Acute Encephalomyelitis Research Articles

Inherited CD59 deficiency, where neurology and genetics intertwine

To describe the clinical phenotype of eight children diagnosed with CD59 deficiency and their ultimate neurological outcome. The data of our cases were extensively reviewed both clinical and ancillary tests; investigations included: neuroimaging, neurophysiological studies, and laboratory tests. All patients presented during early infancy with Guillain-Barre syndrome later they suffered repeated relapses leading to the diagnosis of chronic axonal neuropathy. Recurrent stroke and acute necrotizing encephalopathy were described, 2 patients in each group. One girl developed acute disseminated encephalomyelitis while one boy developed acute transverse myelitis. Overt hemolytic anemia requiring blood transfusion reported in six patients. Inherited CD59 deficiency is an autosomal recessive disorder which can have devastating neurological consequences. First line immunotherapy including intravenous immunoglobin, corticosteroids, and plasma exchange may have transient beneficial effect. Reports of targeted therapy with eculizumab might be lifesaving. Genetic counseling is crucial.

Attack phenotypes and disease course in pediatric MOGAD.

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an autoimmune demyelinating condition that affects children differently than adults. We performed a literature review to assess the presentation and clinical course of pediatric MOGAD. The most common initial phenotype is acute disseminated encephalomyelitis, especially among children younger than five years, followed by optic neuritis (ON) and/or transverse myelitis. Approximately one-quarter of children with MOGAD have at least one relapse that typically occurs within three years of disease onset and often includes ON, even if ON was not present at onset. Clinical risk factors for a relapsing course have not been elucidated.

Leber hereditary optic neuropathy presenting as bilateral visual loss and white matter disease.

Leber hereditary optic neuropathy (LHON) is a rare maternally inherited mitochondrial disorder that typically affects young male adults in their second and third decades of life. It usually manifests as painless, subacute, progressive, bilateral vision loss, with more than 90% of affected individuals losing their vision before age 50. Compared with other diseases that cause optic neuritis (multiple sclerosis or neuromyelitis optica spectrum disorders), LHON has worsening visual function in the first 6-12 months of disease progression, is predominantly male, the optic nerve is affected bilaterally from onset, there is no gadolinium enhancement on MRI, no response to disease-modifying therapy, and there is a family history of mutation in mitochondrial DNA. In this article, we describe an interesting and challenging case of LHON due to a homoplasmic variant in the MT -CO3 gene that was initially misdiagnosed as a monophasic demyelinating disorder (clinically isolated syndrome vs acute disseminated encephalomyelitis vs neuromyelitis optica spectrum disorders).

Exploring the Correlation Between Influenza A Virus (H3N2) Infections and Neurological Manifestations: A Scoping Review.

Influenza A virus (IAV), particularly the H3N2 variant, is known to cause respiratory manifestations, but it can also lead to neurological complications ranging from mild symptoms like headache and dizziness to severe conditions such as encephalitis and acute necrotizing encephalopathy (ANE). In this article, the correlation between the H3N2 variant of the IAVand neurological manifestations is discussed. Additionally, prompt recognition and treatment of influenza-associated neurological manifestations are highlighted to prevent infection-related long-term complications. This review briefly discusses various neurological complications linked to IAV infections, such as encephalitis,febrile convulsions, and acute disseminated encephalomyelitis, and the potential mechanisms involved in the development of neurological complications.

Post Malaria Neurological Syndrome: Unknown, Underestimated, or Underdiagnosed? First Report Done in Gambia

Post-malaria neurological syndrome (PMNS) is a rare self-limiting neurological complication that can occur after recovery from malaria, usually severe falciparum malaria. It is characterized by a myriad of neuropsychiatric manifestations including mild neurological deficit to severe encephalopathy. PMNS is a debated entity, is a rare complication of severe malaria that might be underreported. It can develop up to 2 months after clearance of parasitemia. Clinical features can be variable. Most cases are self-limited, but more severe cases may benefit from steroid therapy. There are several neurological syndromes that can occur following complete recovery from malaria, in particular Plasmodium falciparum. These PMNS include, delayed cerebellar ataxia (DCA), acute inflammatory demyelinating polyneuropathy (AIDP) and acute disseminated encephalomyelitis (ADEM). In 2021 according to WHO report, the African region was home to 95% of all malaria cases and 96% of deaths. Paradoxically, reports of post malaria neurological syndrome in west Africa sub region are practically null, it is not clear if it is due to lack of knowledge regarding the disease, underdiagnosis or combination of factors, the first case of post malaria neurological syndrome in Gambia is described below. The objective is to show that cases exist, but due to lack of knowledge by health personnel they are overwhelmingly underdiagnosed.

Pediatric B Cell Acute Lymphoblastic Leukemia presenting with Paraneoplastic Acute Disseminated Encephalomyelitis

Acute Disseminated Encephalomyelitis (ADEM) is a monophasic demyelinating disease most often triggered by infection or immunization, though associations with malignancy and stem cell transplant have been described. We described the case of a four-year-old boy with new-onset neurological symptoms associated with ADEM, acute leukemia, and equivocal evidence of Mycoplasma pneumoniae infection. He responded to a short course of antibiotics and chemotherapy for B cell acute lymphoblastic leukemia (B-ALL) that included a prolonged course of high dose steroids with immunosuppressive therapy. This case illustrated a possible association between paraneoplastic ADEM and leukemia in pediatric patients.

New Onset Multiple Sclerosis Post-COVID-19 Vaccination and Correlation With Possible Predictors in a Case-Control Study.

Various inflammatory diseases have been associated with the administration of various vaccines. Several reports have associated vaccine administration with the demyelinating diseases of the central nervous system (CNS). However, no clear or strong scientific evidence exists to support the association of vaccine administration with the onset of demyelinating diseases. Some CNS demyelination diseases such as acute disseminated encephalomyelitis (ADEM) and neuromyelitis optica spectrum disorders (NMOSD) were reported following the administration of COVID-19 vaccines. In this study, new onset multiple sclerosis (MS) following COVID-19 vaccine administration was reported. In this longitudinal observational case-control study, a total of 65 participants were studied, who were divided into two groups. Group A included 32 MS patients who were diagnosed post-COVID-19 vaccine administration and group B included 33 participants who received COVID-19 vaccines and did not develop MS. Group B was used as a control. The Chi-square test and logistic regression analysis were carried out using Statistical Product and Service Solutions (SPSS) (IBM SPSS Statistics for Windows, Armonk, NY)software. Univariate and multivariate logistic regression analysis was performed and a significant correlation between the risk factors and the development of MS post-COVID-19 vaccination was identified. The risk factors, identified in this study, can be used as significant independent predictors for developing MS post-COVID-19 vaccinations.

Acute Disseminated Encephalomyelitis (ADEM) Following Completion of Pneumococcal Meningitis Treatment: A Case Report and Review of the Literature

Acute Disseminated Encephalomyelitis (ADEM) Following Completion of Pneumococcal Meningitis Treatment: A Case Report and Review of the Literature

Epidemiological and clinical characteristics of myelin oligodendrocyte glycoprotein antibody-associated disease in a nationwide survey

Background: To our knowledge, no nationwide epidemiological study of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) has been conducted. Objective: We examined the epidemiology and clinical features of MOGAD in Japan. Methods: We distributed questionnaires on the clinical characteristics of patients with MOGAD to neurology, pediatric-neurology, and neuro-ophthalmology facilities throughout Japan. Results: In total, 887 patients were identified. The estimated number of total and newly diagnosed MOGAD patients was 1,695 [95% confidence interval (CI): 1483–1907] and 487 (95% CI: 414–560), respectively. The estimated prevalence and incidence were 1.34/100,000 (95% CI: 1.18–1.51) and 0.39/100,000 (95% CI: 0.32–0.44), respectively. The median age at onset was 28 years (range: 0–84 years). At onset, optic neuritis was present in approximately 40% of patients, irrespective of the onset age. Acute disseminated encephalomyelitis was more frequent in younger patients, whereas brainstem encephalitis, encephalitis, and myelitis were more frequent in elderly patients. Immunotherapy was highly effective. Conclusion: The prevalence and incidence rates of MOGAD in Japan are similar to those in other countries. Notable characteristics such as the preferential occurrence of acute disseminated encephalomyelitis in children exist; however, general characteristics including symptoms and treatment response are common irrespective of the onset age.

Clinical and Radiologic Features Among Children With Myelin Oligodendrocyte Glycoprotein Antibody-Associated Myelitis

BackgroundMyelin oligodendrocyte glycoprotein (MOG) antibody–associated disease (MOGAD) often manifests as optic neuritis, transverse myelitis(TM), and acute disseminated encephalomyelitis. Patients with a TM phenotype are at high risk for neurological sequelae, so recognizing the characteristics of MOG-IgG myelitis is essential for early, accurate diagnosis and treatment. MethodsThis was a single-center retrospective study. Pediatric MOG antibody–associated disease patients who had clinical myelitis were recruited for this study. Data on clinical and radiologic features and outcomes were retrospectively collected. ResultsThirty-four patients (age range: 6 months to 13 years; median age, 7 years; female, 16) were enrolled in this study. As one patient had two clinical episodes of myelitis, 35 episodes were included. Isolated transverse myelitis was the initial manifestation in 28 (82%) patients. The most frequent clinical features of MOG-IgG myelitis were weakness and neurogenic bladder, and 80% were better than wheelchair-dependent at the nadir. There was a high presentation of weakness (91%), bowel/bladder dysfunction (63%), and sensory dysfunction (46%), and 80% were better than wheelchair-dependent at the nadir. In addition, seven patients (20%) had radicular pain, and six had flaccid areflexia. Magnetic resonance imaging features were often longitudinally extensive (63%) and prominently involved gray matter (H-sign) (63%), accompanied by leptomeningeal enhancement (4/14.29%) and spinal root enhancement (6/14.43%). At the final follow-up (median, 28 months; range, 8-109 months), 10 patients (29%) had developed one or more relapses, spinal cord lesions resolved entirely in 11 of 22 children (50%), and none had appreciable spinal cord atrophy. At the final follow-up, most patients had favorable outcomes, with median (interquartile range) Expanded Disability Status Scale scores of 0 (range, 0-2), four patients (12%) had sphincter dysfunction, and one patient had gait problems. ConclusionsPediatric MOG-IgG myelitis clinically presents with weakness and bowel and bladder dysfunctions. Prominent involvement of the gray matter, leptomeningeal enhancement, and spinal root enhancement are common in pediatric MOG-IgG myelitis.

Encephalomyelitis in a patient with monkeypox: an unusual complication

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.

A young female with hypocomplementemic urticarial vasculitis associated with a rare CNS manifestation

This case report represents a rare case of 14-year-old female who diagnosed with hypocompementemic urticarial vasculitis syndrome that presents with glomerulonephritis, diffuse alveolar hemorrhage, and acute disseminated encephalomyelitis. The progression of the symptoms explained in the text below in which the final diagnosis was reached after a challenging approach. Patient was managed properly and followed up after treating with rituximab, although she represents no sign of the disease after a total of two cycles.

A Multidisciplinary Pediatric Neurology Clinic for Systematic Follow-up of Children with Neurologic Sequelae of COVID-19.

Clinical guidance on outpatient follow-up of children hospitalized with acute neurologic complications of SARS-CoV2 infection is needed. We describe the clinical infrastructure of our pediatric neurology post-Covid clinic, including our clinical evaluation and cognitive testing battery specific to this patient population, and a case series of our initial patient cohort. Our findings demonstrate cognitive sequelae in all 4 of our patients months following acute SARS-CoV2 infection with neurologic complications including acute disseminated encephalomyelitis, posterior reversible encephalopathy syndrome, viral encephalitis, and gait difficulties. Verbal and executive function domains were predominantly affected in our cohort, even in patients who did not endorse symptomatic or academic complaints at follow-up. Our recommendations include systematic clinical follow-up for children following hospitalization with SARS-CoV2 infection with a comprehensive cognitive battery to monitor for cognitive sequalae and to assist with developing an individualized education plan for the child as they return to school.

COVID-19 and autoimmune diseases of the nervous system - an update.

Due to a similar pathomechanism, COVID-19 infection may significantly affect the course of autoimmune diseases (AIDs). In our review, we aimed to assess the severity of SARS-CoV-2 infection, response to treatment, and the impact of COVID-19 infection on the course of the underlying disease in patients with neuroimmune diseases. In the time of the COVID-19 pandemic, it was important to determine the influence of COVID-19 infection on the course of autoimmune diseases due to the weakened immune system and immunosuppressive therapies. Many reports have indicated that in patients with AIDs, the existence of the disease is not associated with a worse prognosis in the course of the viral infection. Patients in advanced stages of the disease, elderly patients, and those with comorbidities are at risk of more frequent hospitalisations and higher mortality in the course of COVID-19. Moreover, some drugs used in AIDs have been tested for their efficacy in SARS-CoV-2 infection. Episodes of newly diagnosed myasthenia gravis, Guillain-Barré syndrome, acute disseminated encephalomyelitis (ADEM), and neuromyelitis optica spectrum disorder (NMOSD) secondary to COVID-19 or vaccination have also been reported. Vaccination against this pathogen is highly recommended in most patients with AIDs. Despite many studies on the association between COVID-19 and neuroimmune diseases, more specific data is needed. The approach to patients with AIDs should be individual, since many issues remain unresolved despite the long-lasting pandemic.

Pathophysiology of myelin oligodendrocyte glycoprotein antibody disease.

Myelin Oligodendrocyte Glycoprotein Antibody Disease (MOGAD) is a spectrum of diseases, including optic neuritis, transverse myelitis, acute disseminated encephalomyelitis, and cerebral cortical encephalitis. In addition to distinct clinical, radiological, and immunological features, the infectious prodrome is more commonly reported in MOGAD (37-70%) than NMOSD (15-35%). Interestingly, pediatric MOGAD is not more aggressive than adult-onset MOGAD, unlike in multiple sclerosis (MS), where annualized relapse rates are three times higher in pediatric-onset MS. MOGAD pathophysiology is driven by acute attacks during which T cells and MOG antibodies cross blood brain barrier (BBB). MOGAD lesions show a perivenous confluent pattern around the small veins, lacking the radiological central vein sign. Initial activation of T cells in the periphery is followed by reactivation in the subarachnoid/perivascular spaces by MOG-laden antigen-presenting cells and inflammatory CSF milieu, which enables T cells to infiltrate CNS parenchyma. CD4+ T cells, unlike CD8+ T cells in MS, are the dominant T cell type found in lesion histology. Granulocytes, macrophages/microglia, and activated complement are also found in the lesions, which could contribute to demyelination during acute relapses. MOG antibodies potentially contribute to pathology by opsonizing MOG, complement activation, and antibody-dependent cellular cytotoxicity. Stimulation of peripheral MOG-specific B cells through TLR stimulation or T follicular helper cells might help differentiate MOG antibody-producing plasma cells in the peripheral blood. Neuroinflammatory biomarkers (such as MBP, sNFL, GFAP, Tau) in MOGAD support that most axonal damage happens in the initial attack, whereas relapses are associated with increased myelin damage.

Central nervous system manifestations of dengue infection: data from a tertiary care Centre in South India.

Denguefever is the second most common mosquito-borne disease affecting human beings and neurological manifestations (NMs) of this arboviral infection are increasingly being reported. In this retrospective study, conducted in a tertiary care centre in south India, we sought to describe the spectrum of NMs of dengue fever. The records of all patients admitted in the Department of Internal Medicine over 8 years, with a diagnosis of dengue-based on clinical symptoms and the detection of dengue IgM antibodies or detection of NS1 antigen or nucleic acid by reverse transcriptase-polymerase chain reaction (RT-PCR); with Glasgow coma score (GCS) ≤14, neck stiffness, focal neurological signs, seizures, or visual disturbances-were explored. Of 1121 patients admitted with dengue fever, 341 (30%) had severe dengue and 23 (2%) had NMs. Encephalopathy was seen in 9 patients (39%), encephalitis in 6 (27%), cerebellitis in 4 (17%), acute disseminated encephalomyelitis (ADEM) in 2 (9%), ischaemic stroke in 1 (4%) patient, and neuro-ophthalmological manifestations in 1 (4%) patient. The median duration of hospital stay was 7 days. Nine (39% of) patients required intensive care unit (ICU) admission and 5 (22%) had fatal outcomes. There was no association between thrombocytopenia, metabolic acidosis, transaminitis, hyponatremia, the type of dengue infection, and the various NMs described. The NMs of dengue infection are varied, and a high index of suspicion is needed to identify them in patients who present with lethargy or altered sensorium on the background of an acute febrile illness suggestive of dengue.

A retrospective study on acquired demyelinating diseases in children

Background: Acquired demyelinating diseases (ADS) соnstitute а heterogeneous grоuр of central nervous system disorders of autoimmune origin that causes significant physical and cognitive disabilities. Early recognition and prompt management causes significant improvement in acute episodes of demyelinating disorders. Methods: 33 children diagnosed with demyelinating disorders at Lokmanya Tilal municipal medical college and hospital in Mumbai were enrolled. The study was conducted between January 2013 and November 2022. Demographic data, clinical profile, CSF study, serum antibody, radiological findings were collected and results were analyzed. Statistical Data was analysed using statistical software GraphPad in Stat.V3.0. Data were presented in tables and figures whenever needed. P value <0.05 considered as significant. Results: Of 33 patients, 21 (63%) were cases of acute disseminated encephalomyelitis (ADEM), 6 (18.1%) of transverse myelitis (TM), 1 case of ADEM + TM (3%), 3 (9%) of neuromyelitis optica, 1 (3%) of Optic neuritis and 1 (3%) of multiple sclerosis. ADEM patients presented with encephalopathy and multifocal neurological deficits, 40% were MOG positive. Two patients were of multiphasic ADEM. Patients of transverse myelitis had paraparesis or quadriparesis and sensory + bladder involvement. Patients with NMO presented with bilateral visual impairment with limb weakness and bladder involvement. Steroids were the primary treatment, 3 patients (9%) required intravenous immunoglobulin (IVIG) and 1 (3%) patient received plasma exchange therapy. Conclusions: ADEM is the most common ADS. Early diagnosis and management with steroids therapy improves outcome in most of the patients. Non response to steroids warrants second line treatment options like IV Immunoglobulin (IVIG) or Therapeutic plasma exchange.

Longitudinally extensive transverse myelitis after COVID-19 vaccination in a teenage girl

COVID-19 pandemic has led to the development of a variety of vaccines for protection against SARS-CoV-2 infection, with varying safety profiles and protective efficacy. As with other vaccination, vaccination against COVID-19 has also been found to be associated with various kinds of adverse events following immunization. While most adverse effects are mild, some serious side effects like transverse myelitis and acute disseminated encephalomyelitis have also been reported. However, these serious side effects have been reported in adults. To the best of our knowledge, there is no report of longitudinally extensive transverse myelitis (LETM) in the paediatric population, associated with this vaccination. We report here a case of a teenage girl, who was admitted to our hospital with the symptoms of LETM after receiving COVID-19 vaccination. Her diagnosis was based on history and, laboratory and imaging findings. She was discharged after a full recovery.

Differentiation of MOGAD in ADEM-like presentation children based on FLAIR MRI features.

The differences in magnetic resonance imaging (MRI) between children with classic acute disseminated encephalomyelitis (ADEM) and myelinal oligodendrocyte glycoprotein antibody associated disease (MOGAD) with ADEM-like presentation are controversial. The purpose of this study was to investigate whether the radiological characteristics of the MRI-FLAIR sequence can predict MOGAD in children with ADEM-like presentation and to further explore its imaging differences. We extracted 1041 radiomics features from MRI-FLAIR lesions. Then we used the redundancy analysis (Spearman correlation coefficient), significance test (student test or Mann-Whitney U test), least absolute contraction and selection operator (LASSO) to select potential predictors from the feature groups. The selected potential predictors and MOG antibody test results were used to fit the machine learning model for classification. Combined with feature selection and machine learning classifiers, the optimal model for each subgroup was derived. The resulting models have been evaluated using the receiver operator characteristic curve (ROC) at the lesion level and the model performance was evaluated at the case level using decision curve analysis. We retrospectively reviewed and re-diagnosed 70 ADEM-like presentation cases in our center from April 2015 to January 2020. Including 49 cases with classic ADEM and 21 cases with MOGAD. 30(43%) were female, with a median age of 5.3 years. On the four subgroups by age and gender, the area under the curve (AUC) of the optimal models were 89%, 90%, 98%, and 99%, and the MOGAD detection rates (Specificity) were 83%, 83%, 92%, and 75%, respectively. The machine learning model trained on radiomics features of MR-FLAIR images can effectively predict patients' MOGAD. This study provides a fast, objective, and quantifiable method for MOGAD diagnosis.

The epidemiology and burden of neuromyelitis optica spectrum disorder, multiple sclerosis, and MOG antibody-associated disease in a province in Thailand: A population-based study.

Central nervous system inflammatory demyelinating diseases (CNSIDDs) have notable interracial heterogeneity. The epidemiology of CNSIDDs in Thailand, a mainland Southeast Asian country, is unknown. To determine the cumulative incidence, point prevalence, and disease burden of neuromyelitis optica spectrum disorder (NMOSD) and other CNSIDDs in Thailand using population-based data of Chumphon. Searching for CNSIDD patients at a public secondary care hospital in Chumphon, the only neurology center in the province, from January 2016 to December 2021 was implemented using relevant ICD-10-CM codes. All diagnoses were individually ascertained by a retrospective chart review. Cumulative incidence, point prevalence, attack rate, mortality rate, and disability-adjusted life years (DALYs) were calculated. Aquaporin 4-IgG-positive NMOSD was the most prevalent CNSIDD in the Thai population at 3.08 (1.76-5.38) per 100,000 persons. The prevalence of multiple sclerosis (MS) followed at 0.77 (0.26-2.26) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) at 0.51(0.14-1.87) per 100,000 adults. In the pediatric population, the incidence of acute disseminated encephalomyelitis was 0.28 (0.08-1.02) per 100,000 persons/year. Among other idiopathic demyelinating diseases, idiopathic optic neuritis had the highest incidence at 0.58 (0.24-0.92) per 100,000 persons/year, followed by acute transverse myelitis at 0.44 (0.14-0.74). Idiopathic demyelinating brainstem syndrome was also observed at 0.04 (0.01-0.25) per 100,000 persons/year. Although most had a fair recovery, disability was worst among NMOSD patients with DALYs of 3.61 (3.00-4.36) years per 100,000 persons. Mortality rate was the highest in NMOSD as well. CNSIDDs are rare diseases in Thailand. The prevalence is comparable to that of East Asian populations. A nationwide CNSIDDs registry would better elaborate the epidemiology of these diseases.