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.

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.

Síndrome anti-GQ1b: Descripción de cuatro pacientes y revisión de la literatura / Anti-GQ1b syndrome: Report of four cases

Anti-GQ1b syndrome includes Miller Fisher Syndrome (MFS), Guillain Barré Syndrome (GBS), Bickerstaff`s brain stem encephalitis (BBE) and Acute Ophtamoplegia (AO). We report four patients aged 16 to 76 years, with anti-GQ1b syndrome. All presented with MFS, one of them evolved to GBS pharyngeal-cervical-brachial variant and other to GBS with BBE. All had a previous history of diarrhea or upper respiratory tract infection. All had positive anti-GQ1b serum antibodies. Both brain magnetic resonance imaging and cerebrospinal fluid analysis were normal. Electrophysiology studies were compatible with a demyelinating disease. Two patients needed airway protection with an orotracheal tube and developed dysautonomia. All four patients were treated with immunomodulation. On the sixth month follow-up, patients had only minimal alterations in the neurological examination.

An Adult Case of Fisher Syndrome Subsequent to Mycoplasma pneumoniae Infection

Reported herein is an adult case of Fisher syndrome (FS) that occurred as a complication during the course of community-acquired pneumonia caused by Mycoplasma pneumoniae. A 38-yr-old man who had been treated with antibiotics for serologically proven M. pneumoniae pneumonia presented with a sudden onset of diplopia, ataxic gait, and areflexia. A thorough evaluation including brain imaging, cerebrospinal fluid examination, a nerve conduction study, and detection of serum anti-ganglioside GQ1b antibody titers led to the diagnosis of FS. Antibiotic treatment of the underlying M. pneumoniae pneumonia was maintained without additional immunomodulatory agents. A complete and spontaneous resolution of neurologic abnormalities was observed within 1 month, accompanied by resolution of lung lesions.

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.

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.

Miller Fisher syndrome--an uncommon clinical presentation.

Miller Fisher syndrome is an uncommon disease and it is a variant of Guillain-Barre syndrome. Miller Fisher syndrome also has rarer variants. Combined features of classic Guillain-Barre syndrome and Miller Fisher syndrome are uncommon. Here we are reporting a case of Miller Fisher variant with Guillain-Barre syndrome overlap in which ataxia, are flexia, oculomotor disturbance and limb weakness occurred within few days.

A case of Miller Fisher syndrome with anti GQ1b in Thailand.

Miller-Fisher syndrome (MFS) is a rare variant of Guillain-Barré syndrome (GBS) and is characterized by the clinical triad of ataxia, ophthalmoplegia, and areflexia. The incidence rate in Thailand has not been established but it occurred approximately 1-5% that of GBS. Here, the authors report a Thai patient diagnosed as MFS that had a positive test of antibodies against the ganglioside GQ1b. These antibodies have diagnostic and pathogenic importance to MFS because of high sensitivity and specificity. All other investigations, such as cerebrospinal fluid analysis, electrophysiological studies, and imaging studies had no significant abnormalities. The patient was successfully treated with intravenous immunoglobulin and fully recovered within one month. After eighteen months follow-up, he is still healthy and has had no recurrent symptoms.

Recurrent Miller Fisher syndrome.

Miller Fisher syndrome (MFS) is a variant of Guillan Barre syndrome characterized by the triad of ophthalmoplegia, ataxia and areflexia. Recurrences are exceptional with Miller Fisher syndrome. We are reporting a case with two episodes of MFS within two years. Initially he presented with partial ophthalmoplegia, ataxia. Second episode was characterized by full-blown presentation characterized by ataxia, areflexia and ophthalmoplegia. CSF analysis was typical during both episodes. Nerve conduction velocity study was fairly within normal limits. MRI of brain was within normal limits. He responded to symptomatic measures initially, then to steroids in the second episode. We are reporting the case due to its rarity.

Recurrent Miller fisher syndrome: a case report.

Miller fisher syndrome (MFS) is a variant of Guillain-Barre syndrome characterized by the triad of ophthalmoplegia, ataxia and areflexia. Recurrences are exceptional with MFS. A case with two episodes of MFS within four years is reported. He presented with findings of ophthalmoplegia, ataxia, areflexia, and oropharyngeal weakness and mild distal sensory impairment during both episodes. Electrophysiological findings showed reduced compound muscle action potentials and sensory nerve action potentials with no evidence of conduction blocks. Nerve biopsy showed segmental demyelination. MRI of brain was normal. He responded well to immunoglobulins during both episodes suggesting that immunomodulating drugs have a role in the treatment of MFS.

Síndrome de Miller Fisher e neurite óptica: relato de caso / Miller Fisher syndrome and optic neuritis: case report

Descreve-se um caso de síndrome de Miller Fisher associada a neuropatia óptica desmielinizante bilateral, confirmada pelo exame de potencial evocado visual, sugerindo possível comprometimento do sistema nervoso central nessa síndrome

Guillian Barré syndrome--recent advances.

Guillian Barré Syndrome (GBS) is an acquired disease of the peripheral nerves that is characterized clinically by rapidly progressing paralysis, areflexia, and albumino-cytological dissociation. It affects both genders, involves people of all ages, is reported worldwide, and in the post-polio era, it is the most common cause of an acute generalized paralysis. The clinical features are distinct and a history and an examination generally lead to a high suspicion of the diagnosis that can then be confirmed by supportive laboratory tests and electrodiagnostic studies. This review discusses the recent advances in understanding of the different variants of GBS such as acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN), and the Fisher syndrome. The clinical, electrodiagnostic criteria, immunopathogenesis, and management of GBS and its variants are discussed.

Síndrome de Guillain-Barré: análisis de 36 pacientes / Guillain-Barre syndrome: analysis of 36 patients

Describe las características clínicas y epidemiológicas, métodos, diagnósticos y la terapéutica empleada en los pacientes con síndrome de Guillain-Barré atendidos en el Hospital San Juan de Dios durante el período comprendido entre el 1 de enero de 1994 y el 31 de diciembre de 1998. Se revisaron en forma retrospectiva un total de 36 expedientes de pacientes que egresaron con el diagnóstico de síndrome de Guillain-Barré del Hospital San Juan de Dios. Todos los pacientes fueron valorados por médicos del servicio de neurología y cumplieron con los criterios diagnósticos de Asbury y Cornblath. El 64 por ciento de los pacientes fueron hombres. El grupo etario más frecuente fue el de 30 a 49 años de edad, sin embargo la distribución en todos los grupos de edad fue similar. La incidencia fue mayor durante los meses de mayo a diciembre, época de mayor precipitación lluviosa en el país. Una patología previa se identificó en 25 casos (70 por ciento). La infección previa de las vías respiratorias superiores ocurrió en 13 pacientes (36 por ciento) y el antecedente de enfermedad diarreica fue observado en 6 pacientes (17 por ciento). Se realizó punción lumbar a 94,5 por ciento de los pacientes; el 50 por ciento presentó disociación albúmino citológica en el líquido cefalorraquídeo inicial. Los estudios electrofisiológicos fueron realizados en el 72 por ciento de los casos y todos fueron compatibles con una neuropatía periférica aguda desmielinizante. Todos los pacientes presentaron manifestaciones clínicas motoras, el 97 por ciento presentó afección de los miembros inferiores, el 81 por ciento fue de predominio distal y en el 78 por ciento la progresión fue ascendente. El 36 por ciento presentó compromiso sensitivo, 31 por ciento autonómico y el 31 por ciento afectación de pares craneales. El síndrome de Miller Fisher se presentó en el 5.5 por ciento de los casos. En el 42 por ciento de los pacientes se utilizó la gammaglobulina como terapia específica. El 25 por ciento de los pacientes requirieron ventilación mecánica. La mortalidad fue del 8 por ciento. Las características clínicas de los pacientes con síndrome de Guillain Barré encontradas en nuestro estudio son similares a lo reportado por varios autores. Es de resaltar de hecho que la mayoría de los casos se presentaron durante los meses de mayo a diciembre época en que se observa en Costa Rica con gran frecuencia infecciones de vías respiratorias superiores...