C Scoppetta, M Fontana, R Quadrini, I La Cesa, R Di Lello, A Peppe, V S Tolli, C Casali
{"title":"[Miller Fisher syndrome: review of the literature and presentation of 2 cases].","authors":"C Scoppetta, M Fontana, R Quadrini, I La Cesa, R Di Lello, A Peppe, V S Tolli, C Casali","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>Miller Fisher Syndrome (MFS), which is characterized by ophthalmoplegia, ataxia and tendon areflexia, is generally considered as a clinical variant of Guillain-Barré Syndrome. However some features of the disease are still debated, particularly regarding possible central nervous system involvement. After presenting two new cases of MFS, the authors provide a critical review of the literature and discuss the nosographical position of the disease. The main conclusions can be summarized as follows: MFS is a predominantly axonal inflammatory neuropathy with prevailing involvement of oculomotor nerves. It is associated to spinal multi or polyneuropathy, which in mildly affected cases is manifested by areflexia, while in severe ones it can be responsible of sense and/or motor impairment. In addition to peripheral neuropathy CNS involvement, exclusive or more marked in posterior fossa, occurs not infrequently. The prognosis of the disease is often benign, but disabling or even fatal outcome is possible. Corticosteroid treatment, possibly because of antiinflammatory and/or immunosuppressive action, could be effective in some patients. Finally, in spite of some similarities with GBS, MFS should be considered as a separate entity with its own nosographical position.</p>","PeriodicalId":76494,"journal":{"name":"Rivista di neurologia","volume":"61 4","pages":"137-44"},"PeriodicalIF":0.0000,"publicationDate":"1991-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Rivista di neurologia","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
Miller Fisher Syndrome (MFS), which is characterized by ophthalmoplegia, ataxia and tendon areflexia, is generally considered as a clinical variant of Guillain-Barré Syndrome. However some features of the disease are still debated, particularly regarding possible central nervous system involvement. After presenting two new cases of MFS, the authors provide a critical review of the literature and discuss the nosographical position of the disease. The main conclusions can be summarized as follows: MFS is a predominantly axonal inflammatory neuropathy with prevailing involvement of oculomotor nerves. It is associated to spinal multi or polyneuropathy, which in mildly affected cases is manifested by areflexia, while in severe ones it can be responsible of sense and/or motor impairment. In addition to peripheral neuropathy CNS involvement, exclusive or more marked in posterior fossa, occurs not infrequently. The prognosis of the disease is often benign, but disabling or even fatal outcome is possible. Corticosteroid treatment, possibly because of antiinflammatory and/or immunosuppressive action, could be effective in some patients. Finally, in spite of some similarities with GBS, MFS should be considered as a separate entity with its own nosographical position.
Miller Fisher综合征(MFS)通常被认为是格林-巴-罗综合征的临床变体,以眼麻痹、共济失调和肌腱反射为特征。然而,该疾病的一些特征仍有争议,特别是关于中枢神经系统的可能累及。在提出两个新的MFS病例后,作者提供了一个重要的文献回顾,并讨论了该疾病的医院位置。主要结论可以总结如下:MFS是一种以轴突炎性神经病为主,主要累及动眼神经。它与脊髓多神经或多神经病变有关,在轻度影响的病例中表现为反射性松弛,而在严重的病例中,它可导致感觉和/或运动障碍。除了周围神经病变外,中枢神经系统受累,排他或更明显地发生在后窝,并不罕见。这种疾病的预后通常是良性的,但致残甚至致命的结果是可能的。皮质类固醇治疗,可能是因为抗炎和/或免疫抑制作用,可能对某些患者有效。最后,尽管与GBS有一些相似之处,但MFS应被视为一个独立的实体,具有自己的医院位置。