RESUMEN
Fisher syndrome is recognized as a variant of Guillain-Barré syndrome, encompassing acute onset immune-mediated neuropathies marked by the classical triad of ataxia, areflexia, and ophthalmoplegia. Generally, Fisher syndrome follows a self-limited course with a good prognosis. Ophthalmoplegia, typically bilateral, progresses to complete external ophthalmoplegia within 1-2 weeks. Ataxia, often very severe, may cause an inability to walk without support despite normal strength. Fisher syndrome is also frequently concomitant with additional clinical features, including ptosis, internal ophthalmoplegia, facial nerve palsy, sensory deficits, and bulbar palsy. The confirmation of an antecedent infection is often established. Among the ganglioside antibodies, anti-GQ1b antibodies exhibit positivity in over 80% of patients. The syndrome manifests in three distinct types: a partial subtype exhibiting only a subset of the triad symptoms, Bickerstaff's brainstem encephalitis marked by impaired consciousness and pyramidal tract signs, and an overlapping subtype with Guillain-Barré syndrome, characterized by weakness in the extremities.
Asunto(s)
Síndrome de Miller Fisher , Humanos , Síndrome de Miller Fisher/diagnóstico , Síndrome de Miller Fisher/inmunología , Síndrome de Miller Fisher/terapia , Síndrome de Miller Fisher/fisiopatología , Gangliósidos/inmunología , Pronóstico , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/terapiaRESUMEN
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.
Asunto(s)
Humanos , Masculino , Anciano , Síndrome de Miller Fisher/diagnóstico , Diagnóstico Diferencial , Electromiografía , Polineuropatías , Síndrome de Miller Fisher/terapiaAsunto(s)
Humanos , Masculino , Femenino , Adolescente , Anciano , Cuadriplejía/cirugía , Plasmaféresis/métodos , Síndrome de Miller Fisher/terapia , Inmunización PasivaRESUMEN
O presente estudo relata um caso de síndrome de Miller-Fischer com tetraparesia severa e comprometimento de pares cranianos, que foi tratada com imunoglobulina G humana intravenosa (IgGIV) na dose de 0,35g/kg/dia, apresentando melhora do quadro neurológico, tendo piorado após uma semana. Foi realizada nova sessäo de IgGIV com melhora definitiva. Foi realizada uma revisäo bibliográfica pertinente à patologia. Concluindo-se que o uso da imunoglobulina intravenosa apresenta resultados similares ao da plasmaférese e que é importante uma observaçäo clínica rigorosa nas primeiras semanas após o tratamento com IgGIV