Acute Encephalomyelitis Research Articles

Severe encephalitis: aetiology, management and outcomes over 10 years in a paediatric intensive care unit

ObjectiveTo describe the characteristics, differential diagnoses, management and outcomes of severe encephalitis in children.DesignA 10-year retrospective cohort study in children admitted to a tertiary paediatric intensive care unit (PICU) with...

Scrub Typhus in Children: A Prospective Observational Study in a Tertiary Care Hospital in Eastern India.

Background Scrub typhus is a reemerging, acute, undifferentiating febrile illness and one of the most neglected tropical diseases, calling for an in-depth investigation into its clinical diversity, complications, and mortality, which drives us to carry out this research work. Methods Over a year, prospective observational research was carried out after gaining parental consent and institutional ethical clearance, 206 children of either gender aged between one month and 12 years who had been hospitalized with a fever for at least five days and subsequently tested positive forOrientia​​​​tsutsugamushiwere included in the study. Basic demographic information, clinical characteristics, laboratory findings, complications, related coinfections, and results were gathered and analyzed.A P-value of 0.05 was set as the statistical benchmark. Results The current study found that boys outnumbered girls. The ratio of boys to girls was 1.22:1, and the average age was 5.18 years. All had a fever (100%), and the other most frequently occurring clinical signs and symptoms were abdominal pain (16.99%), vomiting (22.33%), hepatosplenomegaly (49.51%), facial puffiness (39.32%), edema (27.18%), lymphadenopathy (19.90%), eschar (19.90%), macular-erythematous rash (17.96%), cough (21.84%), conjunctival congestion (25.24%), and headache (13.59%). Anemia (81.55%), leucocytosis (20.39%), leucopenia (6.8%), thrombocytopenia (49.51%), thrombocytosis (2.43%), and elevated serum levels of alanine aminotransferase (ALT, 57.28%) and aspartate aminotransferase (AST, 63.59%) were characteristic laboratory results. The coinfections were dengue, enteric fever, urinary tract infections, and malaria.Children who also had dengue were more likely to develop thrombocytopenia, which was statistically significant (P-value = 0.008). With doxycycline medication, early defervescence of fever occurred earlier than with azithromycin, and it was statistically significant (P-value = 0.000). The complications were hepatitis (63.59%), lower respiratory tract infections (LRTIs, 22.82%),scrub typhus meningoencephalitis (STME, 3.88%), acute kidney injury (AKI, 2.91%), myocarditis (1.46%), and acute disseminated encephalomyelitis (ADEM, 0.49%). Except for one who had ADEM, everyone was sent back home after receiving the best care possible. The average duration of hospital stay was 6.89 days. Conclusions Even in the absence of eschar, scrub typhus should be suspected in any febrile child who experiences clinical signs of meningoencephalitis syndrome, capillary leakage, skin rash, conjunctival congestion, LRTI, AKI, lymphadenopathy, hepatosplenomegaly, thrombocytopenia, and liver dysfunction in the post-monsoon season. Strong clinical suspicion and prompt anti-scrub drug administration go a long way in preventing or decreasing the morbidity and mortality of the same.

The Occurrence of Acute Disseminated Encephalomyelitis in SARS-CoV-2 Infection/Vaccination: Our Experience and a Systematic Review of the Literature.

The neurological complications of coronavirus disease 2019 (COVID-19) infection and the side effects of vaccination include immune-mediated diseases of the central nervous system (CNS) such as acute disseminated encephalomyelitis (ADEM). It is an acute-onset demyelinating disease that involves a rapid evolution and multifocal neurological deficits that develops following a viral or bacterial infection or, less frequently, following vaccination. Acute hemorrhagic leukoencephalitis (AHLE) is the hemorrhagic variant of ADEM that presents a more severe evolution which can be followed by coma and death. The objectives of this study consist in evaluating the diagnosis, clinical characteristics, imaging and laboratory features, evolution, and treatment of ADEM and AHLE following COVID-19 infection or vaccination. We performed a systematic review of the medical literature according to PRISMA guidelines that included ADEM cases published between 1 January 2020 and 30 November 2022 following severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and vaccination and also included our own clinical experience regarding this pathology. A total number of 74 patients were diagnosed with ADEM, 45 following COVID-19 infection and 29 after a SARS-CoV-2 vaccine. A total of 13 patients (17.33%) presented AHLE. The moderate form of COVID-19 presented a positive correlation with AHLE (r = 0.691, p < 0.001). The existence of coma and AHLE was correlated with poor outcomes. The following more aggressive immunomodulatory therapies applied in severe cases were correlated with poor outcomes (major sequelae and death): therapeutic plasma exchange (TPE) treatment (r = 382, p = 0.01) and combined therapy with corticosteroids and TPE (r = 0.337, p = 0.03). Vaccinations are essential to reduce the spread of the COVID-19 pandemic, and the monitoring of adverse events is an important part of the strategic fight against SARS-CoV-2. The general benefits and the overall good evolution outweigh the risks, and prompt diagnosis is associated with a better prognosis in these patients.

Intravenous immunoglobulin treatment for acute attacks in myelin oligodendrocyte glycoprotein antibody disease

Background: The potential therapeutic benefit of intravenous immunoglobulins (IVIGs) for acute attacks of myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is unknown. Objective: The objective was to describe the outcomes of IVIG treatment for acute MOGAD attacks. Methods: A retrospective observational study involving seven tertiary neuroimmunology centers. Data collection included patients’ demographics, Expanded Disability Status Scale (EDSS), and visual acuity (VA) before the attack, at the nadir of the attack before IVIG treatment, and at follow-up visits ⩾3 months after treatment. Results: Thirty-nine patients were included, of which 21 (53.8%) were female. The median age was 23 years (range 5–74 years), and the median disease duration was 4 months (range 0–93 months). The most common type of attack treated with IVIG was isolated optic neuritis (ON) (unilateral n = 14, bilateral n = 5, associated with transverse myelitis (TM), n = 1), followed by acute disseminated encephalomyelitis (ADEM) (n = 8), multifocal (n = 7), TM (n = 3), brainstem (n = 1), and other encephalitis (n = 1). A significant improvement in both the EDSS and VA measures was observed at follow-up compared to the time of IVIG treatment initiation (p < 0.0001 for both outcome measures). Conclusion: IVIG may be an effective treatment option for acute MOGAD attacks. Further prospective studies are warranted to validate our results.

Chorea following SARS-CoV-2 infection and vaccination: a systematic review of reported cases

ObjectivesChorea following SARS-CoV-2 infection and vaccination, has been increasingly recognized. We aimed to synthesize clinical and paraclinical characteristics, treatment responses, and outcomes of this neurologic complication. MethodsWe systematically reviewed LitCOVID, the World Health Organization database on COVID-19, and MedRxiv up to March 2023, following a published protocol. ResultsWe included 14 chorea cases in patients with SARS-CoV-2 infection and eight following COVID-19 vaccination. Acute or subacute chorea preceded COVID-19 symptoms within 1-3 days or developed up to 3 months after infection. Frequently it was generalized (85.7%), with associated neurological manifestations (encephalopathy 35.7%; other movement disorders 7.1%). After vaccination, chorea had a sudden onset (87.5%) within 2 weeks (75%); 87.5% of cases presented hemichorea, with hemiballismus (37.5%) or other movement disorders; 12.5% presented additional neurological findings. Cerebrospinal fluid was normal in 50% of infected individuals but abnormal in all vaccinated cases. Brain magnetic resonance imaging detected normal basal ganglia in 51.7% of infection cases and 87.5% following vaccination. ConclusionIn SARS-CoV-2 infection, chorea may present several pathogenic mechanisms: autoimmune response to infection, direct infection-related injury, or an infection-related complication (i.e., acute disseminated encephalomyelitis, cerebral venous sinus thrombosis, hyperglycemia); also, previous Sydenham chorea may relapse. After COVID-19 vaccination, chorea could be due to an autoimmune reaction or other mechanisms (vaccine-induced hyperglycemia, stroke).

Treatment Options for Epstein-Barr Virus-Related Disorders of the Central Nervous System.

Epstein-Barr virus (EBV), a causative agent for several types of lymphomas and mucosal cancers, is a human lymphotropic herpesvirus with the capacity to establish lifelong latent infection. More than 90% of the human population worldwide is infected. The primary infection is usually asymptomatic in childhood, whereas infectious mononucleosis (IM) is common when the infection occurs in adolescence. Primary EBV infection, with or without IM, or reactivation of latent infection in immunocompromised individuals have been associated with a wide range of neurologic conditions, such as encephalitis, meningitis, acute disseminated encephalomyelitis, and cerebellitis. EBV is also involved in malignant lymphomas in the brain. An increasing number of reports on EBV-related disorders of the central nervous system (CNS) including the convincing association with multiple sclerosis (MS) have put in focus EBV-related conditions beyond its established link to malignancies. In this review, we present the clinical manifestations of EBV-related CNS-disorders, put them in the context of known EBV biology and focus on available treatment options and future therapeutic approaches.

Post-COVID-19 Vaccination CNS Magnetic Resonance Imaging Findings: A Systematic Review.

This systematic review aims to synthesize and analyze the available literature on central nervous system (CNS) magnetic resonance imaging (MRI) findings in individuals who have received COVID-19 vaccinations. Our objective is to enhance understanding of potential neurological side effects, inform clinical practice, and guide future research on the neurological implications of COVID-19 vaccination. In this systematic review, we conducted a comprehensive search in PubMed, Scopus, and Web of Science from January 2020 to April 2023, using terms related to COVID-19 vaccination and CNS MRI findings. We evaluated the quality of the study, extracted relevant data, and included 89 eligible studies that covered various vaccines, demographics of patients, symptoms, and MRI findings to provide a thorough understanding of SARS-CoV-2 vaccination-related CNS problems. We investigated CNS MRI findings following COVID-19 vaccination across various vaccine types. Common diseases associated with post-vaccination CNS MRI findings included cerebral venous sinus thrombosis (CVST), vaccine-induced immune thrombotic thrombocytopenia (VITT), acute disseminated encephalomyelitis (ADEM), acute myelitis, autoimmune encephalitis (AE), and others. Patients presented with diverse onset symptoms and neurological manifestations. Abnormalities identified in CNS MRI findings included white matter (WM) hyperintensity. Our analysis offers a comprehensive overview of the current literature on post-vaccination CNS MRI findings. Discussion. We highlight a range of post-COVID-19 vaccination CNS MRI findings, including CVST, with a higher incidence in individuals receiving the ChAdOx1 (AstraZeneca) vaccine. Other notable observations include cases of ADEM, myelitis or transverse myelitis (TM), Guillain-Barré syndrome (GBS), and acute encephalopathy following COVID-19 vaccination. The incidence of these neurological complications is extremely rare, and the benefits of vaccination outweigh the risks. The reviewed studies were primarily case reports or case series, and thus large-scale epidemiological studies and controlled clinical trials are needed to better understand the underlying mechanisms and risk factors associated with these neurological complications following COVID-19 vaccination.

Posterior Reversible Leucoencephalopathy Syndrome: Case Series, Comments, and Diagnostic Dilemma.

To report a series of patients with clinical and radiological features suggestive of posterior reversible encephalopathy syndrome (PRES) related to diverse etiologies emphasizing its pathophysiological basis. Posterior reversible encephalopathy syndrome (PRES) may present with a broad range of clinical symptoms from headache and visual disturbances to seizure and altered mentation. Typical imaging findings include posterior-circulation predominant vasogenic edema. Although there are many well-documented diseases associated with PRES, the exact pathophysiologic mechanism has yet to be fully elucidated. Generally accepted theories revolve around disruption of the blood-brain barrier secondary to elevated intracranial pressures or endothelial injury induced by ischemia from a vasoconstrictive response to rising blood pressure or toxins/cytokines. While clinical and radiographic reversibility is common, long-standing morbidity and mortality can occur in severe forms. In patients with malignant forms of PRES, aggressive care has markedly reduced mortality and improved functional outcomes. Various factors that have been associated with poor outcome include altered sensorium, hypertensive etiology, hyperglycemia, longer time to control the causative factor, elevated C reactive protein, coagulopathy, extensive cerebral edema, and hemorrhage on imaging. Reversible cerebral vasoconstriction syndromes (RCVS) and primary angiitis of the central nervous system (PACNS) are invariably considered in the differential diagnosis of new cerebral arteriopathies. Recurrent thunderclap headache (TCH), and single TCH combined with either normal neuroimaging, border zone infarcts, or vasogenic edema, have 100% positive predictive value for diagnosing RCVS or RCVS-spectrum disorders. Diagnosis of PRES in some circumstances can be challenging and structural imaging may not be sufficient to distinguish it from other differential diagnostic considerations like ADEM. Advanced imaging techniques, such as MR spectroscopy or positron emission tomography (PET) can provide additional information to determine the diagnosis. Such techniques are more useful to understand the underlying vasculopathic changes in PRES and may answer some of the unresolved controversies in pathophysiology of this complex disease. Eight patients with PRES resulting from different etiologies varying from pre-eclampsia/eclampsia, post-partum headache with seizures, neuropsychiatric systemic lupus erythematosus, snake bite, Dengue fever with encephalopathy, alcoholic liver cirrhosis with hepatic encephalopathy, and lastly reversible cerebral vasoconstriction syndrome (RCVS). Additionally, a diagnostic dilemma between PRES and acute disseminated encephalomyelitis (ADEM) was notable in one patient. Some of these patients did not have or only very transiently had arterial hypertension. PRES may underlie the clinical conundrum of headache, confusion, altered sensorium, seizures, and visual impairment. PRES need not necessarily be always associated with high blood pressure. Imaging findings may also be variable. Both clinicians and radiologists need to familiarize themselves with such variabilities.

Acute hemorrhagic encephalitis in pediatric patient: a case report

Acute hemorrhagic encephalomyelitis (AHEM) is considered a rare presentation of acute disseminated encephalomyelitis (ADEM) and is challenging to manage. ADEM is a rare illness with an incidence of 0.2–0.4 per 100,000 children annually. Here we discuss a case report of a 3-year-old patient who presented with the clinical features of ADEM. On neuroimaging patient had a rare form of ADEM as hemorrhagic encephalitis. AHEM incidence is very low in the pediatric age group with poor prognosis. With the use of immunosuppressants and immunoglobulin, the survival of the patient was possible.

Myelin oligodendrocyte glycoprotein antibody associated disease – two distinct clinical presentations

Myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD) is an inflammatory demyelinating disorder caused by the development of immunoglobulin G autoantibody targeting MOG, which is expressed exclusively on the outer surface of the myelin sheath and plasma membrane of oligodendrocyte. MOGAD can present in different clinical spectrum including acute disseminated encephalomyelitis (ADEM), optic neuritis (ON), transverse myelitis and relapsing courses of these presentations. Depending on the severity, acute attacks can be treated with I V methyl prednisolone, plasma exchange or IVIG as in any demyelinating disease. This report is about 2 children with MOG antibody positive disease who presented with different symptomatology, severity and outcome.

Clinical polymorphism of acute disseminated encephalomyelitis: diagnosis, treatment, long-term prognosis. Lecture with the description of clinical cases

Acute disseminated encephalomyelitis is an acute autoimmune demyelinating disease that manifests as a widespread polyfocal asymmetric lesions of the central nervous system and, in some cases, optic nerves with the formation of corresponding neurological symptoms. This lecture presents basic information about acute disseminated encephalomyelitis with consideration of 5 clinical cases demonstrating the variety of manifestations of this disease in the practice of a neurologist.

Ablation of microglia following infection of the central nervous system with a neurotropic murine coronavirus infection leads to increased demyelination and impaired remyelination

Intracranial inoculation of susceptible mice with a glial-tropic strain of mouse hepatitis virus (JHMV), a murine coronavirus, results in an acute encephalomyelitis followed by viral persistence in white matter tracts accompanied by chronic neuroinflammation and demyelination. Microglia are the resident immune cell of the central nervous system (CNS) and are considered important in regulating events associated with neuroinflammation as well as influencing both white matter damage and remyelination. To better understand mechanisms by which microglia contribute to these immune-mediated events, JHMV-infected mice with established demyelination were treated with the small molecular inhibitor of colony stimulating factor 1 receptor (CSF1R), PLX5622, to deplete microglia. Treatment with PLX5622 did not affect viral replication within the CNS yet the severity of demyelination was increased and remyelination impaired compared to control mice. Gene expression analysis revealed that targeting microglia resulted in altered expression of genes associated with immune cell activation and phagocytosis of myelin debris. These findings indicate that microglia are not critical in viral surveillance in persistently JHMV-infected mice yet restrict white matter damage and remyelination, in part, by influencing phagocytosis of myelin debris.

#6167 THERAPEUTIC PLASMA EXCHANGE AS A TOOL IN THE TREATMENT OF NEUROLOGICAL DISEASES: A SINGLE CENTER EXPERIENCE

Abstract Background and Aims Plasmapheresis is an extracorporeal technique that removes immunological active substances, such as antibodies or immune complexes, from plasma. Simultaneously, it can provide essential factors in deficit, when supplementing with plasma. Therapeutic plasma exchange (TPE) can be used in numerous diseases with systemic involvement and, more recently, it has assumed an increasingly relevant role in the treatment of neurological diseases. Our aim was to review neurological indications, technique prescription, clinical outcomes and complications of these procedures. Methods We conducted an observational retrospective single-center study of the neurological diseases treated with TPE in our unit. We analyzed all procedures performed between January 2010 and December 2012 and collected indications, technical factors, complications and clinical outcomes of all treatments conducted. TPE was performed removing 1 to 1.5 of plasma volume per session. All procedures used Human Albumin 5% as replacement fluid and two units of fresh frozen plasma (FFP) were given in the end of each session. Hypocoagulation of the extracorporeal circuit was maintained with unfractionated heparin. Results Seventy-seven cycles of TPE (a total of 421 procedures) were performed on 45 patients [55.6% female; median age of 51 years (SD±16.8)]. The main indications of TPE in neurological diseases included myasthenia gravis (n = 35), Anti-N-methyl-d-aspartate (NMDA) encephalitis (n = 7), Guillain-Barré syndrome (n = 7), neuromyelitis optica spectrum disorders (n = 6), acute disseminated encephalomyelitis (n = 5), paraneoplastic syndromes (n = 4) and multiple sclerosis (n = 2). Intravenous immunoglobulin was given before TPE in 25 cases, 34 patients were on steroid and 6 patients had received pyridostigmine. Eighteen patients had coadjuvant immunosuppression [Mycophenolate mofetil or Azathioprine (n = 13), Rituximab (n = 3), tacrolimus (n = 1), Tocilizumab (n = 1) or Eculizumab (n = 1)]. The median number of TPE sessions per patient was 5 (range 1-20) and most cycles (63.6%) were performed every day for 3 sessions and 2 sessions every other day. Most of TPE (59.7%) was performed in ward, 27.3% in Intensive Care Unit (ICU) and 10 treatments were conducted in ambulatory. Near 84% of treatments were done through central venous catheters (CVC) and one patient had an arteriovenous (AV) fistula. Twenty-four (5.7%) complications were seen in 421 procedures. These complications were infection (18.2%), catheter-related (7.8%) and allergic reactions (1.3%). TPE procedure was terminated in 7.8% of sessions depending on these complications. Overall responses to TPE were noted in 81.8% of patients and 6 patients had at least one relapse during follow-up. Conclusion TPE is an effective and safe treatment option in several neurologic diseases. In our center, principal indications for TPE were myasthenia gravis, NMDA encephalitis and Guillain-Barré syndrome. In this study, clinical outcomes were favorable in the majority of the cases, with a minimum rate of complications per procedure. Therefore TPE should be considered in an early phase of presentation of disease. In relapsing diseases like myasthenia gravis, refractory to immunosuppression therapy, an AV fistula could be an alternative access to minimize the CVC-related complications.

Coronavirus Disease 2019-Associated Neurological Manifestations in Children: A Large Single-Center Experience With Rare Cases

BackgroundWe aimed to analyze pediatric patients with coronavirus disease 2019 (COVID-19) with a diverse spectrum of neurological manifestations in a single center since neurological involvement in children is still poorly understood. MethodsWe performed a retrospective study on 912 children aged between zero and 18 years who had a positive severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) test result and symptoms of COVID-19 from March 2020 to March 2021 in a single center. ResultsAmong 912 patients, 37.5% (n = 342) had neurological symptoms and 62.5% (n = 570) had no neurological symptoms. The mean age of patients with neurological symptoms was significantly higher (14.2 ± 3.7 vs 9.9 ± 5.7; P < 0.001). Three hundred and twenty-two patients had nonspecific symptoms (ageusia, anosmia, parosmia, headache, vertigo, myalgia), whereas 20 patients had specific involvement (seizures/febrile infection-related epilepsy syndrome, cranial nerve palsy, Guillain-Barré syndrome and variants, acute disseminated encephalomyelitis, central nervous system vasculitis). The mean age of the patients with nonspecific neurological symptoms was significantly higher (14.6 ± 3.1 vs 7.7 ± 5.7; P < 0.001). ConclusionThis study presents a large number of patients with a diverse spectrum of neurological manifestations. The rare neurological manifestations reported in our study will contribute to better understanding the neurological involvement of SARS-CoV-2 in children. The study also points out the differences of SARS-CoV-2-related neurological manifestations between patients at different ages. Physicians should be alert about recognizing the early neurological manifestations of the SARS-CoV-2 in children.

Ferroptosis and central nervous system demyelinating diseases.

Ferroptosis is a newly discovered programmed cell death caused by intracellular iron excess and glutathione (GSH) system imbalance, resulting in fatal lipid peroxidation. It is different from necrosis, apoptosis, autophagy, and other forms of cell death. Accumulating evidences suggest that brain iron overload is involved in the pathogenesis of demyelinating diseases of the central nervous system (CNS), such as multiple sclerosis (MS), neuromyelitis optica (NMO), and acute disseminated encephalomyelitis (ADEM). The study of ferroptosis may provide a new understanding of demyelinating diseases and provide a novel therapeutic target for clinical treatment. Herein, we reviewed recent discoveries on mechanisms of ferroptosis, the effects of metabolic pathways on ferroptosis, and its involvement in CNS demyelinating diseases.

Clinical, radiological, therapeutic and prognostic differences between MOG-seropositive and MOG-seronegative pediatric acute disseminated encephalomyelitis patients: a retrospective cohort study.

This study aimed to compare the clinical, radiological, therapeutic, and prognostic differences between pediatric patients showing acute disseminated encephalomyelitis (ADEM) with and without myelin oligodendrocyte glycoprotein (MOG) antibodies. We retrospectively collected all available data of children diagnosed with ADEM and tested for serum MOG antibodies at the Children's Hospital of Chongqing Medical University from January 2017 to May 2021. A total of 62 patients were included in our cohort, of which 35 were MOG-seropositive and 27 were MOG-seronegative. MOG-seropositive ADEM children presented with significantly lower rates of seizures (P = 0.038) and cranial nerve (III-XII) palsy (P = 0.003). Isolated leukocytosis in the blood was more common in ADEM children with MOG antibodies (P < 0.001). The two groups showed no significant differences in the distributions and extent of the MRI lesions as well as the appearance of typical/atypical magnetic resonance imaging (MRI) features. MOG-seropositive children were more likely to relapse (P = 0.017) despite having slower oral prednisolone tapering after acute treatments (P = 0.028). In scoring performed on the basis of two neurological function scoring systems, MOG-seropositive children showed milder neurological disability status at onset (P = 0.017 and 0.025, respectively) but showed no difference during follow-up. In summary, the differences in the clinical manifestations and auxiliary examination findings for MOG-seropositive and MOG-seronegative ADEM children lacked significance and specificity, making early identification difficult. MOG-seropositive children were more likely to relapse and showed slower steroid tapering. Moreover, MOG-seronegative children tended to have more severe neurological impairments at onset with no difference during follow-up.

Multiple sclerosis imaging in clinical practice: a European-wide survey of 428 centers and conclusions by the ESNR Working Group.

To evaluate compliance with the available recommendations, we assessed the current clinical practice of imaging in the evaluation of multiple sclerosis (MS). An online questionnaire was emailed to all members and affiliates. Information was gathered on applied MR imaging protocols, gadolinium-based contrast agents (GBCA) use and image analysis. We compared the survey results with the Magnetic Resonance Imaging in MS (MAGNIMS) recommendations considered as the reference standard. A total of 428 entries were received from 44 countries. Of these, 82% of responders were neuroradiologists. 55% performed more than ten scans per week for MS imaging. The systematic use of 3T is rare (18%). Over 90% follow specific protocol recommendations with 3D FLAIR, T2-weighted and DWI being the most frequently used sequences. Over 50% use SWI at initial diagnosis and 3D gradient-echo T1-weighted imaging is the most used MRI sequence for pre- and post-contrast imaging. Mismatches with recommendations were identified including the use of only one sagittal T2-weighted sequence for spinal cord imaging, the systematic use of GBCA at follow-up (over 30% of institutions), a delay time shorter than 5min after GBCA administration (25%) and an inadequate follow-up duration in pediatric acute disseminated encephalomyelitis (80%). There is scarce use of automated software to compare images or to assess atrophy (13% and 7%). The proportions do not differ significantly between academic and non-academic institutions. While current practice in MS imaging is rather homogeneous across Europe, our survey suggests that recommendations are only partially followed. Hurdles were identified, mainly in the areas of GBCA use, spinal cord imaging, underuse of specific MRI sequences and monitoring strategies. This work will help radiologists to identify the mismatches between their own practices and the recommendations and act upon them. • While current practice in MS imaging is rather homogeneous across Europe, our survey suggests that available recommendations are only partially followed. • Several hurdles have been identified through the survey that mainly lies in the areas of GBCA use, spinal cord imaging, underuse of specific MRI sequences and monitoring strategies.

MRI of fatal course of acute hemorrhagic leukoencephalitis in a child with SARS-CoV-2 omicron BA 2.0 infection.

We present a pediatric case of acute hemorrhagic leukoencephalitis associated with SARS-CoV-2 Omicron BA 2.0 infection. A previously healthy girl presented with ataxia and diplopia three weeks after the COVID-19 confirmation from a nasopharyngeal swab. Acute and symmetrical motor weakness and drowsiness ensued within the following 3days. She then became spastic tetraplegic. MRI revealed multifocal lesions in the cerebral white matter, basal ganglia, and brainstem, with hemorrhagic changes confirmed with T1-hyperintensity and hypointensity on susceptibility-weighted images. Peripheral areas of decreased diffusion, increased blood flow, and rim contrast enhancement were noted in the majority of lesions. She was treated with a combination of intravenous immunoglobulin and methylprednisolone pulse therapy. Neurological deterioration ensued with coma, ataxic respiratory pattern and decerebrate posture. Repeated MRI performed on day 31 revealed progression of abnormalities, hemorrhages and brain herniation. Despite the administration of plasma exchange, she died two months after admission.

Acute encephalomyelitis in a 52-year-old male post messenger ribonucleic acid severe acute respiratory syndrome coronavirus 2 vaccination: a case report

BackgroundAcute disseminated encephalomyelitis is a well-known, but rare, side effect of some vaccines, or symptom following a febrile illness.CaseA 69-year-old, otherwise healthy Hispanic male presented with acute fever, confusion, and later progressive weakness after receiving the first dose of the mRNA-1273 (Moderna) severe acute respiratory syndrome coronavirus 2 vaccine. Considering the progressive deterioration of the patient, despite being on multiple immunosuppressive agents, a brain biopsy was obtained, which revealed nonspecific meningoencephalitis.ConclusionIn this case, we highlight the need for a regulatory framework to assist clinicians and patients with coverage of treatment for acute disseminated encephalomyelitis. The use of intravenous immunoglobulin in conjunction with glucocorticoids seems to be an effective treatment option.

Encefalomielitis diseminada aguda, síndrome de Susac y astrocitopatía anti-PGFA

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease more common in children than in adults, often preceded by a nonspecific infectious process. It is typically characterized by encephalopathy and multifocal symptoms and signs and, in some cases, may take a more aggressive form such as acute hemorrhagic leukoencephalitis. A substantial number of patients will be positive for IgG-MOG (especially in children), and to a lesser extent for IgG-AQP4. ADEM is usually monophasic, but recurrent and/or persistent seropositive forms will require chronic immunomodulation. Susac syndrome is an inflammatory endotheliopathy characterized by occlusion of retinal arterial branches, hearing loss and encephalopathy, with no known biomarker and with neuroimaging findings amenable to differential diagnosis with multiple sclerosis. Anti-glial fibrillary acidic protein astrocytopathy (PGFA) can present with meningitis, encephalitis, myelitis, papillitis and epilepsy, is paraneoplastic in a third of cases and has an exquisite response to corticosteroids.