A case of recurrent Miller-Fisher syndrome with multiple anti-ganglioside antibodies / 中国综合临床

Guillain-Barré syndrome (GBS) defines a kind of Immune-mediated acute inflammatory peripheral neuropathy. Miller-Fisher Syndrome (MFS) is a special variant of GBS, with mostly one-way course and rare clinical recurrence. Only a few recurrent cases have been reported in China. Here we report a case of a young male patient with double vision and progressive aggravation of limb numbness, acute onset, with symptoms of upper respiratory tract infection before onset, accompanied by pupil abnormalities and autonomic nervous dysfunction, who was was admitted to our hospital for similar symptoms 3 years ago and was improved by immunotherapy. The patient had a triad of “ataxia, areflexia and ophthalmoplegia”. Cerebrospinal fluid showed protein-cell separation. Serum anti-Sulfatides antibody IgM, anti-GT1a antibody IgG, anti-GQ1b antibody IgG and anti-GM3 IgM were positive. Recurrent MFS was diagnosed and the symptoms improved after immunotherapy. This case suggests that MFS is clinically heterogeneous, a few patients can present with relapse and generally have a better prognosis with immunotherapy. Pre-existing infection and anti-GQ1b antibody production may be predisposing factors for MFS recurrence.

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.

Síndrome de Miller Fisher, un desafío diagnóstico de oftalmoplejía: reporte de un caso / Miller Fisher syndrome, a diagnostic challenge of ophthalmoplegia: a case report

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.

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.

Clinical application of the new classification criteria in children with Miller Fisher syndrome and clinical characteristics analysis of different types of Miller Fisher syndrome / 中华实用儿科临床杂志

Objective:To investigate the clinical application of the new classification criteria in children with Miller Fisher syndrome (MFS), and to analyze clinical characteristics of different types of MFS.Methods:Clinical data of MFS patients hospitalized in the Department of Neurology, Beijing Children′s Hospital, Capital Medical University from January 2015 to December 2019 were collected and analyzed retrospectively, including demographic characteristics, clinical symptoms, neurological examination findings, laboratory examination data, diagnosis and treatment, and prognosis.The counting data was described by percentage, and the measurement data was described by median.Results:A total of 23 patients were included in the research, including 14 males and 9 females, with a median age of 4 years and 8 months.There were 3 cases of pure MFS; 5 cases of incomplete MFS, including 1 case of acute ptosis and 4 cases of acute ataxia neuropathy; 15 cases of overlapping syndrome, including 13 cases of MFS/Guillain Barre syndrome (GBS), 1 case of MFS/pharyngocervical brachial variant GBS(PCB GBS)and 1 case of MFS/GBS/Bickertaff brainstem encephalitis (BBE). In addition to Ⅲ, Ⅳ and Ⅵ cranial nerve palsy, 11 cases had the involvement of other cra-nial nerves, including 2 cases in pure MFS, 8 cases in MFS/GBS and 1 case in MFS/GBS/BBE.Autonomic nervous dysfunction occurred in 6 cases.Respiratory muscle paralysis occurred in 6 cases, including 5 cases in MFS/GBS and 1 case in MFS/GBS/BBE.Graded by the Hughes scoring system (HG score), 3 cases with pure MFS were graded 4 points; 1 case with acute ptosis was graded 0; 3 cases with acute ataxia neuropathy were graded 2 points, and the other one was graded 3 points; 1 case with MFS/PCB GBS was graded 3 points; 10 cases with MFS/GBS were graded 4 points, 1 case was graded 3 points, and the other 2 cases were graded 2 points; 1 case with MFS/GBS/BBE was graded 4 points.Twenty-two patients were treated with intravenous immunoglobulin.The HG of all patients at discharge decreased at varying degree, which was graded 0 at 6 months of follow-up.Conclusions:The clinical application of the new diagnostic classification method is helpful to the accurate diagnosis of different types of MFS.More than half of MFS cases will develop into the overlapping syndrome.The overlapping of MFS and GBS or BBE is prone to the involvement of cranial nerves except for the external ophthalmic muscles, autonomic nerve dysfunction and respiratory muscle paralysis.The disease course of MFS varies, and its diagnosis should be comprehensively made.All cases of MFS in this study have a satisfactory prognosis.

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.

Clinical features of 19 children with Bickerstaff brainstem encephalitis / 中华儿科杂志

Objective@#To summarize the clinical features of Bickerstaff brainstem encephalitis (BBE) in children.@*Methods@#In this retrospective study, data of 19 patients with BBE (11 males and 8 females) were collected from Department of Neurology, Beijing Children′s Hospital from October 2015 to January 2018. The clinical features, treatment and prognosis were analyzed.@*Results@#The onset age of BBE ranged from 1 year and 8 months to 12 years and 11 months. There were 18 cases with preceding infection. The most common infection was upper respiratory tract infection (9 cases), followed by simple fever (5 cases). The most common initial neurological symptoms were lethargy or disturbance of consciousness (8 cases), followed by limb weakness (5 cases). There were 6 cases of simple BBE and 13 cases of BBE overlapping Guillain-Barré syndrome (GBS). Besides the characteristic triad of altered mental status, ataxia, and ophthalmoplegia, there were other symptoms including convulsion (5 cases), diplopia (3 cases), nystagmus (7 cases), facial muscular weakness (7 cases),bulbar palsy (13 cases) and autonomic nerve symptoms (9 cases). Hypo or areflexia was seen in 16 cases. Positive Babinski′s signs were seen in 8 cases. Hyponatremia was present in 10 cases in whom 4 showed severe hyponatremia. Albumin-cytological dissociation of cerebrospinal fluid was seen in 10 cases. The autoimmune antibodies were examined in all 19 patients. Anti-ganglioside antibodies including anti-GM1 IgG antibody was positive in 2 patients and one of whom was also found with positive anti-GD1b IgG antibody. Anti-GQ1b IgG antibody was present in 2 patients. Electromyography was performed in 14 cases and 8 cases, who were all BBE overlapping GBS, showed neurological damage. A total of 16 cases were monitored by video electroencephalography and 8 cases showed slow waves of background. In addition to, interictal focal discharge was detected in 2 cases. T2 fluid-attenuated inversion recovery (FLAIR) sequence abnormal signals were detected in 3 of 18 cases performed brain magnetic resonance imaging (MRI), and lesions involved with brainstem, basal ganglia, thalamus, cerebellum, corpus callosum and cerebral cortex. Lesions involved cervical and thoracic spinal cord were found in 1 out of 11 cases for whom spinal cord MRI was performed. All of the 4 cases who underwent enhanced MRI of spinal had partial nerve roots enhancement. All of the 19 patients received 1 to 2 courses of intravenous immunoglobulin therapy, and 2 cases also received plasma exchange. Fifteen cases received steroid therapy. The following-up period ranged from 3 months to 2.5 years. Two cases were lost to follow-up. Twelve cases achieved a full recovery within 3 months. Three cases recovered within 6 months. One case still had slight limb weakness and ataxia after 1 year and 8 months of follow-up, and another case had left autonomic nerve symptoms in the follow-up of 2 years and 3 months. Both of them were BBE overlapping GBS.@*Conclusions@#Children′s BBE is similar to that in adults, and is frequently found overlapped with GBS. Furthermore, it is sometimes accompanied by central nervous system demyelination disease. The antiganglioside antibodies are not often detectable. Immunoglobulin therapy could usually achieve good response. The prognosis of simple BBE is good in most situations. For BBE overlapping GBS, the more severe the limb weakness during the peak of disease is, the slower the recovery would be.

Recent Concepts of Guillain-Barré Syndrome

Guillain-Barré syndrome (GBS) is a representative form of post-infectious autoimmune neuropathy with heterogenous manifestations. It was originally considered as an ascending demyelinating polyneuropathy in Western countries. However, the discovery of anti-ganglioside antibodies on the basis of molecular mimicry theory could help us better understand various kinds of focal and regional variants as well as axonal type of GBS those were frequently found from Asian countries. Recent development of new techniques about anti-ganglioside complex antibodies is making more detailed descriptions for specific or unusual clinical manifestations. It has been regarded that GBS has good prognosis if treated properly as early as possible, but it still shows high mortality and morbidity rate with frequent long term neurologic and medical complications. Unfortunately, there are only two options for medical treatment, intravenous immunoglobulin and plasmapheresis, for the last 100 years. Several clinical studies on new immunotherapy targeting complement activating system with background of molecular mimicry using animal model are underway. We hope that these new treatments will be helpful for the future patients.

Pattern of Extraocular Muscle Involvements in Miller Fisher Syndrome

BACKGROUND AND PURPOSE: The most-common initial manifestation of Miller Fisher syndrome (MFS) is diplopia due to acute ophthalmoplegia. However, few studies have focused on ocular motility findings in MFS. This study aimed to determine the pattern of extraocular muscle (EOM) paresis in MFS patients. METHODS: We consecutively recruited MFS patients who presented with ophthalmoplegia between 2010 and 2015. The involved EOMs and the strabismus pattern in the primary position were analyzed. Antecedent infections, other involved cranial nerves, and laboratory findings were also reviewed. We compared the characteristics of the patients according to the severity of ophthalmoplegia between complete ophthalmoplegia (CO) and incomplete ophthalmoplegia (IO). RESULTS: Twenty-five patients (15 males and 10 females) with bilateral ophthalmoplegia were included in the study. The most-involved and last-to-recover EOM was the lateral rectus muscle. CO and IO were observed in 11 and 14 patients, respectively. The patients were aged 59.0±18.4 years (mean±SD) in the CO group and 24.9±7.4 years in the IO group (p<0.01), and comprised 63.6% and 21.4% females, respectively (p=0.049). Elevated cerebrospinal fluid protein was identified in 60.0% of patients with CO and 7.7% of patients with IO (p=0.019) for a mean follow-up time from the initial symptom onset of 3.7 days. CONCLUSIONS: The lateral rectus muscle is the most-involved and last-to-recover EOM in ophthalmoplegia. The CO patients were much older and were more likely to be female and have an elevation of cerebrospinal fluid protein than the IO patients.

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.

Clinical characteristics of patients with recurrent Guillain-barré syndrome / 中国神经精神疾病杂志

Objective To investigate the clinical features of recurrent Guillain-barré syndrome. Methods The data on demographic information and clinical features of 286 inpatients with Guillain-barré syndrome were collected. Patients were divided into recurrent group and non-recurrent group based on the number of episodes. The data from those patients with recurrent Guillain-barré syndrome whose information was missing was collected by telephone. Results we identified 22 (7.7%) cases with recurrent Guillain-barré syndrome including one case of Miller fisher syndrome. The total episodes were 55. Sixteen patients had 2 episodes, 3 patients had 3 episodes, 1 patients had 4 episodes and 2 patients had 5 episodes. Compared with non-recurrent group, patients with recurrent Guillain-barré syndrome had younger onset [(36.36 ±14.51)y vs. (45.72 ±16.13)y, t=-2.633, P=0.009], the shorter interval between the initial manifestations to peak [(2.18±0.73)d vs.(4.24±2.98)d, t=-8.537, P=0.000], the lower GBS disability score (68.2% vs. 31.8% , χ2=4.209, P=0.040) and less involvement in the cranial nerve (13.6% vs. 37.5% , χ2=5.040, P=0.025). Conclusion The recurrent risk may be considered in patients with Guillain-barré syndrome when younger patients, have lower GBS disability score and the shorter interval between the initial manifestations to peak.

Miller-Fisher Syndrome Presenting with Bilateral Pseudo-Internuclear Ophthalmoplegia

Bilateral internuclear ophthalmoplegia (INO) refers to a specific gaze abnormality of bilateral adduction deficits, often accompanied by dissociated abducting nystagmus, caused by medial longitudinal fasciculus lesions usually due to multiple sclerosis or stroke. We report a 63-year-old man with clinical features of Miller-Fisher syndrome (MFS), consisting of ataxia, areflexia, and external ophthalmoplegia which mimicked bilateral-INO without an identifiable central lesion. Although bilateral adduction deficits are usually caused by central lesions, peripheral nervous damage by MFS is needed to be considered.

A Variant Guillain-Barré Syndrome with Anti-Ganglioside Complex Antibody

BACKGROUND: Recently, anti-ganglioside complex (GSC) antibodies were discovered among the various subtypes of Guillain-Barré syndrome. GSC is the novel glycoepitopes formed by two individual ganglioside molecules. CASE REPORT: We present a 36-year-old man with overlap Miller Fisher syndrome and acute bulbar palsy who had anti-GSC antibody that provided diagnostic robustness. CONCLUSION: Anti-GSC testing could be considered important in patients who show atypical manifestation with negative antibody reaction against each constituent ganglioside.

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.

The probability and timing of Miller-Fisher syndrome progressing to Guillain-Barre syndrome or Bickerstaff brainstem encephalitis in childhood / 临床儿科杂志

Objective To investigate the probability and timing of childhood Miller-Fisher syndrome (MFS) progressing to Bickerstaff brainstem encephalitis (BBE), classical Guillain-Barre syndrome (GBS), and pharyngeal-cervical-brachial (PCB-GBS). Methods The clinical data of 128 children with confirmed MFS diagnosis were retrospectively analyzed. Results Among 128 children, 60 cases were simple MFS (ocular muscle paralysis, ataxia, reflexes diminished or disappeared, without limbs weakness and lethargy; laboratory tests suggest cerebrospinal fluid protein-cell separation and/or serum anti-GQ1b antibody positive), 28 cases developed MFS/PCB-GBS (met MFS diagnosis criteria, accompanied by weakness of pharynx, neck and upper limb, weakened or disappeared of upper limb reflex, without weakness of lower limb), 22 cases developed MFS/GBS (met MFS diagnosis criteria, accompanied by weakness of limb), 18 cases developed MFS/BBE (met MFS diagnosis criteria, accompanied by lethargy, pyramidal tract positive). There were no differences in the age at onset, the interval from onset to the start of the treatment, Hughes functional grading, and the percentage of cases having a history of preceding infections, the rate of positive serum anti-GQ1b antibody, the ratio of albumin cytological dissociation in cerebrospinal fluid among 4 groups (P>0.05). The interval from MFS onset to progression to MFS/PCB-GBS, MFS/GBS, or MFS/BBE was within 10 days. Conclusions In children with MFS, 50% developed PCB-GBS, GBS, or BBE, which occurred within 10 days after onset. Clinicians should pay attention to the time window and adjust the medicine rationally.

Clinical analysis of four cases of anti GQ1b syndrome / 中华儿科杂志

Objective@#To investigate the clinical manifestations, laboratory findings, treatment and outcome of anti-GQ1b antibody syndrome.@*Method@#The clinical manifestations, laboratory examination, diagnosis, treatment and prognosis of (4 patients 4 male patients, from 4 to 12 years) with anti-GQ1b syndrome in Beijing Children's Hospital affiliated to Capital Medical University from 2015 to 2016 were retrospectively analyzed.@*Result@#All 4 children presented with ataxia. Case 1 showed impaired speech, ptosis and weakness of arms; case 2 and 3 had external ophthalmoplegia, weakness of limbs; case 4 presented hypersomnia, irritability and hallucinations. Serum anti-GQ1b-IgG antibody was positive in all cases. Case 1-3 received lumber puncture at the course of 1-2 weeks, CSF presented albuminocytological dissociation, case 4 had CSF pleocytosis and increased protein level. Brain MRI of Case 1-2 were normal; Case 3 showed long T1 and T2 signal in cerebellar dentate nucleus, pons and corpus callosum; Case 4 showed long T1 and T2 signal in bilateral centrum semiovale, basal ganglia, external capsule, insula and cerebellum. Electromyograms of case 1-3 showed peripheral axonal lesion. All children were treated with IVIG. After treatment, condition of all patients were improved. According to the clinical manifestation, laboratory examination, and outcome after treatment, case 1 was diagnosed as anti-GQ1b antibody syndrome (Pharyngeal-Cervical-Brachial weakness overlapped with Miller Fisher syndrome), case 2 and 3 were diagnosed as anti-GQ1b antibody syndrome (Miller Fisher syndrome overlapped with Guillain Barré syndrome) and case 4 was diagnosed as anti-GQ1b antibody syndrome (acute ataxia hypersomnolence).@*Conclusion@#When patients with the presence of prodromic infections, monophasic course, drowsiness, ataxia, ophthalmoplegia, weakness and the symptoms/signs are relatively symmetric, anti-GQ1b antibody syndrome should be considered. Anti-GQ1b antibody has important significance for diagnosis. Most children have a good prognosis. Early correct diagnosis can avoid unnecessary examinations and guide appropriate use of immunotherapy.