Assessment of potential adverse events following the 2022-2023 seasonal influenza vaccines among U.S. adults aged 65 years and older.

BACKGROUND: While safety of influenza vaccines is well-established, some studies have suggested potential associations between influenza vaccines and certain adverse events (AEs). This study examined the safety of the 2022-2023 influenza vaccines among U.S. adults ≥ 65 years. METHODS: A self-controlled case series compared incidence rates of anaphylaxis, encephalitis/encephalomyelitis, Guillain-Barré Syndrome (GBS), and transverse myelitis following 2022-2023 seasonal influenza vaccinations (i.e., any, high-dose or adjuvanted) in risk and control intervals among Medicare beneficiaries ≥ 65 years. We used conditional Poisson regression to estimate incidence rate ratios (IRRs) and 95 % confidence intervals (CIs) adjusted for event-dependent observation time and seasonality. Analyses also accounted for uncertainty from outcome misclassification where feasible. For AEs with any statistically significant associations, we stratified results by concomitant vaccination status. RESULTS: Among 12.7 million vaccine recipients, we observed 76 anaphylaxis, 276 encephalitis/encephalomyelitis, 134 GBS and 75 transverse myelitis cases. Only rates of anaphylaxis were elevated in risk compared to control intervals. With all adjustments, an elevated, but non-statistically significant, anaphylaxis rate was observed following any (IRR: 2.40, 95% CI: 0.96-6.03), high-dose (IRR: 2.31, 95% CI: 0.67-7.91), and adjuvanted (IRR: 3.28, 95% CI: 0.71-15.08) influenza vaccination; anaphylaxis IRRs were 2.54 (95% CI: 0.49-13.05) and 1.64 (95% CI: 0.38-7.05) for persons with and without concomitant vaccination, respectively. CONCLUSIONS: Rates of encephalitis/encephalomyelitis, GBS, or transverse myelitis were not elevated following 2022-2023 seasonal influenza vaccinations among U.S. adults ≥ 65 years. There was an increased rate of anaphylaxis following influenza vaccination that may have been influenced by concomitant vaccination.

Vaccine patterns among patients diagnosed with Guillain-Barré Syndrome and matched counterparts in a Medicare supplemental population, 2000-2020.

Some vaccines have a small risk of Guillain-Barré Syndrome (GBS), a rare autoimmune disorder characterized by paralysis if untreated. The CDC's Advisory Committee on Immunization Practices (ACIP) guidelines do not consider GBS a precaution for future vaccines unless GBS developed within six weeks after a tetanus-toxoid-containing vaccine or influenza vaccine. Our goal was to describe vaccine patterns before and after GBS diagnosis. We matched each of 709 patients diagnosed with GBS from 2002 to 2020 with Medicare supplemental insurance to 10 counterparts without GBS (1:10) on age and sex. Propensity score-based weighting balanced covariates between groups, and we estimated weighted mean cumulative counts (wMCC) of vaccines/person before and after GBS diagnosis. Among patients with GBS, 7% were diagnosed within 42 days after a vaccine. Prior to GBS diagnosis, the wMCC of vaccines per person was similar between GBS cases and matched counterparts, but after two years of follow-up, GBS patients received 21 fewer vaccines/100 people than counterparts (wMCC difference -0.21 vaccines/person, 95% CI -0.24 to -0.18); GBS patients received 16 vaccines/100 people while matched counterparts received 36/100. Vaccine use was reduced following GBS diagnosis despite no ACIP precaution for most (93%) patients in this study. The observed drop in vaccines after GBS diagnosis indicates a disconnect between clinical practice and current recommendations.

Immune-mediated polyneuropathy in cats: Clinical description, electrodiagnostic assessment, and treatment.

BACKGROUND: Suspected immune-mediated polyneuropathy has been increasingly reported in cats, especially in the last decade, but the condition remains poorly understood. OBJECTIVES: Refine the clinical description and review the classification of this condition based on electrodiagnostic investigation and evaluate the benefit of corticosteroid treatment and L-carnitine supplementation. ANIMALS: Fifty-five cats presented with signs of muscular weakness and electrodiagnostic findings consistent with polyneuropathy of unknown origin. METHODS: Retrospective, multicenter study. Data from the medical records were reviewed. The owners were contacted by phone for follow-up at the time of the study. RESULTS: The male-to-female ratio was 2.2. The median age of onset was 10 months, with 91% of affected cats being <3 years of age. Fourteen breeds were represented in the study. The electrodiagnostic findings supported purely motor axonal polyneuropathy. Histological findings from nerve biopsies were consistent with immune-mediated neuropathy in 87% of the tested cats. The overall prognosis for recovery was good to excellent, as all but 1 cat achieved clinical recovery, with 12% having mild sequelae and 28% having multiple episodes during their lifetime. The outcome was similar in cats with no treatment when compared with cats receiving corticosteroids or L-carnitine supplementation. CONCLUSIONS AND CLINICAL IMPORTANCE: Immune-mediated motor axonal polyneuropathy should be considered in young cats with muscle weakness. This condition may be similar to acute motor axonal neuropathy in Guillain-Barré syndrome patients. Based on our results, diagnostic criteria have been proposed.

Patient-related healthcare costs for diarrhoea, Guillain Barré syndrome and invasive non-typhoidal salmonellosis in Gondar, Ethiopia, 2020.

BACKGROUND: Globally, foodborne diseases result in a significant disease burden with low- and middle-income countries disproportionately affected. Estimates of healthcare costs related to foodborne disease can aid decision makers to take action to mitigate risks and prevent illness. However, only limited data on the African continent are available, especially related to more severe sequelae. We provide estimates of direct and indirect (non)-medical costs of patients with diarrhoea, Guillain-Barré syndrome (GBS), and invasive non-typhoidal salmonellosis (iNTS) in three healthcare facilities in Gondar, Ethiopia. METHODS: We used healthcare data from patient records, interviews with family caregivers and 2020 healthcare resource unit costs. Descriptive statistical analysis was performed. For diarrhoea, differences in mean and median transformed costs between healthcare facilities and etiologies (Campylobacter spp., enterotoxigenic Escherichia coli, non-typhoidal Salmonella enterica) were analysed with ANOVA and chi squared tests. Contribution of healthcare facility, dehydration severity, sex, age and living area to transformed costs was identified with linear regression. Results are in 2020 USD per patient. To extrapolate to national level, 2017 national incidence estimates were used. RESULTS: Mean direct medical costs were 8.96 USD for diarrhoea (health centre 6.50 USD, specialised hospital 9.53 USD, private clinic 10.56 USD), 267.70 USD for GBS, and 47.79 USD for iNTS. Differences in costs between diarrhoea patients were mainly associated with healthcare facility. Most costs did not differ between etiologies. Total costs of a diarrhoea patient in the specialised hospital were 67 USD, or 8% of gross national income per capita. For direct medical plus transport costs of a GBS and iNTS patient in the specialised hospital, this was 33% and 8%, respectively. Of the 83.9 million USD estimated national non-typhoidal Salmonella enterica related cost, 12.2% was due to iNTS, and of 187.8 million USD related to Campylobacter spp., 0.2% was due to GBS. CONCLUSION: Direct medical costs per patient due to GBS and iNTS were 30 respectively five times those due to diarrhoea. Costs of a patient with diarrhoea, GBS or iNTS can be a substantial part of a household's income. More severe sequalae can add substantially to cost-of-illness of foodborne hazards causing diarrheal disease.

Cardiovascular Autonomic Assessment in Guillain-Barré Syndrome: A Longitudinal Study.

Background: Severe dysautonomia is typically seen during acute phase of Guillain-Barré syndrome (GBS). Objective: To investigate the relationship of cardiovascular autonomic dysfunction with motor recovery in GBS. Materials and Methods: Consecutive GBS patients presented to our hospital were recruited. Clinical assessment was evaluated with the Medical Research Council (MRC) sum score and GBS disability score (GDS). All patients had series of autonomic testing on admission and after treatment at 6 and 24 weeks. Both computation-dependent tests (heart rate variability [HRV] and baroreflex sensitivity [BRS]) and autonomic maneuvers were performed. Age- and gender-matched healthy controls (HC) were recruited. The data obtained at admission, 6 weeks and 24 weeks were compared within groups for statistical difference. Results: Six patients (4 men; mean age 39.5 ± 14.3 years) were recruited over one year. Five had GBS and one Miller Fisher syndrome. The mean MRC sum score and GDS on admission were 52.3 ± 4.3 and 3.5 ± 0.8 respectively. During admission, time-domain average RR interval (AVNN) and BRS were significantly poorer among cases compared to HC. Active standing 30:15 ratio and cold pressor test at admission were also significantly abnormal when compared with HC. All the autonomic parameters had normalized by 6 weeks and these were significant for the high frequency-HRV, BRS, and active standing 30:15 ratio. For MRC and GDS, there were significant improvements in the scoring over a period of 24 weeks. Conclusions: Dysautonomia in GBS improved gradually and in keeping with motor and disability recovery.

Assessment of neurological sequelae and new-onset symptoms in the long-term follow-up of paediatric Guillain-Barre syndrome: A longitudinal study from India.

BACKGROUND: Guillain-Barre syndrome (GBS) is the commonest cause of acute flaccid paralysis in children. There is a paucity of studies that assess the long-term outcome of paediatric GBS. AIM: To assess the frequency of neurological sequelae and the new-onset symptoms in the long-term follow-up of paediatric GBS and to identify the risk factors associated with them. METHODS: This longitudinal study involved 78 children with GBS treated between January 2015 and 2021. The parents of those children were contacted to visit the hospital for a detailed neurological examination and to look for new-onset symptoms after the initial treatment for GBS. RESULTS: Of the 78 children, acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, and acute motor-sensory axonal neuropathy variants were observed in 30 (38.5%), 27 (34.6%) and 11 (14.1%) children, respectively. The median (interquartile range (IQR)) duration of follow-up was 3 (2, 4.5) years. The median (IQR) time to independent ambulation was 30 (13.5, 105) days. The neurological sequelae were found in 22 (28.2%) children. GBS disability score at admission (odds ratio (OR) = 4.6; 95% confidence interval (CI): 1.1-19.8; P = 0.04) and axonal variant of GBS (OR = 4.1; 95% CI: 1.5-20.8; P = 0.04) were found to be independent predictors of neurologic sequelae. A total of 28 children experienced new-onset symptoms after GBS, with frequent falls while running and fatigue being the predominant symptoms. Those children with demyelinating variant achieved independent ambulation earlier than the axonal group on survival analysis (log-rank P value = 0.04). CONCLUSION: The presence of neurological sequelae and new-onset symptoms were found in 28.2 and 35% of the GBS children, respectively. High GBS disability score at admission and axonal variant of GBS were independent predictors of neurological sequelae. Knowledge about these would help in devising a plan for rehabilitation.

Inmunoglobulina endovenosa vs. plasmaféresis para el tratamiento del Sd. Guillain-Barré relacionado a infección por Zika en población clave / Intravenous immunoglobulin vs. plasmapheresis for the treatment of Sd. Guillain-Barré related to Zika infection in the key population

INTRODUCCIÓN: Este documento técnico se realiza a solicitud de la Estrategia sanitaria nacional de prevención y control de enfermedades metaxénicas y otras transmitidas por vectores. a. Cuadro clínico: El Síndrome de Guillain-Barré (SGB) es un trastorno autoinmune poco frecuente en el que el propio sistema inmunitario de una persona ataca a los nervios periféricos. Aproximadamente dos tercios de personas con SGB presentan una infección respiratoria o entérica previa entre 2 y 4 semanas antes del inicio de los síntomas. Mientras que en el año 2016, se estableció una relación causal entre virus del Zika (ZIKV) y el desarrollo del SGB. La inmunoterapia por administración de inmunoglobulina intravenosa (IgIV) y la plasmaféresis (PE) son dos tratamientos que han sido usados para el tratamiento del SGB relacionado a infección previa por ZIKV, habiéndose extrapolado esta práctica de la experiencia al tratar el SGB no relacionado a ZIKV. b. Tecnología sanitária: Existen varios medicamentos aprobados contra el SGB como la IgIV. IgIV fue aprobada para la indicación del SGB en Europa en el 2006 por la European Medicines Agency (EMA), en los Estados Unidos en el 2008 por la United States Food and Drug Administration (FDA), y en el Perú en 2016 por la Dirección General de Medicamentos, Insumos y Drogas (DIGEMID), aunque en este último caso sólo para la inmunoglobulina humana normal 5% inyectable para la indicación del SGB agudo. El uso de la PE, por otro lado, para el tratamiento del SGB fue descrito por primera vez entre el 1978 y 1981. OBJETIVO El objetivo del presente documento es evaluar la eficacia y seguridad, así como documentos relacionados a la decisión del uso de la IgIV y PE en el tratamiento del SGB relacionado a infección por Zika en adultos, gestantes y niños. METODOLOGÍA: Se realizó una búsqueda en las principales bases de datos bibliográficas: MEDLINE, LILACS, COCHRANE, así como en buscadores genéricos de Internet incluyendo Google Scholar y TRIPDATABASE. Adicionalmente, se hizo una búsqueda dentro de la información generada por las principales instituciones internacionales de infectología, y agencias de tecnologías sanitarias que realizan revisiones sistemáticas (RS), evaluación de tecnologías sanitarias (ETS) y guías de práctica clínica (GPC). RESULTADOS: Se seleccionaron una RS, una ECA, 4 evaluaciones económicas, y un estudio observacional. Se identificaron 8 guías de práctica clínica y una ETS. Lin et al. publicaron una revisión sistemática para comparar la eficacia de varios tratamientos para el SGB en octubre de 2021. Se incluyeron ECAs y ensayos clínicos no aleatorizados con adultos y niños con SGB de todos los grados de severidad (28 estudios; n=2474). El análisis indicó que la IgIV y la PE eran ambas eficaces para el tratamiento del SGB y, aunque la IgIV fue el tratamiento más efectivo, no hubo una diferencia significativa comparado con la PE (diferencia de medias [DM] = 0,073; IC95% ­ 0,26 ­ 0,41. El uso de distintas dosis de PE o IgIV, y la combinación de PE y IgIV, no demostraron tener un beneficio significativo ante el tratamiento estándar de PE (4-5 sesiones) o de IgIV (0,4-0,5g/kg/día por 4-5 días). Se encontró además otro ECA no incluido en la RS anterior (n=41, edad promedia=37.4 ± 9.2 años), publicada en 2014 que encontró que el tiempo de hospitalización era significativamente menor en los pacientes tratados con IgIV (p=0,03), además de poder ser retirados de la ventilación mecánica antes (p=0.01) y tener un tiempo hasta el inicio de la recuperación motora menor (p=0.04) comparado con pacientes tratados con PE. Un estudio observacional publicado en los Estados Unidos en 2020 coincide con el ECA anterior y encontró que los pacientes tratados con PE tenían un tiempo de hospitalización mayor (PE = 17,78 días vs. IgIV = 10,24 días), unos costos totales mayores (PE: US$149 143 vs. IgIV: US$103 223), y una tasa de mortalidad mayor (PE = 3,8% vs. IgIV = 1,4%; OR = 2,78) comparado con los pacientes tratados con IgIV. Existen varias GPC para el diagnóstico y tratamiento del SGB. La más reciente se publicó en Argentina en octubre de 2021 y indica que la IgIV (0,4g/kg/día durante 5 días) y la PE (200-250ml/kg en 5 sesiones) son tratamientos igualmente eficaces para el SGB. La guía indica que ambos tratamientos conllevan riesgos comparables a eventos adversos, aunque la PE tiene más probabilidades de interrumpirse que la IgIV. Debido a esto, y dado que la IgIV también es más fácil de administrar y, en general, tiene una mayor disponibilidad que la plasmaféresis, suele ser el tratamiento de elección. La guía también indica que aunque la evidencia en embarazadas y niños es limitada, no existen contraindicaciones de estos tratamientos en estos grupos. Sin embargo, dado que la PE requiere consideraciones y seguimiento adicionales, y como la PE solo está disponible en centros con experiencia. en su uso y parece producir mayor malestar y mayores tasas de complicaciones que la IgIV en niños, se tiende a preferir el uso de IgIV en estos grupos. Estas recomendaciones coinciden con las descritas en la guía de práctica clínica para el SGB en niños y adolescentes desarrollada por expertos en Alemania, publicada en enero de 2020. Adicionalmente, esta guía indica que en caso de contraindicaciones a la IgIV en niños y adolescentes con SGB severo, o si el tratamiento con IgIV es ineficaz, se recomienda el uso de PE, aunque no se recomienda el inicio de la PE en las 2 semanas precedentes al tratamiento con IgIV. Se encontraron otras 4 GPC internacionales: guía publicada en 2021 en Brasil por la CONITEC (por sus siglas en portugués Comissão Nacional de Incorporação de Tecnologias no SUS), guía internacional publicada en 2016 por la Organización Mundial de la Salud (OMS), guía europea publicada en 2008 por la EFNS (por sus siglas en inglés European Federation of the Neurological Societies), y guía publicada en 2003 en los Estados Unidos por la AAN (por sus siglas en inglés American Academy of Neurology). Todas estas guías coinciden con las recomendaciones descritas anteriormente. Cabe recalcar que la guía publicada por la OMS es la única en la que se encontró recomendaciones para el diagnóstico y manejo del SGB relacionado específicamente a infección previa por ZIKV. En este contexto, y después de recomendar el uso de IgIV y la PE debido a su misma eficacia, la OMS recomendó lo siguiente: 1) la selección del tratamiento debe basarse en la disponibilidad, costo y viabilidad de la administración, 2) se requiere entrenamiento en la administración apropiada de ambos tratamientos, y 3) antes de la administración de IgIV debe realizarse un muestreo de sangre para el ZIKV y otros flavivirus. También se encontraron dos GPC recientes para SGB peruanas: la primera publicada en 2018 por la oficina de calidad y el servicio de Neurocirugía del Instituto Nacional de Ciencias Neurológicas (INCN) del Ministerio de Salud (MINSA), y la segunda publicada en 2019 por el Instituto de Evaluación de Tecnologías en Salud e Investigación (IETSI). Ambas guías coinciden con las recomendaciones en las guías anteriores. Se seleccionó un estudio de minimización de costos entre el uso de la IgIV vs. PE en pacientes con SGB realizado en Colombia y publicado en 2016. El estudio encontró una diferencia significativa (p=0,002) en el costo total de la atención de los pacientes tratados con IgIV (9,976 US$) comparado con PE (23,354 US$). El costo de la atención de los pacientes tratados con PE se vio afectado por el alto costo en cuanto a complicaciones y mayor estancia hospitalaria en este grupo, ya que no se encontraron diferencias significativas en el costo del procedimiento. Por otro lado, un estudio de minimización de costos en Brasil concluyó que los costos directos estimados para el tratamiento con IgIV eran significativamente mayores al tratamiento con PE. Sin embargo, este estudio solamente incluyó los costos directos del tratamiento con IgIV o PE, y excluyó costos indirectos como aquellos relacionados con complicaciones del tratamento. CONCLUSIONES: La evidencia con respeto al tratamiento del SGB con inmunoglobulina intravenosa (IgIV) o plasmaféresis (PE) en adultos es abundante y se basa en ensayos clínicos aleatorizados y noaleatorizados. No hay evidencia que el tratamiento del SGB relacionado a infección previa por Zika debe ser distinto al tratamiento estándar del SGB. Una RS analizando ECAs publicados indicó que ambos tratamientos son igual de eficaces para el tratamiento del SGB, y que el uso de distintas dosis o combinación de tratamientos no demostraron tener un beneficio significativo ante el tratamiento estándar de PE o de IgIV. Por otro lado, estudios observacionales indican que las complicaciones y el tiempo de hospitalización en pacientes tratados con PE son mayores, lo que también afecta a los costos totales del paciente, siendo mayores en pacientes tratados con PE comparado con aquellos tratados con IgIV, a pesar de que los costos directos del medicamento y sanitarios sean menores para el tratamiento con PE. Debido a esto, la mayoría de las GPC nacionales e internacionales recomiendan el tratamiento del SGB con IgIV o PE de acuerdo a la disponibilidad, costo, viabilidad de la administración y ausencia de contraindicaciones a los tratamentos. La evidencia con respeto al tratamiento del SGB con IgIV o PE en embarazadas y niños es limitada, aunque no hay evidencia actual de contraindicaciones de estos tratamientos en estos grupos. A pesar de ello, por su mayor facilidad de administración y tolerancia, tiende a haber una preferencia para el uso de IgIV en embarazadas y niños.

Proposal for the functional assessment of acute inflammatory neuropathy (FAAIN) in Guillain-Barré syndrome.

BACKGROUND: Guillain Barré syndrome (GBS) functional assessment is necessary in clinical practice, research and clinical trials. Existing instruments are not sensitive to change and are not applicable to the current GBS clinical spectrum. OBJECTIVE: To construct a functional assessment for acute inflammatory neuropathies (FAAIN-GBS), inclusive for current GBS spectrum that assesses extension and intensity separately. METHODS: FAAIN-GBS subscales were constructed. Its structure and interpretation were defined. It was validated using data from medical record of 167 GBS patients admitted to the Institute of Neurology and Neurosurgery. Cronbach α was used for items reduction and reliability analysis. Bartlett sphericity test was performed. Exploratory factor analysis (EFA) of the main components, with varimax rotation, was applied to evaluate dimensionality and content validity. Hughes scale was used as gold standard for criterion validity. Sensitivity, specificity and area under the receiver operating characteristic curves (AUROC), were calculated. Construct validity was assessed by confirmatory factor analysis (CFA). RESULTS: FAAIN-GBS is made up of two subscales (extension and intensity). The final score is obtained by averaging both dimensions. Internal consistency was acceptable (Cronbach 0.745). EFA showed three dimensions: intensity, spinal extension and cranial extension. Spearman correlation between FAAIN-GBS and Hughes scale was 0.463. Sensitivity (0.714) and specificity (0.986) values showed the good behavior of the scale; AUROC was 0.93. CONCLUSION: FAAIN-GBS was constructed and a first step of validation was made, showing good internal consistency and validity. New prospective studies with large populations will be necessary to perfect this instrument that could be useful in neurological practice.

A Broad Safety Assessment of the Recombinant Herpes Zoster Vaccine.

The recombinant herpes zoster vaccine (RZV), approved as a 2-dose series in the United States in October 2017, has proven highly effective and generally safe. However, a small risk of Guillain-Barré syndrome after vaccination was identified after approval, and questions remain about other possible adverse events. This data-mining study assessed RZV safety in the United States using the self-controlled tree-temporal scan statistic, scanning data on thousands of diagnoses recorded during follow-up to detect any statistically unusual temporal clustering of cases within a large hierarchy of diagnoses. IBM MarketScan data on commercially insured persons at least 50 years of age receiving RZV between January 1, 2018, and May 5, 2020, were used, including 56 days of follow-up; 1,014,329 doses were included. Statistically significant clustering was found within a few days of vaccination for unspecified adverse effects, complications, or reactions to immunization or other medical substances/care; fever; unspecified allergy; syncope/collapse; cellulitis; myalgia; and dizziness/giddiness. These findings are consistent with the known safety profile of this and other injected vaccines. No cluster of Guillain-Barré syndrome was detected, possibly due to insufficient sample size. This signal-detection method has now been applied to 5 vaccines, with consistently plausible results, and seems a promising addition to vaccine-safety evaluation methods.

In older adults, use of a recombinant zoster vaccine was associated with Guillain-Barré syndrome.

SOURCE CITATION: Goud R, Lufkin B, Duffy J, et al. Risk of Guillain-Barré syndrome following recombinant zoster vaccine in Medicare beneficiaries. JAMA Intern Med. 2021;181:1623-30. 34724025.

Catastrophic health expenditure in patients with Guillain Barre syndrome: Retrospective study.

INTRODUCTION: Catastrophic health expenditure (CHE) is a reliable measure of the financial unpreparedness of the studied population to meet unexpected health issues. The alarming proportion of patients who incur CHE in the wake of an acute neurological illness like Guillain Barre Syndrome (GBS) is of serious concern in a country like India where a large majority of households are uninsured. METHODOLOGY: Medical records of patients diagnosed with at a tertiary care centre in Delhi were analysed retrospectively to determine the rate of CHE. Clinical details and other contributory variables were also recorded. RESULTS: 53 patients with a median age of 29 years (10.5-46.5) were included in the study. Tow- third of patients were less than 40 years of age and 58.5% were male. 90.6% of patients incurred CHE with a median amount INR 273 300 spent out of pocket. CONCLUSION: The enormous magnitude of financial distress and crisis emerging out of an acute neurological illness needs to be addressed with urgency to prevent impoverishment of already weakened households.

Costs of Guillain-Barré Syndrome in the Brazilian Federal District: the patients' perspective.

BACKGROUND: Although rare, Guillain-Barré Syndrome (GBS) has a high economic burden, with consequences for families and society. This study aimed to estimate the total cost of GBS, per individual and per variant of the disease, as well as its effect on household income, from the perspective of patients. METHODS: This was a cost-of-illness study from the perspective of patients and their families, with a time horizon from disease onset to 6 mo after discharge. The total cost of GBS was estimated by bottom-up microcosting, considering direct and indirect costs. RESULTS: The median cost of GBS per individual was US$1635.5, with direct costs accounting for 64.3% of this amount. Among the variants analyzed, acute motor sensory axonal neuropathy (US$4660.1) and acute inflammatory demyelinating polyneuropathy (US$2017.0) exhibited the highest costs compared with acute motor axonal neuropathy (US$1635.5) and Miller Fisher Syndrome (US$1464.8). The costs involved compromise more than 20% of the household income of 22 (47.8%) patients. CONCLUSIONS: This study demonstrated how costly GBS can be. It is hoped that decision-makers will analyze these results with a view to improving the structure of healthcare services.

iMAX: A new tool for assessment of motor axon excitability. A multicenter prospective study.

OBJECTIVE: This study was undertaken to establish by a multicentric approach the reliability of a new technique evaluating motor axon excitability. METHODS: The minimal threshold, the lowest stimulus intensity allowing a maximal response by 1 mA increments (iUP) and then by 0.1 mA adjustments (iMAX) were prospectively derived from three nerves (median, ulnar, fibular) in four university centers (Liège, Marseille, Fraiture, Nice). iMAX procedure was applied in 28 healthy volunteers (twice) and 32 patients with Charcot-Marie-Tooth (CMT1a), chronic inflammatory demyelinating polyneuropathy (CIDP), Guillain-Barré syndrome (SGB) or axonal neuropathy. RESULTS: Healthy volunteers results were not significantly different between centers. Correlation coefficients between test and retest were moderate (> 0.5). Upper limits of normal were established using the 95th percentile. Comparison of volunteers and patient groups indicated significant increases in iMAX parameters especially for the CMT1a and CIDP groups. In CMT1a, iMAX abnormalities were homogeneous at the three stimulation sites, which was not the case for CIDP. CONCLUSIONS: The iMAX procedure is reliable and allows the monitoring of motor axon excitability disorders. SIGNIFICANCE: The iMAX technique should prove useful to monitor motor axonal excitability in routine clinical practice as it is a fast, non-invasive procedure, easily applicable without specific software or devices.

Risk of Guillain-Barré syndrome following herpes zoster, United States, 2010-2018.

Epidemiologic data regarding the risk of Guillain-Barré syndrome (GBS) following herpes zoster (HZ) are limited. We conducted a self-controlled case series analysis using two large national data sources to evaluate the risk of GBS following HZ among U.S. adults. We analyzed medical claims from the IBM® MarketScan® Commercial Claims and Encounters (persons 18-64 years during 2010-2018) and Centers for Medicare and Medicaid Services Medicare (persons ≥65 years during 2014-2018) databases. HZ cases were defined as persons with an outpatient claim with a primary or secondary ICD-9 or ICD-10 diagnostic code for HZ. GBS cases were defined as persons with an inpatient claim with a principle diagnostic code for GBS and an associated procedural code. We compared the rates of GBS following HZ in the 1-42-day risk window versus primary (100-365-day) or secondary (43-99-day) control windows. We identified 489,516 persons 18-64 years of age and 650,229 persons ≥65 years of age with HZ, among whom 11 and 41, respectively, developed GBS 1-365 days following HZ. The risk of GBS following HZ was increased during the risk window as compared to the primary control window for both groups, with a rate ratio of 6.3 (95% CI, 1.8-21.9) for those 18-64 years and 4.1 (95% CI, 1.9-8.7) for those ≥65 years. This study provides new and methodologically rigorous epidemiologic support for an association between HZ and GBS, and useful context regarding the benefits versus potential risks of zoster vaccination.

Risk of Guillain-Barré Syndrome Following Recombinant Zoster Vaccine in Medicare Beneficiaries.

Importance: Guillain-Barré syndrome can be reported after vaccination. This study assesses the risk of Guillain-Barré syndrome after administration of recombinant zoster vaccine (RZV or Shingrix), which is administered in 2 doses 2 to 6 months apart. Objective: Use Medicare claims data to evaluate risk of developing Guillain-Barré syndrome following vaccination with zoster vaccine. Design, Setting, and Participants: This case series cohort study included 849 397 RZV-vaccinated and 1 817 099 zoster vaccine live (ZVL or Zostavax)-vaccinated beneficiaries aged 65 years or older. Self-controlled analyses included events identified from 2 113 758 eligible RZV-vaccinated beneficiaries 65 years or older. We compared the relative risk of Guillain-Barré syndrome after RZV vs ZVL, followed by claims-based and medical record-based self-controlled case series analyses to assess risk of Guillain-Barré syndrome during a postvaccination risk window (days 1-42) compared with a control window (days 43-183). In self-controlled analyses, RZV vaccinees were observed from October 1, 2017, to February 29, 2020. Patients were identified in the inpatient, outpatient procedural (including emergency department), and office settings using Medicare administrative data. Exposures: Vaccination with RZV or ZVL vaccines. Main Outcomes and Measures: Guillain-Barré syndrome was identified in Medicare administrative claims data, and cases were assessed through medical record review using the Brighton Collaboration case definition. Results: Amongst those who received RZV vaccinees, the mean age was 74.8 years at first dose, and 58% were women, whereas among those who received the ZVL vaccine, the mean age was 74.3 years, and 60% were women. In the cohort analysis we detected an increase in risk of Guillain-Barré syndrome among RZV vaccinees compared with ZVL vaccinees (rate ratio [RR], 2.34; 95% CI, 1.01-5.41; P = .047). In the self-controlled analyses, we observed 24 and 20 cases during the risk and control period, respectively. Our claims-based analysis identified an increased risk in the risk window compared with the control window (RR, 2.84; 95% CI, 1.53-5.27; P = .001), with an attributable risk of 3 per million RZV doses (95% CI, 0.62-5.64). Our medical record-based analysis confirmed this increased risk (RR, 4.96; 95% CI, 1.43-17.27; P = .01). Conclusions and Relevance: Findings of this case series cohort study indicate a slightly increased risk of Guillain-Barré syndrome during the 42 days following RZV vaccination in the Medicare population, with approximately 3 excess Guillain-Barré syndrome cases per million vaccinations. Clinicians and patients should be aware of this risk, while considering the benefit of decreasing the risk of herpes zoster and its complications through an efficacious vaccine, as risk-benefit balance remains in favor of vaccination.

Global, regional, and national burden of Guillain-Barré syndrome and its underlying causes from 1990 to 2019.

BACKGROUND: This article presents the first detailed analysis of the prevalence and disability burden of Guillain-Barré syndrome (GBS) from 1990 to 2019 by cause, age, sex, and Socio-demographic Index (SDI) in 204 countries and territories. METHODS: Data from the Global Burden of Diseases Study (GBD) 2019 were used. GBD 2019 modelled the prevalence of GBS using hospital and claims data. Years lived with disability (YLDs) were estimated as the product of the GBS prevalence and the disability weight. This article also reported proportions in the age-standardised prevalence rate that were due to six underlying causes of GBS. RESULTS: In 2019, there were 150,095 [95% uncertainty intervals (UI) 119,924 to 188,309] total cases of GBS worldwide, which resulted in 44,407 (95% UI 28,016 to 64,777) YLDs. Globally, there was a 6.4% (95% UI 3.6 to 9.5) increase in the age-standardised prevalence of GBS per 100,000 population between 1990 and 2019. High-income Asia Pacific [1.9 (95% UI: 1.5 to 2.4)] and East Asia [0.8 (95% UI: 0.6 to 1.0)] had the highest and lowest age-standardised prevalence rates (per 100,000), respectively, in 2019. Nationally, Japan [6.4 (95% UI: 5.3 to 7.7)] and China [0.8 (95% UI: 0.6 to 1.0)] had the highest and lowest age-standardised prevalence rates (per 100,000). The age-standardised burden of GBS increased with increasing age and was higher in males in all age groups. Furthermore, the age-standardised prevalence of GBS (per 100,000) had a positive association with the level of development, as measured by SDI, although this association was not strong. Upper respiratory infections and unknown causes accounted for the highest proportions of underlying causes. CONCLUSIONS: Globally, the prevalence of GBS continues to increase. Geographical differences and strategies aimed at preventing infectious diseases should be considered in future health policy planning and decision-making processes. This study had several limitations, such as using the same disability weight for all causes and a reliance on hospital- and self-reported data, which should be addressed in future research.

Sensitivity of Guillain-Barre Syndrome Surveillance in the Brazilian Federal District, using the Capture-Recapture Method.

INTRODUCTION: Guillain-Barre Syndrome (GBS) is an acute immune-mediated polyneuropathy that compromises the peripheral and cranial nerves. It is characterized by rapid-onset paresthesia accompanied by progressive weakness in the lower extremities followed by symmetric ascending paralysis. METHODOLOGY: assessment of sensitivity to detect GBS between March 2017 and May 2019 in a public referral hospital, using the capture-recapture method based on the Chapman estimator and comparing three GBS data sources: the hospital-based sentinel surveillance system (VSBH), Human Immunoglobulin Dispensing Records System (RDIH), and Hospital Information System (SIH). RESULTS: A total of 259 possible cases were identified (captured). Of these, 58 were confirmed and most resided in the Federal District. The VSBH showed the greatest sensitivity in case identification. The temporal distribution of cases showed periods with no cases identified, and more were registered during the rainy season from October to May, when high temperatures also occur. CONCLUSIONS: Increased circulation of arboviruses and gastrointestinal infections during the rainy season may explain the greater concentration of GBS cases. It is important to note that one-third of the cases identified in the different data sources do not converge, demonstrating that no single surveillance system is 100% effective. The severity and possible increase in cases related to GBS demonstrates the need for an improved surveillance system capable of monitoring and following-up cases involving neurological syndromes, regardless of the event preceding infection.

Guillain-Barré syndrome in low-income and middle-income countries: challenges and prospects.

The epidemiology, clinical characteristics, management and outcome of Guillain-Barré syndrome (GBS) differ between low-income and middle-income countries (LMIC) and high-income countries (HIC). At present, limited data are available on GBS in LMIC and the true incidence of GBS in many LMIC remains unknown. Increased understanding of GBS in LMIC is needed because poor hygiene and high exposure to infections render populations in LMIC vulnerable to GBS outbreaks. Furthermore, insufficient diagnostic and health-care facilities in LMIC contribute to delayed diagnosis in patients with severe presentations of GBS. In addition, the lack of national clinical guidelines and absence of affordable, effective treatments contribute to worse outcomes and higher mortality in LMIC than HIC. Systematic population-based surveillance studies, cohort and case-control studies are required to understand the incidence and risk factors for GBS. Novel, targeted and cost-effective treatment strategies need to be developed in the context of health system challenges in LMIC. To ensure integrative rehabilitation services in LMIC, existing prognostic models must be validated, and responsive outcome measures that are cross-culturally applicable must be developed. Therefore, fundamental and applied research to improve the clinical management of GBS in LMIC should become a critical focus of future research programmes.

Pediatric acute demyelinating syndromes: identification and management in the emergency department.

Acute demyelinating disorders can present with vague complaints and subtle abnormalities of the neurological examination. A thorough history and physical examination are important for narrowing the differential diagnosis and determining which diagnostic studies are indicated. This issue focuses on the most common acute demyelinating disorders in children: Guillain-Barré syndrome and acute transverse myelitis. Common presenting signs and symptoms of these conditions are reviewed, and evidence-based recommendations are provided for the initial assessment and management of Guillain-Barré syndrome and acute transverse myelitis in the emergency department.