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OBJECTIVES: To explore the challenges in diagnosing acute flaccid myelitis (AFM) and evaluate clinical features and treatment paradigms associated with under recognition. STUDY DESIGN: This was a retrospective multicenter study of pediatric patients (≤18 years) who were diagnosed with AFM from 2014 to 2018 using the Centers for Disease Control and Prevention's case definition. RESULTS: In 72% of the cases (126 of 175), AFM was not considered in the initial differential diagnosis (n = 108; 61.7%) and/or the patient was not referred for acute care (n = 90; 51.4%) at the initial clinical encounter, and this did not improve over time. Although many features of the presentation were similar in those initially diagnosed with AFM and those who were not; preceding illness, constipation, and reflexes differed significantly between the 2 groups. Patients with a non-AFM initial diagnosis more often required ventilatory support (26.2% vs 12.2%; OR, 0.4; 95% CI, 0.2-1.0; P = .05). These patients received immunomodulatory treatment later (3 days vs 2 days after neurologic symptom onset; 95% CI, -2 to 0; P = .05), particularly intravenous immunoglobulin (5 days vs 2 days; 95% CI, -4 to -2; P < .001). CONCLUSIONS: Delayed recognition of AFM is concerning because of the risk for respiratory decompensation and need for intensive care monitoring. A non-AFM initial diagnosis was associated with delayed treatment that could have a clinical impact, particularly as new treatment options emerge.
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Central Nervous System Viral Diseases , Enterovirus Infections , Myelitis , Neuromuscular Diseases , Child , Humans , Myelitis/diagnosis , Myelitis/therapy , Neuromuscular Diseases/diagnosis , Neuromuscular Diseases/therapy , Central Nervous System Viral Diseases/diagnosis , Central Nervous System Viral Diseases/therapy , Retrospective Studies , Enterovirus Infections/diagnosis , Enterovirus Infections/therapySubject(s)
Mpox (monkeypox) , Humans , Mpox (monkeypox)/epidemiology , Monkeypox virus , Disease Outbreaks , ResearchABSTRACT
Objectives: To analyze the symptoms and severity of coronavirus disease 2019 (COVID-19) in people with multiple sclerosis (pwMS) on disease-modifying therapies using data from the COVID-19 in multiple sclerosis (MS) Global Data Sharing Initiative dataset. Methods: The open-access COVID-19 in MS Global Data Sharing Initiative dataset was obtained through credentialed access using PhysioNet. The variables analyzed included BMI, symptoms of COVID-19, age, current use of disease-modifying therapy (DMT), efficacy of DMT, comorbidities, hospitalization status, and type of MS. A linear regression analysis was completed. Data analysis and visualization were completed using STATA v15, R-Studio v1.1.447, Python v3.8, and its associated libraries, including NumPy, Pandas, and Matplotlib. Results: A total of 1141 participants were included in the analysis. 904 women and 237 men were diagnosed with MS. Among the pwMS included in the study; 208 (19.54%) had a suspected infection with COVID-19 and only 49 (5.25%) were confirmed. Any COVID-19 symptom was present in 360 individuals. The commonly reported DMT agents included dimethyl fumarate (12.71%) and fingolimod (10.17%). 101 in total (8.85%) reported not using any DMT. Factors associated with hospitalization and/or admission to the ICU included having any comorbidity (P=0.01), neuromuscular disorder (P=0.046), hypertension (P=0.005), chronic kidney disease (P<0.001), and immunodeficiency (P=0.003). The type of MS, the duration of the disease, and high-efficacy DMT therapy did not have a statistically significant influence on hospitalization. Conclusion: This study underscores the importance of comorbidities, especially neuromuscular disorders, hypertension, chronic kidney disease, and immunodeficiencies, as possible prognostic indicators for worse outcomes of COVID-19 in pwMS. On the contrary, the type of MS, the duration of the disease, and the efficacy of disease-modifying therapy did not significantly affect the severity of the symptoms of COVID-19 in this cohort.
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Objectives: This study aimed to analyze the Vaccine Adverse Event Reporting System (VAERS) database and systematically review the literature to provide a comprehensive analysis of reversible cerebral vasoconstriction syndrome (RCVS) and posterior reversible encephalopathy syndrome (PRES) secondary to vaccination. Methods: The authors analyzed the VAERS database and conducted a systematic review following PRISMA guidelines. The inclusion criteria for VAERS data were a score of ≥3 on the RCVS2 score and/or radiographic findings consistent with the diagnosis of RCVS or PRES. The systematic review was registered with PROSPERO. Results: Our combined data set included 29 cases (9 RCVS and 20 PRES). Most cases were women (72.4%) with a mean age of 50.7 years (SD 19.4 years). Most cases were associated with COVID-19 mRNA vaccines (58.6% Moderna, 20.7% Pfizer). Hypertension (37.9%), hyperlipidemia (13.7%), chronic kidney disease (CKD) (10.3%), and end-stage renal disease (6.8%) were common comorbidities. Furthermore, 20.6% (6/29) of cases were on immunosuppression therapy for various reasons. The mean time to symptom onset was 10.49 days after vaccination (SD 18.60), and the mean duration of hospitalization was 7.42 days (SD 5.94). The symptoms reported the most frequently were headache (41.3%), elevated blood pressure (31.0%), and emesis (17.2%). Typical radiographic findings included T2/FLAIR hyperintensities affecting the parieto-occipital lobes, indicative of vasogenic and/or cytotoxic edema. Conclusions: This study provides a comprehensive analysis of postvaccine RCVS and PRES. Both disease states were seen most often in those with pre-existing risk factors such as female sex, age over 50, hypertension, renal disease, and immunosuppression. Vaccines and their associated immune response may cause endothelial dysfunction leading to cerebral vasospasm and loss of cerebral autoregulation. However, further research is required to understand the underlying pathophysiological mechanisms. Despite the associations found, the absolute risk of these syndromes remains extremely low compared to the immense benefits of vaccination.
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Neuromyelitis optica, an autoimmune inflammatory disorder affecting the central nervous system, can occur in a paraneoplastic context, although rare. We report an intriguing case of a 71-year-old woman with a history of triple-negative infiltrating ductal breast carcinoma, manifesting with paraneoplastic neuromyelitis optica that led to significant respiratory failure and required a cervical laminectomy. The patient presented with pain in the left breast, weakness in the lower extremities, and neck pain. The neurological evaluation showed 2/5 muscle strength in all extremities, diffuse hyperreflexia, and loss of multimodal sensation below the shoulder. She developed acute respiratory failure that required mechanical ventilation. Magnetic resonance imaging highlighted a diffuse abnormal increase in T2 signal intensity throughout the posterior and central portion of the cervical and thoracic spinal cord consistent with longitudinally extensive transverse myelitis, and significant cervical cord compression at C3-C4. Magnetic resonance imaging of the brain showed non-enhancing T2/fluid-attenuated inversion recovery (FLAIR) white matter hyperintensities and cerebellar hemispheres. The serum cell-based assay study demonstrated a high anti-aquaporin-4 immunoglobulin G titer (>1:160) confirming the diagnosis of neuromyelitis optica. She was taken for bilateral laminectomy from C3 to C6. Despite intravenous methylprednisolone and plasmapheresis treatment, no significant recovery was achieved, necessitating tracheostomy and percutaneous endoscopic gastrostomy. Subsequent rituximab treatment led to a mild improvement, with no new lesions on repeat magnetic resonance imaging. This case raises suspicion of the potential for neuromyelitis optica to occur as a paraneoplastic phenomenon, strengthening the need for vigilance in patients with malignancies.
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The coronavirus disease 2019 (COVID-19) pandemic has unveiled a wide array of clinical biomarkers, and neurological manifestations in affected patients, necessitating further exploration. Methods: This single-center retrospective study evaluated clinical and neurological sequelae, demographics, as well as laboratory markers, in hospitalized COVID-19 patients from January to September 2020. Results: Among 1248 inpatients (median age: 68 years; 651 women), 387 (31%) were admitted to the ICU. Central nervous system (CNS) manifestations were present in 521 (41.74%) patients, while peripheral nervous system manifestations were observed in 84 (6.73%). COVID-19-related mortality occurred in 314 (25.16%) cases. ICU-admitted patients were predominantly male (P<0.0001), older (age≥60; P=0.037) and had more comorbidities such as diabetes (P=0.001), hyperlipidemia (P=0.043), and coronary artery disease (P=0.015). ICU patients exhibited more CNS manifestations (P=0.001), including impaired consciousness (P<0.0001) and acute cerebrovascular disease (P=0.023). Biomarkers linked to admission to the ICU included elevated white blood cell count, ferritin, lactate dehydrogenase, creatine kinase, blood urea nitrogen, creatinine, and acute phase reactants (e.g. erythrocyte sedimentation rate and C-reactive protein). ICU patients demonstrated lower lymphocyte and platelet counts compared to non-ICU patients. Those with CNS involvement in the ICU often exhibited elevated blood urea nitrogen, creatinine, and creatine kinase levels. Higher mortality from COVID-19 was observed in ICU patients (P<0.0001). Conclusions: Multiple serum biomarkers, comorbidities, and neurological manifestations in COVID-19 patients have been consistently documented and may be linked to increased morbidity, ICU admission, and mortality. Recognizing and addressing these clinical and laboratory markers is essential for effective COVID-19 management.
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In the midst of the coronavirus disease 2019 (COVID-19) pandemic, intensive care units (ICUs) around the world have been pushed to their limits as they grapple with the effects of the severe acute respiratory syndrome coronavirus 2 virus. Identifying prognostic factors that influence mortality in COVID-19 patients admitted to the ICU could offer valuable insights for clinicians seeking to prevent disease progression. A retrospective analysis was conducted on COVID-19 patients admitted to the ICU between January and September 2020. The analysis considered patient demographics, comorbidities, neurological and non-neurological symptoms, as well as laboratory markers. The multivariate logistic regression analysis aims to uncover associations between these factors and patient outcomes. Of the 387 patients included in this study, nearly half (48.5%) of the ICU patients succumbed to COVID-19. Factors that contributed to increased mortality included being 60 years of age or older, impaired consciousness, lung disease, elevated international normalized ratio (INR), and elevated blood urea nitrogen (BUN) levels. Surprisingly, symptoms such as dizziness/lightheadedness, myalgia, and headache were associated with a higher likelihood of survival. In addition, elevated D-dimer and aspartate aminotransferase (AST) levels, as well as lymphopenia, were more commonly observed in deceased patients. The study concluded that those who died in the ICU tended to be older, white, and burdened with more comorbidities and impaired consciousness. With the intriguing link between specific symptoms and survival, further research is essential to uncover the underlying pathophysiological mechanisms that influence ICU patient outcomes in the context of COVID-19.
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COVID-19 , Humans , Retrospective Studies , Risk Factors , SARS-CoV-2 , Intensive Care UnitsABSTRACT
BACKGROUND AND OBJECTIVES: Multiple sclerosis (MS) prevalence varies widely by country and world region, variation that is often attributed to latitude and its association with vitamin D exposure. Given that increasing latitude is also associated with higher national wealth, this study investigated associations between MS prevalence and other factors driving regional differences, with a focus on sociodemographic, health systems, and lifestyle factors on a national and regional level. METHODS: Utilizing data from multilateral organizations and scientific literature, an ecological study was conducted to evaluate associations between age- and sex-adjusted MS prevalence and pre-specified sociodemographic (gross domestic product [GDP] per capita and gross national income [GNI] per capita), health systems (current health expenditure per capita and by percentage of GDP, universal health coverage [UHC] index, medical doctors per capita), neurology-specific (MRI unit density, neurologists per capita) and lifestyle (obesity, tobacco use) factors. National, regional and income-stratified data were aggregated and employed in relevant univariable and multivariable regression models. Stepwise variable selection techniques identified independent predictors of MS prevalence. RESULTS: Univariable regression analyses showed significant associations at the national level for all investigated factors, except obesity prevalence and tobacco use. Latitude was significantly associated with MS prevalence in all world regions (ß=0.16-2.16), while UHC index was significantly associated in five of six world regions (ß=0.18-3.17). MS prevalence was significantly associated with all factors except lifestyle factors and MRI unit density in high-income countries, but no associations were observed in low-income countries. Latitude was associated with MS prevalence for all income strata except low-income countries (ß=0.55-1.62). In multivariable analyses, current health expenditure per capita (ß = 0.083, 95% CI = 0.048 - 0.12, p < 0.01) and latitude (ß = 1.05, 95% CI = 0.63 - 1.47, p < 0.01) remained significantly associated with MS prevalence. DISCUSSION: Health expenditure per capita is strongly associated with national MS prevalence, suggesting theories that attribute variations in MS prevalence primarily to latitude effects on vitamin D are incomplete. Healthcare access significantly contributes to the global variations in MS prevalence, especially since national wealth rises with latitude and likely results in significant underestimation of MS prevalence in countries with lower health expenditure.