcomes following ANE are poor overall and worse in those with brainstem involvement. Specific management is often directed toward modulating immune responses given the potential role of systemic inflammation and cytokine storm in potentiating neurologic injury in ANE, though benefits of such approaches remain unclear. The finding that many patients have mutations in the nucleoporin gene RANBP2, which encodes a multifunctional protein that plays a key role in nucleocytoplasmic transport, may allow for the development of disease models that provide insights into pathogenic mechanisms and novel therapeutic approaches.

Automated quantification of deep grey matter structures and white matter lesions using magnetic resonance imaging in relapsing remission multiple sclerosis

BackgroundBrain volume loss (BVL) is widespread in MS and occurs throughout the disease course at a rate considerably greater than in the general population. In MS, brain volume correlates with and predicts future disability, making BVL a relevant measure of diffuse CNS damage leading to clinical disease progression, as well as serving as a useful outcome in evaluating MS therapies. The aim of our study was to evaluate the role of automated segmentation and quantification of deep grey matter structures and white matter lesions in Relapsing Remitting Multiple Sclerosis patients using MR images and to correlate the volumetric results with different degrees of disability based on expanded disability status scale (EDSS) scores.ResultsAll the patients in our study showed relative atrophy of the thalamus and the putamen bilaterally when compared with the normal control group. Statistical analysis was significant for the thalamus and the putamen atrophy (P value < 0.05). On the other hand, statistical analysis was not significant for the caudate and the hippocampus (P value > 0.05); there was a significant positive correlation between the white matter lesions volume and EDSS scores (correlation coefficient of 0.7505). On the other hand, there was a significant negative correlation between the thalamus and putamen volumes, and EDSS scores (correlation coefficients < − 0.9), while the volumes of the caudate and the hippocampus had a very weak and non-significant correlation with the EDSS scores (correlation coefficients > − 0.35).ConclusionsThe automated segmentation and quantification tools have a great role in the assessment of brain structural changes in RRMS patients, and that it became essential to integrate these tools in the daily medical practice for the great value they add to the current evaluation measures.

Comparison of Thalamus and Basal Ganglia Signs Between Multiple Sclerosis and Primary Angiitis of the Central Nervous System: An Exploratory Study.

Based on the symptoms, especially those affecting small vessels, it is difficult to distinguish multiple sclerosis (MS) from primary angiitis of the central nervous system (PACNS). Magnetic resonance imaging (MRI) helps understand the characteristics of deep gray matter lesions (DGML) in MS and PACNS. We aimed to compare the MRI characteristics of thalamus and basal ganglia lesions between relapsing-remitting MS and PACNS. In our study, 49 relapsing-remitting MS patients and 16 PACNS with MRI-confirmed thalamus or basal ganglia lesions were enrolled. Among the DGMLs in basal ganglia, putamen had significantly higher (P = 0.037) involvement in PACNS than in MS. More importantly, larger lesion sizes in thalamus helps to distinguish PACNS (12.4 ± 4.3 mm) from MS (7.9 ± 3.7 mm) (P = 0.006). But using lesions in basal ganglia, researchers were unable to differentiate the two disorders. Presently, our study shows that MRI performances of deep gray matter differ between MS and PACNS.

Clinical and Prognostic Analysis of Autoantibody-Associated CNS Demyelinating Disorders in Children in Southwest China.

Objective: To analyze the positive and recurrence rates of different autoantibody-associated demyelination disorders in children in Southwest China, and describe the clinical, radiological, and prognostic features of the myelin oligodendrocyte glycoprotein antibody (MOG-ab) and aquaporin-4 antibody (AQP4-ab) associated disease. This study also summarizes steroid maintenance therapy approaches for MOG-ab-positive children.Methods: A total of 160 children presenting with acquired demyelinating syndromes (ADS) between January 2016 and December 2019 were tested for MOG-ab and AQP4-ab. Clinical data, MRI scans, and survival analyses were compared between MOG-ab-positive and AQP4-ab-positive children. Evolution of serologic status and treatment response to immunosuppressants were collected in MOG-ab-positive children.Results: Of the 160 included children, the MOG-ab positivity rate (47.4%) was significantly higher than the AQP4-ab (5%) positivity rate. The recurrence rate for AQP4-ab disease (71.4%) was higher than that of MOG-ab disease (30.1%). For 135 children with both MOG-ab and AQP4-ab tested, the median age at onset was 7 (interquartile range [IQR] 5–10) years, and the median follow-up period was 19 (IQR 13–27.5) months. MOG-ab-positive children more frequently presented with acute disseminated encephalomyelitis, had deep gray matter lesions on MRI, had a better clinical and radiological recovery, and were less likely to have sustained disability than AQP4-ab-positive children. In MOG-ab-positive and AQP4-ab-positive children, maintenance therapy was a protective factor for recurrence, but presenting optic neuritis was a predictor of earlier relapse. A high Expanded Disability Status Scale score at onset was associated with sustained disability. Steroid maintenance therapy longer than 6 months after the initial attack was associated with a lower risk of a second relapse in MOG-ab-positive children. On serial serum MOG antibody analysis, clinical relapse occurred in 34.6% of children with persistent seropositivity, but none of the children who converted to seronegative status experienced relapse.Conclusion: The MOG antibody is more common in children with ADS than the AQP4 antibody. MOG-ab-positive children are characterized by distinct clinical and radiological features. Although some MOG-ab-positive children experience relapsing courses or have persistently seropositive status, they still predict a better outcome than AQP4-ab-positive children.

Neuroradiologic Features Associated With Severe Restriction of Functional Mobility in Children With Cerebral Palsy in North India.

Few studies have focused on magnetic resonance imaging (MRI) brain findings associated with functional mobility in cerebral palsy. To determine association between MRI findings and Gross Motor Functional Classification System (GMFCS) levels in cerebral palsy. Prospective-observational study conducted in Pediatric Neurology Clinic at a public teaching hospital, Northern India. First 3 new cases of cerebral palsy were enrolled on particular neuro-clinic day per week for 1 year. Functional mobility was classified according to GMFCS. Association between MRI findings, cerebral palsy type, and GMFCS levels were evaluated using χ2 test. A total of 138 cases (mean age 2.71 [SD = 1.91] years; male [64.5%]) were enrolled. Reported types of cerebral palsy were as follows: spastic quadriplegia (47.8%), spastic diplegia (28.35%), spastic hemiplegia (11.6%), extrapyramidal (6.5%), and ataxic/hypotonic (5.8%). GMFCS were classified into level 1 (13%), level 2 (7.2%), level 3 (4.3%), level 4 (10.9%), and level 5 (64.5%). Spastic quadriplegia and extrapyramidal cerebral palsy were significantly associated with higher (severe) levels (IV and V), whereas spastic diplegia and hemiplegia were significantly associated with lower (mild) levels (I-III) of GMFCS. MRI features of periventricular white matter injury, deep gray matter injury, basal ganglia and thalamic changes, and superficial gray matter injury were significantly associated with severe levels of GMFCS (V and IV). MRI was normal in 8 children (5 = mild category, 3 = severe category). Severe cerebral palsy is most often associated with spastic quadriplegia, extrapyramidal cerebral palsy, superficial gray matter lesions, deep gray matter lesions, and periventricular white matter injury. This information is useful for anticipating and addressing the needs of children with cerebral palsy and for prognostication.

In vivo tensor-valued diffusion MRI of focal demyelination in white and deep grey matter of rodents

BackgroundMultiple sclerosis (MS) is a chronic inflammatory demyelinating disease leading to damage of white matter (WM) and grey matter (GM). Magnetic resonance imaging (MRI) is the modality of choice to assess brain damage in MS, but there is an unmet need in MRI for achieving higher sensitivity and specificity to MS-related microstructural alterations in WM and GM. ObjectiveTo explore whether tensor-valued diffusion MRI (dMRI) can yield sensitive microstructural read-outs for focal demyelination in cerebral WM and deep GM (DGM). MethodsEight rats underwent L-α-Lysophosphatidylcholine (LPC) injections in the WM and striatum to introduce focal demyelination. Multimodal MRI was performed at 7 Tesla after 7 days. Tensor-valued dMRI was complemented by diffusion tensor imaging, quantitative MRI and proton magnetic resonance spectroscopy (MRS). ResultsQuantitative MRI and MRS confirmed that LPC injections caused inflammatory demyelinating lesions in WM and DGM. Tensor-valued dMRI illustrated a significant decline of microscopic fractional anisotropy (µFA) in both LPC-treated WM and DGM (P < 0.005) along with a marked increase of isotropic kurtosis (MKI) in DGM (P < 0.0001). ConclusionTensor-valued dMRI bears considerable potential for microstructural imaging in MS, suggesting a regional µFA decrease may be a sensitive indicator of MS lesions, while a regional MKI increase may be particularly sensitive in detecting DGM lesions of MS.

Detection of Cortical and Deep Gray Matter Lesions in Multiple Sclerosis Using DIR and FLAIR at 3T.

The comparative detection rates of deep gray matter (GM) multiple sclerosis (MS) lesions using double inversion recovery (DIR) and fluid-attenuated inversion-recovery (FLAIR) on 3T MR imaging remain unknown. We aimed to assess the detectability of cortical and deep GM MS lesions using DIR and FLAIR on 3T clinical exams and evaluate the relationship between deep GM lesions and brain atrophy. One hundred fifty consecutive MS patients underwent routine brain MRI that included 3D DIR and 2D T2 FLAIR on the same 3T scanner. Three neuroradiologists independently reviewed all exams for cortical and deep GM lesions. Statistical parametric mapping (SPM) and FMRIB software library (FSL)-FIRST pipelines were used to determine normalized whole brain and deep GM volumes. A total of 65 cortical and 98 deep lesions were detected on DIR versus 24 and 20, respectively, on FLAIR. Among all 150 patients, the number and percentage of patients with GM lesions on DIR and FLAIR were as follows: cortical 43 (28.7%) versus 24 (16.0%) (P < .001), thalamus 47 (31.3%) versus 20 (13.3%) (P<.001), putamen 10 (6.7%) versus 3 (2.0%) (P = .02), globus pallidus 9 (6.0%) versus 3 (1.3%) (P = .02), and caudate 5 (3.3%) versus 1 (0.7%) (P = .125). Presence of deep GM lesions weakly correlated with deep GM volume fractions. Deep GM MS lesions can be detected using routine clinical brain MRI including DIR and FLAIR at 3T. Future studies to optimize these sequences may improve the detection rates of cortical and deep GM lesions. The presence of GM lesions showed weak correlation with GM atrophy.

Clinical management of cerebral small vessel disease: a call for a holistic approach.

Cerebral small vessel disease (SVD) is a common global brain disease that causes cognitive impairment, ischemic or hemorrhagic stroke, problems with mobility, and neuropsychiatric symptoms. The brain damage, seen as focal white and deep grey matter lesions on brain magnetic resonance imaging (MRI) or computed tomography (CT), typically accumulates “covertly” and may reach an advanced state before being detected incidentally on brain scanning or causing symptoms. Patients have typically presented to different clinical services or been recruited into research focused on one clinical manifestation, perhaps explaining a lack of awareness, until recently, of the full range and complexity of SVD.In this review, we discuss the varied clinical presentations, established and emerging risk factors, relationship to SVD features on MRI or CT, and the current state of knowledge on the effectiveness of a wide range of pharmacological and lifestyle interventions. The core message is that effective assessment and clinical management of patients with SVD, as well as future advances in diagnosis, care, and treatment, will require a more “joined-up”’ approach. This approach should integrate clinical expertise in stroke neurology, cognitive, and physical dysfunctions. It requires more clinical trials in order to improve pharmacological interventions, lifestyle and dietary modifications. A deeper understanding of the pathophysiology of SVD is required to steer the identification of novel interventions. An essential prerequisite to accelerating clinical trials is to improve the consistency, and standardization of clinical, cognitive and neuroimaging endpoints.

Distribution of Deep Gray Matter Lesions on Magnetic Resonance Imaging in Lymphomatosis Cerebri.

We herein report the distribution of gray matter lesions on magnetic resonance imaging (MRI) in two patients with lymphomatosis cerebri (LC). In our patients, the fluid-attenuated inversion recovery sequence of brain MRI demonstrated a bilateral and diffuse high signal intensity, not only in the white matter but also in the thalamus, globus pallidus, putamen, and hippocampus. Among the deep gray matter, the caudate head and putamen (striatum) were relatively spared when compared with the globus pallidus, thalamus, and hippocampus. Interestingly, we found seven previous reports of similar MRI findings, with relative sparing of the striatum, in patients with LC. This finding may be characteristic of LC and help facilitate its diagnosis. Further investigations of a larger number of LC patients are necessary to confirm these findings.

Risk factors for academic difficulties in children with myelin oligodendrocyte glycoprotein antibody-associated acute demyelinating syndromes.

To describe cognitive abilities through the evaluation of academic difficulties in children with acute demyelinating syndromes (ADS) and myelin oligodendrocyte glycoprotein (MOG) antibodies. This was an observational, retrospective study of a French paediatric cohort that included children aged 18 years and younger. Clinical, biological, and imaging data were collected and academic outcome was measured. Seventy-six children were included in the study with a mean (SD) follow-up of 4 years 7 months (6y 4mo). Median age at disease onset was 9 years 1 months (interquartile range=4y 7mo-13y 11mo; 36 females, 40 males). Thirty-six children relapsed and 20 had academic difficulties at the last follow-up. Academic difficulties, as well as deep grey matter and putaminal lesions (p=0.047 and p=0.006 respectively), were significantly more prevalent in children aged 10 years and younger (p=0.02). Using univariate binary regression analysis, we found that age at disease onset of 10 years and younger (odds ratio [OR] 3.72 [95% confidence interval {CI} 1.19-11.64]; p=0.024), acute disseminated encephalomyelitis at disease onset (OR 52.5 [95% CI 5.97-461.4]; p<0.001), and deep grey matter lesions (OR 17.33 [95% CI 3.87-77.72]; p<0.001) were associated with academic difficulties. MOG antibody-associated ADS have distinct clinical and radiological patterns that are age-dependent. Indirect cognitive evaluation through academic difficulties was prevalent in younger children and is associated with specific clinical and magnetic resonance imaging factors that need to be considered earlier on when assessing this patient population.