High dose immunoglobulin therapy is effective in the Miller Fisher syndrome

Zifko, Udo; Drlicek, M.; Senautka, G.; Grisold, Wolfgang

doi:10.1007/bf00868348

Cited by 28 publications

(12 citation statements)

References 10 publications

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“…In these reports, patients who received IVIG experienced clinical recovery in a slightly shorter time frame than would be expected based on the natural history of the illness. [23][24][25][26][27][28][29] On the basis of the single retrospective analysis and the case reports listed above, it is difficult to clearly define the role of IVIG in treating Fisher syndrome. The literature suggests that best medical management may suffice for many patients.…”

Section: Fisher Syndromementioning

confidence: 98%

Consensus statement: The use of intravenous immunoglobulin in the treatment of neuromuscular conditions report of the aanem AD HOC committee

et al. 2009

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Intravenous immunoglobulin (IVIG) is a therapeutic biologic agent that has been prescribed for over two decades to treat various neuromuscular conditions. Most of the treatments are given off-label, as little evidence from large randomized trials exists to support its use. Recently, IGIV-C has received an indication for the treatment of chronic inflammatory demyelinating polyneuropathy (CIDP). Because of the lack of evidence, an ad hoc committee of the AANEM was convened to draft a consensus statement on the rational use of IVIG for neuromuscular disorders. Recommendations were categorized as Class I-IV based on the strength of the medical literature. Class I evidence exists to support the prescription of IVIG to treat patients with Guillain-Barré syndrome (GBS), CIDP, multifocal motor neuropathy, refractory exacerbations of myasthenia gravis, Lambert-Eaton syndrome, dermatomyositis, and stiff person syndrome. Treatment of Fisher syndrome, polymyositis, and certain presumed autoimmune neuromuscular disorders is supported only by Class IV studies, whereas there is no convincing data to substantiate the treatment of inclusion body myopathy (IBM), idiopathic neuropathies, brachial plexopathy, or diabetic amyotrophy using IVIG. Treatment with IVIG must be administered in the context of its known adverse effects. There is little evidence to advise the clinician on the proper dosing of IVIG and duration of therapy.

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Section: Fisher Syndromementioning

confidence: 98%

Consensus statement: The use of intravenous immunoglobulin in the treatment of neuromuscular conditions report of the aanem AD HOC committee

et al. 2009

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“…The use of plasmapheresis85, 96, 171 with success has mostly been described in case reports, as was the use of intravenous immunoglobulin 181. However, the latter should be used judiciously; a case of cerebral infarction was reported during treatment of an MFS patient,161 and posterior reversible encephalopathy has also occurred 108…”

Section: Treatmentmentioning

confidence: 99%

Clinical and immunological spectrum of the Miller Fisher syndrome

2007

Muscle and Nerve

157

132

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The Miller Fisher syndrome (MFS), characterized by ataxia, areflexia, and ophthalmoplegia, was first recognized as a distinct clinical entity in 1956. MFS is mostly an acute, self-limiting condition, but there is anecdotal evidence of benefit with immunotherapy. Pathological data remain scarce. MFS can be associated with infectious, autoimmune, and neoplastic disorders. Radiological findings have suggested both central and peripheral involvement. The anti-GQ1b IgG antibody titer is most commonly elevated in MFS, but may also be increased in Guillain-Barré syndrome (GBS) and Bickerstaff's brainstem encephalitis (BBE). Molecular mimicry, particularly in relation to antecedent Campylobacter jejuni and Hemophilus influenzae infections, is likely the predominant pathogenic mechanism, but the roles of other biological factors remain to be established. Recent studies have demonstrated the presence of neuromuscular transmission defects in association with anti-GQ1b IgG antibody, both in vitro and in vivo. Collective findings from clinical, radiological, immunological, and electrophysiological techniques have helped to define MFS, GBS, and BBE as major disorders within the proposed spectrum of anti-GQ1b IgG antibody syndrome.

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“…IVIG is effective in immune-mediated disorders such as MFS and GBS12). IVIG is also effective in treatment of BBE with hypothesized immune-mediated etiology, which is similar to the clinical and immunologic features of MFS or GBS, which suggests an overlapping spectrum of MFS and GBS.…”

Section: Discussionmentioning

confidence: 84%

Overlapping Guillain-Barré syndrome and Bickerstaff's brainstem encephalitis associated with Epstein Barr virus

Rho

2014

Korean J Pediatr

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A flaccid tetraparesis in Bickerstaff's brainstem encephalitis (BBE) is presumed to be a sign of overlapping Guillain-Barré syndrome (GBS). In addition, BBE and Fisher syndrome, which are clinically similar and are both associated with the presence of the immunoglobulin G anti-GQ1b antibody, represent a specific autoimmune disease with a wide spectrum of symptoms that include ophthalmoplegia and ataxia. A 2-year-old boy presented with rapidly progressive ophthalmoplegia, ataxia, hyporeflexia, weakness of the lower extremities, and, subsequently, disturbance of consciousness. He experienced bronchitis with watery diarrhea and had laboratory evidence of recent infection with Epstein-Barr virus (EBV). He was diagnosed as having overlapping GBS and BBE associated with EBV and received treatment with a combination of immunoglobulin and methylprednisolone, as well as acyclovir, and had recovered completely after 3 months. In addition, he has not experienced any relapse over the past year. We suggest that combinations of symptoms and signs of central lesions (disturbance of consciousness) and peripheral lesions (ophthalmoplegia, facial weakness, limb weakness, and areflexia) are supportive of a diagnosis of overlapping GBS and BBE and can be helpful in achieving an early diagnosis, as well as for the administration of appropriate treatments.

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High dose immunoglobulin therapy is effective in the Miller Fisher syndrome

Cited by 28 publications

References 10 publications

Consensus statement: The use of intravenous immunoglobulin in the treatment of neuromuscular conditions report of the aanem AD HOC committee

Consensus statement: The use of intravenous immunoglobulin in the treatment of neuromuscular conditions report of the aanem AD HOC committee

Clinical and immunological spectrum of the Miller Fisher syndrome

Overlapping Guillain-Barré syndrome and Bickerstaff's brainstem encephalitis associated with Epstein Barr virus

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