Evaluation of pediatric patients presenting with acute-onset unilateral transient acquired blepharoptosis / Avaliação de pacientes pediátricos apresentando blefaroptose transitória unilateral de início agudo

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.

Síndrome de Miller-Fisher durante el embarazo

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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,

Disfagia e Disfonia em um caso de Síndrome de Miller Fisher: Avaliação fonoaudiológica / Dysphagia and Dysphonia in a case of Miller Fisher Syndrome: Speech therapy evaluation

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.

Casos clínicos de los síndromes de Guillain-Barré y Miller-Fisher relacionados con la COVID-19 / Clinical cases of Guillain-Barré and Miller-Fisher syndromes linked to COVID-19

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.

Síndrome de Miller Fisher tratado con plasmaféresis durante el embarazo: reporte de un caso y revisión de la literatura / Miller Fisher syndrome treated with plasmapheresis during pregnancy: Case report and review of the literature

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.

Síndrome anti-GQ1b en un niño con síndrome de Miller-Fisher-Bickerstaff / Anti-GQ1b syndrome. A child with Miller-Fisher-Bickerstaff syndrome

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

Síndrome de Miller Fisher: relato de caso / Miller Fisher syndrome: case report

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.

Sobreposición del síndrome de Miller Fisher y encefalitis de Bickerstaff en pediatría: reporte de caso / Overlap of Miller Fisher syndrome and Bickerstaff encephalitis in pediatrics: Case report

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.

Síndrome de Miller Fisher en un niño de 12 años / Miller Fisher Syndrome in a 12 year old boy

El Síndrome de Miller Fisher (SMF) es una variante del Síndrome de Guillain Barré (SGB), caracterizado por la tríada clínica de oftalmoplejía, ataxia y areflexia. Se presenta el caso de un niño de 12 años de edad, examinado con un tiempo de enfermedad de 4 días y con una variedad de síntomas que incluían ptosis palpebral, somnolencia, marcha tambaleante y debilidad muscular, asociados a antecedente de infección respiratoria de vías altas. El examen clínico demostró paresia del III, IV, y VI nervios craneales de ambos ojos, arreflexia y debilidad distal en extremidades. Se instaló tratamiento con Inmunoglobulina intravenosa que condujo a una evolución clínica satisfactoria.

The Miller Fisher Syndrome (MFS) is a variant of the Guillain Barre Syndrome (GBS), characterized by the clinical trial of ophthalmoplegia, ataxia and areflexia. The case of a 12 year old boy is examined with a 4-day long history characterized by symptoms such as palpebral ptosis, somnolence, ataxia and muscle weakness, associated with a history of upper respiratory infection. Clinical examination showed paresis of III, IV, and VI cranial nerves of both eyes, areflexia, and distal weakness in the extremities. Treatment with intravenous immunoglobulin was established, leading to a satisfactory clinical evolution.

Síndrome de Bickerstaff / Bickerstaff Syndrome

La encefalitis de Bickerstaff es una entidad infrecuente de origen post infeccioso de presentación grave, que pertenece al espectro de las patologías asociadas a los antigangliósidos, donde el paciente se presenta con compromiso subagudo de oftalmoplejia, ataxia, debilidad con signos piramidales y alteración del estado de conciencia con antecedentes recientes de infecciones respiratorias, gastrointestinales o ambas. Se reporta un caso de encefalitis de Bickerstaff presentado en el Hospital Nacional de Niños.

Bickerstaff encephalitis is a rare and severe inflammatory disease with a serious presentation, which belongs to the spectrum of diseases associated with antiganglioside. Patients present a subacute ophthalmoplegia, ataxia, weakness with pyramidal signs and altered mental status with a recent history of respiratory infections, gastrointestinal infections or both. We report a case of Bickerstaff encephalitis that was diagnosed and treated at the Hospital Nacional de Niños (National Children's Hospital).

Síndrome de Miller Fisher: a propósito de un caso clínico / Miller Fisher syndrome: apropos of clinical case

INTRODUCCIÓN: El síndrome de Miller Fisher es la variante más frecuente del síndrome de Guillain Barré, llegando a una prevalencia del 5 por ciento de entre todas sus variantes. Fue descrito por Charles Miller Fisher en 1956, quien lo expuso como una variante inusual de una polineuritis idiopática aguda. Se caracteriza por la presentación aguda de una polineuropatía, asociada a la tríada de oftalmoplejía, ataxia y arreflexia; pudiendo progresar a un compromiso respiratorio con riesgo vital. PRESENTACIÓN DEL CASO: En el siguiente artículo, se expone el caso clínico de un paciente pesquisado en una revisión retrospectiva de los egresos del servicio de neurología del Hospital Clínico Herminda Martin, el que fue diagnosticado como síndrome de MillerFisher. Se trata del caso de un paciente varón de 77 años, con antecedentes de cardiopatía no precisada e hipercolesterolemia, que se presenta en policlínico de neurología con disartria, ataxia, paraparesia y oftalmoplejía. DISCUSIÓN: Se realizará un análisis contrastado entre la presentación, evolución, tratamiento y datos pesquisados durante su estadía, en relación al manejo y características encontradas en la bibliografía consultada.

INTRODUCTION: Miller Fisher syndrome is the most common variant of Guillain Barré syndrome, reaching a prevalence of 5 percent among all its variants. It was described by Charles Miller Fisherin 1956, who exposed him as an unusual variant of acute inflammatory demyelinating polyneuropathy. It is characterized by acute onset of polyneuropathy, associated with the triad of ophthalmoplegia, ataxia and areflexia, and may progress to life-threatening respiratory compromise. CASE REPORT: The following article presents a case of a patient researched in a retrospective review of the hospital discharges of the department of neurology of Hospital Clínico Herminda Martin, who was diagnosed as Miller Fisher syndrome. This is the case of a 77years old male patient with a history of unspecified coronary heart disease, and hypercholesterolemia, who was presented in the emergency unit with dysarthria, ataxia, paraparesis and ophthalmoplegia. DISCUSSION: We will make an analysis contrast between the presentation, treatment and data collected made during his stay, in relation to the handling and features found in the literature.

Síndrome de Miller Fisher: considerações diagnósticas e diagnósticos diferenciais / Miller Fisher syndrome: diagnosis considerations and differentials diagnosis

JUSTIFICATIVA E OBJETIVOS: A síndrome de Miller Fisher apresenta a tríade oftalmoplegia, ataxia e arreflexia e, em muitas situações pode ser confundida com a doença de Guillian-Barré ou com a encefalite de Bickerstaff, em que ocorre um acometimento dos níveis de consciência. O objetivo deste estudo foi alertar para o possível diagnóstico de Miller Fisher, uma variante da síndrome de Guillain-Barré, abordando algumas recentes descobertas envolvidas com sua fisiopatologia como também, considerando alguns dos seus principais diagnósticos diferenciais. CONTEÚDO: A presença de anticorpos IgG anti-GQ1b pode ser um mecanismo importante na fisiopatologia da síndrome, porém ainda há controvérsias, desde que a simples presença desses anticorpos não garante o aparecimento da síndrome e alguns nervos em que se encontram depósitos desses anticorpos não apresentam alterações. Infecções respiratórias ou do trato gastrintestinal pregressas podem ser encontradas em 70% dos casos de Miller Fisher o que sugere um processo imunológico com reação cruzada aos agentes etiológicos dessas condições clínicas. CONCLUSÃO: A identificação precoce do quadro e o tratamento com gamaglobulina e/ou plasmaférese pode modificar em muito a evolução do quadro e permitir um prognóstico mais favorável, mesmo que ainda não se saiba o real processo fisiopatológico envolvido nessa doença.

BACKGROUND AND OBJECTIVES: Miller Fisher syndrome comes with ophtalmoplegia, ataxia, and arreflexia and, in many situations, has a similar presentation as Guillain-Barré syndrome or Bickerstaff disease. In this last condition, loss of conscious nessmay be present. In this review we were warning for a possible Miller Fisher syndrome, a Guillain-Barre syndrome variant,showing some news pathophysiology aspects and some differentials diagnosis. CONTENTS: IgG anti GQb1 antibodies can play an important role in the pathophysiology but controversies exist, since the presence of these antibodies does not guarantee the presence of the disease. Some peripheral nerves where antibodies are deposited do not present alterations. Respiratory or gastrointestinal tract infections may precede Miller Fisher in 70% of the cases,suggesting that an immunologic cross reaction can trigger thesyndrome. CONCLUSION: The precocious identification of this clinical picture as well an early treatment with gammaglobulin and or plasmapheresis can deeply modify the progression of the disease and allow a much better prognosis.

Encefalitis de Bickerstaff, síndrome oespectro de Fisher Bickerstaff, reporte de dos casos / Bickerstaff encephalitis/Fisher Bickerstaff syndrome, report of two cases

La encefalitis de Bickerstaff es una entidad infrecuente de origen posinfeccioso, de presentación grave y con un curso clínico generalmente benigno, cuya diferenciacióncon el síndrome de Fisher aún no es clara y su asociación con el síndrome de Guillain - Barré está ya establecida. Presentamos dos casos atendidos en el Hospital Universitario San Ignacio...

Bickerstaff encephalitis is a rare post-infectious origin disease, with an acute severe presentation form, but generally with a benign course, its differentiation from Fisher syndrome is unclear, and its association with Guillain - Barre syndrome is alreadyestablished. We report two cases treated at the Hospital Universitario San Ignacio...

Síndrome de Miller Fisher. A propósito de un caso / Miller Fisher syndrome. A case report

El síndrome de Miller Fisher es una variedad clínica del síndrome de Guillain-Barré, que se caracteriza por oftalmoplejía, arreflexia y ataxia. Se presenta el caso de una paciente de 6 años de edad, atendida en el Hospital Pediátrico Docente Pepe Portilla de Pinar del Río, en noviembre de 2010; con antecedentes de salud anterior, que 5 días previos al inicio del cuadro tuvo una infección respiratoria alta aguda, seguida de un cuadro de debilidad bucofaríngea y facial, ataxia y debilidad muscular intensa de las extremidades. Se interconsulta con el servicio de Neurología, donde se constata diplejía facial periférica, parálisis del IV par izquierdo, ataxia e hiporreflexia; se le realizan varias investigaciones e ingresa en la Unidad de Cuidados Intensivos con el diagnóstico de síndrome de Miller Fisher. Se comienza el tratamiento con terapéuticocon, intacglobin y vitaminoterapia, logrando una evolución satisfactoria de la paciente a los 10 días de su ingreso en el servicio de UCI con regresión del cuadro.

Miller Fisher syndrome is a clinical variant of Guillain-Barré syndrome it is characterized by ophthalmoplegia, areflexia and ataxia. A six-year old female patient attended to "Pepe Portilla" Children Hospital, Pinar del Rio in November 2010; with past health records, who 5 days before the onset of the disorder suffered from an acute upper respiratory infection that was followed by a picture of buccopharyngeal and facial weakness, ataxia and acute muscular debility of the limbs. A referral to Neurology service verified peripheral facial diplegia, paralysis of the IV left pair, ataxia and hyporeflexia; several examinations were performed before admitting her to the Intensive Care Unit (ICU) with the diagnosis of Miller Fisher syndrome. The therapeutic treatment included intacglobin and vitamins, achieving a satisfactory progress; the patient was discharged from the ICU 10 days after her admission with a total regression of the clinical picture.

Síndrome de Miller Fisher associada à infecção por escherichia coli / Miller Fisher syndrome linked to infeccion by escherichia coli

A síndrome de Miller Fisher (SMF), uma variante da Síndrome de Guillain-Barré, é uma doença incomum na prática médica. Esta doença é caracterizada por inflamação e desmielinização dos nervos periféricos de provável causa infecciosa. Estão descritos casos associados a infecções respiratórias e digestivas. O presente relato descreve o caso de uma paciente de 54 anos que apresentou SMF após sete dias de infecção urinária por Escherichia coli.

Miller Fisher syndrome (MFS), a variant of Guillain-Barré syndrome, is an uncommon disease in medical practice. It is characterized by inflammation and demyelination of peripheral nerves of probable infectious etiology. Cases are associated with respiratory and digestive infections. This report describes the case of a 54-year-old female patient who presented with MFS after seven days of urinary tract infection with Escherichia coli.

Síndrome de Guillain Barré en pediatría / Guillain-Barré syndrome in pediatrics

Este trabajo revisa el conocimiento actual sobre el síndrome de Guillain-Barré (SGB) en niños. El SGB se define como una parálisis flácida arrefléxica aguda y se clasifica en 4 subgrupos: polirradiculopatía aguda inflamatoria desmielinizante (AIDP), neuropatía axonal sensitivo-motora aguda (AMSAN), neuropatía axonal motora aguda (AMAN) y síndrome de Miller-Fisher (SMF). La AIDP se asocia en un 30-50% a compromiso de pares craneales, lo cual no se observa en la AMAN. El SMF se caracteriza por ataxia, oftalmoplejía y arreflexia, pero puede presentar también compromiso de pares craneales. Datos recientes de la anatomía patológica y la fisiopatología del SGB destacan la importancia de la infección por Campylobacter jejuni en la generación de anticuerpos anti-gangliósidos (GM1 en AIDP, GQ1b en SMF y GD1a en AMAN) que lesionan la mielina en AIDP y SMF y el axón en AMAN. El diagnóstico diferencial debe descartar enfermedades del sistema nervioso central (SNC) (encefalitis, encefalomielitis, mielitis), síndromes miasténicos, neuropatías tóxicas por metales pesados, fármacos, substancias químicas o toxinas animales y cuadros miopáticos, especialmente la miositis aguda infecciosa benigna y la neuromiopatía del paciente en la unidad de cuidados intensivos. Es importante el tratamiento con inmunoglobulina en dosis total de 2 gramos por kilogramo a administrar en 48 horas. La plasmaféresis puede ser igualmente eficaz. El SGB tiene buen pronóstico en niños, con una recuperación total en el 85% de los casos. La rehabilitación es fundamental para lograr una recuperación más rápida e integral.

This paper reviews the current knowledge about Guillain- Barré syndrome (GBS). GBS is defined as an acute, areflexic, flaccid paralysis, which is classified into 4 subgroups: acute inflammatory demyelinating polyneuropathy (AIDP), acute motor-sensory axonal neuropathy (AMSAN), acute motor axonal neuropathy (AMAN) and Miller-Fisher syndrome (MFS). AIDP is associated in 30-50% of cases with cranial nerve involvement, which is not observed in AMAN. MFS is characterized by ataxia, ophthalmoplegia and areflexia, but it may also present cranial nerve dysfunction. Recent data on the pathology and pathophysiology of GBS emphasize the important role of Campylobacter jejuni infection in generating anti-ganglioside antibodies (GM1 in AIDP, GQ1b in MFS and GD1a in AMAN), which damage myelin in AIDP and MFS and axons in AMAN. The differential diagnosis must rule out other disorders of the central nervous system (encephalitis, encephalomyelitis, myelitis), myasthenic syndromes, toxic neuropathies induced by heavy meals, drugs, chemical substances or animal toxins, and myopathic conditions, especially acute benign infectious myositis and neuromyopathy of the intensive care unit patient. It is important the treatment with immune globulin, at a total dose of 2 grams per kilogram administered over 48 hours. Plasmapheresis can be equally effective. GBS has a good prognosis in children with a total recovery in 85% of cases. Rehabilitation is crucial to attain a more rapid and global improvement.