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Brain Communications 2023Acute Flaccid Myelitis is a paralytic illness with significant similarities to poliomyelitis, and which affects predominantly children. It was first fully delineated...
Acute Flaccid Myelitis is a paralytic illness with significant similarities to poliomyelitis, and which affects predominantly children. It was first fully delineated only in 2014 in the USA, occurring in epidemic clusters with a likely overall increasing incidence. It has subsequently rapidly been identified in Europe, the UK, and Australasia and the Far East, confirming it to be an emerging, global, infectious neurological disease. It has, however, been very little studied in low- and middle-income countries-reflecting partly of the global imbalance in science and medical research, and partly the extremely low provision of neurological care in most low- and middle-income countries: Uganda currently has no specialized neurology services outside the capital Kampala. During extended visits over a 2-year period with involvement in acute adult and paediatric internal medicine, one of us (NS) encountered at least six new patients with acute flaccid myelitis, suggesting that both the geographical reach and the frequency of the disorder may be significantly greater than previously thought. Here, these cases are described together with their clinical features and, where available, course and (limited) investigation results. These observations have significant implications concerning the current, and potentially the future geographical spread of the disease, and its clinical phenomenology. In addition, they highlight serious problems concerning the global applicability of the current Acute Flaccid Myelitis diagnostic criteria.
PubMed: 37860825
DOI: 10.1093/braincomms/fcad246 -
Cureus Aug 2023Idiopathic inflammatory demyelinating diseases of the central nervous system (IIDCDs) are wide-ranging disorders due to their similarities and differences. In order to...
BACKGROUND AND OBJECTIVES
Idiopathic inflammatory demyelinating diseases of the central nervous system (IIDCDs) are wide-ranging disorders due to their similarities and differences. In order to address these conditions, studying their characteristics is essential. The endpoints of our study were to assess the incidence, presenting features, MRI findings, and predictors of disease progression of prevalent demyelinating disorders.
MATERIAL AND METHODS
This prospective, observational study was conducted at Srirama Chandra Bhanja (SCB) Medical College and Hospital, India, from August 2018 to November 2021. Individuals of 18-65 years of age with common demyelinating disorders were assessed at baseline, six, 12, and 24 months. Univariate and multivariate analyses were performed for the assessment of predictors. We used R software (version 4.2.1; R Foundation for Statistical Computing, Vienna, Austria) for data analysis.
RESULTS
Two hundred twenty (79%) of 278 enrolled participants completed this study. The mean age of the study population was 52.3±11.4 years. One hundred thirty-eight (63%) of them were males. The most common IIDCD in our study was neuromyelitis optica spectrum disorder (NMOSD: 87, 39.5%), followed by multiple sclerosis (MS: 72, 32.7%), acute transverse myelitis (ATM: 35, 15.9%), and acute disseminated encephalomyelitis (ADEM: 26, 11.8%). The univariate analysis revealed that male gender, diabetes mellitus, and history of smoking or alcoholism were significant predictors of the disease progression.
CONCLUSION
The IIDCDs were polysymptomatic at the initial presentation. Male diabetics are more prone to progressive disorders. However, multivariate analysis did not provide statistically significant results.
PubMed: 37731435
DOI: 10.7759/cureus.43775 -
Autoimmune glial fibrillary acidic protein astrocytopathy: clinical analysis and review of 15 cases.Acta Neurologica Belgica Aug 2023To review clinical characteristics, auxiliary examination results, treatment effects, and outcomes of patients with autoimmune glial fibrillary acidic protein...
BACKGROUND
To review clinical characteristics, auxiliary examination results, treatment effects, and outcomes of patients with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A).
METHODS
We collated and retrospectively analyzed clinical data of 15 patients admitted with clinical characteristics of an autoimmune GFAP-A acute encephalitis or meningitis phenotype.
RESULTS
All patients were diagnosed with acute-onset meningoencephalitis and meningoencephalomyelitis. Initial presentations included pyrexia and headache at onset; dual symptoms of prominent tremor with urinary and bowel dysfunction; ataxia, psychiatric and behavioral abnormalities, and impaired consciousness; neck resistance; reduced extremity muscle strength; blurred vision; epileptic seizures; and reduced basic blood pressure. Cerebrospinal fluid (CSF) examination showed that the degree of protein elevation was significantly higher than the degree of increase in white blood cells. Moreover, in the absence of obvious low chloride and glucose levels, CSF chloride levels decreased in 13 patients, accompanied by a CSF glucose level decrease in four. Brain abnormalities were found in magnetic resonance imaging of ten patients, with a linear radial perivascular enhancement present in the lateral ventricles of two patients and symmetric abnormalities in the splenium of the corpus callosum in three patients.
CONCLUSIONS
Autoimmune GFAP-A may be a spectrum disorder, with acute- or subacute-onset meningitis, encephalitis, and myelitis being the main phenotypes. When used for acute stage treatment, combined hormone and immunoglobulin therapy was superior to hormone pulse therapy or immunoglobulin pulse therapy alone. However, hormone pulse therapy alone without immunoglobulin pulse therapy was associated with a greater number of remaining neurological deficits.
Topics: Humans; Autoimmune Diseases of the Nervous System; Glial Fibrillary Acidic Protein; Retrospective Studies; Chlorides; Encephalitis; Meningitis; Immunoglobulins; Glucose; Autoantibodies
PubMed: 37079256
DOI: 10.1007/s13760-023-02268-0 -
Emerging Infectious Diseases Mar 2024Surveillance for emerging pathogens is critical for developing early warning systems to guide preparedness efforts for future outbreaks of associated disease. To better... (Review)
Review
Surveillance for emerging pathogens is critical for developing early warning systems to guide preparedness efforts for future outbreaks of associated disease. To better define the epidemiology and burden of associated respiratory disease and acute flaccid myelitis (AFM), as well as to provide actionable data for public health interventions, we developed a multimodal surveillance program in Colorado, USA, for enterovirus D68 (EV-D68). Timely local, state, and national public health outreach was possible because prospective syndromic surveillance for AFM and asthma-like respiratory illness, prospective clinical laboratory surveillance for EV-D68 among children hospitalized with respiratory illness, and retrospective wastewater surveillance led to early detection of the 2022 outbreak of EV-D68 among Colorado children. The lessons learned from developing the individual layers of this multimodal surveillance program and how they complemented and informed the other layers of surveillance for EV-D68 and AFM could be applied to other emerging pathogens and their associated diseases.
Topics: Child; Humans; Enterovirus D, Human; Colorado; Prospective Studies; Retrospective Studies; Wastewater; Wastewater-Based Epidemiological Monitoring; Respiratory Tract Diseases; Myelitis; Neuromuscular Diseases; Central Nervous System Viral Diseases
PubMed: 38407198
DOI: 10.3201/eid3003.231223 -
Frontiers in Neurology 2024The Neuromyelitis Optica Spectrum Disorders (NMOSD) constitute a spectrum of rare autoimmune diseases of the central nervous system characterized by episodes of... (Review)
Review
The Neuromyelitis Optica Spectrum Disorders (NMOSD) constitute a spectrum of rare autoimmune diseases of the central nervous system characterized by episodes of transverse myelitis, optic neuritis, and other demyelinating attacks. Previously thought to be a subtype of multiple sclerosis, NMOSD is now known to be a distinct disease with unique pathophysiology, clinical course, and treatment options. Although there have been significant recent advances in the diagnosis and treatment of NMOSD, the field still lacks clinically validated biomarkers that can be used to stratify disease severity, monitor disease activity, and inform treatment decisions. Here we review many emerging NMOSD biomarkers including markers of cellular damage, neutrophil-to-lymphocyte ratio, complement, and cytokines, with a focus on how each biomarker can potentially be used for initial diagnosis, relapse surveillance, disability prediction, and treatment monitoring.
PubMed: 38817544
DOI: 10.3389/fneur.2024.1415535 -
Internal Medicine (Tokyo, Japan) Oct 2023A 33-year-old woman developed paresthesia in her right thumb approximately 30 minutes after receiving the BNT162b2 (Pfizer-BioNTech) severe acute respiratory syndrome...
A 33-year-old woman developed paresthesia in her right thumb approximately 30 minutes after receiving the BNT162b2 (Pfizer-BioNTech) severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccine. The paresthesia gradually spread to her right-side limbs and trunk, and cervical magnetic resonance imaging (MRI) revealed a localized lesion in the right dorsal column. After glucocorticoid therapy, her symptoms and MRI findings improved. Although disease developing less than 24 hours after vaccination is considered an unlikely cause of immuno-associated adverse events following vaccination, we discuss the possible mechanisms involved in early-onset central nervous system inflammation after vaccination in view of preexisting immunopathological susceptibility.
Topics: Adult; Female; Humans; BNT162 Vaccine; COVID-19; COVID-19 Vaccines; Myelitis; Paresthesia; SARS-CoV-2; Vaccination
PubMed: 37532545
DOI: 10.2169/internalmedicine.2339-23 -
Frontiers in Neurology 2023Neuromyelitis optica spectrum disorder (NMOSD) is a devastating inflammatory disease of the central nervous system that is often severely disabling from the outset. The...
BACKGROUND
Neuromyelitis optica spectrum disorder (NMOSD) is a devastating inflammatory disease of the central nervous system that is often severely disabling from the outset. The lack of pathognomonic aquaporin 4 (AQP4) antibodies in seronegative NMOSD not only hinders early diagnosis, but also limits therapeutic options, in contrast to AQP4 antibody-positive NMOSD, where the therapeutic landscape has recently evolved massively.
CASE PRESENTATION
We report a 56-year-old woman with bilateral optic neuritis and longitudinally extensive myelitis as the index events of a seronegative NMOSD, who was successfully treated with inebilizumab.
CONCLUSION
Treatment with inebilizumab may be considered in aggressive seronegative NMOSD. Whether broader CD19-directed B cell depletion is more effective than treatment with rituximab remains elusive.
PubMed: 38073644
DOI: 10.3389/fneur.2023.1297341 -
The Lancet. Infectious Diseases May 2024With continued wild poliovirus transmission in Afghanistan and Pakistan and circulating vaccine-derived poliovirus in certain countries, there exists an ongoing risk of... (Review)
Review
With continued wild poliovirus transmission in Afghanistan and Pakistan and circulating vaccine-derived poliovirus in certain countries, there exists an ongoing risk of importation of polioviruses into other countries, including those that have been polio-free for decades. Diversifying the poliovirus outbreak response toolkit is essential to account for different public health and epidemiological contexts. In this Personal View, we discuss data on intestinal and pharyngeal mucosal immunity induced by inactivated poliovirus vaccine (IPV), previous programmatic experience of poliovirus outbreak response with IPV, and outbreak response guidelines in countries that exclusively use IPV. With recent reports of poliovirus detection in polio-free countries such as the USA and the UK, it is important to assess the interplay of virus transmission dynamics, vaccine impact on preventing paralysis and virus spread, and regulatory complexities of using oral poliovirus vaccine (OPV) and IPV options for outbreak response. As the global eradication programme navigates through cessation of routine OPV use with replacement by IPV and stockpiling of novel OPVs, clarity on the impact of IPV use will be important for informed decision making by global, regional, and national policy makers.
Topics: Humans; Poliomyelitis; Disease Outbreaks; Poliovirus Vaccine, Inactivated; Poliovirus; Poliovirus Vaccine, Oral; Global Health; Disease Eradication
PubMed: 38012892
DOI: 10.1016/S1473-3099(23)00505-4 -
Neurology Aug 2023Hemorrhage in the setting of myelitis is rarely seen in clinical practice. We report a series of 3 women aged 26, 43, and 44 years, who presented with acute hemorrhagic...
Hemorrhage in the setting of myelitis is rarely seen in clinical practice. We report a series of 3 women aged 26, 43, and 44 years, who presented with acute hemorrhagic myelitis within 4 weeks of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Two required intensive care, and 1 had severe disease with multiorgan failure. Serial MRI of the spine demonstrated T2-weighted hyperintensity with T1-weighted postcontrast enhancement in the medulla and cervical spine (patient 1) and thoracic spine (patients 2 and 3). Hemorrhage was identified on precontrast T1-weighted, susceptibility-weighted, and gradient echo sequences. Distinct from typical inflammatory or demyelinating myelitis, clinical recovery was poor in all cases, with residual quadriplegia or paraplegia, despite immunosuppression. These cases highlight that although hemorrhagic myelitis is rare, it can occur as a post/parainfectious complication of SARS-CoV-2 infection.
Topics: Humans; Female; COVID-19; SARS-CoV-2; Myelitis; Magnetic Resonance Imaging; Hemorrhage
PubMed: 36990723
DOI: 10.1212/WNL.0000000000207213 -
Cureus Dec 2023Osteomyelitis, a severe bone infection, poses a multifaceted challenge to healthcare professionals. While its pathophysiology and treatment have been extensively... (Review)
Review
Osteomyelitis, a severe bone infection, poses a multifaceted challenge to healthcare professionals. While its pathophysiology and treatment have been extensively studied, the impact of osteomyelitis on skeletal growth, particularly in pediatric patients, is an area that warrants attention. This abstract highlights the significance of understanding and managing growth disturbances in osteomyelitis, providing key findings and recommendations for clinicians. Understanding growth disturbance in osteomyelitis is essential because it can lead to lifelong consequences for pediatric patients. The infection may affect the growth plate, leading to limb length discrepancies, angular deformities, and functional impairments. These complications not only diminish the quality of life but also pose a substantial economic burden on the healthcare system. Therefore, early recognition and intervention are crucial. Key findings indicate that the risk of growth disturbances in osteomyelitis is particularly high in pediatric patients due to the vulnerability of the growth plate. Timely diagnosis, appropriate management, and targeted interventions can mitigate the long-term sequelae of growth disturbances. These include utilizing advanced imaging techniques to assess the extent of growth plate involvement, optimizing antibiotic therapy, and employing surgical techniques like epiphysiodesis, guided growth, or corrective osteotomies. Additionally, fostering a multidisciplinary approach that involves orthopedic surgeons, infectious disease specialists, and pediatric endocrinologists is vital to achieving successful outcomes. Recommendations for managing growth disturbance in osteomyelitis encompass early detection, meticulous monitoring, and a tailored treatment plan. Healthcare providers should remain vigilant for signs of growth plate involvement in osteomyelitis patients, especially in the pediatric population. A thorough evaluation, including advanced imaging and clinical assessment, is essential for accurate diagnosis. Close collaboration between specialists to address the infection and its skeletal consequences is crucial. Furthermore, patient and family education plays a pivotal role in fostering compliance with the treatment regimen. In conclusion, understanding and managing growth disturbances in osteomyelitis is paramount, particularly in pediatric patients. The implications of growth plate involvement are significant, and timely intervention is essential to prevent lifelong consequences. By implementing a comprehensive approach that combines accurate diagnosis, multidisciplinary collaboration, and patient education, healthcare professionals can enhance the quality of life and well-being of those affected by this challenging condition.
PubMed: 38226077
DOI: 10.7759/cureus.50631