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Treatment for Fisher syndrome, Bickerstaff's brainstem encephalitis and related disorders.
Cochrane Database Syst Rev. 2007 Jan 24;1:CD004761.
Overell J, Hsieh S, Odaka M, Yuki N, Willison H.
BACKGROUND:
Fisher syndrome is one of the regional variants of Guillain-Barre
syndrome, characterised by impairment of eye movements
(ophthalmoplegia), incoordination (ataxia) and loss of tendon reflexes
(areflexia). It can occur in more limited forms, and may overlap with
Guillain-Barre syndrome.
A further variant is associated with upper
motor neuron signs and disturbance of consciousness (Bickerstaff's
brainstem encephalitis). All of these variants are associated with
anti-GQ1b IgG antibodies. Intravenous immunoglobulin (IVIg) and plasma
exchange are often used as treatments in this patient group. This
review was undertaken to systematically assess any available randomised
controlled data on acute immunomodulatory therapies in Fisher Syndrome
or its variants. OBJECTIVES: To provide the best available evidence
from randomised controlled trials on the role of acute immunomodulatory
therapy in the treatment of Fisher Syndrome and related disorders.
SEARCH STRATEGY: We searched the Cochrane Neuromuscular Disease Trials
register (March 2004), MEDLINE (from January 1966 to November 2004),
EMBASE (from January 1980 to November 2004), CINAHL (from January 1982
to November 2004) and LILACS (from January 1982 to November 2004) for
randomised controlled trials, quasi-randomised trials, historically
controlled studies and trials with concurrent controls. We adapted this
strategy to search MEDLINE from 1966 and EMBASE from 1980 for
comparative cohort studies, case-control studies and case series.
SELECTION CRITERIA: All randomised and quasi-randomised controlled
clinical trials (in which allocation was not random but was intended to
be unbiased, e.g. alternate allocation, and non-randomised controlled
studies were to have been selected. Since no such clinical trials were
discovered, all retrospective case series containing five or more
patients were assessed and summarised in the discussion section. DATA
COLLECTION AND ANALYSIS: All studies of Fisher Syndrome and its
clinical variants were scrutinised for data on patients treated with
any form of acute immunotherapy. Information on the outcome was then
collated and summarised. MAIN RESULTS: We found no randomised or
non-randomised prospective controlled trials of immunotherapy in Fisher
Syndrome or related disorders. We summarised the results of
retrospective series containing five or more patients in the discussion
section. AUTHORS' CONCLUSIONS: There are no randomised controlled
trials of immunomodulatory therapy in Fisher Syndrome or related
disorders on which to base practic
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