Acute Encephalomyelitis Research Articles

Homonymous hemianopia following yellow fever vaccination: a case of acute disseminated encephalomyelitis.

A 42-year-old Caucasian man developed left homonymous hemianopia sixteen days after receipt of a live-attenuated 17D-204 yellow fever virus vaccine. MRI imaging of the brain revealed right occipital and left parieto-occipital lesions with marked hyperintensity consistent with demyelination, and a diagnosis of yellow fever vaccine-associated acute disseminated encephalomyelitis was made.

Acute hemorrhagic leukoencephalitis: a case report and systematic review of factors associated with severe disability and death.

Acute hemorrhagic leukoencephalitis (AHLE), a rare form of acute disseminated encephalomyelitis (ADEM), has a generally poor prognosis. However, significant variation is observed, and even complete recovery has been reported. The recent increase in the frequency of AHLE case reports is possibly contributed by the advent of COVID-19 and may have added to the heterogeneity of cases. We report a fatal case of AHLE with a preceding unspecified respiratory infection, then perform a systematic review of AHLE, in an effort to delineate factors that may be associated with an ultimate outcome of severe disability (defined as modified Rankin scale score of 4 or 5) or death. Descriptions of 31 cases of AHLE were found in 21 identified articles, with our case being the 32nd case. The most common antecedent event was an infection (20 patients, 62.5%), with nearly half of these being COVID-19 (9 patients). The majority of patients had a subacute progression (1 to 10days) from onset to clinical nadir. We found that an altered mental status (AMS) and a Glasgow Coma Scale (GCS) score of less than12 were associated with a final outcome of severe disability or death. An abnormal upgoing plantar response was associated with a final outcome of death. COVID-19 and its vaccines were not associated with either outcome. AMS, depressed GCS, and an upgoing plantar response at presentation may be associated with a poor outcome in AHLE. Our findings may serve as a springboard to much-needed research into the stratification of AHLE.

Clinical pattern, neuroimaging findings and outcome of Acute Disseminated Encephalomyelitis in children: A retrospective study.

To determine the clinical spectrum, neuroimaging findings, and outcome of Acute Disseminated Encephalomyelitis (ADEM) in children. We conducted a descriptive cross sectional study of all children aged 6 months to 18 years, diagnosed with ADEM at Aga Khan University Hospital, Karachi from January 2018 till December 2022. This retrospective study enrolled 30 cases of ADEM, with a mean age of 6.43 ± 4.079, including 13 males and 17 females. The average hospital stay was 7.29 ± 4.379 days. The most common clinical features were fever, headache, and altered consciousness, while motor deficit was observed in 15 (53.5%) patients. Abnormal cerebrospinal fluid was found in 14 (46.6%) patients. Brain MRI identified bilateral and multifocal lesions in 22 (78.6%) patients, with brainstem lesions detected in 7 (25%) patients. Treatment included IV methylprednisolone (22; 73%), IVIG (9; 30%), or both (6; 20%). Clinical improvement was observed in 25 (89.3%) patients, with residual weakness present in eight (26%) patients at discharge. There was one reported death. Long-term complications included motor deficits, seizures, poor scholastic performance, and behavioral issues. The clinical presentation of ADEM is variable, but the most common symptoms are fever, headache, and altered consciousness. Despite generally favorable outcome, long-term monitoring revealed that patients may experience motor deficits, seizures, cognitive impairment, and academic difficulties.

Evaluating Therapeutic Plasma Exchange in Pediatric Acute Disseminated Encephalomyelitis: A Comprehensive Review.

Acute disseminated encephalomyelitis (ADEM) is a rare autoimmune disorder characterized by brain and spinal cord inflammation. In pediatric patients, ADEM presents unique challenges due to its potential for rapid progression and long-term neurological sequelae. Therapeutic plasma exchange (TPE) has emerged as a potential treatment option by targeting the underlying autoimmune process and modulating the inflammatory response. This comprehensive review evaluates the role of TPE in pediatric ADEM, synthesizing evidence from clinical studies and providing insights into its efficacy, safety, and potential benefits. The review highlights the variability in TPE efficacy based on disease severity and patient-specific factors. Implications for clinical practice include considering TPE as a therapeutic option, particularly in severe or refractory cases, and emphasizing the importance of early intervention. Recommendations for future research include long-term prospective studies, comparative effectiveness trials, and efforts to standardize TPE protocols. Overall, continued investigation and innovation in managing pediatric ADEM are essential for improving outcomes and quality of life for affected children and their families.

Spinal Cord Involvement in Children with Acquired Demyelinating Syndromes: A Single Centre Study

Abstract Background Myelitis may be the first presentation of relapsing acquired demyelinating syndromes (ADS) such as neuromyelitis optica (NMO) or multiple sclerosis (MS) and in association with connective tissue diseases. It may also occur in the context of acute disseminated encephalomyelitis (ADEM). Early recognition of myelitis in these disorders will allow better management plan including theprognosis. Objective To evaluate the frequency of myelitis among children with acquired demyelinating neurological diseases, and the variability of clinical features, neuroimaging findings and treatments given. Methods A pilot observational study with both retrospective and prospective components was conducted on children and adolescents fulfilling the diagnostic criteria for any of the acquired demyelination syndromes admitted inChildren’s Hospital, Ain Shams University whose ages were below 18 years during the period from July 2020 to February 2022. Results Forty-three children were included in our study with mean (SD)age of onset 7.63 (4.28) years. The most common diagnosis was ADEM (n (%) =20 (46.5%), followed by transverse myelitis (TM) (n (%) =11 (25.6%) and NMO (n (%) =7(16.3%). Progression to develop encephalopathy was recorded in 55.8%of cases (100% of ADEM and autoimmune encephalitis (AE) cases). Seizures developed in 23.3% of cases (35% of ADEM, 50% of AE and 14.29% of NMO cases). MRI brain abnormalities were observed in 76.7% of cases (100% of ADEM, NMO, AE and MS cases), and abnormalities of MRI spine were seen in 59% of cases (100% of TM and MS cases, 85.7% of NMO, 33.3% of AE and 23.5% of ADEM cases). Spinal cord affection was most commonly cervical(86.9%) and mostly short segment. All cases received pulsed methylprednisolone therapy, while intravenous immunoglobulins (IVIG) were used in 32.5% of cases and plasmapheresis in 13.9% of cases as a stepwise approach. Nearly half of the cases (53.5%) had monophasic illnesses without the need for long term therapy. Conclusion Spinal cord involvement is frequently observed in childhood acute demyelinating syndromes, and is most commonly cervical and short-segment. MRI spine should be regarded as a sensitive tool for diagnosis of myelitis in the context of ADEM and MS. MRI Brain is a sensitive tool accounting forfindings in 100% of ADEM, NMO, AE and MS cases. Patients with ADEM and AE has higher risk to develop encephalopathy and seizures unlike TM, NMO and MS patients.

Examining cognition and brain networks using magnetoencephalography in paediatric autoimmune encephalitis and acute disseminated encephalomyelitis: a preliminary study.

Paediatric autoimmune encephalitis, including acute disseminated encephalomyelitis, are inflammatory brain diseases presenting with cognitive deficits, psychiatric symptoms, seizures, MRI and EEG abnormalities. Despite improvements in disease recognition and early immunotherapy, long-term outcomes in paediatric autoimmune encephalitis remain poor. Our aim was to understand functional connectivity changes that could be associated with negative developmental outcomes across different types of paediatric autoimmune encephalitis using magnetoencephalography. Participants were children diagnosed with paediatric autoimmune encephalitis at least 18 months before testing and typically developing children. All completed magnetoencephalography recording at rest, T1 MRI scans and neuropsychology testing. Brain connectivity (specifically in delta and theta) was estimated with amplitude envelope correlation, and network efficiency was measured using graph measures (global efficiency, local efficiency and modularity). Twelve children with paediatric autoimmune encephalitis (11.2 ± 3.5 years, interquartile range 9 years; 5M:7F) and 12 typically developing controls (10.6 ± 3.2 years, interquartile range 7 years; 8M:4F) participated. Children with paediatric autoimmune encephalitis did not differ from controls in working memory (t(21) = 1.449; P = 0.162; d = 0.605) but had significantly lower processing speed (t(21) = 2.463; P = 0.023; Cohen's d = 1.028). Groups did not differ in theta network topology measures. The paediatric autoimmune encephalitis group had a significantly lower delta local efficiency across all thresholds tested (d = -1.60 at network threshold 14%). Theta modularity was associated with lower working memory (β = -0.781; t(8) = -2.588, P = 0.032); this effect did not survive correction for multiple comparisons (P(corr) = 0.224). Magnetoencephalography was able to capture specific network alterations in paediatric autoimmune encephalitis patients. This preliminary study demonstrates that magnetoencephalography is an appropriate tool for assessing children with paediatric autoimmune encephalitis and could be associated with cognitive outcomes.

Life-Threatening MOG Antibody-Associated Hemorrhagic ADEM With Elevated CSF IL-6.

Acute disseminated encephalomyelitis (ADEM) is one characteristic manifestation of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). A previously healthy man presented with retro-orbital headache and urinary retention 14 days after Tdap vaccination. Brain and spine MRI suggested a CNS demyelinating process. Despite treatment with IV steroids, he deteriorated, manifesting hemiparesis and later impaired consciousness, requiring intubation. A repeat brain MRI demonstrated new bilateral supratentorial lesions associated with venous sinus thrombosis, hemorrhage, and midline shift. Anti-MOG antibody was present at a high titer. CSF IL-6 protein was >2,000 times above the upper limits of normal. He improved after plasma exchange, then began monthly treatment alone with anti-IL-6 receptor antibody, tocilizumab, and has remained stable. This case highlights how adult-onset MOGAD, like childhood ADEM, can rapidly become life-threatening. The markedly elevated CSF IL-6 observed here supports consideration for evaluating CSF cytokines more broadly in patients with acute MOGAD.

Imaging Biomarkers for Differential Diagnosis of Central Nervous System Inflammatory Diseases

Inflammatory diseases of the central nervous system (CNS) include multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and acute disseminated encephalomyelitis. In addition, various rheumatoid autoimmune diseases, autoimmune encephalitis, and medications such as immune checkpoint inhibitors can cause inflammation in the CNS. Radiological findings are very useful for us in differentiating inflammatory diseases of the CNS. On MRI, the shape, location, length/size, and gadolinium enhancement pattern of the lesions as well as the recovery and atrophy of the lesions following the acute phase, serve as important biomarkers for differentiating the diseases. In this review, we will highlight the typical characteristics MRI of optic neuritis, brain lesions, and myelitis in MS, NMOSD, and MOGAD, and outline the features of other inflammatory diseases of CNS.

Imaging Biomarkers for Differential Diagnosis of Central Nervous System Inflammatory Diseases

Inflammatory diseases of the central nervous system (CNS) include multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and acute disseminated encephalomyelitis. In addition, various rheumatoid autoimmune diseases, autoimmune encephalitis, and medications such as immune checkpoint inhibitors can cause inflammation in the CNS. Radiological findings are very useful for us in differentiating inflammatory diseases of the CNS. On MRI, the shape, location, length/size, and gadolinium enhancement pattern of the lesions as well as the recovery and atrophy of the lesions following the acute phase, serve as important biomarkers for differentiating the diseases. In this review, we will highlight the typical characteristics MRI of optic neuritis, brain lesions, and myelitis in MS, NMOSD, and MOGAD, and outline the features of other inflammatory diseases of CNS.

A Case Report and Literature Review of New-Onset Myasthenia Gravis after COVID-19 Infection

Myasthenia gravis (MG) is an autoimmune disorder affecting the neuromuscular junction caused by a B-cell-mediated, T-cell-dependent immunologic attack at the end plate of the postsynaptic membrane. Attack on muscle acetylcholine receptors (AChR) of the postsynaptic membrane due to the AChR, muscle-specific tyrosine kinase, or lipoprotein receptor-related peptide 4 antibodies lead to symptoms of painless, fluctuating weakness of muscle groups and often begins with ocular signs and symptoms. Coronavirus disease 2019 (COVID-19) is an acute respiratory disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a novel coronavirus closely related to SARS-CoV. Serious neurologic complications are infrequent and diverse with reported cases of stroke, encephalitis/meningitis, Guillain-Barré syndrome, acute disseminated encephalomyelitis, ataxia, and unspecified limb weakness. MG is a rarely reported sequela of COVID-19 infection. To date, there are 15 reported cases of post-COVID-19 MG. In this article, we present a case of post-COVID-19 MG and a concise review of other reported cases. An 83-year-old Caucasian male with a medical history of atrial fibrillation status post-ablation and non-ischemic cardiomyopathy was initially admitted for COVID-19 pneumonia. He was treated with remdesivir, convalescent plasma, and supplemental oxygen therapy but did not require invasive mechanical intubation. One month after discharge, he started experiencing fatigue with muscle weakness and progressive dyspnea. He progressed to develop dysphonia, especially at the end of the day. After extensive workup, he was diagnosed with MG with a positive antibody against the AChR. The chronological events of developing slowly worsening muscular weakness after recovering from COVID-19 infection and positive AChR antibody led to the diagnosis of post-COVID-19 new-onset MG. Post-COVID-19 fatigue, long-term use of steroids, and intensive care unit-related physical deconditioning ....

Genetic Characterization and Pathology of Porcine Teschovirus (PTV) in India

Background: The porcine teschovirus (PTV) is common swine pathogen which causes a wide range of illnesses ranging from asymptomatic infection to acute fatal encephalomyelitis, diarrhoea and pneumonia. Despite of its economical importance very limited studies are available on the pathology of PTV. The present study was conducted with the objective to investigate the PTV infection and associated pathology in piglets. Methods: A total of 78 piglets below 3 months of age were necropsied and representative tissue samples along with intestinal content were collected for histopathological examination and molecular investigation during April 2019 to November 2020. The 5' UTR region of PTV was amplified via RT-PCR and further confirmed by sequencing. Thereafter, genetic characterization of the Indian PTV isolate was done on the basis of 5' UTR gene. Result: A total of seven out of 78 cases were found positive for the PTV. On necropsy the intestinal wall was thinned and distended with yellowish coloured diarrhoeic content. Congestion of serosal and mucosal vessels along with severe meningeal congestion was observed. Microscopic lesions included congestion of mucosal vessels and destruction of villous structure of jejunum along with perivascular cuffing, focal gliosis, neuronophagia, congestion of meningeal and cerebral vessels and interstitial pneumonia. The phylogenetic analysis revealed that the isolate PTV/India/IVRI-381/2020 (MW022462) and PTV/India/IVRI-1093/2020 (MW022463) were clustered with PTV-13 strain wild boar/WB2C-TV/2011/HUN (JQ429405) and PTV-2 strain Vir 6711-12/83 (AF296107) of Hungary and Germany respectively. This study reported the genotypic and pathological investigation of PTV from Indian piglets. Further explorative surveillance along with studies in suitable cell lines and animal model will pave the way for better understanding of PTV among Indian pigs.

When Guillain–Barré Syndrome Defies Expectations: A Case of Atypical Features

Abstract Guillain–Barré syndrome (GBS) is one of the most common causes of acute flaccid paralysis. In typical GBS, there are no central nervous system (CNS) features. GBS with prominent CNS involvement can be seen in uncommon variants of GBS (such as Miller Fisher Syndrome and Bickerstaff brainstem encephalitis [BBE]) or rarely, when it coexists with other conditions such as acute disseminated encephalomyelitis (ADEM). We report a case of a 35-year-old male who presented with fever followed by ascending lower motor neuron type paraparesis suggestive of GBS which progressed rapidly to involve the respiratory muscles necessitating invasive mechanical ventilation. The patient had many atypical features, namely, persistent fever, early bladder and bowel involvement, facial twitching, ophthalmoplegia with ptosis, and a comatose state. Based on these clinical features and the investigations done, a diagnosis of GBS with BBE with a possibility of ADEM was made. The patient was managed intensively, but there was no improvement in the neurological manifestations and the patient succumbed to the illness due to ventilator-associated complications.

Long-term outcomes of ADEM-like and tumefactive presentations of CNS demyelination: a case-comparison analysis

A minority of initial multiple sclerosis (MS) presentations clinically or radiologically resemble other central nervous system (CNS) pathologies, acute disseminated encephalomyelitis (ADEM) or tumefactive demyelination (atypical demyelination presentations). With the aim of better defining the long-term outcomes of this group we have performed a retrospective cohort comparison of atypical demyelination versus ‘typical’ MS presentations. Twenty-seven cases with atypical presentations (both first and subsequent demyelinating events) were identified and compared with typical MS cases. Disease features analysed included relapse rates, disability severity, whole brain and lesion volumes, lesion number and distribution. Atypical cases represented 3.9% of all MS cases. There was considerable overlap in the magnetic resonance imaging (MRI) features of ADEM-like and tumefactive demyelination cases. ADEM-like cases tended to be younger but not significantly so. Atypical cases showed a trend towards higher peak expanded disability severity score (EDSS) score at the time of their atypical presentation. Motor, cranial nerve, cerebellar, cerebral and multifocal presentations were all more common in atypical cases, and less likely to present with optic neuritis. Cerebrospinal fluid (CSF) white cell counts were higher in atypical cases (p = 0.002). One atypical case was associated with peripheral blood myelin oligodendrocyte glycoprotein (MOG) antibodies, but subsequent clinical and radiological course was in keeping with MS. There was no difference in long-term clinical outcomes including annualised relapse rates (ARR), brain volume, lesion numbers or lesion distributions. Atypical demyelination cases were more likely to receive high potency disease modifying therapy early in the course of their illness. Despite the severity of initial illness, our cohort analysis suggests that atypical demyelination presentations do not confer a higher risk of long-term adverse outcomes.

An Insight into Acute Disseminated Encephalomyelitis.

Acute disseminated encephalomyelitis (ADEM), a neural and immune related state that occur when cerebrospinal system's damaged by extensive swelling. Although manifestation is possible no matter the age, adolescents have a greater probability that adults. The purpose of present manuscript is to provide recent advancement and enhance knowledge of the disease. The literature search on etiology, pathophysiology, diagnosis and treatment was carried out using the online database of Scifinder, Medline, Pubmed and GoogleScholar, Scopus etc. Result: Although the cause of ADEM remains unclear, it is believed to be caused by the inflammation in those with genetic sensitivity to an environmental stimulation. When people have altered levels of awareness or multifocal neurological abnormalities, ADEM is a possibility as a diagnosis. The diagnosis of ADEM is dependent on a combination of clinical, radiologic symptoms and the exclusion of illnesses that mimic ADEM; there is no one test that can establish the diagnosis. The inflammation in a child's brain and spinal cord is treated with medication. Prednisone will occasionally be given to youngsters for a brief amount of time. Most children with ADEM improve with high doses of methylprednisolone. Cyclophosphamide and hypothermia was need to individual. Most investigations show that 50%-75% of individuals completely recover between the first and sixth month of their condition.

Glial Fibrillary Acidic Protein Astrocytopathy: Review of Pathogenesis, Imaging Features, and Radiographic Mimics.

Glial fibrillary acidic protein (GFAP) astrocytopathy is a recently described autoimmune inflammatory disorder of the CNS characterized by the presence of specific antibodies targeting the intracellular filament protein in mature astrocytes. The pathogenesis is heterogeneous and poorly understood, with around 20%-34% of cases occurring as a paraneoplastic syndrome, most frequently associated with ovarian teratomas. It presents clinically as acute or subacute encephalomyelitis, and the diagnosis relies on imaging and detection of GFAP-Immunoglobulin (GFAP-IgG) in the CSF. Characteristic imaging findings include linear perivascular enhancement in the white matter extending in a radial pattern. Other imaging findings include periependymal enhancement, longitudinally extensive cord signal changes, intramedullary enhancement, optic neuritis, and papillitis. There is significant imaging overlap with other neuroinflammatory diseases like neuromyelitis optica spectrum disorder and lymphoproliferative conditions. GFAP astrocytopathy is characteristically responsive to steroids with, however, a significant rate of relapse. Currently, literature on this novel entity is limited with no established diagnostic criteria or standard treatment regimen. This comprehensive review explores the clinical, radiographic, and histopathologic aspects of GFAP astrocytopathy, shedding light on its complex nature and potential diagnostic challenges. The paper highlights the neuroimaging findings with a focus on differentiating GFAP astrocytopathy from other neuroinflammatory disorders.

Clinical, Demographic, and Radiological Characteristics of Patients Demonstrating Antibodies Against Myelin Oligodendrocyte Glycoprotein

Optic neuritis, myelitis, and neuromyelitis optica spectrum disorder (NMOSD) have been associated with antibodies against myelin oligodendrocyte glycoprotein-immunoglobulin G (anti-MOG-IgG). Furthermore, patients with radiological and demographic features atypical for multiple sclerosis (MS) with optic neuritis and myelitis also demonstrate antibodies against aquaporin-4 and anti-MOG-IgG. However, data on the diagnosis, treatment, follow-up, and prognosis in patients with anti-MOG-IgG are limited. To evaluate the clinical, radiological, and demographic characteristics of patients with anti-MOG-IgG. Multicenter, retrospective, observational study. Patients with blood samples demonstrating anti-MOG-IgG that had been evaluated at the Neuroimmunology laboratory at Ondokuz Mayıs University’s Faculty of Medicine were included in the study. Of the 104 patients with anti-MOG-IgG, 56.7% were women and 43.3% were men. Approximately 2.4% of the patients were diagnosed with MS, 15.8% with acute disseminated encephalomyelitis (ADEM), 39.4% with NMOSD, 31.3% with isolated optic neuritis, and 11.1% with isolated myelitis. Approximately 53.1% of patients with spinal involvement at clinical onset demonstrated a clinical course of NMOSD. Thereafter, 8.8% of these patients demonstrated a clinical course similar to MS and ADEM, and 28.1% demonstrated a clinical course of isolated myelitis. The response to acute attack treatment was lower and the disability was higher in patients aged > 40 years than patients aged < 40 years at clinical onset. Oligoclonal band was detected in 15.5% of the patients. For patients with NMOSD and without anti-NMO antibodies, the diagnosis is supported by the presence of anti-MOG-IgG. Furthermore, advanced age at clinical onset, Expanded Disability Status Scale (EDSS) score at clinical onset, spinal cord involvement, and number of attacks may be negative prognostic factors in patients with anti-MOG-IgG.

Background Rates of Adverse Events of Special Interest for COVID-19 Vaccine Safety Monitoring in South Korea

Objectives: This study aimed to estimate background incidence rates (IR) for adverse events of special interest (AESI) associated with COVID-19 vaccines in South Korea. Methods: We used data from the Korean National Health Insurance Service-National Sample Cohort from January 1, 2015 to December 31, 2019, with IR calculated annually. Eight AESIs including acute myocarditis, acute pericarditis, acute disseminated encephalomyelitis (ADEM), acute transverse myelitis (ATM), encephalitis, anaphylaxis, Bell’s palsy, and lymphadenitis were evaluated. Two operational strategies were implemented: a broad definition using ICD-10 codes for primary or secondary diagnoses to enhance sensitivity, and a narrow definition integrating ICD-10 codes with procedure and drug codes for enhanced accuracy and specificity. IR was calculated by dividing the number of incident cases by the total person-time at risk, with separate analyses by sex and age. Results: During the study period, the incidence of anaphylaxis, Bell’s palsy, and lymphadenitis under the broad definition continued to increase. Under the narrow definition, there were no reported cases of acute myocarditis or Bell’s palsy from 2015 to 2019. Additionally, the incidence rates for acute pericarditis, ADEM, ATM, encephalitis, anaphylaxis, and lymphadenitis were lower than those calculated under the broad definition. Males generally had higher rates, except for Bell’s palsy and lymphadenitis. Most AESIs were more common in individuals aged 65 and older, whereas ADEM and lymphadenitis were higher in children aged 0-19 years. Conclusions: Variability in AESI rates emphasizes the importance of considering definitions and demographics when establishing background rates for surveillance, which is a critical aspect of monitoring vaccine safety.

Neurological findings associated with neuroimaging in COVID-19 Patients

Patients with COVID-19 and other corona virus infections have been documented as having a variety of neurologic symptoms. In this article, we conducted a thorough evaluation of the imaging results of individuals who had been identified as having neurological symptoms linked to coronavirus infections. The use of CT and MRI has revealed varying radiologic outcomes in the setting of various neurologic presentations. Despite the fact that the majority of patients have normal imaging analyses, certain patients exhibit intra- and extra-axial abnormalities. There have been reports of encephalomyelitis, meningitis, acute disseminated encephalomyelitis (ADEM), ischemic and hemorrhagic strokes, and encephalopathy. In especially during the present COVID-19 pandemic, familiarity with these radiologic patterns can help radiologists and referring doctors investigate coronavirus infections in patients with worsening or progressing neurologic symptoms.

Closing the Diagnostic Gap in Encephalitis and Acute Disseminated Encephalomyelitis through Digital Case Classification and Viral Metagenomics

Encephalitis and acute disseminated encephalomyelitis (ADEM) are often caused or triggered by viruses—but the specific pathogen commonly remains unidentified in routine care. We explored the use of viral metagenomic next-generation sequencing (mNGS) in addition to PCR testing of non-invasive stool samples to see if unbiased testing could potentially increase diagnostic yield. To identify specific clinical cases at the point of care, we took advantage of a previously published digital app allowing instant clinical case classification based on consensus case criteria, the VACC-Tool. This hospital-based prospective digital surveillance program assessed 100 pediatric patients (mean age: 11 years, range: 0.15–17.85; 49% male) with case-confirmed encephalitis and/or ADEM. Analysis of case classification at the point of care revealed that in routine care, 96% of confirmed encephalitis/ADEM cases had been missed. Overall agreement of routine care diagnoses with digital encephalitis/ADEM case classification was &lt;50%. Also in routine care, only 13% of cases held a virus-related diagnosis, i.e., herpesvirus (n = 8) and enterovirus infection (n = 5). Use of mNGS increased the yield of virus detection by 77% (n = 23 virus hits). Specifically, mNGS identified 10 additional virus species beyond herpes- and enteroviruses. Of the additional 23 virus hits detected with mNGS, PCR confirmation was possible post hoc in 14 cases (61%). Linking digital case classification, mNGS, and PCR testing may not be feasible in routine care at this point but may help to provide hints to the pathogenesis of encephalitis/ADEM in childhood, warranting further research and exploration.

Myelin Oligodendrocyte Glycoprotein-Antibody Associated Disease: An Updated Review of the Clinical Spectrum, Pathogenetic Mechanisms and Therapeutic Management.

Clinical syndromes associated with antibodies against myelin oligodendrocyte glycoprotein (MOG) are now recognized as a distinct neurological disease entity, and are gaining increasing attention. The pathogenic mechanisms underlying MOG-antibody disease (MOGAD) remain incompletely understood. Case series, facilitated by registries, and observational studies over the past few years have shed increasing light on the clinical aspects and therapeutic approaches of MOGAD. MOGAD may manifest with a variety of clinical syndromes, including acute disseminated encephalomyelitis (ADEM), autoimmune encephalitis, optic neuritis (ON) and transverse myelitis (TM). MOGAD can be either monophasic or relapsing. This review aims to provide a comprehensive updated description of the clinical spectrum, paraclinical features, and prognosis of MOG-antibody disease, as well as summarize its therapeutic considerations. Randomized clinical trials, standardized diagnostic criteria and treatment guidelines are the steps forward.