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Background and Objectives: Antibodies to myelin oligodendrocyte glycoprotein (MOG-Ab) have recently been reported in patients with encephalitis who do not fulfill criteria for acute disseminated encephalomyelitis (ADEM). We evaluated a cohort of these children and compared them with children with ADEM., Methods: This retrospective, multicenter cohort study comprised consecutive patients <18 years of age with MOG-Ab who fulfilled criteria for autoimmune encephalitis. These patients were stratified into (1) children not fulfilling criteria for ADEM (encephalitis phenotype) and (2) children with ADEM. Clinical/paraclinical data were extracted from the electronic records. Comparisons were made using the Mann-Whitney U test and χ 2 Fisher exact test for statistical analysis., Results: From 235 patients with positive MOG-Ab, we identified 33 (14%) with encephalitis and 74 (31%) with ADEM. The most common presenting symptoms in children with encephalitis were headache (88%), seizures (73%), and fever (67%). Infective meningoencephalitis was the initial diagnosis in 67%. CSF pleocytosis was seen in 79%. Initial MRI brain was normal in 8/33 (24%) patients. When abnormal, multifocal cortical changes were seen in 66% and unilateral cortical changes in 18%. Restricted diffusion was demonstrated in 43%. Intra-attack new lesions were seen in 7/13 (54%). When comparing with children with ADEM, children with encephalitis were older (median 8.9 vs 5.7 years, p = 0.005), were more likely to be admitted to intensive care (14/34 vs 4/74, p < 0.0001), were given steroid later (median 16.6 vs 9.6 days, p = 0.04), and were more likely to be diagnosed with epilepsy at last follow-up (6/33 vs 1/74, p = 0.003)., Discussion: MOG-Ab should be tested in all patients with suspected encephalitis even in the context of initially normal brain MRI. Although exclusion of infections should be part of the diagnostic process of any child with encephalitis, in immunocompetent children, when herpes simplex virus CSF PCR and gram stains are negative, these features do not preclude the diagnosis of immune mediated disease and should not delay initiation of first-line immunosuppression (steroids, IVIG, plasma exchange), even while awaiting the antibody results.

Objective: Our aim is to emphasize the varied presentation of acute disseminated encephalomyelitis (ADEM) to help health care professionals improve recognition of the disease in a timely manner, thereby allowing for the selection of an appropriate treatment regimen. Therefore, this may avoid neurocognitive consequences and the ultimate fatality of the patient., Patients and Methods: This is a retrospective case series involving 7 cases of children presenting to the Pediatric Emergency Department of Hackensack University Medical Center who were ultimately diagnosed with ADEM., Results: In many of the cases, a preceding viral-like illness with nonspecific symptomatology made it difficult to accurately establish an initial diagnosis. Ultimately, the neurologic symptoms spontaneously resolved or improved with administration of high-dose steroids., Conclusions: Children presenting to the emergency department with nonspecific symptoms associated with any neurological deficits should undergo further investigation using magnetic resonance imaging and lumbar puncture to rule out rare yet possibly fatal diseases such as ADEM., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.)

A 38-year-old gentleman, following an uncomplicated dengue fever 2 weeks back, developed acute onset bilateral lower limb weakness and numbness for 5 days, associated with bladder and bowel incontinence and a band-like sensation in T4 dermatome. On examination, he had paraparesis with normal cranial nerves except for left upper motor neuron-type 7th cranial nerve palsy and normal higher mental function. Magnetic resonance imaging (MRI) of the brain and spine detected multiple demyelinating lesions. A diagnosis of postdengue acute disseminated encephalomyelitis (ADEM) was made as part of postinfective inflammatory process after the fever had subsided. Cerebrospinal fluid study ruled out active infection. He was treated with intravenous steroids and is currently recovering. An interesting point in our case was that the patient had significant imaging findings in MRI of the brain with no symptoms or signs suggestive of intracranial involvement-ADEM without evidence of encephalitis., (© Journal of the Association of Physicians of India 2024.)

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder that typically follows an infection or recent vaccination. Symptoms such as encephalopathy and focal neurological deficits appear weeks after the initial illness, leading to swift and progressive neurological decline. While ADEM in the brain has been well documented, reports of ADEM, specifically in the spinal cord, are relatively limited. A 58-year-old male presented with rapidly progressive bilateral lower extremity tingling, numbness, and mild gait disturbance approximately two days prior to visiting the emergency room. Spinal magnetic resonance imaging revealed a diffuse, longitudinal, high-signal lesion with mild enlargement of the conus and proximal cauda equina. The lesions were predominantly localized in the distal conus and cauda equina, and serial electrodiagnostic studies showed that the lesions progressed toward the proximal conus in tandem with symptom evolution and lacked clear lateralization. The patient was subsequently treated with high-dose steroids for seven days (intravenous methylprednisolone, 1 mg/kg). The patient's lower extremity weakness gradually improved and he was able to walk independently under supervision three weeks after symptom onset. In this case of spinal ADEM in a middle-aged adult, high-dose steroid treatment led to outstanding neurological recovery from both the initial occurrence and subsequent attacks.

Thiamine pyrophosphate (TPP), the substrate of Thiamine pyrophosphate kinase (TPK), is an important cofactor in carbohydrate metabolism, specifically as a cofactor of the Pyruvate dehydrogenase complex (PDH) complex. The nervous system is particularly dependent on TPP due to its reliance on glucose metabolism. In this case, a four-year-old girl had a previously unreported pathogenic variant of the gene encoding TPK (TPK1) which presented as Thiamine metabolism dysfunction syndrome 5 (THMD5; OMIM 614458). She had been diagnosed with acute disseminated encephalomyelitis and autism spectrum disorder (ASD), and initially presented with fever and agitation following vaccinations. After follow-up with genetic testing, our patient was found to have compound heterozygous pathogenic variants of TPK1. After treatment with biotin and thiamine her clinical status improved, and her ASD features resolved. The presentation of our patient was consistent with previous reports and adds to the evidence that thiamine and biotin are effective treatments of TPK1 related metabolic deficiencies. The improvement of neurobehavioral symptoms in this case was marked, highlighting the importance of early identification and therapeutic intervention in this condition., (© 2023 The Authors. American Journal of Medical Genetics Part A published by Wiley Periodicals LLC.)

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.

The spectrum of severe neurological complications following COVID-19 vaccination includes cerebrovascular events, inflammatory diseases of the CNS, cranial and peripheral nerve involvement and muscle affections. Post-vaccinal acute disseminated encephalomyelitis (ADEM) and acute encephalitis are rare. We report on a patient suffering from acute encephalitis and another with post-vaccinal monophasic ADEM. Beside imaging features typical for acute autoimmune associated inflammation, cranial MRI disclosed also transient haemorrhagic signal alterations in some cerebral lesions. To our best knowledge, this has not been mentioned before in literature. Competing causes were excluded by extensive laboratory investigations including serial CSF analysis. In line with the literature, repeated iv high-dosage corticosteroid therapy resulted in impressive improvement of neurological symptoms in both patients., Competing Interests: All authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

A new outbreak of monkeypox has been reported worldwide with CNS complications like encephalitis or myelitis being extremely rare. We present a case of a 30-year-old man with PCR-confirmed diagnosis of monkeypox who developed rapid neurological deterioration with extensive inflammatory involvement of the brain and spinal cord on MRI. Because of the clinical and radiological resemblance to acute disseminated encephalomyelitis (ADEM), it was decided to indicate treatment with high-dose corticosteroids for 5 days (without concomitant antiviral management due to lack of availability in our country). Given the poor clinical and radiological response, 5 days of immunoglobulin G were administered. During follow-up the patient's clinical condition improved, physiotherapy was started and all associated medical complications were controlled. To our knowledge, this is the first reported monkeypox case with severe CNS complications treated with steroids and immunoglobulin in the absence of specific antiviral treatment., (© 2023. The Author(s).)

The epidemiology of Dengue fever in the Indian subcontinent has been substantially increased. Acute disseminating encephalomyelitis is a rare complication of dengue and is characterized by multifocal white matter involvement and encephalopathy with neurological deficits. Treatment is usually steroids, IVIG and therapeutic plasma exchange (TPE). We report a case of 46 year old female patient who was a non responder to steroid treatment and successfully treated by TPE., (Copyright © 2022 Société française de transfusion sanguine (SFTS). Published by Elsevier Masson SAS. All rights reserved.)

Background: Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated inflammatory demyelinating disease of the central nervous system. We report a case of ADEM presenting with bilateral optic neuritis temporally associated with the ChAdOx1 vaccine against SARS-COVID19 virus., Case Presentation: A 36-year-old female presented with bilateral optic neuritis following her first dose of the ChAdOx1 vaccine. Initial MRI Brain showed evidence of demyelination within the subcortical white matter, with no radiological involvement of the optic nerves. Visual evoked potentials were consistent with bilateral optic neuritis which was confirmed radiologically on follow up MRI. She was treated with intravenous steroids with improvement both in symptoms and radiological appearance. A pseudo-relapse occurred which was treated with a further course of intravenous steroids followed by an oral taper. The clinical, radiological and serological results were most consistent with diagnosis of ADEM., Conclusions: ADEM is an exceedingly rare complication of ChAdOx1 vaccine despite millions of doses. While it is imperative clinicians remain aware of neurological complications of vaccines, the importance of vaccination to control a pandemic should not be undermined., (© 2022. The Author(s).)

We describe a case of rapidly progressive dementia (RPD) in a nonagenarian. Dementia was caused by an acute disseminated encephalomyelitis (ADEM). Although not frequently diagnosed in the very elderly acute disseminated encephalomyelitis should not be overlooked for it is a treatable condition. A recent infection followed by rapid cognitive deterioration and multifocal neurologic signs should raise the attention to curable autoimmune diseases. Although the cause of ADEM is still unclear, immune suppression is the mainstay of treatment. Most patients improve on high-dose glucocorticoids and eventually immune globulin treatment or plasma exchange if steroid-unresponsive.

Acute disseminated encephalomyelitis (AEM) and acute transverse myelitis (OPM) are autoimmune demyelinating diseases of the central nervous system. Two clinical observations of AEM and OPM developed after suffering acute coronavirus infection (SARS-CoV-2) are presented. Differential diagnosis was carried out with multiple sclerosis, encephalitis of an infectious nature, compressive myelopathy, and opticomyelitis. Both observations show an almost complete recovery of lost functions. The pathogenetic mechanisms of the development of AEM and OPM in patients with coronavirus infection are discussed. The onset of central nervous system dysimmune lesion in the context of coronavirus infection makes it necessary to monitor the clinical situation with the involvement of a neurologist for timely diagnosis and determination of therapeutic tactics that can reduce the degree of disability of patients.

Background: Acute disseminated encephalomyelitis (ADEM) followed by optic neuritis (ADEM-ON) is characterized by the following features: early onset, monophasic or multiphasic ADEM followed by one or more episodes of ON, and the presence of serum anti-myelin oligodendrocyte glycoprotein (MOG) antibodies., Case Report: We report a case of ADEM-ON without anti-MOG antibodies in a 78-year-old woman. The patient developed acute-onset neurological findings and was diagnosed with ADEM. She was treated with intravenous methylprednisolone (IVMP), and oral corticosteroids. Her clinical symptoms and MRI findings subsequently improved. Left optic neuritis emerged 6 months later, and we made a diagnosis of ADEM-ON. A brain biopsy performed during the acute phase of ADEM showed perivascular infiltration of macrophages with demyelination., Conclusion: The majority of the reported ADEM-ON cases are pediatric cases with serum anti-MOG antibodies, but our patient was the elderly, without anti-MOG antibodies. Moreover, the pathological features of our case were similar to those observed in patients with typical ADEM and in patients with anti-MOG antibody-positive ADEM. Although ADEM-ON is related to the presence of anti-MOG antibodies, factors other than anti-MOG antibodies could contribute to the development of ADEM-ON., (© 2021. Fondazione Società Italiana di Neurologia.)

Acute disseminated encephalomyelitis (ADEM) has been reported after coronavirus disease 2019 (COVID-19). In this review, we systematically included worldwide reported cases on this association. We included 30 case reports (pediatric and adults) and explored epidemiological and clinical evidence. We described time to diagnosis, clinical, imaging, and laboratory features, response to treatment regimens, and differences regarding severity. Also, an original case report was presented. Neurologists must be alert to the occurrence of multifocal neurological symptoms with or without encephalopathy in patients recovered from COVID-19. Timely MRI studies should be performed to establish the diagnosis and to consider early corticosteroid-based treatment., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Introduction: Sars-CoV-2 is a single-strained RNA virus belonging to Coronaviridae's family. In pediatric age, the majority of patients is asymptomatic; however, several neurological manifestations associated with Sars-CoV-2 infection have been detected in a percentage of cases ranging from 17.3 to 36.4%. Acute disseminated encephalomyelitis (ADEM) has been recently included among the potential complications of Sars-Cov2 infection. The available data regarding pediatric patient show only one case., Case Report: We present a case regarding a 6-year-old patient suffering from Fisher-Evans syndrome who was given sirolimus and thalidomide therapy. After 10 days since the first positive nasopharyngeal swab for Sars-CoV-2, in which he had no symptoms, he presented an episode of generalized tonic-clonic seizure with spontaneous resolution. The patient underwent MRI which showed the typical picture of acute disseminated encephalomyelitis. His clinical course was favorable, with a good response to cortisone therapy and a progressive improvement of the neuroradiological and electroencephalographic picture., Conclusions: According to our knowledge, this is the second case of an acute disseminated encephalomyelitis following SARS-CoV-2 infection in a pediatric patient, characterized by monosymptomatic onset, in which the immunosuppressive therapy practiced for the Fisher-Evans syndrome has probably contributed to a favorable evolution of ADEM, in contrast to other case described in the literature., (© 2021. Fondazione Società Italiana di Neurologia.)

We present a case of an immunocompromised systemic lupus erythematosus female patient admitted to our hospital for general impairment, monoparesis, and temporary cognitive disability. The case represented a significant diagnostic and therapeutic challenge primarily because of a wide range of differential diagnosis options (CNS lupus, ischemic cerebrovascular disease, viral meningoencephalitis, progressive multifocal leukoencephalopathy, limbic encephalitis, and acute disseminated encephalomyelitis-ADEM). Brain MRI findings were compatible with ADEM, and microbiological tests showed a cytomegalovirus infection (CMV) which is rarely associated with ADEM despite the increasing number of immunocompromised patients prone to symptomatic CMV reactivation. Our patient was treated with intravenous methylprednisolone, immunoglobulin (IVIG), along with antiviral therapy resulting in a favorable therapeutic effect. In conclusion, only a few described ADEM cases have been associated with CMV, and none of them, to the best of our knowledge, in an immunocompromised patient. In this case, a multidisciplinary approach and broad diagnostic considerations were decisive for successful treatment and outcome.

Acute disseminated encephalomyelitis (ADEM) is a monophasic demyelinating disorder of central nervous system occurring in children with a wide range of clinical manifestations after infection or vaccination. There are few case reports in literature, describing atypical presentations of ADEM with fever of unknown origin, autonomic dysfunction, complex movement disorders such as myoclonus, dystonia and chorea, acute psychosis and myocarditis. Here, we report four cases of ADEM with atypical features like uniocular blindness, myelin oligodendrocyte glycoprotein antibodies negative multiphasic disseminated encephalomyelitis, ADEM mimicking Guillain-Barre syndrome at presentation and isolated spinal ADEM. Treatment with high-dose steroids elicited an excellent neurological outcome in all patients. A high index of clinical suspicion along-with awareness of atypical features, magnetic resonance imaging and cerebrospinal fluid studies are of paramount importance in establishing ADEM diagnosis and initiation of early treatment for better outcome., (© The Author(s) [2021]. Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Absrtract Acute disseminated encephalomyelitis (ADEM) is an immune-mediated inflammatory demyelinating disorder of the central nervous system that predominantly affects the pediatric population, and it is characterized by an acute or subacute onset of multifocal neurological symptoms. ADEM is typically a monophasic illness, and the majority of cases are associated with either a preceding infection or vaccination. First-line therapy for the disease is intravenous administration of high-dose methylprednisolone, and the long-term prognosis of ADEM is generally favorable.

Recently, the problem of demyelinating diseases in children is still very acute. This occurs, on the one hand, by high access and specificity of diagnostic methods and, on the other hand - by high morbidity of children different neuroinfectious diseases which can lead to demyelinating diseases. This literature review presents the currently available information on the autoantibodies and neurospecific protein role in the development of multiple sclerosis and acute disseminative encephalitis in children. The authors also describe their experience of complex etiopatogenic therapy and cytoflavin use that helps to reduce frequency and expression of demyelinating process and endothelium dysfunction in case of active herpesvirus infection.

The relevance of the study of demyelinating diseases is due to their increasing frequency in children, clarification of the role of infectious agents in their genesis, as well as the possibility of transformation of disseminated encephalomyelitis into multiple sclerosis. The literature review presents the currently available information on the causes of the development of demyelinating diseases, biomarkers of disseminated encephalomyelitis and multiple sclerosis, the causes of an unfavorable course and possible laboratory parameters indicating the transition from one disease to another, which can be used as prognostic factors. The authors also noted the experience of the authors on the importance of adequate etiopathogenetic therapy in changing the nature of the course of the disease, in particular, when confirming the relationship between the frequency of exacerbations of ADEM and MS with the activation of herpesvirus infections, courses of specific antiviral therapy are effective, as well as pathogenetic therapy aimed at correcting endothelial dysfunction using the drug cytoflavin.

A 53-year-old man admitted to the critical care secondary to respiratory failure due to COVID-19 developed agitation and global hypotonia. Brain MRI revealed bilateral hyperintense lesions throughout the brain and cerebrospinal fluid identified oligoclonal bands. Intravenous high-dose glucocorticoids were administered followed by an oral tapering dose and the patient clinically improved. Acute disseminated encephalomyelitis should be considered in patients with COVID-19 who present with altered mentation and polyfocal neurological deficits., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Intravenous corticosteroids have been regarded as the first-line therapy of anti-myelin-oligodendrocyte glycoprotein antibody (MOG-Ab)-positive acute disseminated encephalomyelitis (ADEM). While steroids are the first-choice therapy, MOG-Ab-positive ADEM has a high relapse rate. In some cases, MOG-Ab-positive ADEM relapses even in a low-MOG-Abs state. There is no evidence-based rule supporting steroid tapering. We herein report a case of MOG-Ab-positive ADEM in which recurrence was preventing by tapering steroids under MOG-Ab seronegativity confirmation. In some cases, the MOG-Ab titer may be an important index for tapering steroids to prevent relapse., Competing Interests: Declaration of Competing Interest There is no conflict of interest for this work., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Objective: The purpose of this study was to report three patients COVID-19 infection with severe respiratory syndrome requiring intubation, who developed acute demyelinating encephalomyelitis (ADEM)., Methods: Patient data were obtained from medical records from the North Memorial Hospital, Robbinsdale, MN, USA., Results: Three patients (two men and one woman, aged 38-63) presented with fatigue, cough, and fever leading to acute respiratory distress syndrome secondary to COVID-19 infection requiring ventilatory support. Two patients were unresponsive and the third patient had severe diffuse weakness. MRI in all patients showed findings consistent with ADEM. CSF showed elevated protein in all patients with normal cell count and no evidence of infection, including negative COVID-19 PCR. All three patients were treated with intravenous corticosteroids and one improved markedly. The other two had minimal response to steroids and no further improvement after IVIG., Conclusion: Neurological complications from COVID-19 are being rapidly recognized. Our three cases highlight the occurrence of ADEM as a postinfectious/immune-mediated complication of COVID-19 infection, which may be responsive to corticosteroid treatment., Competing Interests: None

Describing the variable clinical features, laboratory findings, neuroimaging findings, and treatments given to children who presented with ADEM and following them up both clinically and radiologically. 21 patients were recruited: 14 new cases, and 7 old ones presenting over the preceding 5 years (retrospective review of existing data). 11 males and 10 females, with a mean age of 4.4 years ± 2.7 SD, were included. All new patients were subject to full history, examination and a panel of investigations including MRI of the brain. Treatment was given in the form of pulsed methyl prednisolone or intravenous immunoglobulin (IVIG), followed by clinical and radiological follow-up every 3 months as needed. 11 cases occurred in spring, 8 post vaccine, of which 5 were after oral polio vaccine (OPV). MRI was done for all 21 patients and was abnormal in all of them, CT was done in only 10 patients as was normal in 9. Hyponatremia was seen in 11 patients. All patients who received corticosteroids showed prompt improvement. 6 out of 10 patients who received IVIG first failed treatment. Of the 17 treated patients, 10 had no sequelae and 10 had total lesion resolution on MRI at 3 months, versus 1 and 0 patients, respectively, in the untreated group. We found a disproportionately large number of post vaccination cases, especially after OPV. The association of ADEM with hyponatremia needs further study. MRI is central to diagnosis. Outcome is much better with treatment with steroids being far superior to IVIG. Excess use of IVIG should be discouraged.

Acute disseminated encephalomyelitis (ADEM) is a demyelinating, autoimmune disease of the central nervous system (CNS). It causes motor and sensory deficits, altered mental status and other neurological symptoms. Though rarely fatal, it has been associated with residual motor and neurocognitive deficits. Our case consisted of a 4-year-old girl who presented with fatigue and unsteady gait after a respiratory illness. During her hospital course, she became progressively weaker and experienced seizures. Imaging showed sections of demyelination in the CNS, and appropriate treatment was started. Additional labs resulted in positive Mycoplasma pneumoniae serum serology. Antimyelin oligodendrocyte glycoprotein (anti-MOG) antibodies were also found, which is a risk factor for relapsing, multiphasic ADEM. To our knowledge, this is the first case of anti-MOG antibody-associated ADEM due to M. pneumoniae infection. Our patient has made a complete recovery. The parents only report slightly increased fatigue and irritability., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Tumefactive demyelination is a phenomenon involving the radiographic resemblance of an acute demyelinating process in the central nervous system to neoplasia. Although this has been described and characterized for multiple sclerosis, it has been reported in a few cases in patients with acute disseminated encephalomyelitis (ADEM) within the past decade. While it may be challenging to establish a diagnosis of tumefactive ADEM according to clinical and radiological data alone, a thorough review of the clinical history and following the patient over time can be supportive of the same. The principal diagnostic confounds include neoplastic disease and a first attack of multiple sclerosis. A definitive diagnosis can be made by biopsy, which reveals perivenular demyelination and mononuclear cell infiltration in ADEM, in contrast to confluent plaque-like areas of demyelination in patients with multiple sclerosis. Histopathologic evidence of neoplastic disease includes characteristic features, including nuclear atypia and polymorphism, cellular hyperproliferation, mitoses, necrosis, endothelial proliferation, rosettes, and/or pseudorosettes. ADEM responds excellently to treatment with corticosteroids and is monophasic, with recurrence occurring infrequently. We review the literature on tumefactive ADEM and discuss the clinical manifestations, imaging characteristics, and histopathologic findings used to distinguish it from other conditions., Competing Interests: None

Background: Acute disseminated encephalomyelitis is generally preceded by an infection, and it is usually self-limiting and non-recurrent. However, when there are multiple attacks of acute disseminated encephalomyelitis followed by optic neuritis, it is defined as acute disseminated encephalomyelitis-optic neuritis. To the best of our knowledge, there are no previous reports of acute disseminated encephalomyelitis and optic neuritis preceded by autoinflammation, triggered by periodic fever syndrome. We report on a case of acute disseminated encephalomyelitis with optic neuritis and periodic fever syndrome in a 12-year-old Ecuadorian Hispanic boy with several relapses over the past 10 years, always preceded by autoinflammatory manifestations and without evidence of infectious processes. Whole exome sequencing was performed, and although the results were not conclusive, we found variants in genes associated with both autoinflammatory (NLRP12) and neurological (POLR3A) phenotypes that could be related to the disease pathogenesis having a polygenic rather than monogenic trait., Conclusion: We propose that an autoinflammatory basis should be pursued in patients diagnosed as having acute disseminated encephalomyelitis and no record of infections. Also, we show that our patient had a good response after 1 year of treatment with low doses of intravenous immunoglobulin and colchicine.

Background: Acute disseminated encephalomyelitis (ADEM) is an uncommon, treatable, primarily pediatric, immune-mediated disease. Diagnosis of ADEM requires two essential elements: typical clinical presentation and magnetic resonance imaging (MRI) findings. The aim of this study was to evaluate how clinical findings in the initial emergency department (ED) presentation influenced the timing of MRI., Methods: A retrospective chart review was conducted of children diagnosed with ADEM, over a 12-year period, in a tertiary care pediatric center. Clinical presentation at ED admission was recorded and patients who underwent an MRI as part of their ED evaluation (early MRI) with those who had MRI performed during ward hospitalization (late MRI) were compared., Results: 30 patients were diagnosed with ADEM during the study period. Encephalopathy and polyfocal neurological signs were described in 80% and 50% of patients ED charts, respectively. Seven patients underwent early MRI and polyfocal neurological signs were more common in this group (p = 0.006). Fever was more common in the late MRI group (p = 0.02). Following diagnosis, all patients were treated with immune-modulation therapy, improved clinically, and were discharged., Conclusion: 20% of ADEM patients were not encephalopathic at ED presentation. Polyfocal neurological signs and absence of fever at ED presentation were related to earlier MRI utilization and thus earlier diagnosis and treatment. Familiarity with the ADEM constellation of signs, and a high index of suspicion, may help the ED clinician in early diagnosis and treatment of this rare disease., (Copyright © 2019. Published by Elsevier Inc.)

Introduction: Acute disseminated encephalomyelitis (ADEM) is an idiopathic inflammatory demyelinating disorder of the central nervous system (CNS). Early treatment is the key for neurological recovery., Methods: A case of ADEM associated with varicella-zoster virus infection was presented, in which magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) examinations were included., Results: Magnetic resonance imaging of the brain revealed multiple hyperintense lesions at the subcortical level on fluid-attenuated inversion recovery (FLAIR), and MRI of the spinal cord revealed longitudinally segmented hyperintense lesions at the spinal cord on T2-weighted images. The patient was treated with methylprednisolone and gancyclovir, and had a favorable recovery. Subsequent MRI of the brain and cervical cord showed the previous abnormal hyperintensities had markedly disappeared., Conclusion: A rare case of ADEM with longitudinal segmented hyperintense lesions at the spinal cord on T2-weighted images was presented. Excellent response to ADEM treatment with high-dose steroids was reported resulting in a remarkable neurological recovery. A long-term follow-up is needed for prognosis., (© 2019 The Authors. Brain and Behavior published by Wiley Periodicals, Inc.)

Acute disseminated encephalomyelitis (ADEM) is exceptionally uncommon, with approximately 3 pediatric cases reported in the United States each year. Given the uncommon nature of ADEM, most of the current data rely heavily on case reports. The overwhelming majority of cases have been reported after an acute viral infection or vaccination. Although up to 90% of cases exhibit full remission after intravenous steroids, those in which treatment is delayed can display debilitating sequelae. Here, we present a case of ADEM in a 7-year-old boy who presented with double vision and imbalance with no recent history of acute viral infections or vaccinations.

A 2-year-old presented with lethargy, acute visual loss, fixed dilated pupils and severe bilateral retinal haemorrhages. The retinal findings raised concerns about abusive head trauma, but subsequent investigations confirmed the diagnosis of bilateral optic neuritis associated with acute disseminated encephalomyelitis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

There are four neurological complications that can occur after malaria treatment at a time when the patient is aparasitaemic: delayed cerebellar ataxia, acute inflammatory demyelinating polyneuropathy, post-malaria neurological syndrome and acute disseminated encephalomyelitis (ADEM). The authors describe a case of a 54-year-old male who presented with encephalopathy and generalized seizures forty-three days after complete recovery from acute malaria by Plasmodium falciparum. Diagnosis of post-malaria ADEM was made based on the acute onset of the neurological symptoms, characteristic findings in magnetic resonance imaging of the brain and prompt response to steroid therapy. ADEM is an autoimmune demyelinating disease of the central nervous system that usually arises after an infection or vaccination. Its occurrence after malaria infection is relatively rare, and to the best of our knowledge there are only thirteen cases described in the literature., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system that usually affects children and young adults. It most commonly has a monophasic course, although relapses are reported. Clinical presentation of the disease includes encephalopathy and multifocal neurological deficits. There are no established reliable criteria for diagnosis of ADEM and sometimes it is difficult to distinguish it from first attack of multiple sclerosis, especially in adults. The diagnosis of ADEM is based on clinical, radiological and laboratory findings. In the treatment of ADEM, high doses of corticosteroids, plasmapheresis and immunoglobulins are used. We report a case of a young adult female patient with ADEM who fully recovered after prompt administration of high dose methylprednisolone and immunoglobulins.

Acute disseminated encephalomyelitis is a rare inflammatory demyelinating multifocal disease of the central nervous system that typically occurs in children following vaccination or exanthematous viral infections and conveys an elevated risk of neurological sequelae unless promptly recognized and treated. We describe an adult case of acute disseminated encephalomyelitis following vaccination against Mumps, Measles and Rubella, presenting with fever and progressive neurological deficits which improved under systemic corticosteroid therapy. Considering the ongoing public debate regarding universal vaccination and the surge of previously controlled infectious diseases, we aim not only to underline the need for a rigorous assessment of vaccination safety on adult patients in order to prevent misguidance of public opinion, but also to alert clinicians for an early diagnosis of acute disseminated encephalomyelitis in these patients, the incidence of which we speculate may be rising.

The two main demyelinating diseases in children are reviewed. Acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS). For its physiopathological characteristics, probable etiologies, clinical manifestations, diagnosis, treatment, prognosis, evolution, as well as atypical alterations that complicate its diagnosis, the smaller the child is, more study is needed before reaching the diagnosis. The International Study Group of Multiple Pediatric Sclerosis, published the operating definitions for demyelinating diseases acquired from the central nervous system in children: the ADEM is monophasic, polysymptomatic and with encephalopathy. Its duration is up to 3 months, with fluctuating symptoms and magnetic resonance findings. MS is an isolated monofocal or polyfocal syndrome, without encephalopathy. Currently, two different and distinguishable diseases are considered from the onset of symptoms.

Myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) are a well-recognized cause of acquired demyelinating syndromes in both adult and children. Despite basal ganglia involvement on imaging, movement disorder is not a cardinal feature. We describe a 2-year-9-month-old girl who presented with severe encephalopathy with aphasia, seizures and a complex movement disorder with dystonic posturing and tonic eye deviation. Neuroimaging revealed subtle asymmetrical predominantly white matter signal changes. MOG-Abs were positive in the serum. Other known pathogenic autoantibodies including N-methyl-D-aspartate receptor antibodies (NMDAR-Abs) were negative. The patient made a complete recovery following 2-week corticosteroid treatment. This case highlights the need for MOG-Ab testing in children with suspected autoimmune encephalopathies.

Purpose of Review: Diagnostic criteria for pediatric-onset multiple sclerosis (POMS) and related demyelinating disorders have been updated, neuroimaging studies have revealed new insights, biological assays identify patients with specific antibodies that influence both diagnosis and treatment, clinical trials are informing on treatment efficacy and safety, and longitudinal studies of neurological, cognitive and quality of life outcomes are informing on the impact of these diseases. We provide updates to assist providers caring for these children., Recent Findings: The recent 2017 McDonald Criteria for MS provide a simplified means to confirm diagnosis at onset and over time, and have been shown to be equally applicable for POMS. MRI analyses demonstrate that brain volume is reduced at onset, and that both volumetric and tissue integrity measures decline over time, indicating that POMS shares the degenerative aspects that also characterize adult-onset disease. The presence of myelin oligodendrocyte glycoprotein (MOG) antibodies at onset is detected in more than 50% of children with acute disseminated encephalomyelitis. When persistent over time, they are associated with relapsing disease. The first randomized clinical trials of disease supports superiority of fingolimod over subcutaneous interferon beta 1a, and demonstrated a favorable safety profile. Finally, while Expanded Disability Status Scale (EDSS) scores remain low in the first 10 years post-onset, POMS is associated with high rates of patient-reported fatigue and reduced engagement in exercise and carries a risk for cognitive impairment. The past 15 years have borne witness to a marked expansion in recognition and research in POMS. There are now more specific diagnostic criteria, antibodies to CNS proteins appear to define diagnostically distinct disorders, clinical trials have successfully launched and one has completed, and we are gaining increasing appreciation of the impact of MS and related disorders on the lived experience of children and adolescents.

Background and Purpose: Acute disseminated encephalomyelitis followed by optic neuritis (ADEM-ON) is a rare demyelinating syndrome that is different from multiple sclerosis and neuromyelitis optica spectrum disorder. The aim of this study was to describe the disease course, treatment response and outcome of children with ADEM-ON., Methods: Children of <18 years of age were identified from six countries of the EU Paediatric Demyelinating Disease Consortium. Patients fulfilled the diagnostic criteria for ADEM followed by at least one ON. Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies were tested in all patients., Results: In this study of 17 patients (nine boys) with ADEM-ON, anti-myelin oligodendrocyte glycoprotein (MOG) antibodies were identified in 16 patients. Age at onset was 6.1 years (interquartile range, 5.1-9.2 years). Twelve patients received oral prednisolone and 10 received maintenance immunosuppression (e.g. azathioprine, intravenous immunoglobulins, Rituximab). During a follow-up of 5.3 years (interquartile range, 1.8-10.2 years), 54 relapses occurred with a median of 3 relapses per patient (range, 1-9 per patient). Patients relapsed on all treatments but no relapses occurred on a prednisolone dose >10 mg/day. Visual and cognitive residual deficits were common in this group., Conclusions: Acute disseminated encephalomyelitis followed by optic neuritis is an anti-MOG antibody-associated relapsing disorder that can have a heterogeneous disease course. Patients were refractory for maintenance immunosuppression and appeared to be corticosteroid-dependent. Further international collaborations are now required to unify guidelines in this difficult-to-manage group of patients., (© 2018 EAN.)

Demyelinating diseases are a group of conditions of autoimmune etiology directed against the myelin of the central nervous system. In many cases, the onset of the illness is preceded by a nonspecific viral infection. Multiple sclerosis is a disease that evolves with relapses and remissions with polyfocal neurological deficits, being the most frequent optic neuritis, transverse myelitis and encephalic trunk involvement. Typically, magnetic resonance image (MRI) shows peri-ventricular, peri-callosal, cerebellum, brain stem and spinal cord hyperintensive lesions in T2 and FLAIR weighted images. Optic neuromyelitis is characterized by the presence of optic neuritis and transverse myelitis associated with the postrema and diencephalic area syndrome. MRI lesions are distributed in sectors rich with aquaporine-4 channels (AQP-4): hypothalamus, third and fourth ventricle, optic nerves and spinal cord. Finding anti AQP4 antibodies is useful for the diagnosis although they are not essential for it. Acute disseminated encephalomyelitis is typically a monophasic condition characterized by acute encephalopathy associated with hyperintense MRI large, bilateral and irregular asymmetric lesion in T2 and FLAIR weighted images. In all three cases, cerebral spine fluid (CSF) can show pleocytosis and hyperproteinorrachia. The presence of oligoclonal bands in CSF is characteristic of multiple sclerosis. In all cases, acute treatment includes high dose intravenous corticosteroids and plasmapheresis in non-responsive cases. Both multiple sclerosis and optic neuromyelitis require long-term treatment to prevent relapse and recurrent diseases.

Chronic graft versus host disease (cGVHD) of the central nervous system is a rare condition that could occur after allogeneic hematopoietic stem cell transplantation (SCT) but has been poorly documented. Here, we report a patient diagnosed with recurrent acute disseminated encephalomyelitis (ADEM) with longitudinal extensive transverse myelitis (LETM) as cGVHD after HLA haploidentical peripheral blood SCT using posttransplantation cyclophosphamide for mixed-phenotype acute leukemia. We observed clinical and radiological improvement after the rituximab treatment of the condition that had been refractory to steroids. To the best of our knowledge, no report of cGVHD presented recurrent ADEM with LETM after allogeneic SCT and successfully treated with rituximab. Hence, ADEM should be included in the differential diagnosis of neurological symptoms in posttransplant patients with cGVHD.