Guillain-Barré syndrome associated with SARS-CoV-2 infection: a scoping review

SUMMARY BACKGROUND: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections can affect the nervous system, triggering problems such as the Guillain-Barre Syndrome (GBS), an association that can bring complications to the patient. OBJECTIVE: This scoping review aimed to clarify the clinical features and analyze patients with GBS associated with SARS-CoV-2 infection, looking at morbidity, mortality, and neurological outcomes. SEARCH STRATEGY: The search was conducted through Medline, Web of Science, Embase, CINAHAL, Latin-American and Caribbean Literature in Health Sciences (LILACS), clinicaltrials.gov, SCOPUS, and the Cochrane Central Register of Controlled Trials. SELECTION CRITERIA: Observational studies, published after 2019, describe patients with GBS associated with SARS-CoV-2 infection. There were no language restrictions while selecting the studies. DATA COLLECTION AND ANALYSIS: Three authors, Kleyton Santos de Medeiros, Luíza Thomé de Araújo Macêdo, and Wederson Farias de Souza, independently screened the search results using titles and abstracts. Duplicate studies were excluded. The same authors then went through the entire text to determine whether the studies met the inclusion criteria. Discrepancies were resolved by other reviewers, Ana Paula Ferreira Costa, Ayane Cristine Sarmento, and Ana Katherine Gonçalves. Finally, the selection of the studies was summarized in a PRISMA flow diagram. MAIN RESULTS: Main manifestations were fever, coughing, dyspnea, sore throat, ageusia, anosmia, and respiratory failure, in addition to paresthesia of the upper and lower limbs, tetraparesis, facial diplegia, areflexia, asthenia, mastoid pain, acute ataxia, fatigue, numbness, swallowing disorder, and moderate low back pain. CONCLUSION: Coronavirus disease 2019 (COVID-19) can trigger the GBS, despite the few studies on this topic. Patients had clinical manifestations of COVID-19 infection and neurological manifestations characterizing GBS.

Síndrome de Guillain Barré e hidrocefalia en un lactante con Síndrome de Wiskott Aldrich / Guillain-Barré Syndrome and Hydrocephalus in an infant with Wiskott-Aldrich Syndrome

Resumen: Introducción: El Síndrome de Guillain-Barré (SGB) es raramente diagnosticado en lactantes menores de 1 año. Su asociación con el Síndrome de Wiskott Aldrich (SWA), es aún menos frecuente, y ha sido previa mente reportada sólo en dos pacientes a nuestro conocimiento. La hidrocefalia, es una complicación conocida, pero infrecuente del SGB. Objetivo: presentar el caso clínico de un lactante en el que se asocian las patologías de SGB, SWA e hidrocefalia. Caso Clínico: varon de 9 meses, portador de SWA hospitalizado en unidad de cuidados intensivos por hipotonía aguda y compromiso del estado gene ral. Evolucionó con parálisis fláccida, falla ventilatoria y arreflexia generalizada. Una punción lumbar mostró disociación albuminocitológica, y el estudio electrofisiológico mostró signos de polineuropatía desmielinizante severa. Se trató con inmunoglobulina, evolucionando en forma satisfactoria. Por bradicardia intermitente, se realizó tomografla axial computada cerebral (TAC), que mostró signos de una hidrocefalia aguda, manejada mediante válvula derivativa ventrículo peritoneal con favorable respuesta. En el largo plazo, se sometió a trasplante de médula ósea y debió ser reintervenido por complicaciones valvulares, sin embargo, su desarrollo psicomotor es normal sin secuelas neurológi cas evidentes hasta los 3 años. Conclusión: Presentamos el tercer caso de SGB en un paciente porta dor de SWA, destacando ser el primero de ellos en un lactante menor de 1 año. Adicionalmente, este niño presentó una hidrocefalia aguda como complicación del SGB. Consideramos relevante tener presente estas comorbilidades, debido a que su pronto diagnóstico y manejo oportuno, permiten una mejor recuperación neurológica y evitan complicaciones potencialmente letales.

Abstract: Introduction: Guillain-Barre Syndrome (GBS) is rarely diagnosed in the first year of life. The association of GBS with Wiskott-Aldrich syndrome (WAS) is even less frequent and has been previously reported in only two children to our knowledge. Hydrocephalus is a known but rare complication of GBS. Objective: To describe the case of an infant in which GBS, WAS and hydrocephalus appear clinically associated. Clinical Case: A nine-months-old male infant with a history of WAS was admitted to our ICU with acute hypotonia and poor general condition. He developed flaccid paralysis, absent deep tendon reflexes, and respiratory failure. A lumbar puncture showed albuminocytologic dissociation. GBS was suspected and an electromyography was performed, showing a demyelinating polyneuropathy. He was successfully treated with intravenous immunoglobulins. During hospitalization, he presented intermittent bradycardia, so a brain CT scan was performed, showing acute hydrocephalus which was managed through an external ventricular drain achieving favorable response. In the long term, the patient underwent bone marrow transplant and had to be reoperated due to valve-related complications. However, his psychomotor development is normal, with no obvious neurological sequelae. Conclusion: We present the third case of GBS in a patient with WAS, which is the first infant younger than one year. Additionally, he presented acute hydrocephalus as a complication of GBS. We suggest considering these three comorbidities since their early diagnosis and prompt management allow bet ter neurological recovery and avoid potentially lethal complications.

Síndrome de Guillain-Barré e sua relação com o calendário vacinal brasileiro: uma revisão de literatura / Guillain-Barré syndrome and its relationship with the brazilian vaccine calendar: a literature review

A síndrome de Guillain-Barré (SGB) é uma polineuropatia inflamatória desmielinizante aguda, geralmente pós-infecciosa e mediada pelo sistema imune, levando a graus variados de fraqueza progressiva e ascendente, podendo atingir os membros superiores e a face. A SGB é a causa mais frequente de paralisia flácida aguda e subaguda desde a erradicação da poliomielite. Estudos mostram a associação de SGB e diversas vacinas do calendário vacinal brasileiro. Este artigo tem por objetivo estabelecer as vacinas como fator desencadeante de SGB que compõem o Programa Nacional de Imunização. Com o emprego em larga escala das vacinas em nosso meio, estudos mostram a associação temporal significante com a SGB. Recomenda-se, portanto, a descrição dos casos suspeitos dessa associação. A vacina continua sendo o método mais efetivo para prevenir doenças graves e morte.(AU)

Guillain-Barré syndrome (GBS) is a post-infectious, immune-mediated, acute inflammatory demyelinating polyneuropathy, leading to varying degrees of progressive and ascending weakness, reaching the upper limbs and a face. GBS is the most frequent cause of acute and subacute flaccid paralysis since poliomyelitis eradication. Studies show an association between GBS and several vaccines of the Brazilian vaccine calendar. This article aims to establish GBS as a triggering factor for some vaccines that make up the National Immunization Program. With the large-scale use of vaccines in our country, studies show a significant temporal association with a GBS, it is therefore recommended a description of the suspected cases of the association. The vaccine continues to be the most effective method to prevent grave diseases and death.(AU)

Syndrome of inappropriate antidiuretic hormone secretion related to Guillain-Barré syndrome after laparoscopic cholecystectomy / Síndrome da secreção inapropriada do hormônio antidiurético relacionada à síndrome de Guillain-Barré após colecistectomia videolaparoscópica / Síndrome de secreción inapropiada de hormona antidiurética relacionado con el síndrome de Guillain-Barré tras colecistectomía laparoscópica

Background and objectives: Guillain-Barré Syndrome is one of the most common causes of acute polyneuropathy in adults. Recently, the occurrence of Guillain-Barré Syndrome after major and minor surgical operations has been increasingly debated. In Guillain-Barré syndrome, syndrome of inappropriate antidiuretic hormone secretion and dysautonomy are generally observed after maximal motor deficit. Case report: A 44-year-old male patient underwent a laparoscopic cholecystectomy for acute cholecystitis. After the development of a severe headache, nausea, diplopia, and attacks of hypertension in the early postoperative period, a computer tomography of the brain was normal. Laboratory tests revealed hyponatremia linked to syndrome of inappropriate antidiuretic hormone secretion, the patient's fluids were restricted, and furosemide and 3% NaCl treatment was initiated. On the second day postoperative, the patient developed numbness moving upward from the hands and feet, loss of strength, difficulty swallowing and respiratory distress. Guillain-Barré syndrome was suspected, and the patient was moved to intensive care. Cerebrospinal fluid examination showed 320 mg/dL protein, and acute motor-sensorial axonal neuropathy was identified by electromyelography. Guillain-Barré syndrome was diagnosed, and intravenous immune globulin treatment (0.4 g/kg/day, 5 days) was initiated. After 10 days in the intensive care unit, at which the respiratory, hemodynamic, neurologic and laboratory results returned to normal, the patient was transferred to the neurology service. Conclusions: Our case report indicates that although syndrome of inappropriate antidiuretic hormone secretion and autonomic dysfunction are rarely the initial characteristics of Guillain-Barré syndrome, the possibility of postoperative syndrome of inappropriate antidiuretic hormone secretion should be kept in mind. The presence of secondary hyponatremia ...

Justificativa e objetivos: a síndrome de Guillain-Barré é uma das causas mais comuns de polineuropatia aguda em adultos. Recentemente, a ocorrência após grandes e pequenas intervenções cirúrgicas tem sido cada vez mais debatida. Na Guillain-Barré, a síndrome da secreção inapropriada do hormônio antidiurético e a disautonomia são geralmente observadas após déficit motor máximo. Relato de caso: paciente do sexo masculino, 44 anos, submetido a colecistectomia videolaparoscópica para colecistite aguda. Após desenvolver uma forte dor de cabeça, náusea, diplopia e ataques de hipertensão no período pós-operatório imediato, uma tomografia computadorizada do cérebro revelou-se normal. Os exames laboratoriais revelaram hiponatremia associada à síndrome de secreção inadequada de hormônio antidiurético; os líquidos foram restritos e tratamento com furosemida e NaCl a 3% foi iniciado. No segundo dia pós-operatório, o paciente desenvolveu dormência que se propagava a partir das mãos e dos pés, perda de força, dificuldade para engolir e respirar. Suspeitou-se de síndrome de Guillain-Barré e o paciente foi transferido para a unidade de tratamento intensivo. Exame do líquido cefalorraquidiano revelou 320 mg/dL de proteína e neuropatia axonal sensório-motora aguda foi identificada por eletromiografia. Síndrome de Guillain-Barré foi diagnosticada e tratamento intravenoso com imunoglobuliva (0,4 g/kg/dia, cinco dias) foi iniciado. Após 10 dias na unidade de terapia intensiva, durante os quais os parâmetros respiratório, hemodinâmico, neurológicos e laboratoriais voltaram ao normal, o paciente foi transferido para o serviço de neurologia. Conclusões: nosso relato de caso indica que, ...

Justificación y objetivos: el síndrome de Guillain-Barré es una de las causas más comunes de polineuropatía aguda en adultos. Recientemente, la aparición del síndrome de Guillain-Barré después de cirugías mayores o menores se ha convertido en objeto de debate cada vez mayor. En el síndrome de Guillain-Barré, generalmente se observan síndrome de secreción inapropiada de hormona antidiurética y disautonomía después de un déficit motor máximo. Relato de caso: paciente de sexo masculino, 44 años, sometido a colecistectomía laparoscópica por colecistitis aguda. Después de comenzar con un fuerte dolor de cabeza, náuseas, diplopía y ataques de hipertensión en el período postoperatorio inmediato, una tomografía computadorizada del cerebro del paciente se reveló normal. Las pruebas de laboratorio revelaron hiponatremia asociada con el síndrome de secreción inadecuada de hormona antidiurética; los líquidos se restringieron y se inició tratamiento con furosemida y NaCl al 3%. En el segundo día del postoperatorio, el paciente presentó adormecimiento que se propagaba desde las manos y los pies, pérdida de fuerza, dificultad para tragar y para respirar. Se sospechó síndrome de Guillain-Barré y el paciente fue derivado a la unidad de cuidados intensivos. El examen del líquido cefalorraquídeo reveló 320 mg/dL de proteína y mediante electromiografía se identificó neuropatía axonal sensorial y motora aguda. Se diagnosticó síndrome de Guillain-Barré y se inició el tratamiento intravenoso con inmunoglobulina (0,4 g/kg/día, durante 5 días). Después de 10 días en la unidad de cuidados intensivos, durante los cuales los parámetros respiratorio, hemodinámico, neurológico ...

Síndrome de Guillain-Barré em associação temporal com a vacina influenza A / Guillain-Barré syndrome in temporal association with influenza A vaccine

OBJETIVO:Descrever um caso de síndrome de Guillain-Barré em associação temporal com a vacina influenza A (H1N1) 2009. DESCRIÇAO DO CASO: Menino de quatro anos com queixa inicial de dor em coxa direita e perda de força muscular ascendente 15 dias após a segunda dose da vacina influenza A (H1N1) 2009. Ao exame neurológico apresentava tetraparesia e arreflexia, com predomínio em membros inferiores. A eletroneuromiografia evidenciou redução da velocidade e bloqueio de condução neuronal, com discreta perda axonal secundária. Foi tratado com imunoglobulina por via intravenosa, atingiu platô no quarto dia de evolução da doença e, depois, houve melhora progressiva da força muscular. COMENTÁRIOS: Com o emprego em larga escala da vacina influenza A (H1N1) 2009 em nosso meio e os dados preliminares do sistema de vigilância norte-americano mostrando associação temporal significante com a síndrome de Guillain-Barré, recomenda-se a descrição dos casos suspeitos dessa associação. A vacina continua sendo o método mais efetivo para prevenir doença grave e morte por influenza.

OBJECTIVE: To report a case of Guillain-Barré syndrome following influenza A (H1N1) 2009 vaccine. CASE DESCRIPTION: A four-year-old boy presented right thigh pain and ascending muscular weakness 15 days after the second dose of influenza A (H1N1) 2009 vaccine. The neurological examination revealed tetraparesis and areflexia. Electroneuromyography showed lower velocity and conduction blockage with small secondary axonal loss. Treated with intravenous immunoglobulin, the patient reached a plateau in the 4th day, followed by progressive muscular strength improvement. COMMENTS: The employment of large-scale influenza A (H1N1) 2009 vaccination and the preliminary reports from the American Surveillance Program suggest a significant association between Guillain-Barré syndrome and influenza A H1N1 2009 vaccination. All suspected cases of this association should be published for further evaluation. Vaccination remains the most effective method to prevent serious illness and death related to influenza.

Acute inflammatory demyelinating polyradiculoneuropathy (Guillain-Barré syndrome) following dengue fever / Polirradiculoneuropatia desmielinizante inflamatória aguda (síndrome de Guillain-Barré) após dengue

This paper reports a case of dengue in a six-year-old female child who suddenly developed excruciating headaches, fever, myalgia and paresis. Laboratory examinations included blood count, platelet count, biochemical tests (BUN, creatinine, aminotransferases, and total bilirubin and bilirubin fractions) and specific IgM titers (enzyme-immunoassay with recombinant tetravalent dengue). After ten days of hospitalization and having already been in a home environment, a new clinical image emerged, characterized by dysphagia, dysphonia, weakness, peripheral facial palsy and paresthesia. The diagnosis of Guillain-Barré Syndrome was based on clinical findings, cerebrospinal fluid examination, electrophysiological findings and the exclusion of other pathologies. Our case, as some shown in previous reports, calls attention to the possibility that Guillain-Barré Syndrome may occur in association with dengue.

Este trabalho relata o caso de uma criança, sexo feminino, seis anos que desenvolveu subitamente cefaléia lancinante, febre, mialgia e paresia. Os exames de investigação clínica, que incluíram hemograma, contagem de plaquetas, dosagens bioquímicas (uréia, creatinina, transferases e billirrubina total e frações) e por títulos específicos de IgM, por enzima-imunoensaio (EIA) com antígeno tetravalente de dengue. Após dez dias de internação e já em ambiente domiciliar, novo quadro clínico surgiu caracterizado por disfagia, disfonia, paresia, paralisia facial periférica e parestesias. O diagnóstico do dengue e da Síndrome de Guillain-Barré foi baseado nos achados clínicos, no exame do líquido cefalorraquidiano, achados eletrofisiológicos e na exclusão de outras patologias. Neste caso, como em alguns relato anteriores, chama a atenção para a possibilidade de que Síndrome de Guillain-Barré pode ocorrer em associação com a dengue.

Guillain Barre syndrome in a family: a case report of four siblings

Although generally a sporadic disease, a few cases of Guillain Barre syndrome clustered in families have been reported. We describe four siblings of a Pakistani family from a consanguineous marriage out of whom two developed definite GBS and two had probable GBS at different times. This suggests that there may be a genetic element in the pathogenesis of at least some forms of Guillain Barre syndrome

Dengue infection in patients presenting with neurological manifestations in a dengue endemic population / Infección de dengue en pacientes que se presentan con manifestaciones neurológicas en una población con dengue endémico

The evaluation of the contribution of neurological dengue in suspected central nervous system (CNS) viral infections is essential to better understand the impact of neurological dengue on morbidity and mortality in dengue endemic regions such as Jamaica. For this study, 401 cases of suspected viral CNS infections were investigated for evidence of dengue infection. The frequency of neurological dengue among these CNS cases was found to be 13.5% (54/401). Fifty-three cases were confirmed serologically by haemagglutination inhibition assay (HI) and IgM antibody (ELISA) and the virus was isolated in one case only. Clinical manifestations among dengue positive CNS cases included encephalitis in 51.8% (28/54), meningitis in 33.3% (18/54), seizures in 11.1% (6/54) and acute flaccid paralysis/Guillain-Barré syndrome in 3.7% (2/54). The clinical diagnosis of dengue neurological infection corresponded with laboratory confirmation in 22.2% (12/54) of cases only. Deaths occurred in 3.7% (2/54) of cases and were associated with patients with dengue neurological infection. The high risk of dengue among patients with suspected viral CNS infections in this study supports the need for an increased index of suspicion of dengue in patients presenting with neurological manifestations in dengue endemic countries.

La evaluación de la contribución del dengue neurológico en las infecciones virales sospechadas del sistema nervioso central (SNC) resulta esencial para un mejor entendimiento del impacto del dengue neurológico en la morbilidad y mortalidad en regiones donde el dengue es endémico tales como Jamaica. Para este estudio 401 casos de infecciones virales sospechadas del SNC fueron investigados en busca de evidencia de infección por dengue. Se haló entonces que la frecuencia del dengue neurológico entre estos casos de SNC, fue de 13.5% (54/401). Cincuenta y tres casos fueron confirmados por serología mediante ensayos de inhibición de hemaglutinación (IH) y ELISA para la detección de anticuerpos IgM, siendo el virus aislado sólo en un caso. Las manifestaciones clínicas entre los casos que resultaron positivos al dengue, incluyeron encefalitis en 51.8 % (28/54), meningitis en 33.3% (18/54), convulsiones en 11.1% (6/54) y parálisis facial aguda/síndrome Barré Guillain en 3.7% (2/54). El diagnóstico clínico de infección neurológica por dengue estuvo en correspondencia con la confirmación del laboratorio sólo en el 22.2% (12/ 54) de los casos. Se produjeron muertes en el 3.7% (2/54) de los casos, las cuales estuvieron asociadas con pacientes con infección neurológica por dengue. El alto riesgo de dengue entre los pacientes con sospecha de infecciones virales de SNC en este estudio, apunta a la necesidad de aumentar el índice de sospecha de dengue en pacientes que se presentan con manifestaciones neurológicas en países donde el dengue es endémico.

Guillain-Barré syndrome after brachial plexus trauma: case report / Síndrome de Guillain-Barré após traumatismo de plexo braquial: relato de caso

The Guilllain-Barré syndrome (GBS) is an acute predominantly demyelinating polyneuropathy. In many cases GBS is preceding by infection, immunization, surgery or trauma. Although there are a few reports of GBS after head trauma, there is no report of this syndrome after brachial plexus injury. We report on a 51 years-old man who presented GBS fifteen days after a brachial plexus trauma. The polineuropathy resolved completely in a few weeks. We believe that GBS was triggered by the trauma that evoked an immune mediated disorder producing inflammation and demyelination of the peripheral nerves.

A síndrome de Guillain-Barré (SGB) é uma polineuropatia predominantemente desmielinizante, que ocorre na maioria das vezes após uma infecção, vacinação, cirurgia ou traumatismo. Embora tenham sido descritos alguns casos após traumatismo crânio encefálico, ainda não foi referido caso de SGB após traumatismo do plexo braquial. Relatamos o caso de um homem de 51 anos que 15 dias após ter apresentado paralisia traumática do plexo braquial, desenvolveu SGB. Recuperou-se inteiramente em algumas semanas. Achamos que em nosso caso a SGB foi desencadeada pelo traumatismo, que provocou distúrbios imunológicos com conseqüente acometimento dos nervos periféricos.

Anestesia em criança com síndrome de Guillain-Barré após vacina de sarampo: relato de caso / Anesthesia in children with Guillain-Barré syndrome after measles vaccination: case report

JUSTIFICATIVA E OBJETIVOS: A síndrome de Guillain-Barré após vacina de sarampo é rara. O diagnóstico muitas vezes é tardio, o que leva a um aumento da morbidade. O presente relato apresenta um caso avançado e os cuidados especiais exigidos durante a anestesia. RELATO DO CASO: Paciente do sexo masculino, com quatro anos de idade com síndrome de Guillain-Barré desde um ano de idade, foi submetido a gastrostomia sob anestesia geral sem intercorrências, com sevoflurano e sem bloqueadores neuromusculares. CONCLUSÕES: O caso ilustra a raridade etiológica de uma síndrome importante na prática anestésica assim como os eventos adversos pós-vacinação, a melhor escolha para a equipe anestésica e as complicações da síndrome de Guillain-Barré na infância.

Guillain-Barre syndrome associated with acute hepatitis E.

A 58-year-old woman presented with a brief icteric illness followed by progressive bilateral symmetrical hypotonic areflexic muscular weakness and unilateral infranuclear facial palsy. She was diagnosed to be suffering from Guillain-Barre syndrome and acute hepatitis E. Such an association has not been described till date.

Guillain-Barré syndrome in the course of dengue: case report

Este relato de caso descreve os achados de uma mulher de 45 anos, branca, que desenvolveu mialgia, febre e eritema macular. Ela recebeu o diagnóstico de dengue, tomando por base os achados clínicos e títulos específicos de IgM. Uma semana depois do início dos primeiros sintomas de dengue, a paciente desenvolveu fraqueza muscular, tetraplegia e insuficiência respiratória. A eletromiografia mostrou evidência de uma neuropatia desmielinizante e o liquor apresentou quadro de dissociação albuminocitológica. Os achados neurológicos foram consistentes com o diagnóstico de síndrome de Guillain-Barré. A paciente foi tratada com imunoglobulina e metilprednisolona. Ventilação mecânica foi iniciada uma semana após a admissão hospitalar, sendo mantida por quatro semanas. Após seis semanas de internamento a paciente teve alta em cadeira de rodas, apresentando fraqueza muscular e perda dos reflexos patelar e aquileu. Quando a paciente foi vista no ambulatório, três semanas após a alta hospitalar, ela já era capaz de andar com ajuda do acompanhante. Este relato de caso sugere uma possível associação entre dengue e síndrome de Guillain-Barré.

Report of five children with Guillain-Barré syndrome following a nationwide oral polio vaccine campaign in Turkey.

Five children with Guillain-Barré syndrome (GBS), following a national oral polio vaccination campaign to eradicate disease, are reported. Clinical examination, cerebrospinal fluid and electromyographic findings conformed to the classical description of GBS. Four of them received therapeutic dose of intravenous immunoglobulin G. Two children succumbed to the disease. It was observed that the number of cases of GBS in children increased during the period of the oral polio vaccination campaign in Turkey, suggesting a causal relationship.

Síndrome de Guillain Barré / Guillian Barré's syndrome

El síndrome de Guillain Barré es una polineuropatía desmielinizante inflamatoria aguda de origen desconocido. Su fisiopatología no está completamente aclarada y se señala que un organismo infeccioso induce una respuesta inmunológica, de origen tanto humoral como celular, la que produce una reacción cruzada contra la vaina de mielina de los nervios periféricos que causa su destrucción. Se realizó una exposición de los principales eventos fisiopatológicos que desencadenan la insuficiencia respiratoria. Se comentaron ampliamente los criterios diagnósticos actuales, que incluyen los resultados de los estudios electrofisiológicos, se señalaron los criterios establecidos para iniciar la asistencia respiratoria. Por último, se presentaron las principales medidas terapéuticas, sus indicaciones, dosis, y los diferentes estudios que avalan el uso de cada una de ellas

Guillain-Barre syndrome in a case of acute lymphoblastic leukaemia. A case report

To report a case of severe Guillain-Barr‚ syndrome in a 32-year old female patient diagnosed with acute lymphoblastic leukaemia who was on chemotherapy. Clinical Presentation and Intervention: The patient received chemotherapy including vincristine and steroids according to the Medical Research Council United Kingdom Acute Lymphoblastic Leukaemia-12 [MRC UKALL-12] protocol. On the 21st day of the first induction course she developed acute fulminant quadriparesis with total areflexia. The clinical features, nerve conduction and the cerebrospinal fluid studies were consistent with acute Guillain-Barr‚ syndrome. She was treated with a 5-day course of intravenous immunoglobulins [IVIG] that resulted in only partial improvement. A second course of IVIG was given 2 weeks later that improved her condition slowly and steadily over a period of 12-16 weeks; the patient was able to walk with minimal support. The fulminant neuropathy was most likely due to the association between Guillain-Barr‚ syndrome and leukaemia rather than vincristine neurotoxicity. IVIG was an effective and non-invasive treatment for Guillain-Barr‚ syndrome associated with the malignancy

Síndrome de Guillain-Barré: etiología y patogénesis / Guillain-Barre syndrome: etiology and pathogenesis

Guillain-Barre syndrome (GBS) is a reactive, self-limited, monophasic disease triggered by a preceding bacterial or viral infection. GBS has also been linked to underlying systemic diseases, certain malignancies, surgery, pregnancy, trauma severe infection, and tissue transplantation (bone marrow and organs). Although its pathogenesis is unclear, it is likely to be a consequence of an immune mediated process. Therefore, we believe that GBS results from an aberrant immune response that somehow mistakenly attacks the nerve tissue of its host, most probably by recognizing a molecular similar epitope mechanism (molecular mimicry). Immune reactions against these epitopes result in acute inflammatory demyelinating neuropathy or acute axonal forms. GBS has a worldwide distribution with an annual incidence of approximately 1.2-8.6 cases per 100,000 people. Both genders are at similar risk (but there is a slight male predominance). All ages are affected, although the distribution is bimodal. The supporting measures are critically important to provide optimal treatment. Immunomodulation with plasma exchange and intravenous immunoglobulin treatments shorten the disease course. Outcome is generally good, with virtually full recovery in 70-80 of the patients. In this review physiopathological aspects and clinical implications of GBS are fully discussed.

Guillain-Barre syndrome and Campylobacter infection.

Campylobacter infection of the gastrointestinal tract has been observed as an antecedent illness in some patients with Guillain-Barre syndrome (GBS); these patients have been reported to have poor prognosis. We investigated 29 patients with GBS, admitted to our hospital from January 1996 to December 1999 for recent Campylobacter enteritis by culture of their stool specimens. Campylobacter upsaliensis and C. jejuni were isolated from stools of one patient each with acute motor axonal neuropathy (AMAN) and acute inflammatory demyelinating polyradiculoneuropathy (AIDP) respectively. The patient with C. upsaliensis infection was a 7 year-old male child who developed features of AMAN, 7 days after onset of diarrhea. He recovered gradually within 24 days with residual deficit in the form of foot drop. This deficit has persisted for last three and half years. The other patient with C. jejuni infection was a 9 year-old boy, who developed AIDP after 9 days of acute diarrhea. This patient recovered completely within 28 days of illness without any deficit. None of the patients had relapse of GBS. The present findings indicate the need of planned systematic studies to explore the role of C. upsaliensis and other campylobacters as agents of antecedent diarrhea in patients of GBS with different clinical presentations and prognosis.