Treatment for Fisher syndrome, Bickerstaff's brain stem encephalitis and related disorders

Fisher syndrome is an uncommon paralysing illness, usually caused by autoimmune inflammation of nerves following an infection. Fisher syndrome is characterised by impairment of eye movements, abnormal co-ordination and loss of tendon reflexes. It is a variant of Guillain-Barré syndrome. Unlike Guillain-Barré syndrome, it does not cause limb or respiratory muscle weakness. In Western countries Fisher syndrome represents between 5% and 10% of Guillain-Barré syndrome cases, but it is more common in Eastern Asia, for example 25% of cases in Japan. Bickerstaff's brain stem encephalitis shares many clinical features but also includes altered consciousness and signs of central nervous system inflammation. Treatment strategies tried have been immunotherapies such as plasma exchange or intravenous immunoglobulin which are used in Guillain-Barré syndrome. This systematic review found no randomised controlled trials of treatments for Fisher syndrome, Bickerstaff's brain stem encephalitis or their variants. Observational studies suggest that Fisher syndrome always and Bickerstaff's brain stem encephalitis usually recovers completely. Randomised controlled trials are needed to establish the value of immunotherapies or other treatments.

Authors' conclusions: 

There are no randomised controlled trials of immunomodulatory therapy in Fisher Syndrome or related disorders on which to base practice.

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Background: 

Fisher syndrome is one of the regional variants of Guillain-Barré 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-Barré 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 Specialized Register (February 2009), MEDLINE (January 1966 to February 2009), EMBASE (January 1980 to February 2009), CINAHL (January 1982 to February 2009) and LILACS (January 1982 to February 2009) for randomised controlled trials and quasi-randomised trials.

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.

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