Incidence Of Acute Disseminated Encephalomyelitis Research Articles

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.

Association between seasonal influenza vaccines and the increased risk of acute disseminated encephalomyelitis, estimated using the Vaccine Adverse Event Reporting System.

Acute disseminated encephalomyelitis (ADEM) is a rare and immune-mediated inflammatory disorder of the central nervous system (CNS) that can be triggered by infections and vaccinations. To date, only anecdotal case studies have reported the association between ADEM incidence and seasonal influenza vaccines, and multiple studies have found no association. This study aimed to investigate the association between the incidence of ADEM and seasonal influenza vaccines in a real-world setting using data from the United States Vaccine Adverse Event Reporting System (VAERS). Further, propensity score matching and disproportionality analysis was performed by calculating the adjusted reporting odds ratio (ROR) of reported ADEM cases associated with seasonal influenza vaccines using multiple logistic regression. Additionally, we analysed the time-to-onset using Weibull shape parameters (WSPs). The VAERS database contained 390,352 adverse events reported from January 2011 to December 2020. The ROR of seasonal influenza vaccines for ADEM was 3.02 (95% confidence interval: 1.72-5.33). The median duration (interquartile range) of ADEM was 11.0 (5.0-33.0) days. The median duration of ADEM induced by egg culture-based influenza vaccine (Egg-based vaccine) and cell culture-based influenza vaccine (Cell-based vaccine) was 10.0 (5.0-24.0) and 91.0 (79.0-125.0) days (P < 0.001), respectively. Only Cell-based cases had WSP β > 1, indicating a wear-out failure type. The incidence of ADEM within 30 days after administration of egg- and Cell-based vaccines was 78.6% and 0.0%, respectively. Our findings indicate that ADEM incidence is associated with seasonal influenza vaccines; thus, careful monitoring of ADEM is required within the first month of Egg-based vaccination and after two months of Cell-based vaccination. Neurologists and general practitioners should exercise caution, as the timing for careful monitoring varies depending on the vaccine type.

The emerging spectrum of COVID-19 neurology: clinical, radiological and laboratory findings.

Preliminary clinical data indicate that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is associated with neurological and neuropsychiatric illness. Responding to this, a weekly virtual coronavirus disease 19 (COVID-19) neurology multi-disciplinary meeting was established at the National Hospital, Queen Square, in early March 2020 in order to discuss and begin to understand neurological presentations in patients with suspected COVID-19-related neurological disorders. Detailed clinical and paraclinical data were collected from cases where the diagnosis of COVID-19 was confirmed through RNA PCR, or where the diagnosis was probable/possible according to World Health Organization criteria. Of 43 patients, 29 were SARS-CoV-2 PCR positive and definite, eight probable and six possible. Five major categories emerged: (i) encephalopathies (n = 10) with delirium/psychosis and no distinct MRI or CSF abnormalities, and with 9/10 making a full or partial recovery with supportive care only; (ii) inflammatory CNS syndromes (n = 12) including encephalitis (n = 2, para- or post-infectious), acute disseminated encephalomyelitis (n = 9), with haemorrhage in five, necrosis in one, and myelitis in two, and isolated myelitis (n = 1). Of these, 10 were treated with corticosteroids, and three of these patients also received intravenous immunoglobulin; one made a full recovery, 10 of 12 made a partial recovery, and one patient died; (iii) ischaemic strokes (n = 8) associated with a pro-thrombotic state (four with pulmonary thromboembolism), one of whom died; (iv) peripheral neurological disorders (n = 8), seven with Guillain-Barré syndrome, one with brachial plexopathy, six of eight making a partial and ongoing recovery; and (v) five patients with miscellaneous central disorders who did not fit these categories. SARS-CoV-2 infection is associated with a wide spectrum of neurological syndromes affecting the whole neuraxis, including the cerebral vasculature and, in some cases, responding to immunotherapies. The high incidence of acute disseminated encephalomyelitis, particularly with haemorrhagic change, is striking. This complication was not related to the severity of the respiratory COVID-19 disease. Early recognition, investigation and management of COVID-19-related neurological disease is challenging. Further clinical, neuroradiological, biomarker and neuropathological studies are essential to determine the underlying pathobiological mechanisms that will guide treatment. Longitudinal follow-up studies will be necessary to ascertain the long-term neurological and neuropsychological consequences of this pandemic.

Does area postrema syndrome occur in myelin oligodendrocyte glycoprotein-IgG-associated disorders (MOGAD)?

Myelin oligodendrocyte glycoprotein-IgG is a biomarker associated with CNS demyelinating diseases.1,2 MOGAD and aquaporin-4-IgG–positive neuromyelitis optica spectrum disorder (AQP4-IgG + NMOSD) have overlapping clinical features including optic neuritis and myelitis. However, there are several clinical and radiologic distinguishing features of MOGAD including lack of female predominance, higher incidence of acute disseminated encephalomyelitis (ADEM), higher proportion of bilateral optic neuritis, and T2-signal confined to gray matter and conus involvement in myelitis.1,3,4

Nursing Interventions of Children with Acute Disseminated Encephalomyelitis

This paper aims to investigate the clinical features and nursing interventions of children with acute disseminated encephalomyelitis (ADEM). By randomly selecting the children with ADEM treated in our hospital as the study subjects, the relevant clinical data of these patients was analysed and then their clinical characteristics were compared. According to the randomized table method, the patients were divided into control group and study group, where the control group was given routine nursing care, while the study group was given comprehensive care, so as to compare and analyse the effects of the two groups. The results show that the incidence of ADEM in children has no significant difference in the four seasons, and the predisposing factors are related to respiratory tract infection, viral infection, gastrointestinal tract infection and vaccination. In terms of clinical typing, it includes brain type and cerebrospinal type. The clinical manifestations of ADEM are fever, limb spasms, and convulsions. Therefore, the clinical symptoms of ADEM are rather clear, and the timely symptomatic treatment and assisted effective nursing intervention can improve the therapeutic effect.

Multiple sclerosis in Belgian children: Amulticentre retrospective study.

Although the diagnosis of multiple sclerosis (MS) in the paediatric population remains challenging, paediatric-onset MS is increasingly recognized worldwide. We report on the clinical and biochemical features of a Belgian multicentre cohort of paediatric MS patients in a national retrospective descriptive study. Twenty one paediatric MS patients from four Belgian University Hospitals were included. In nine patients, onset of MS was before the age of ten years which makes the study cohort of special interest. We report a higher incidence of acute disseminated encephalomyelitis (ADEM)-like first MS attacks and an overall higher proportion of polysymptomatic episodes than in adult and most paediatric cohorts reported in the literature. The clinical presentation in our cohort was rather severe with high median EDSS-score during the first clinical manifestation and barely more than half of our study patients showing full recovery after their first clinical manifestation. Also, a significant proportion of children in our cohort has severe disease progression despite disease modifying therapy and 9.5% of patients showed transition to secondary progressive multiple sclerosis during adolescence. An early and correct diagnosis of paediatric MS is essential to start early adequate treatment. As illustrated by our study cohort, current treatment options in childhood are unsatisfactory.

Incidence of acute disseminated encephalomyelitis in the Jiangsu province of China, 2008-2011.

BackgroundIt is important to have an estimate of the incidence of acute disseminated encephalomyelitis (ADEM) because the incidence of ADEM is unknown and the outcomes undefined in China.ObjectivesThis study attempts to describe ADEM incidence in large Chinese populations located in four geographically different and moderately distant areas of the same province.MethodsA retrospective investigation was conducted with ADEM patients in Nanjing, Nantong, Yancheng and Xuzhou. The survey was carried out in regions that might have received patients meeting the case definition of ADEM provided by the International Pediatric MS Study Group from 2008 to 2011. A total of 125 hospitals were included and 412 patients were identified through the hospital information systems (HIS).ResultsThe incidence of ADEM was 0.32/100,000/year. There are two peaks on the age-specific ADEM rates curve. One is 0.77/100,000/year among 0- to 9-year-olds, the other is 0.45/100,000/year in those aged 50–59 years. The incidence rate found for ADEM in males was 0.34/100,000/year, and in females was 0.29/100,000/year. The highest incidence rate was in Nanjing (0.40/100,000/year).ConclusionsThe average annual incidence of ADEM was 0.32/100,000/year. The peak age of onset was 50–59 years old and 0–9 years old. The incidence among males was insignificantly higher than that among females. There was no significant difference in incidence by seasonal variation.

Review on domestic published papers regarding the acute disseminated encephalomyelitis and its relations with rabies vaccination

To explore whether the case of acute disseminated encephalomyelitis (ADEM) who had been inoculated with rabies vaccination reported in domestic literatures were related to the vaccination. Data on 1368 cases of ADEM (from 99 papers) were collected by computer from the Chinese Journal Full-text Database in CNKI and Wanfang database with the key words of acute disseminated encephalomyelitis/rabies vaccination/abnormal reaction in preventive vaccination during 1995 and 2011. All the cases were divided into the two queues according to the situation of rabies vaccination. Cohort study was used to analyze the relations between ADEM and vaccination. The incidence of ADEM, standardized by age that having received immunization with rabies vaccine, was 9.41/1 million person year, higher than 0.29/1 million person year (u = 428.35, P < 0.01) among those who had not. Among people who had been inoculated, the adjusted RR value of cause ADEM was 31.05 (95%CI: 26.33 - 36.62). RR values increased along with the times of rabies vaccine injection. Correlation was seen between inoculated rabies vaccine and ADEM.

Decompressive Hemicraniectomy for Acute Disseminated Encephalomyelitis: Case Report

Acute disseminated encephalomyelitis (ADEM) varies widely in symptoms and severity. Some cases are associated with massive life-threatening cerebral edema refractory to conventional medical management. A 51-year-old woman with ADEM who developed severe brain swelling and herniation despite aggressive medical management is described. A decompressive hemicraniectomy and durotomy led to rapid improvement and an excellent outcome. This case report reinforces the place of this procedure in the armamentarium of treatment options for patients with medically refractory brain swelling and elevated intracranial pressure caused by ADEM. The potential for an increase in the incidence of ADEM with more frequent smallpox vaccinations emphasizes the significance of redefining the full range of management options for this treatable disease.

Acute disseminated encephalomyelitis in childhood: epidemiologic, clinical and laboratory features.

Acute disseminated encephalomyelitis (ADEM) is a central nervous system demyelinating disease that usually follows an apparently benign infection in otherwise healthy young persons. The epidemiology, infectious antecedents and pathogenesis of ADEM are poorly characterized, and some ADEM patients are subsequently diagnosed with multiple sclerosis (MS). We retrospectively (1991-1998) and prospectively (1998-2000) studied all persons aged < 20 years diagnosed with ADEM from the 3 principal pediatric hospitals in San Diego County, CA, during 1991-2000. Acute neurologic abnormalities and imaging evidence of demyelination were required for study inclusion. Epidemiologic variables, risk factors, clinical course, laboratory and radiographic findings, neuropathology and treatment data were analyzed. Interleukin (IL)-12, interferon-gamma (IFN-gamma) and IL-10 were assayed in blinded manner on cerebrospinal fluid (CSF) obtained prospectively from a subset of ADEM cases and compared with CSF from patients with enteroviral (EV) meningoencephalitis confirmed by polymerase chain reaction (PCR) and controls without pleocytosis. Data were analyzed on 42 children and adolescents diagnosed with ADEM during 1991-2000, and CSF IL-12, IFN-gamma and IL-10 levels were compared among ADEM (n = 14), EV meningoencephalitis (n = 14) and controls without pleocytosis (n = 28). Overall incidence of ADEM was 0.4/100,000/year; incidence quadrupled during 1998-2000 compared with earlier years. No gender, age stratum, ethnic group or geographic area was disproportionately affected. A total of 4 (9.5%) patients initially diagnosed with ADEM were subsequently diagnosed with MS after multiple episodes of demyelination. Although most children eventually recovered, 2 died, including 1 of the 3 ultimately diagnosed with MS. Magnetic resonance imaging was required for diagnosis among 74% of patients; computerized tomography findings were usually normal. Patients with EV had significantly higher mean CSF IFN-gamma (P = 0.005) and IL-10 (P = 0.05) than patients with ADEM and controls without CSF pleocytosis. CSF from ADEM patients had CSF cytokine values statistically similar to those of 3 patients subsequently diagnosed with MS. ADEM is a potentially severe demyelinating disorder likely to be increasingly diagnosed as more magnetic resonance imaging studies are performed on patients with acute encephalopathy. Further characterization of the central nervous system inflammatory response will be needed to understand ADEM pathogenesis, to improve diagnostic and treatment strategies and to distinguish ADEM from MS.