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With the outbreak of coronavirus disease 2019 (COVID-19), the whole world was impacted by a pandemic. With the passage of time and knowledge about the dynamics and viral propagation of this disease, the short-, medium- and long-term repercussions are still being discovered. During this period, it has been learned that various manifestations of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can affect the nervous system. In recent months, a variety of studies and case reports have proposed an association between COVID-19 and Guillain-Barré syndrome (GBS). The present work aims to systematically review the publications available to date to verify the relationship between these two pathologies and the characteristics of post-COVID GBS. There were 156 studies included in this work, resulting in a total of 436 patients. The findings show a mean age of the patients of 61,38 years and a male majority. The GBS symptoms began on average 19 days after the onset of COVID-19 infection. Regarding GBS, the main manifestations found included generalized weakness, reflex reduction, facial paresis/paralysis and hypoesthesia. As expected, the most common result in cerebrospinal fluid (CSF) analysis was albuminocytological dissociation. A pattern of blood analysis findings common to all patients was not observed due to non-standardization of case reports. Regarding electrodiagnostic studies, acute inflammatory demyelinating polyneuropathy (AIDP) appeared as the most common subtype of GBS in this study. There have been reports, to a lesser extent, of acute motor axonal neuropathy (AMAN), acute sensorimotor axonal neuropathy (AMSAN), the pharyngeal-cervical-brachial variant (PCB), and Miller-Fisher syndrome (MFS). The GBS treatment used was mainly intravenous immunoglobulin (IVIG) and plasma exchange (PLEX). Therefore, the present study reports a high prevalence of hospitalization and intensive care units ICU admissions, conjecturing a relationship between the development of GBS and the severity of COVID-19. Despite the severity, most patients showed improvement in GBS symptoms after treatment, and their residual symptoms did not include motor involvement. Therefore, the development of GBS seems to be related to COVID-19 infection, as reported by the present systematic review.

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ABSTRACT Purpose: To evaluate the clinical features of pediatric patients with acute-onset, unilateral transient acquired blepharoptosis. Methods: In this retrospective study, the clinical records of patients between April 2015 and June 2020 were reviewed for evaluation of demographic features, accompanying neurological and ophthalmologic manifestations, symptom duration, etiological cause, and imaging findings. Patients with congenital and acquired blepharoptosis with chronic etiologies were excluded. Results: Sixteen pediatric patients (10 boys and 6 girls) with acquired acute-onset unilateral transient blepharoptosis were included in this study. The patients' mean age was 6.93 ± 3.16 years. The most commonly identified etiological cause was trauma in 7 patients (43.75%) and infection (para-infection) in 5 patients (31.25%). In addition, Miller Fisher syndrome, Horner syndrome secondary to neuroblastoma, acquired Brown's syndrome, and pseudotumor cerebri were identified as etiological causes in one patient each. Additional ocular findings accompanied blepharoptosis in 7 patients (58.33%). Blepharoptosis spontaneously resolved, without treatment, in all the patients, except those with Miller Fisher syndrome, neuroblastoma, and pseudotumor cerebri. None of the patients required surgical treatment and had ocular morbidities such as amblyopia. Conclusion: This study demonstrated that acute-onset unilateral transient blepharoptosis, which is rare in childhood, may regress without the need for surgical treatment in the pediatric population. However, serious pathologies that require treatment may present with blepharoptosis.

RESUMO Objetivo: Avaliar as características clínicas de pacientes pediátricos com blefaroptose adquirida unilateral, transitória e de início agudo. Métodos: Neste estudo retrospectivo, foram revisados prontuários clínicos entre abril de 2015 e junho de 2020. Os pacientes foram avaliados em termos de características demográficas, manifestações neurológicas e oftalmológicas associadas, duração dos sintomas, etiologia e achados de imagem. Foram excluídos pacientes com blefaroptose congênita e com blefaroptose adquirida de etiologia crônica. Resultados: Foram incluídos neste estudo 16 pacientes pediátricos (10 masculinos e 6 femininos) com blefaroptose adquirida transitória unilateral de início agudo. A média de idade dos pacientes foi de 6,93 ± 3,16 anos. As causas etiológicas mais comumente identificadas foram trauma em 7 pacientes (43,75%) e infecção (casos parainfecciosos) em 5 pacientes (31,25%). Além disso, a síndrome de Miller-Fisher, a síndrome de Horner secundária a neuroblastoma, a síndrome de Brown adquirida e pseudotumor cerebral foram determinados como causas etiológicas em um paciente cada uma. Achados oculares adicionais estavam associados à blefaroptose em 7 pacientes (58,33%). Foi observada a resolução espontânea da blefaroptose, sem tratamento, em todos os pacientes, exceto nos pacientes com síndrome de Miller-Fisher, neuroblastoma e pseudotumor cerebral. Nenhum paciente precisou de tratamento cirúrgico. Morbidades oculares, como ambliopia, não foram encontradas em nenhum paciente. Conclusão: Este estudo demonstrou que a blefaroptose transitória unilateral de início agudo, rara na infância, pode regredir sem a necessidade de tratamento cirúrgico na população pediátrica. No entanto, também não deve ser esquecido que patologias graves que requerem tratamento podem se apresentar com blefaroptose.

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INTRODUCTION: Miller-Fisher Syndrome (MFS) is a variant of Guillain-Barré syndrome (GBS), an acute immune-mediated neuropathy, which manifests as a rapidly evolving areflex motor paralysis. This syndrome presents as a classic triad: ophthalmoplegia, areflexia, and ataxia. MFS is usually benign and self-limited. CASE REPORT: A Caucasian patient was admitted to our hospital with the flu, loss of bilateral strength in the lower limbs and upper limbs and sudden-onset ataxia 7 days after receiving a first dose of the Oxford/AstraZeneca COVID-19 vaccine. On neurological examination, the patient had Glasgow Coma Scale score of 15, with absence of meningeal signs; negative Babinski sign; grade 2 strength in the lower limbs and grade 4 strength in the upper limbs; axial and appendicular cerebellar ataxia; and peripheral facial diparesis predominantly on the right, without conjugate gaze deviation. Cerebrospinal fluid (CSF) was collected on admission, and analysis revealed albuminocytological dissociation with CSF protein of 148.9 mg/dL; leukocytes, 1; chlorine, 122; glucose, 65 mg/mL; red cells, 2; and non-reactive venereal disease research laboratory test result. The COVID-19 IgG/IgM rapid immunological test was negative. Electroneuromyography revealed a recent moderate-grade and primarily sensory and motor demyelinating polyneuropathy with associated proximal motor block. DISCUSSION AND CONCLUSION: Miller-Fisher Syndrome may be related to events other than infections prior to neuropathy, as in the case reported here. The patient presented strong correlations with findings for MFS reported in the literature, such as the clinical condition, the results of electroneuromyography, and results of the CSF analysis typical for MFS. When treatment was provided as proposed in the literature, the disease evolved with improvement. Ultimately, the diagnosis of incomplete MFS was made, including acute ataxic neuropathy (without ophthalmoplegia).

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Introduction: Guillain-Barré syndrome (GBS) in association with arboviruses, such as Zika, chikungunya, and dengue, has been previously documented; however, Miller-Fisher Syndrome (MFS) and other GBS subtypes are rarely reported. Methods: We identified a series of GBS and MFS cases that were followed during the Zika virus outbreak in Salvador, Brazil (2015-2016). Blood and CSF samples were collected for virus diagnosis. In addition, serological studies to verify previous arboviral infection and electromyography (EMG) were performed. Results: Of the 14 patients enrolled, 10 were diagnosed with GBS, including three GBS subtypes (two cases of bifacial weakness with paresthesia and one case of paraparetic GBS), and four as MFS. IgM antibodies against one or more of three arboviruses were present in 11 (78.6%) patients: anti-zika IgM positivity in eight (57%), anti-Chikungunya IgM in three (21%), and anti-Dengue in one (7%) individual. A single case was positive for both anti-Dengue IgM and anti-Chikungunya IgM, suggesting co-infection. EMG revealed an AIDP pattern in all nine patients analyzed. Conclusion: The current case series contributes to our knowledge on the clinical presentation of arbovirus-associated GBS and its subtypes, including MFS, and serves as an alert to clinicians and other healthcare professionals in regions affected by arbovirus outbreaks. We highlight the importance of recognizing arboviruses in diagnosing GBS and its subtypes.

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Miller-Fisher syndrome is a rare, acute, autoimmune, demyelinating disorder which is considered a variant of Guillain-Barré syndrome. The pathologic mechanism is unclear, but acute demyelinating polyneuropathies may be triggered by bacterial or viral infections, major surgical interventions, or vaccination. Pregnancy may be a trigger of the immune response causing the onset of the syndrome. Miller-Fisher syndrome is characterized by acute onset, with predominant involvement of the facial and cranial nerves resulting in ophthalmoparesis, ataxia, and areflexia/hyporeflexia. Diagnosis is based on clinical suspicion together with the determination of specific ganglioside antibodies and other laboratory and imaging tests. Treatment consists of intravenous immunoglobulin and plasmapheresis, together with supportive measures. There are few reports of the syndrome occurring in pregnant women. A case of Miller-Fisher syndrome during pregnancy is presented.

INTRODUCTION Miller-Fisher syndrome (MFS) is a rare disorder that is characterized by acute onset of ophthalmoparesis, ataxia and hyporeflexia / areflexia(1). It was recognized 60 years ago as a variant of Guillain-Barré syndrome (GBS). The annual incidence is 0.09 per 100,000 persons and affects more males than females with a 2:1 ratio(2). GBS usually follows Campylobacter jenuni, cytomegalovirus, Epstein-Barr and influenza virus infections or secondary to major surgery, pregnancy, or vaccination(3,4). MFS accounts for 5%-10% of GBS cases and may have a major autoimmune component due to the presence of anti-ganglioside antibodies. During the acute phase of the disease, these antibodies have a diagnostic sensitivity and specificity of 92% and 97%, respectively(5). MFS during pregnancy is rare and there are only reports of 5 cases in pregnant women. A case of Miller-Fisher syndrome during pregnancy is presented. CLINICAL CASE The patient was 16 years old, primigravida of 20 weeks, who was referred for presenting nausea and incoercible vomiting of five days of evolution, accompanied by double vision, generalized weakness, ataxia,

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RESUMO Relato de caso de um paciente do sexo masculino internado no pronto socorro de um hospital com síndrome de Miller Fisher (SMF). A SMF é caracterizada pela presença de uma tríade de sinais: oftalmoparesia, arreflexia e ataxia, podendo apresentar outros sinais menos frequentes como alterações de fala e de deglutição. A partir da avaliação fonoaudiológica foi possível identificar disfagia orofaríngea de grau grave, hipernasalidade vocal e outras alterações fonoarticulatorias. O paciente apresentou boa evolução a partir de uma terapêutica multiprofissional integrada, incluído atendimento fonoaudiológico.

SUMMARY The case report of a male patient admitted to the emergency hospital with the diagnosis of Miller Fisher syndrome (MF), is presented. MFS is characterized by the presence of a triad of signs: ophthalmoparesis, areflexia and ataxia, and may present other less frequent signs such as speech and swallowing disorders. On the basis of findings during the speech therapy evaluation it was possible to identify severe oropharyngeal dysphagia, vocal hypernasality and other speech disorders. The patient presented a good evolution following an integrated multi-professional treatment program, including speech-language therapy.

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coronavirus disease 2019 (COVID-19), caused by the new coronavirus SARS-CoV-2, has been associated with the development of neurological diseases such as Guillain-Barré syndrome (GBS) and its variants. In the present work, two cases of demyelinating syndromes associated with COVID-19 are reported. Clinical cases: 53-year-old male with GBS and and 29-year-old female with Miller-Fisher syndrome (MFS) variant, respectively. Both patients presented the classic neurological signs and symptoms of demyelinating polyneuropathy that characterizes the syndromes. From the paraclinical biochemical tests, the increase of proteins in cerebrospinal fluid was distinctive. The positivity of the RT-qPCR for SARS-CoV-2 suggested the association of GBS and MFS with COVID-19. Both patients were treated with intravenous immunoglobulin showing improvement. Electromyography performed weeks ahead still showed chronic demyelinating involvement. Conclusion: The cases of GBS and MFS, along with other similar cases reported around the world, provide further evidence for SARS-CoV-2 as a new possible etiology of these rare neurological diseases.

Introducción: la enfermedad por coronavirus del 2019 (COVID-19), causada por el nuevo coronavirus SARS-CoV-2, se ha asociado con el desarrollo de enfermedades neurológicas como el síndrome de Guillain-Barré (SGB) y sus variantes. En el presente trabajo se reportan dos casos de síndromes desmielizantes asociados con la COVID-19. Casos clínicos: hombre de 53 años con SGB y mujer de 29 años con la variante del síndrome de Miller-Fisher (SMF), respectivamente. Ambos presentaron los signos y síntomas neurológicos clásicos de polineuropatía desmielinizante que caracterizan a estos síndromes. De las pruebas bioquímicas paraclínicas, el aumento de proteínas en líquido cefalorraquídeo fue distintiva. La positividad de la RT-qPCR para el SARS-CoV-2 indicó la asociación de los SGB y SMF con la COVID-19. Ambos pacientes se trataron con inmunoglobulina intravenosa y mostraron mejoría. La electromiografía realizada en semanas posteriores aún mostraba afectación desmielinizante crónica. Conclusión: los casos de los SGB y SMF, junto con otros casos similares reportados en todo el mundo, proporcionan más evidencia para el SARS-CoV-2 como nueva posible etiología de estas raras enfermedades neurológicas.

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Miller Fisher syndrome (MFS) is a rare variant of Guillain-Barré syndrome that is characterised by ataxia, ophthalmoplegia, and areflexia. Its relation with other autoimmune diseases is scarcely found in the literature, and in those few cases, treatment has been especially difficult. We report a case of a 28-year-old woman who presented with ophthalmoplegia and ptosis, later developing facial palsy and hyporeflexia. She had positive GD1a, GT1a, GQ1b antibodies confirming MFS. She also had positive antinuclear and lupus anticoagulant antibodies confirming antiphospholipid syndrome. She had a mild clinical course. MFS can present with multiple autoimmunity; it is unclear if there is cross-reactivity due to myelin damage.

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Introducción: la enfermedad por coronavirus del 2019 (COVID-19), causada por el nuevo coronavirus SARSCoV-2, se ha asociado con el desarrollo de enfermedades neurológicas como el síndrome de Guillain-Barré (SGB) y sus variantes. En el presente trabajo se reportan dos casos de síndromes desmielizantes asociados con la COVID-19. Casos clínicos: hombre de 53 años con SGB y mujer de 29 años con la variante del síndrome de Miller-Fisher (SMF), respectivamente. Ambos presentaron los signos y síntomas neurológicos clásicos de polineuropatía desmielinizante que caracterizan a estos síndromes. De las pruebas bioquímicas paraclínicas, el aumento de proteínas en líquido cefalorraquídeo fue distintiva. La positividad de la RT-qPCR para el SARS-CoV-2 indicó la asociación de los SGB y SMF con la COVID-19. Ambos pacientes se trataron con inmunoglobulina intravenosa y mostraron mejoría. La electromiografía realizada en semanas posteriores aún mostrabaafectación desmielinizante crónica. Conclusión: los casos de los SGB y SMF, junto con otros casos similares reportados en todo el mundo, proporcionan más evidencia para el SARS-CoV-2 como nueva posible etiología de estas raras enfermedades neurológicas.

Background: coronavirus disease 2019 (COVID-19), caused by the new coronavirus SARS CoV-2, has been associated with the development of neurological diseases such as Guillain-Barré syndrome (GBS) and its variants. In the present work, two cases of demyelinating syndromes associated with COVID-19 are reported. Clinical cases: 53-year-old male with GBS and and 29-yearold female with Miller-Fisher syndrome (MFS) variant, respectively. Both patients presented the classic neurological signs and symptoms of demyelinating polyneuropathy that characterizes the syndromes. From the paraclinical biochemical tests, the increase of proteins in cerebrospinal fluid was distinctive. The positivity of the RT-qPCR for SARSCoV-2 suggested the association of GBS and MFS with COVID-19. Both patients were treated with intravenous immunoglobulin showing improvement. Electromyography performed weeks ahead still showed chronic demyelinating involvement. Conclusion: The cases of GBS and MFS, along with other similar cases reported around the world, provide further evidence for SARS-CoV-2 as a new possible etiology of these rare neurological diseases.

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El síndrome de Guillain-Barré (SGB), y sus derivados, entre ellos el síndrome de Miller Fisher (SMF); junto a otras patologías de origen neurológico como la Polineuropatía desmielinizante inflamatoria crónica (CIDP), las polineuropatías de causa metabólica, miastenia gravis, esclerosis lateral amiotrófica (ELA), síndrome de Lambert-Eaton, encefalopatía de Wernicke entre otras; presentan signos y síntomas neurológicos de presentación común. De este modo, la importancia del examen neurológico acabado; y los exámenes de apoyo diagnóstico como: laboratorio -destacando el líquido cefalorraquídeo (LCR)-, electromiografía, y toma de imágenes, son cruciales para esclarecer el diagnóstico. Así, es posible ofrecer un tratamiento de forma precoz, basado en la evidencia, y con el objetivo de disminuir la letalidad de la enfermedad. En el presente texto se plasma un subgrupo de patología de SGB, el SMF, el cual posee una incidencia significativamente baja, una clínica característica, y un pronóstico bastante ominoso sin un tratamiento adecuado. En el presente texto se plasma el reporte de un caso abordado en el Hospital San Pablo de Coquimbo, Chile.

Guillain-Barré syndrome (GBS) and its derivatives, including Miller Fisher syndrome (MFS), along others pathologies of neurological origin such as chronic inflammatory demyelinating polyneuropathy (CIDP), metabolic polyneuropathies, myasthenia gravis, amyotrophic lateral sclerosis (ALS), Lambert-Eaton syndrome, Wernicke's encephalopathy and well as others, have common neurological signs and symptoms. In this way, the importance of a thorough neurological examination, and supporting diagnostic tests such as: laboratory, -cerebrospinal fluid (CSF)-electromyography, and imaging, are crucial to clarify the diagnosis. Thus, it is possible to offer early, evidence-based treatment with an aim of reducing the disease's lethality. In the text below we present a subgroup of GBS pathology, MFS, which has a significantly low incidence, a characteristic clinical picture, and a rather ominous prognosis without adequate treatment. In the following text/paper is shown the report of a case approached in San Pablo Hospital, from Coquimbo, Chile.

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BACKGROUND AND PURPOSE: Miller Fisher syndrome (MFS) is a subtype of Guillain-Barré syndrome characterized by the triad of ophthalmoparesis, areflexia, and ataxia. Although cases of MFS have been associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, no studies have synthesized the clinical characteristics of patients with this condition. METHODS: In this rapid systematic review, we searched the PubMed database to identify studies on MFS associated with SARS-CoV-2 infection. RESULTS: This review identified 11 cases, of whom 3 were hospitalized with motor and/or sensory polyneuropathy as the first sign of SARS-CoV-2 infection. SARS-CoV-2 RNA was not detected in analyses of cerebrospinal fluid, suggesting a mechanism of immune-mediated injury rather than direct viral neurotropism. However, antiganglioside antibodies were found in only two of the nine patients tested. It is possible that target antigens other than gangliosides are involved in MFS associated with SARS-CoV-2 infection. CONCLUSIONS: The present patients exhibited clinical improvement after being treated with intravenous immunoglobulin. Although rare, patients with SARS-CoV-2 infection may present neurological symptoms suggestive of MFS. Early recognition of the MFS clinical triad is essential for the timely initiation of treatment.

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Objective: To report the case of pregnant woman with Guillain-Barré syndrome (GBS) presenting as the Miller Fisher variant, and to review the literature on the diagnosis, treatment and prognosis of this GBS variant during gestation. Materials and Methods: Pregnant woman presenting at 27 weeks of gestation with Miller Fisher syndrome (MFS), treated in a military referral hospital with a satisfactory course after 15 days, continuation of normal pregnancy and delivery of a healthy neonate at 38 weeks. A search of the literature was conducted in the Medline via PubMed, Lilacs, SciELO, ScienceDirect and Ovid databases using the terms "Pregnancy," "Miller Fisher syndrome," "Guillain-Barré syndrome". Cohorts, case series and case reports of pregnant women with MFS were included. Data on diagnostic methods, treatment and maternal and perinatal prognosis were extracted. The search was made on June 2020, with no restriction by date, but restriction by language (Spanish and English). Results: Overall, 423 titles were identified, three studies met the inclusion criteria, the three of them corresponding to case reports. All cases were found to be seropositive for anti-GQ1b ganglioside antibodies. No imaging abnormalities were found in any of the cases. Two patients received IV immunoglobulin and the third patient was kept under observation. No obstetric complications have be documented so far. Conclusion: There are few cases of MFS reported during pregnancy. Intravenous immunoglobulin is the most frequently used treatment option. Plasmapheresis was used in the case presented here. The impact of the Miller Fisher variant on the normal course of gestation and on long-term perinatal outcomes is unknown. Further studies that look into the diagnosis, treatment and prognosis of this condition are required.

Objetivo: reportar el caso de una paciente gestante con síndrome de Guillain-Barré (SGB) presentado en la variante denominada síndrome de Miller Fisher (SMF), y realizar una revisión en torno al diagnóstico, tratamiento y pronóstico de esta variedad de SGB durante la gestación. Materiales y métodos: se presenta el caso de una gestante de 27 semanas con síndrome de Miller Fisher, quien fue tratada con plasmaféresis en un hospital militar de referencia, con evolución satisfactoria a los 15 días y continuación normal del embarazo, parto a las 38 semanas con recién nacido sano. Se realizó una búsqueda bibliográfica en bases de datos electrónicas: Medline vía PubMed, Lilacs, SciELO, ScienceDirect, Ovid, con los términos "Embarazo", "Síndrome de Miller Fisher", "Síndrome de Guillain-Barré". Se incluyeron cohortes, series y reportes de casos de mujeres gestantes con síndrome de Miller Fisher; se extrajo información sobre los métodos diagnósticos, el tratamiento utilizado y el pronóstico materno y perinatal. La búsqueda se hizo en junio de 2020, sin restricción por fecha, pero sí por tipo de idioma (español e inglés). Resultados: se identificaron 423 títulos, tres estudios cumplieron los criterios de inclusión, los tres correspondieron a reportes de caso. Todos los casos mostraron seropositividad para antigangliósidos GQ1b positivos; en ningún caso hubo alteración imagenológica. Dos pacientes recibieron inmunoglobulina intravenosa y la tercera paciente se dejó en observación. Hasta el momento no se documentan complicaciones obstétricas. Conclusión: existen pocos casos reportados de SMF durante la gestación, el diagnóstico se basa en el examen clínico; el tratamiento con inmunoglobulina IV representa la alternativa utilizada con mayor frecuencia. En el caso presentado se utilizó la plasmaféresis. Se desconoce el impacto de la variedad del síndrome de Miller Fisher sobre el curso normal de la gestación y sobre los resultados perinatales a largo plazo. Se requieren más estudios que aborden el diagnóstico, el tratamiento y el pronóstico de esta entidad.

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Resumen Objetivo: Reportar el caso de una paciente gestante con síndrome de Guillain-Barré (SGB) presentado en la variante denominada síndrome de Miller Fisher (SMF), y realizar una revisión en torno al diagnóstico, tratamiento y pronóstico de esta variedad de SGB durante la gestación. Materiales y métodos: Se presenta el caso de una gestante de 27 semanas con síndrome de Miller Fisher, quien fue tratada con plasmaféresis en un hospital militar de referencia, con evolución satisfactoria a los 15 días y continuación normal del embarazo, parto a las 38 semanas con recién nacido sano. Se realizó una búsqueda bibliográfica en bases de datos electrónicas: Medline vía PubMed, Lilacs, SciELO, ScienceDirect, Ovid, con los términos "Embarazo", "Síndrome de Miller Fisher", "Síndrome de Guillain-Barré". Se incluyeron cohortes, series y reportes de casos de mujeres gestantes con síndrome de Miller Fisher; se extrajo información sobre los métodos diagnósticos, el tratamiento utilizado y el pronóstico materno y perinatal. La búsqueda se hizo en junio de 2020, sin restricción por fecha, pero sí por tipo de idioma (español e inglés). Resultados: Se identificaron 423 títulos, tres estudios cumplieron los criterios de inclusión, los tres correspondieron a reportes de caso. Todos los casos mostraron seropositividad para antigangliósidos GQ1b positivos; en ningún caso hubo alteración imagenológica. Dos pacientes recibieron inmunoglobulina intravenosa y la tercera paciente se dejó en observación. Hasta el momento no se documentan complicaciones obstétricas. Conclusión: Existen pocos casos reportados de SMF durante la gestación, el diagnóstico se basa en el examen clínico; el tratamiento con inmunoglobulina IV representa la alternativa utilizada con mayor frecuencia. En el caso presentado se utilizó la plasmaféresis. Se desconoce el impacto de la variedad del síndrome de Miller Fisher sobre el curso normal de la gestación y sobre los resultados perinatales a largo plazo. Se requieren más estudios que aborden el diagnóstico, el tratamiento y el pronóstico de esta entidad.

Abstract Objective: To report the case of pregnant woman with Guillain-Barré syndrome (GBS) presenting as the Miller Fisher variant, and to review the literature on the diagnosis, treatment and prognosis of this GBS variant during gestation. Materials and Methods: Pregnant woman presenting at 27 weeks of gestation with Miller Fisher syndrome (MFS), treated in a military referral hospital with a satisfactory course after 15 days, continuation of normal pregnancy and delivery of a healthy neonate at 38 weeks. A search of the literature was conducted in the Medline via PubMed, Lilacs, SciELO, ScienceDirect and Ovid databases using the terms "Pregnancy," "Miller Fisher syndrome," "Guillain-Barré syndrome". Cohorts, case series and case reports of pregnant women with MFS were included. Data on diagnostic methods, treatment and maternal and perinatal prognosis were extracted. The search was made on June 2020, with no restriction by date, but restriction by language (Spanish and English). Results: Overall, 423 titles were identified, three studies met the inclusion criteria, the three of them corresponding to case reports. All cases were found to be seropositive for anti-GQ1b ganglioside antibodies. No imaging abnormalities were found in any of the cases. Two patients received IV immunoglobulin and the third patient was kept under observation. No obstetric complications have be documented so far. Conclusion: There are few cases of MFS reported during pregnancy. Intravenous immunoglobulin is the most frequently used treatment option. Plasmapheresis was used in the case presented here. The impact of the Miller Fisher variant on the normal course of gestation and on long-term perinatal outcomes is unknown. Further studies that look into the diagnosis, treatment and prognosis of this condition are required.

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Classic and overlapping Miller-Fisher syndrome (MFS) have divergent clinical courses. Few studies have addressed the electrophysiological evaluation of MFS patients, most of them carried out in Asia. This work describes and compares their clinical and neurophysiological characteristics. From a Guillain-Barré syndrome (GBS) patient cohort, we made a selection of twenty MFS cases. We defined classic and overlapping MFS, as stated by Wakerley et al. (Nat Rev Neurol 10(9):537-544, 2014). We describe and compare clinical, biochemical, and electrodiagnostic parameters between groups. Seventy-five percent were men, mean age was 42.2 ± 13.6 years, and 45% had a Hughes score ≥ 3. MFS/GBS was the most frequent clinical subtype with 50%. Almost one-third had unaltered electrophysiological studies. Comparative analysis between groups showed statistically significant differences in length of stay, dysautonomia presence, and treatment type. Kaplan-Meier survival analysis showed that 100% of the patients had an independent walk at 3 months. This study reports Mexican MFS patient's characteristics and represents the most extensive case series in Latin America. We observed a high proportion of overlapping syndromes, a good recovery profile, and no significant severe complications.

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BACKGROUND AND AIMS: During the last two decades important advances have been made regarding the knowledge of immunomediated neuropathies, which entitled them to evolve from a theoretical existence to subtype differentiation. Nevertheless, questions regarding antigen topography or antibody's pathogenicity still need to be answer. Moreover, antibody typification is not necessarily available during clinical practice and usually diagnostic decisions or follow-up are done without it. The aim of this study is to raise awareness of the importance of divergent clinical findings in patients with immunomediated neuropathies. METHODS: We present an acute and a chronic case of anti-ganglioside neuropathy immunologically studied with the best anti-ganglioside antibody panel available nowadays in our country along with an update of the current knowledge of these entities. RESULTS: Although there is no consensus regarding the pathogenicity of these antibodies, diverging clinical features (mainly ataxia and ophthalmoparesis) and disease prognosis (as markers of axonal damage) are known to be associated with different antibodies. CONCLUSIONS: Antibody characterization in anti-ganglioside neuropathies is an evolving field, which we believe should periodically updated and minded for patient's diagnosis and follow-up, even if laboratory confirmation is not immediate, if available at all.

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El síndrome anti-GQ1b reúne el síndrome de Miller-Fisher y la encefalitis del tronco cerebral de Bickerstaff, entre otras entidades. Tienen etiopatogenia común, constituida por la presencia de anticuerpos anti-GQ1b que reaccionan contra los sitios GQ1b del sistema nervioso según sea su accesibilidad. La prevalencia anual del síndrome de Miller-Fisher es de 0,09 casos por 100 000 habitantes por año y no existen estudios epidemiológicos sobre la encefalitis del tronco cerebral de Bickerstaff, que sería menos frecuente. De evolución natural hacia la mejoría, se beneficia del tratamiento con gammaglobulina endovenosa.Se presenta a un paciente de 12 años con síndrome de Miller-Fisher­Bickerstaff tras un episodio de diarrea aguda por Campylobacter jejuni en el que los anticuerpos anti-GQ1b resultaron positivos. Es nuestro objetivo comunicar sobre un síndrome de presentación poco habitual en pediatría a fin de advertir acerca de la necesidad de su sospecha precoz y solicitud de estudios de laboratorio específico

Miller-Fisher syndrome and Bickerstaff brainstem encephalitis, among others, constitute the anti-GQ1b syndrome, with a common immune pathophysiologic pathway characterized by the presence of anti-GQ1b antibodies, which react against the different nervous system GQ1b sites according to their different accessibility. The Miller-Fisher syndrome has a prevalence of 0.09 cases per 100 000 people-year but there are not epidemiological studies about Bickerstaff brainstem encephalitis, that it seems to be less frequent. In spite of having a good natural outcome, the immunoglobulin administration has been established as efficacious at improving it. A twelve-year-old boy suffering from Miller-Fisher-Bickerstaff syndrome after an acute Campylobacter jejuni diarrhea with positive titers of anti-GQ1b and anti-QGT1a antibodies is presented. We communicate a very uncommon pediatric disease with the aim of warning about the importance of its early suspicion and the need of specific laboratory determination

Assuntos

RESUMO

Miller-Fisher syndrome and Bickerstaff brainstem encephalitis, among others, constitute the anti-GQ1b syndrome, with a common immune pathophysiologic pathway characterized by the presence of anti-GQ1b antibodies, which react against the different nervous system GQ1b sites according to their different accessibility. The Miller-Fisher syndrome has a prevalence of 0.09 cases per 100 000 people-year but there are not epidemiological studies about Bickerstaff brainstem encephalitis, that it seems to be less frequent. In spite of having a good natural outcome, the immunoglobulin administration has been established as efficacious at improving it. A twelveyear- old boy suffering from Miller-Fisher-Bickerstaff syndrome after an acute Campylobacter jejuni diarrhea with positive titers of anti-GQ1b and anti-QGT1a antibodies is presented. We communicate a very uncommon pediatric disease with the aim of warning about the importance of its early suspicion and the need of specific laboratory determinations.

El síndrome anti-GQ1b reúne el síndrome de Miller-Fisher y la encefalitis del tronco cerebral de Bickerstaff, entre otras entidades. Tienen etiopatogenia común, constituida por la presencia de anticuerpos anti-GQ1b que reaccionan contra los sitios GQ1b del sistema nervioso según sea su accesibilidad. La prevalencia anual del síndrome de Miller-Fisher es de 0,09 casos por 100 000 habitantes por año y no existen estudios epidemiológicos sobre la encefalitis del tronco cerebral de Bickerstaff, que sería menos frecuente. De evolución natural hacia la mejoría, se beneficia del tratamiento con gammaglobulina endovenosa. Se presenta a un paciente de 12 años con síndrome de Miller- Fisher­Bickerstaff tras un episodio de diarrea aguda por Campylobacter jejuni en el que los anticuerpos anti-GQ1b resultaron positivos. Es nuestro objetivo comunicar sobre un síndrome de presentación poco habitual en pediatría a fin de advertir acerca de la necesidad de su sospecha precoz y solicitud de estudios de laboratorio específicos.

Assuntos

RESUMO

A síndrome de Miller Fisher é uma desmielinização dos nervos cranianos e periféricos, gerando graves consequências para o paciente, como, por exemplo, redução ou ausência dos reflexos, paralisia do III, IV e VI nervos cranianos e ataxia. Este relato descreveu o caso de uma mulher de 51 anos, natural e procedente de Penápolis (SP), admitida em um hospital de Araçatuba (SP) com quadro de arreflexia, ataxia e oftalmoplegia. No contexto clínico, foi suspeitada a hipótese de síndrome de Miller Fisher e, assim, começou o processo de investigação, com base nos critérios diagnósticos. O caso foi diagnosticado como síndrome de Miller Fisher, e o tratamento teve início.

Miller Fisher Syndrome is a demyelinating disease affecting cranial and peripheral nerves, leading to severe problems to the patient, such as reduced or absent reflexes, III, IV and VI cranial nerves palsy, and ataxia. This report describes the case of a 51-year-old woman from the city of Penápolis, in the state of São Paulo, who was admitted to the hospital in the city of Araçatuba, in the same state, with ataxia, areflexia and ophthalmoplegia. In the clinical context, the suspicion of Miller Fisher Syndrome was raised, and then investigation ensued for the disease, based on the diagnostic criteria. After evaluation, Miller Fisher Syndrome was confirmed and treatment was started.

Assuntos

RESUMO

RESUMEN Se describe el caso en pediatría de la sobreposición del síndrome de Miller Fisher y la encefalitis de Bickerstaff en presencia de perfil de anticuerpos positivos para anti-GQ1b en un niño de 6 años, quien presenta un compromiso tronco-encefálico y luego entra en una encefalopatía con compromiso de nervio periférico. El presente caso es relevante en relación con los escases de artículos semejantes en la literatura pediátrica, con pocos precedentes en la literatura publicada hasta la fecha.

SUMMARY To describe the pediatric case of the overlap of Miller Fisher syndrome and Bickerstaff encephalitis in the presence of an anti-GQ1b positive antibody profile in a 6-year-old boy who presents with a brainstem compromise and progress to encephalopathy with peripheral nerve compromise, the present case is relevant in relation to the scarcity of similar articles in pediatric literature with few precedents in the literature published to date.

Assuntos