Ventilación mecánica, tiempo de hospitalización, muertes y discapacidad según las variantes del síndrome de Guillain-Barré: revisión sistemática y metaanálisis / Mechanical ventilation, hospitalization time, deaths and disability according to the variants of Guillain-Barré syndrome: systematic review and meta-analysis

RESUMEN Objetivos. Determinar el requerimiento y tiempo para ventilación mecánica y Unidad de Cuidados Intensivos (UCI), hospitalización y tiempo de hospitalización, muerte y discapacidad de las variantes axonales del Síndrome de Guillain-Barré (SGB) en comparación con la variante aguda desmielinizante en pacientes de todas las edades. Materiales y métodos. Revisión sistemática que incluyó pacientes con SGB; la exposición fueron las variantes axonales y el comparador la polineuropatía desmielinizante inflamatoria aguda (AIDP) los desenlaces fueron el requerimiento y tiempo en ventilación mecánica (VM), requerimiento y tiempo en la UCI, tiempo de hospitalización, discapacidad y muerte. Se utilizó la escala NewCasttle-Ottawa (NOS) para evaluar el riesgo de sesgo. Se realizó un metaanálisis para calcular las diferencias de medias y los riesgos relativos (RR) con sus intervalos de confianza (IC) del 95% utilizando varianzas inversas y modelos de efectos aleatorios. Resultados. De los 3116 artículos encontrados, 46 cumplieron los criterios de selección. El tiempo en VM fue 7,42 días (IC95%: 0,36 a 1,48) y el tiempo de hospitalización fue 3,11 (IC95%: 0,73 a 5,49) días en las variantes axonales. Las variantes axonales tuvieron un RR de 0,47 (IC95%: 0,24 a 0,92) para el requerimiento de VM en adultos, pero en niños fue de 1,68 (IC95%: 1,25 a 2,25). Hubo una alta heterogeneidad estadística. Conclusiones. Las variantes axonales tienen en promedio mayor tiempo de VM y de hospitalización, en total y por subgrupos. Se observó un mayor requerimiento de VM para las variantes axonales en niños; mientras que en los adultos fue menor.

ABSTRACT Objectives. To determine the requirement and time to mechanical ventilation and Intensive Care Unit (ICU), hospitalization and hospitalization time, death and disability of the axonal variants of Guillain-Barré Syndrome (GBS) in comparison with the acute demyelinating variant in patients of all the ages. Materials and methods. The systematic review that included patients with GBS. The exposure variable was the axonal variants and the comparator was acute inflammatory demyelinating polyneuropathy (AIDP). The outcomes were the requirement and time on mechanical ventilation (MV), requirement and time in the ICU, hospitalization time, disability and death. The NewCasttle-Ottawa Scale (NOS) was used to assess risk of bias. A meta-analysis was conducted to calculate mean differences and relative risks (RR) with their 95% confidence intervals (CI) using inverse variances and random effects models. Results. Of the 3116 articles found, 46 met the selection criteria. The time on MV was 7.42 days (95% CI: 0.36 to 1.48) and the hospitalization time was 3.11 (95% CI: 0.73 to 5.49) days for the axonal variants. The axonal variants had a RR of 0.47 (95% CI: 0.24 to 0.92) for the requirement of MV in adults, but it was 1.68 (95% CI: 1.25 to 2.25) in children. There was a high statistical heterogeneity. Conclusions. Axonal variants showed, on average, longer MV and hospitalization time, overall and by subgroups. A high MV requirement was found for axonal variants in children; it was lower for adults.

Rehabilitación neurológica en la variante faringo-cérvico-braquial del síndrome de Guillain-Barré: reporte de un caso pediátrico / Neurological rehabilitation in the pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a pediatric case report

RESUMEN Introducción: El síndrome de Guillain-Barré (SGB) es la principal causa de parálisis en niños, y la variante faringo-cérvico-braquial (FCB) es poco común. Esta variante atípica se asocia con secuelas y requiere rehabilitación prolongada. Dado el enfoque predominante durante el tratamiento agudo, este estudio describe la evolución motora, respiratoria y funcional de un niño con SGB, variante FCB, en un centro de neurorrehabilitación durante el período subagudo. Presentación del caso: Un niño de 11 años ingresó con cuadriparesia fláccida y un mayor compromiso en los miembros superiores (MMSS), traqueotomizado y dependiente para las actividades de la vida diaria. Recibió un programa de evaluación y abordaje interdisciplinario intensivo. Logró la decanulación, la marcha y la independencia funcional con adaptaciones, y fue dado de alta luego de 16 semanas. Conclusión: La evolución del caso concuerda con lo reportado en la literatura sobre la presencia de secuelas y los tiempos prolongados de recuperación de la variante FCB. El niño recibió rehabilitación interdisciplinaria intensiva durante 16 semanas y, aún luego de un año, continuaba presentando debilidad a nivel distal de los MMSS y fatiga. Estos hallazgos resaltan la importancia de una intervención temprana y una rehabilitación integral en pacientes pediátricos con esta variante particular del SGB.

ABSTRACT Introduction: The Guillain-Barré syndrome (GBS) is the primary cause of paralysis in children, with the pharyngeal-cervical-brachial (PCB) variant being rare. This atypical form is associated with sequelae and requires prolonged rehabilitation. Given the predominant focus during acute treatment, this study describes the motor, respiratory, and functional clinical course of a child with GBS, PCB variant, in a neurorehabilitation center during the subacute period. Case presentation: An 11-year-old boy was admitted with flaccid quadriparesis and greater involvement of the upper limb (UL), tracheotomized, and dependent for activities of daily living. He underwent an intensive interdisciplinary evaluation and treatment program. The patient achieved decannulation, gait, and functional independence with adaptations, and was discharged after 16 weeks. Conclusion: The clinical course of the case aligns with the literature regarding the presence of sequelae and prolonged recovery times of the PCB variant. The child underwent intensive interdisciplinary rehabilitation for 16 weeks and, even after one year, continued to experience weakness in the distal UL and fatigue. These findings highlight the importance of early intervention and comprehensive rehabilitation in pediatric patients with this particular variant of GBS.

Use of Biomedical Engineering for Rehabilitation of Patients with Disability Caused by Guillain-Barré Syndrome: a Systematic Review / Uso de la Ingeniería Biomédica para Rehabilitación de Pacientes con Discapacidad Causada por el Síndrome de Guillain-Barré: Una Revisión Sistemática

Abstract This systematic review aims to assess the extent to which biomedical engineering has been applied in the rehabilitation of patients suffering from Guillain-Barré Syndrome (GBS), given the scarcity of information on this topic. We conducted a thorough analysis of research articles, conference abstracts, and case reports published between 2000 and 2023, specifically from ScienceDirect, PubMed, IEEE Xplore, Springer, and Dimensions. 19 articles were extensively discussed, complemented by an additional 40 information sources providing supplementary information. Each paper underwent a meticulous review process by the four authors, where each separately examined the title and abstract of the papers and subsequently provided a thorough examination of the full text; when conflicts arose, a clear consensus was reached through discussion. The analysis of the articles revealed a notable improvement in upper and lower limb function of GBS patients that was facilitated by both custom-made and commercial devices. Likewise, a small handful of other devices have been used (e.g., to improve urinary retention issues). There is a clear opportunity for new research, innovation and applications.

Resumen Esta revisión sistemática tiene como objetivo evaluar hasta qué punto se ha aplicado la ingeniería biomédica en la rehabilitación de pacientes que padecen el Síndrome de Guillain-Barré (SGB), dada la escasez de información sobre este tema. Realizamos un análisis exhaustivo de artículos de investigación, resúmenes de conferencias e informes de casos publicados entre 2000 y 2023, específicamente de ScienceDirect, PubMed, IEEE Xplore, Springer y Dimensions. Se discutieron ampliamente 19 artículos, complementados con 40 fuentes de información adicionales. Cada artículo pasó por un meticuloso proceso de revisión por parte de los cuatro autores, donde cada uno examinó por separado el título y el resumen de los artículos y posteriormente proporcionó un examen exhaustivo del texto completo; cuando surgieron conflictos, se alcanzó un consenso mediante la discusión. El análisis de los artículos reveló una mejora notable en la función de las extremidades superiores e inferiores de los pacientes con SGB que fue facilitada por dispositivos tanto hechos a medida como comerciales. Asimismo, se han creado un pequeño puñado de otros dispositivos, (por ejemplo, para mejorar los problemas de retención urinaria). Existe una clara oportunidad para nueva investigación, innovación y aplicaciones.

Arboviruses epidemic associates with Guillain-Barré syndrome: temporal series in northeast Brazil / Epidemia de arboviroses associada à síndrome de Guillain-Barré: série temporal no nordeste brasileiro / Epidemia de arbovirosis asociada al síndrome de Guillain-Barré: serie temporal en el noreste de Brasil

Arboviruses cause public health problems in several countries, and records show that they can generate central and peripheral neurological complications with permanent sequelae. However, it is not certain which arbovirus is responsible for outbreaks of the Guillain-Barré Syndrome (GBS), especially in Brazil. Thus, the objective of this study is to verify if there is a coincidence between the GBS outbreak and the most common arboviruses in Northeastern Brazil, as well as their relationship. An ecological time series study was designed with the federative units of Northeastern Brazil, using hospitalizations for Guillain-Barré syndrome and notifications of arbovirus infections between 2014 and 2019 as a data source. Distribution incidence curves were constructed for the conditions studied, and generalized estimating equations (GEE) models were applied to estimate the relationship between arboviruses and Guillain-Barré. The results showed a similar distribution for the incidences of Chikungunya virus (z=7.82; p=0.001), Zika virus (z=3.69; p=0.03), and Guillain-Barré syndrome (z=2.98; p=0.05) from 2014 to 2019. The GEE model revealed that the distribution of Chikungunya incidence is associated with the distribution of GBS incidence in each year (x2Wald=3,969; p=0.046). This pattern was repeated in seven of the nine states, while the Zika virus had a significant relationship with GBS in only two states. The outbreak of GBS in Northeastern Brazil appears to be probabilistically related to outbreaks of the Chikungunya virus.

As arboviroses são problemas de saúde pública em vários países e há registros de que podem produzir complicações neurológicas centrais e periféricas com sequelas permanentes. Entretanto, não se sabe ao certo qual delas é realmente responsável pelos surtos da Síndrome de Guillain-Barré (SGB), principalmente no Brasil. Assim, o objetivo é verificar se há coincidência entre o surto de SGB e as arboviroses mais comuns no Nordeste do Brasil e suas relações. Foi desenhado um estudo ecológico de série temporal com as unidades federativas do Nordeste do Brasil, adotando como fonte de dados as internações Guillain-Barré e as notificações de infecções por arbovírus entre 2014 e 2019. Curvas de distribuição de incidência foram construídas para as condições estudadas, e foram aplicados modelos de equações generalizadas estimadas (GEE) para estimar a relação entre arbovírus e Guillain-Barré. Evidencia-se que há distribuição semelhante para as incidências do vírus Chikungunya (z=7,82; p=0,001), vírus Zika (z=3,69; p=0,03) e síndrome de Guillain-Barré (z=2,98; p=0,05) entre 2014 e 2019. O modelo GEE revelou que a distribuição da incidência de Chikungunya está associada à distribuição da incidência de SGB em cada ano (x2Wald=3,969; p=0,046). Esse padrão se repetiu em sete dos nove estados, enquanto o zika vírus teve uma relação significativa com o GBS em apenas dois estados. Conclui-se, então, que o surto de SGB no Nordeste do Brasil parece estar probabilisticamente relacionado aos surtos do vírus Chikungunya.

Los arbovirus causan problemas de salud pública en varios países y, según indican los reportes, pueden producir complicaciones neurológicas centrales y periféricas con secuelas permanentes. Sin embargo, no se sabe cuál de ellos es realmente el responsable de los brotes del síndrome de Guillain-Barré (SGB), especialmente en Brasil. Así, el objetivo de este estudio es verificar si existen coincidencias entre el brote del SGB y los arbovirus más comunes en el Noreste de Brasil y sus asociaciones. Se diseñó un estudio de series temporales ecológico en las unidades federativas del Noreste de Brasil, adoptando como fuente de datos las hospitalizaciones y las notificaciones de arbovirosis de Guillain-Barré entre 2014 y 2019. Se construyeron curvas de distribución de incidencia para las condiciones científicas, y se aplicó una ecuación estimada generalizada (GEE) para estimar la relación entre arbovirus y Guillain-Barré. Se encontró que existe una distribución similar en las incidencias de virus del chikunguña (z=7,82; p=0,001), virus del Zika (z=3,69; p=0,03) y síndrome de Guillain-Barré (z =2,98; p=0,05) entre 2014 y 2019. El modelo GEE reveló que la distribución de la incidencia de chikunguña está asociada con la distribución de la incidencia de SGB en cada año (x2Wald=3,969; p=0,046). Este patrón se repitió en siete de los nueve estados, mientras que el virus del Zika presentó una relación significativa con el SGB en solo dos estados. El brote del SGB en el Noreste de Brasil parece estar relacionado probabilísticamente con los brotes del virus del chikunguña.

The clinical observation of Yangming meridian acupuncture combined with needle pushing based on the theory of"treating flaccidity through Yangming meridian"in the treatment of Guillain-Barre syndrome / 实用医学杂志

Objective To observe the clinical effect of Yangming meridian acupuncture combined with acupuncture push based on the theory of"treating flaccence and taking Yangming"in the treatment of Guillain-Barre syndrome.Methods 52 cases of patients with conventional rehabilitation combined with Yangming meridian acupuncture combined with acupuncture based on the theory of"treating potence and taking Yangming alone"were taken as the study group and 52 cases of patients with conventional rehabilitation alone as the control group.Limb muscle strength score,clinical efficacy,limb sensory function,limb motor function,upper limb median nerve electrophysiology and daily living ability were compared between the two groups.Results After treatment,muscle strength scores of proximal lower extremity,distal lower extremity,proximal upper extremity,distal upper extremity,median sensory nerve action potential(SNAP),motor conduction velocity(MCV),sensory conduction velocity(SCV)and modified Barthel index(MBI)were increased in 2 groups(P＜0.05).The study group was more obvious(P＜0.05).The total effective rate of the study group(86.54%)was higher than control group(69.23%)(P＜0.05).After treatment,the sensory function and motor function of limbs in 2 groups were better than before treatment(P＜0.05),especially in the study group(P＜0.05).After treatment,the distal motor latency(DML)of upper limb median nerve was decreased in 2 groups(P＜0.05),and more significantly in the study group(P＜0.05).Conclusion Yangming meridian acupuncture combined with acupuncture pushing based on the theory of"treating impotence and taking Yangming"is effective in the treatment of Guillain-Barre syndrome,which can improve the muscle strength,sensation and motor function of limbs,and regulate nerve electrophysiology.

One case of diabetic peripheral neuropathy complicated with chronic Guillain-Barre syndrome / 中华内分泌代谢杂志

Chronic Guillain-Barre syndrome, also known as chronic inflammatory demyelinating polyradiculoneuropathy(CIDP), is an immune-mediated demyelinating peripheral neuropathy. This article analyzes the clinical data of a CIDP patient presenting primarily with limb numbness, pain, and weakness. Along with literature review, this study explores the differential diagnosis between CIDP and diabetic peripheral neuropathy in terms of the pathogenesis, clinical manifestations, laboratory tests, and treatment.

Four cases of COVID-19 associated Guillain-Barré syndrome / 中华神经科杂志

COVID-19 associated Guillain-Barré syndrome (GBS) caused by peripheral nerve damage after SARS-CoV-2 infection is one of the most common COVID-19 related nervous system inflammatory diseases, with high incidence of respiratory failure and mortality. Positive SARS-CoV-2 RNA in cerebrospinal fluid of COVID-19 associated GBS patients has been rarely reported. This paper reports 4 patients with COVID-19 associated GBS in China who developed neurological symptoms 4-15 days after fever and were confirmed SARS-CoV-2 infection. All patients presented with progressive weakness of both lower limbs, 3 patients with autonomic dysfunction such as defecation and urination disorders, and 1 patient with polycranial neuritis and Miller-Fisher syndrome such as bilateral facial palsy, dysphagia, diplopia and ataxia. Nerve conduction velocity and F wave were abnormal in 3 patients, and motor conduction pathway was abnormal in 1 patient. Anti-ganglioside antibodies were tested in 3 patients, and GD1a-IgG was positive in 1 patient. All 4 patients underwent metagenomic next-generation sequencing examination in blood and cerebrospinal fluid. SARS-CoV-2 RNA was positive in blood and cerebrospinal fluid of 3 patients, and SARS-CoV-2 RNA was positive in cerebrospinal fluid of 1 patient.

Comparative analysis of cerebrospinal fluid laboratory test results between multiple sclerosis and Guillain-Barré syndrome / 中华检验医学杂志

Objective:To compare the characteristics of cerebrospinal fluid (CSF) oligoclonal band electrophoresis examination results between patients with multiple sclerosis (MS) and Guillain-Barré syndrome (GBS), and to provide a basis for the differential diagnosis of the two types of neurological demyelinating diseases.Methods:Case analysis.The retrospective study method was used, and the patients who visited Beijing Tiantan Hospital, Capital Medical University from January 2020 to August 2023 were selected as the research subjects, including 70 MS patients[19 males and 51 females, aged 34 (28, 44) years] and 70 GBS patients [44 males and 26 females, aged 50 (36, 61) years]. The oligoclonal band electrophoresis and immunoglobulin G(IgG) index (IgG I) were performed on the clinical specimens from MS and GBS patients, and CSF routine, CSF biochemistry (glucose, chloride, protein), lactate, interleukin-6 (IL-6), interleukin-8 (IL-8), and tumor necrosis factor-α (TNF-α), antibodies to herpes simplex virus (HSV), cytomegalovirus (CMV), rubella virus (RV), toxoplasma gondii (TOX), Epstein-Barr virus (EBV), and coxsackievirus were detected simultaneously. The enumeration data were treated with the chi-square test. The measurement data didn′t accord with normal distribution, and were treated with the Mann-Whitney U test. Results:The positive rate of oligoclonal band (OCB) electrophoresis in MS and GBS patients were 80.00% (56/70) and 4.29% (3/70), respectively. The positive rate in MS patients was significantly higher than that in GBS patients (χ 2=82.289, P<0.001). The white blood cells count [5.50 (3.00, 11.00)/μl] and the level of chlorine [127 (125, 128) mmol/L] in CSF of MS patients was higher than that of GBS patients [3.50(2.00, 7.00)/μl, 126(124, 128) mmol/L] ( U=-2.245, P<0.05; U=-2.028, P<0.05), while the levels of CSF protein [33.40(27.61, 39.17)mg/L], glucose [3.59(3.36, 3.88) mmol/L], and lactate [1.55(1.40, 1.73) mmol/L] of MS patients were lower than those of GBS patients [6.71(43.78, 138.30) mg/L, 3.97(3.55, 4.54) mmol/L, 1.80(1.60, 2.00) mmol/L]( U=-6.747, P<0.001; U=-3.651, P<0.001; U=-4.531, P<0.001). The levels of IL-6 [3.36(2.34, 5.02) pg/ml], IL-8 [55.40(46.75, 66.40) pg/ml], and TNF-α [5.63(4.25, 6.63) pg/ml] in CSF of MS patients were lower than those of GBS patients [6.12(3.61, 11.73) pg/ml, 120.00(74.90, 187.80) pg/ml, 6.57(5.25, 8.03) pg/ml]( U=-3.463, P<0.05; U=-5.225, P<0.001; U=-2.785, P<0.05). The positive rates of CMV IgG, TOX IgG, and EBVCA IgG in CSF of MS patients were 36.36% (24/66), 0 and 0, respectively,and the positive rates of those of GBS patients were 85.71% (54/63), 30.16% (19/63), and 19.05% (12/63), respectively. The positive rates of CMV IgG, TOX IgG, and EBVCA IgG in CSF of MS patients were significantly lower than those of GBS patients (χ 2=32.839, P<0.001; χ 2=23.343, P<0.001; χ 2=13.861, P<0.001). Conclusions:The MS patients mainly showed the higher positive rates of OCB. The GBS patients showed elevated CSF protein levels but no significant increase in white blood cell count, namely albuminocytologic dissociation in CSF. Meanwhile, the GBS patients showed elevated levels of intrathecal immunity and inflammation indicators, and a higher positive rate of pathogen antibodies.

Ideas and Methods of Acupuncture for Guillain-Barré Syndrome based on the Core Principle of “To Treat Flaccidity， Select the Yangming （阳明） Channel only” / 中医杂志

This study explored the ideas and methods of acupuncture for Guillain-Barré Syndrome （GBS） with the core principle of “to treat flaccidity， select the yangming （阳明） channel only”. The main pathological mechanism of GBS is deficiency of qi and blood in the yangming channel， malnutrition of all sinews， diminished spleen and stomach function leading to the production of pathogenic damp-heat qi， which obstructs the meridians， and gradually affects the liver and kidneys， consuming essence and damaging blood. Concurrently， dysfunction of the dumai （督脉） pivotal mechanism and lack of moisture in sinews and vessels result in symptoms such as skin numbness， paralysis， and muscle wastage. In clinical diagnosis and treatment， a combination of syndrome and channel differentiation is taken. Treatment primarily focuses on acupoints of yangming channel， aiming to supplement qi and blood， and acupoints of du mai are combined to open the vessel and fill the marrow. Specific acupoints are selected based on syndrome differentiation， providing comprehensive regulation to promote harmonization of qi and blood， relieve meridians， and the smooth generation and circulation of whole body fluids. This， in turn， enhances the strength of muscles and bones， and fosters a robust and freely moving body.

Diplejía facial en paciente con nefropatía por virus de inmunodeficiencia humana

Introducción: El síndrome de Guillain-Barré comprende un grupo heterogéneo de polirradiculoneuropatías inflamatorias agudas autoinmunes, las cuales se caracterizan por debilidad simétrica de extremidades con pérdida de reflejos miotáticos. Presenta gran variabilidad clínica, donde la afectación facial es habitual, sin embargo, incluye manifestaciones atípicas que dificultan el diagnóstico temprano de la enfermedad. Objetivo: Describir el comportamiento de un caso atípico de diplejía facial en un paciente con nefropatía por virus de inmunodeficiencia humana. Caso clínico: Se presentó el caso de un paciente portador de nefropatía por virus de la inmunodeficiencia humana, que comenzó con parálisis facial bilateral, como forma de presentación atípica de este síndrome. Acudió a los servicios de salud refiriendo decaimiento marcado, náuseas, vómitos, hipo y dos deposiciones líquidas. Se realizaron estudios que corroboran el diagnóstico. El tratamiento empleado facilitó la recuperación paulatina de la afección. Conclusiones: El diagnóstico precoz de las variantes atípicas de presentación del síndrome, permite un tratamiento oportuno, donde las posibilidades de complicaciones en el paciente son reducidas, así como la mortalidad asociada a la enfermedad.

Introduction: Guillain-Barré Syndrome comprises a heterogeneous group of autoimmune acute inflammatory polyradiculoneuropathies, which are characterized by symmetrical limb weakness with loss of stretch reflexes. It presents great clinical variability, where facial involvement is common; however, it includes atypical manifestations that make early diagnosis of the disease difficult. Objective: To describe the behavior of an atypical case of facial displejía in a patient with nephropathy due to Human Immunodeficiency Virus. Case report: A case of a patient with HIV nephropathy is presented, which begins with bilateral facial paralysis, as an atypical presentation of this syndrome. The patient went to the health services reporting marked weakness, nausea, vomiting, hiccups and two liquid stools. Studies were performed that corroborate the diagnosis. The treatment used facilitated the gradual recovery of the condition. Conclusions: Early diagnosis of the atypical presentation variants of the syndrome allows timely treatment, where the chances of complications in the patient are reduced, as well as the mortality associated with the disease.

The sural-sparing pattern in clinical variants and electrophysiological subtypes of Guillain-Barré syndrome / Padrão de preservação do sural nas variantes clínicas e subtipos eletrofisiológicos da síndrome de Guillain-Barré

Abstract Background Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide and can be classified into electrophysiological subtypes and clinical variants. Objective This study aimed to compare the frequency of the sural-sparing pattern (SSP) in subtypes and variants of GBS. Methods This retrospective cohort study analyzed clinical and electrophysiological data of 171 patients with GBS hospitalized in public and private hospitals of Natal, Rio Grande do Norte, Brazil, between 1994 and 2018; all cases were followed up by the same neurologist in a reference neurology center. Patients were classified according to electrophysiological subtypes and clinical variants, and the SSP frequency was compared in both categories. The exact Fisher test and Bonferroni correction were used for statistical analysis. Results The SSP was present in 53% (57 of 107) of the patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 8% (4 of 48) of the patients with axonal subtypes, and 31% (5 of 16) of the equivocal cases. The SSP frequency in the AIDP was significantly higher than in the axonal subtypes (p < 0.0001); the value was kept high after serial electrophysiological examinations. Only the paraparetic subtype did not present SSP. Conclusion The SSP may be present in AIDP and axonal subtypes, including acute motor axonal neuropathy, but it is significantly more present in AIDP. Moreover, the clinical variants reflect a specific pathological process and are correlated to its typical electrophysiological subtype, affecting the SSP frequency.

Resumo Antecedentes A síndrome de Guillain-Barré (GBS) é a causa mais comum de paralisia flácida aguda em todo o mundo e pode ser classificada em subtipos eletrofisiológicos e variantes clínicas. Objetivo Este estudo teve como objetivo comparar a frequência do padrão de preservação do sural (SSP) em subtipos e variantes de GBS. Métodos É um estudo de coorte retrospectivo que analisou dados clínicos e eletrofisiológicos de 171 pacientes com GBS internados em hospitais públicos e privados de Natal, Rio Grande do Norte, Brasil, entre 1994 e 2018. Todos os casos foram acompanhados pelo mesmo neurologista em centro de referência em neurologia. Os pacientes foram classificados de acordo com os subtipos eletrofisiológicos e variantes clínicas e a frequência do SSP foi comparada em ambas as categorias. O teste exato de Fisher e a correção de Bonferroni foram utilizados para análise estatística. Resultados O SSP esteve presente em 53% (57 de 107) dos pacientes com polirradiculoneuropatia desmielinizante inflamatória aguda (PDIA), em 8% (4 de 48) dos pacientes com subtipos axonais e em 31% (5 de 16) dos casos não definidos. A frequência do SSP no AIDP foi significativamente maior do que nos subtipos axonais (p < 0,0001); o valor manteve-se elevado após exames eletrofisiológicos seriados. Apenas o subtipo paraparético não apresentou SSP. Conclusão O SSP pode estar presente na PDIA e nos subtipos axonais, incluindo a neuropatia axonal motora aguda, mas está significativamente mais presente na PDIA. Além disso, as variantes clínicas refletem um processo patológico específico e estão correlacionadas ao seu subtipo eletrofisiológico típico, afetando a frequência do SSP.

Guillain-Barré syndrome: a review of immunotherapy, rehabilitation, and long-term outcomes

Guillain-Barré syndrome (GBS) is a complex autoimmune disorder characterized by the rapid onset of motor weakness and sensory disturbances, often precipitated by preceding infections. GBS is considered a rare disorder, with an estimated annual incidence ranging from 0.6 to 4.0 cases per 100,000 individuals. It constitutes a medical emergency due to its potential for severe disability or mortality. The hallmark clinical features of GBS include progressive symmetric muscle weakness and sensory disturbances. Diagnosis relies on clinical evaluation, cerebrospinal fluid analysis, and electrodiagnostic studies, with the Brighton criteria providing standardized diagnostic criteria. The hallmark clinical features include progressive symmetric muscle weakness, sensory deficits, and areflexia or hyporeflexia. The management of GBS primarily involves supportive care and immunomodulatory therapies. Plasmapheresis, or plasma exchange, and intravenous immunoglobulin (IVIG) are the mainstay treatments aimed at modulating the immune response and shortening the illness duration. Rehabilitation, encompassing physical and occupational therapy, is pivotal for restoring functionality and improving long-term outcomes.

Síndrome de Guillain-Barré recurrente asociado a vacuna anti-COVID-19. Informe de caso

El Síndrome de Guillain-Barré constituye la causa más frecuente de parálisis flácida aguda en el mundo. Su diagnóstico es clínico y debe ser respaldado por estudios del líquido cefalorraquídeo y de conducción nerviosa. Se produce como consecuencia de una respuesta inmune cruzada ante antígenos de diferentes patógenos, y se ha asociado también a inmunizaciones previas. Existe escasa literatura sobre su recurrencia asociado a la vacunación anti-COVID-19 en el mundo, y hasta donde los autores tienen conocimiento, este es el único informe asociado a la vacuna Abdala (CIGB-66). Se presenta el caso de un paciente masculino de 55 años de edad que refirió pérdida aguda de la fuerza muscular, el cual presentó complicaciones durante su estancia en la Terapia Intensiva, asociadas a la ventilación mecánica. La dificultad en realizar un diagnóstico rápido y certero, especialmente en países subdesarrollados, donde los estudios especializados no se encuentran siempre disponibles y debe hacerse un diagnóstico eminentemente clínico, motivó a los autores a presentar este caso atípico.

Guillain-Barré Syndrome is the most frequent cause of acute flaccid paralysis in the world. Its diagnosis is clinical and must be supported by studies of the cerebrospinal fluid and nerve conduction. It is produced as a consequence of a crossed immune response to antigens from different pathogens, and it has also been associated with previous immunizations. There is little literature on its recurrence associated with anti-COVID-19 vaccination in the world, and as far as the authors are aware, this is the only report associated with the Abdala vaccine (CIGB-66). We present the case of a 55-year-old male patient who reported acute loss of muscle strength, who presented complications during his stay in the Intensive Care Unit, associated with mechanical ventilation. The fact that it is so difficult to give a quick and accurate diagnosis, especially in underdeveloped countries, where specialized studies are not always available and an eminently clinical diagnosis must be made motivated the authors to present this atypical case.

A Síndrome de Guillain-Barré é a causa mais frequente de paralisia flácida aguda no mundo. Seu diagnóstico é clínico e deve ser apoiado por estudos do líquido cefalorraquidiano e da condução nervosa. É produzida como consequência de uma resposta imune cruzada a antígenos de diferentes patógenos, e também tem sido associada a imunizações anteriores. Há pouca literatura sobre sua recorrência associada à vacinação anti-COVID-19 no mundo e, até onde os autores sabem, este é o único relato associado à vacina Abdala (CIGB-66). Apresentamos o caso de um paciente do sexo masculino, 55 anos, que relatou perda aguda de força muscular, que apresentou complicações durante sua internação na Unidade de Terapia Intensiva, associada à ventilação mecânica. A dificuldade de um diagnóstico rápido e preciso, principalmente em países subdesenvolvidos, onde nem sempre há estudos especializados e deve ser feito um diagnóstico eminentemente clínico, motivou os autores a apresentarem este caso atípico.

Características clínicas y laboratoriales de los diez primeros pacientes con diagnóstico de Guillain-Barré en Piura, 2023

El síndrome de Guillain Barré es una enfermedad derivada del compromiso en las neuronas del sistema nervioso periférico por una respuesta descontrolada del sistema inmune que conduce daño axonal y/o desmielinización. El objetivo de este reporte fue describir los 10 primeros casos sospechosos de Síndrome de Guillain Barré en Piura. Se logró identificar la presencia de Campylobacter jejuni en las muestras de heces del 80% de los pacientes reportados. Es muy importante reconocer rápida y oportunamente al paciente con diagnóstico sospechoso de Guillain Barré, y realizar los estudios necesarios en un brote para identificar los agentes desencadenantes del cuadro.

Guillain Barré syndrome is a disease derived from compromise in neurons of the peripheral nervous system by an uncontrolled response from the immune system that leads to axonal damage and/or demyelination. The objective of this report was to describe the first 10 suspected cases of Guillain Barre Syndrome in Piura. It was possible to identify the presence of Campylobacter jejuni in the stool samples of 80% of the reported patients. It is very important to quickly and opportunely recognize the patient with a suspected diagnosis of Guillain Barré, and to carry out the necessary studies in an outbreak to identify the triggering agents of the condition.

Expect the unexpected: a case report of bacterial meningitis causing Guillain-Barré syndrome in an immunocompetent person

This is the case report of a 36-year-old male presenting as acute onset of progressive bilateral lower extremity weakness, initially managed as a case of Guillain-Barré Syndrome (GBS). However, his cerebrospinal fluid tested positive for Neisseria meningitidis Y, Neisseria meningitidis W135, Streptococcus group B antigen, and Streptococcus pneumoniae. The patient was later treated for bacterial meningitis. Most cases of GBS are preceded by an upper respiratory infection or gastroenteritis. There are only a few reported cases of bacterial meningitis occurring coincidentally with GBS, much more in an immunocompetent individual. This is a rare case of such with good patient recovery and outcome.

Síndrome de Guillain-Barré antes y durante la pandemia de COVID-19 en un centro de referencia de México / Guillain-Barre syndrome before and during the COVID-19 pandemic in a referral center of Mexico

Resumen Antecedentes: Durante la pandemia de COVID-19 se ha reportado incremento de casos de síndrome de Guillain-Barré (SGB). Objetivo: Describir características clínicas y pronóstico de pacientes con SGB antes y durante la pandemia de COVID-19. Material y métodos: Cohorte prospectiva de pacientes con SGB estratificados en dos subgrupos: antes (2018-2019) y durante (2020-2021) la pandemia de COVID-19. Se registraron características clínicas, paraclínicas y defunciones. Se definió como buen pronóstico a la recuperación de la marcha independiente a los tres meses. Resultados: Se incluyeron 201 pacientes (123 durante la pandemia y 78 antes), 69 % del sexo masculino, edad de 45 ± 16 años, 2.5 % de muertes intrahospitalarias. Durante la pandemia se observó mayor frecuencia de la variante desmielinizante (50 %), afección de nervios craneales bulbares (44 % versus 28 %), antecedente de vacunación (16 % versus 0 %) y menor puntuación en la escala MRC (30 ± 16.7 versus 34.3 ± 17.7); se observó aumento de casos de julio a septiembre (38 versus 13). No existieron diferencias significativas en la recuperación de la marcha independiente y número de defunciones. Conclusiones: Durante la pandemia se atendió mayor número de casos de SGB, 16 % asociado a la vacuna contra SARS-CoV-2; los pacientes no presentaron peor pronóstico.

Abstract Background: During the COVID-19 pandemic, an increase in the number of Guillain-Barre syndrome (GBS) cases has been reported. Objective: To describe the clinical characteristics and prognosis of patients with GBS before and during the COVID-19 pandemic. Material and methods: Prospective cohort of GBS patients divided in two subgroups: before (2018-2019) and during (2020-2021) the COVID-19 pandemic. Clinical and paraclinical characteristics, as well as deaths, were recorded. A good prognosis was defined as independent ambulation recovery at three months. Results: Two-hundred and one patients were included (123 during and 78 before the pandemic), out of whom 69 % were males; age was 45 ± 16 years, and there was 2.5 % of in-hospital deaths. During the pandemic, a higher frequency of the demyelinating variant (50 %), bulbar cranial nerves involvement (44 % vs. 28 %), prior history of vaccination (16 % vs. 0 %), and a lower MRC score (30 ± 16.7 vs. 34.3 ± 17.7) were documented. An increase in the number of cases was observed from July to September (38 vs. 13). There were no significant differences in independent ambulation recovery or in the number of deaths. Conclusions: During the COVID-19 pandemic, a higher number of GBS cases were treated, out of which 16 % were associated with the SARS-CoV-2 vaccine; patients treated during the pandemic did not have a worse prognosis.

Independência funcional de pacientes com síndrome de Guillain-Barré internados para reabilitação / Functional independence of patients with Guillain-Barré syndrome hospitalized for rehabilitation

Objetivo: Analisar a evolução da independência funcional de pacientes com Síndrome de Guillain-Barré (SGB) internados para reabilitação. Método: Estudo retrospectivo e longitudinal. Foram analisados prontuários de pacientes com diagnóstico de SGB internados para reabilitação, de janeiro de 2015 a março de 2020, que possuíam a Medida de Independência Funcional (MIF) na admissão e alta hospitalar devidamente preenchida, sem distinção de idade. A comparação da MIF antes e depois da internação para reabilitação foi feita por meio do teste t de Student e McNemar. Resultados: A amostra foi composta por 26 pacientes, com média de idade de 41,96 ± 19,67 anos. Os participantes tinham em média 66,07 ± 69,56 dias entre os primeiros sintomas e a admissão para internação para reabilitação. O tempo médio de internação foi de 38,96± 28,36 dias. Houve diferença significativa entre as médias das pontuações, na admissão e alta, nos domínios motor (37,58 - 59,62; p<0,001) e cognitivo (23,19 - 33,35; p<0,001) e escore total da MIF (60,77 - 92,96; p<0,001). Observou-se também aumento do número de pacientes com independência completa após internação para reabilitação (2 - 15; p<0,001). Conclusão: Pacientes com SGB internados para reabilitação apresentam melhora da independência funcional. É importante acesso a esta modalidade terapêutica multiprofissional aos pacientes com SGB.

Objective: To analyze the evolution of the functional independence of patients with Guillain-Barré Syndrome (GBS) hospitalized for rehabilitation. Method: Retrospective and longitudinal study. Medical records of patients with diagnosis of GBS hospitalized for rehabilitation were analyzed, from January 2015 to March 2020, who had the Functional Independence Measure (FIM) at admission and hospital discharge duly filled, regardless of age. The comparison of FIM before and after hospitalization for rehabilitation was performed using Student's t-test and McNemar's test. Results: The sample consisted of 26 patients, with a mean age of 41.96 ± 19.67 years. Participants had a mean of 66.07 ± 69.56 days between the first symptoms and hospital admission for rehabilitation. The mean length of stay was 38.96 ± 28.36 days. There was a significant difference between the mean scores, at admission and discharge, in the motor domain (37.58-59.62; p<0.001) and cognitive domain (23.19-33.35; p<0.001) and total FIM score (60.77-92.96; p<0.001). There was also an increase in the number of patients with complete independence after hospitalization for rehabilitation (2-15; p<0.001). Conclusion: Patients with GBS hospitalized for rehabilitation show improved functional independence. Access to this multiprofessional therapeutic modality is important for patients with GBS.

Concurrencia de síndrome de Guillain-Barré y colangitis biliar primaria no vinculado a SARS-CoV-2: reporte de caso / Concurrence of Guillain-Barré syndrome and primary biliary cholangitis not related to SARS-CoV-2: case report

INTRODUCCIÓN: El síndrome de Guillain-Barré es una polirradiculoneuropatia de origen autoinmune, considerada la causa más frecuente de parálisis flácida aguda. Se han reportado diversas asociaciones del síndrome de Guillain-Barré con otras enfermedades autoinmunes no neurológicas, algunas de ellas extremadamente raras, como la que ocurre con la colangitis biliar primaria, una enfermedad crónica de etiología autoinmune cuyo diagnóstico se sustenta, además del cuadro clínico, en la alteración de las enzimas hepáticas y la presencia de anticuerpos anti-mitocondriales. CASO CLÍNICO: Paciente varón de 38 años, sin antecedente de comorbilidades previas, quien luego de presentar enfermedad diarreica dos semanas antes, desarrolló debilidad ascendente de inicio subagudo asociado a parestesias en cuatro extremidades que progresó hasta generar cuadriplejia y dificultad respiratoria. Se le realizó examen citoquímico de líquido cefalorraquídeo que evidenció disociación albumino-citológica y electromiografía que mostró hallazgos compatibles con neuropatía axonal motora aguda. Recibió tratamiento con inmunoglobulina intravenosa a dosis de 0,4 gramos por kilogramo al día, logrando mejoría del cuadro neurológico. Desde su ingreso y durante la hospitalización, presentó alteración persistente de las enzimas hepáticas que seguía un patrón colestásico. Además, se agregó dolor abdominal de leve intensidad y prurito generalizado, por lo cual fue evaluado por gastroenterología, quienes solicitaron anticuerpos anti-mitocondriales que resultaron positivos. Con esta prueba, se comprobó el diagnóstico de colangitis biliar primaria. CONCLUSIÓN: El presente caso muestra una asociación extremadamente rara de dos enfermedades autoinmunes; síndrome de Guillain-Barré y colangitis biliar primaria, tanto así que representa el primer caso reportado, no vinculado a SARS-CoV-2.

INTRODUCTION: Guillain-Barré syndrome is a polyradiculoneuropathy of autoimmune origin, considered the most frequent cause of acute flaccid paralysis. Various associations of Guillain-Barré syndrome with other non-neurological autoimmune diseases have been reported, some of them extremely rare, such as that which occurs with primary biliary cholangitis, a chronic disease of autoimmune etiology whose diagnosis is also supported by the clinical picture. , in the alteration of liver enzymes and the presence of anti-mitochondrial antibodies. CLINICAL CASE: A 38-year-old male patient, with no history of previous comorbidities, who, after presenting with diarrheal disease two weeks prior, developed subacute onset ascending weakness associated with paresthesias in four extremities that progressed to quadriplegia and respiratory distress. Cerebrospinal fluid cytochemistry was performed, which showed albuminocytological dissociation and electromyography, which showed findings compatible with acute motor axonal neuropathy, for which he received treatment with intravenous immunoglobulin at 0.4g/kg/day, achieving improvement in the neurological condition. Since admission and during hospitalization, he presented persistent changes in liver enzymes which followed a cholestatic pattern, in addition to mild abdominal pain and generalized itching, for which he was evaluated by gastroenterology, who requested anti-mitochondrial antibodies that were positive. Concluding in the diagnosis of primary biliary cholangitis. CONCLUSION: The present case shows an extremely rare association of two autoimmune diseases Guillain-Barré syndrome and primary biliary cholangitis, so much so that it represents the first case reported, not linked to SARS-CoV-2.

Neuropsychiatric systemic lupus erythematosus: a rare case of acute polyneuropathy

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease which presents most commonly in middle aged females and affects multiple organ systems. Amongst the many systems involved, the nervous system generally gets affected later in the course of the disease. We report a case of a male patient who developed quadriparesis as the initial presentation of SLE who progressed to lupus nephritis. The patient was started on methylprednisolone, and later planned on IVIg when he did not respond to the initial treatment. Unfortunately, the patient developed diaphragmatic paralysis and succumbed to the illness.

Clinical features of Guillain-Barré syndrome with positive anti sulfatide antibody in CSF and hyponatremia(report of one case) / 临床神经病学杂志

Objective To summarize the diagnosis and treatment experience of patients with Guillain-Barré syndrome(GBS)with positive anti sulfatide antibody in CSF.Methods The clinical data of a case of patient with GBS with positive anti sulfatide antibody in CSF in Department of Neurology,The Affiliated Brain Hospital of Nanjing Medical University in January,2023 were retrospectively analyzed,and the relevant literatures were reviewed.Results Two cases with GBS with positive anti sulfatide antibody in CSF from 2 literatures were retrieved,a total of 3 cases were retrieved.All cases were males.The onset duration was 4-6 d.Two patients with GBS with positive anti-sufatide antibody in CSF developed limb weakness,severe back and limb pain.Albuminocytologic dissociation in CSF and inefficacy of immunoglobin were found in the two cases.Severe hyponatremia secondary to intravenous immunoglobin was observed in our case.One patient presented with cranial nerve damage with mild elevation of CSF protein and improvement after immunoglobulin.Conclusion The plasmapheresis was recommended for the patients presenting with limb weakness with positive anti-sulfatide antibody in CSF in order to prevent inefficacy and severe hyponatremia secondary to intravenous immunoglobin.