Miller-Fisher Syndrome
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Type of Guillain-Barre syndrome, featuring ataxia, areflexia, and ophthalmoplegia. Specific autoantibodies associated.
Miller-Fisher syndrome Miller-Fisher Syndrome (MFS) was first described in 1956 by a Canadian Stroke Neurologist, Charles Miller-Fisher [1] (who is also recognised as providing the first description of transient ischaemic attacks). The disease is a variant of Guillain-Barre syndrome, and is currently the subject of much research, as it is a good model for immune mediated injury in the nervous system.
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Aetiology
Patients often have a preceding insult to their immune system, and this could be as diverse as vaccination, respiratory tract infection, herpes or mycoplasma infection. It has been noted in particular that many patients will have a preceding infection with Campylobacter jejuni [2], and this offers interesting insights into disease pathogenesis . It is thought that a number of antigenic epitopes exist on the surface of Campylobacter that are structurally similar to certain complex gangliosides. Through a process of molecular mimicry, antibodies raised to Campylobacter also bind to gangliosides found in the body, and produce injury.
Clinical
The classic clinical triad of: ataxia, areflexia, and ophthalmoplegia are present. Bulbar palsy may often be a feature, necessitating referral to ITU. Other variants of GBS ( Ataxic Guillain-Barre Syndrome and Acute Ophthalmoparesis [3] [4]) share many of the features of Miller-Fisher Syndrome, and these conditions are thought to represent a spectrum of MFS
Investigations
CT scan will be normal. LP may show an acellular fluid with elevated protein. EMG may not be diagnostic, but could show evidence of demyelination or conduction block. Anti-ganglioside antibodies, particularly to GQ1b will be positive in 90% of cases [5].
Treatment
Intravenous immunoglobulin therapy and plasmapharesis are used.[6] Main restrictions at the moment are cost and accessibility.
A recent Cochrane review did not show any evidence that steroids alone produce an improvement in symptoms, and indeed their use was associated with a slight (statistically insignificant) increase in steroid related complications including diabetes and hypertension.[7] However, one study did suggest that the time to independent walking is improved if intravenous steroid is given in conjunction with immunoglobulin, and it can therefore be used as an adjunct treatment in cases where the patient’s recovery is prolonged.[8]
References
- ↑ Fisher, M An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia). 1956 Jul 12;255(2):57-65.
- ↑ Endtz,H.P., Ang,C.W., Van den,B.N., Duim,B., Rigter,A., Price,L.J., Woodward,D.L., Rodgers,F.G., Johnson,W.M., Wagenaar,J.A. et al. 2000. Molecular characterization of Campylobacter jejuni from patients with Guillain-Barre and Miller Fisher syndromes. J.Clin.Microbiol. 38:2297-2301
- ↑ Willison HJ, Yuki N. Peripheral neuropathies and anti-glycolipid antibodies 2002 Dec;125(Pt 12):2591-625.
- ↑ Odaka,M., Yuki,N., and Hirata,K. Anti-GQ1b IgG antibody syndrome: clinical and immunological range. 2001 Jan;70(1):50-5
- ↑ Chiba,A., Kusunoki,S., Obata,H., Machinami,R., and Kanazawa,I. Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barre syndrome: clinical and immunohistochemical studies Peripheral neuropathies and anti-glycolipid antibodies 1993 Oct;43(10):1911-7
- ↑ Shahar,E.Current therapeutic options in severe Guillain-Barre syndrome. 2006 Jan-Feb;29(1):45-51.
- ↑ Hughes RA, Swan AV, van Koningsveld R, van Doorn PA. Corticosteroids for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2006 Apr 19;(2):CD001446. Review.
- ↑ The Dutch Guillain-Barre Study Group. Treatment of Guillain-Barre syndrome with high-dose immune globulins combined with methylprednisolone: a pilot study. Ann Neurol. 1994 Jun;35(6):749-52. Erratum in: Ann Neurol 1994 Sep;36(3):457.
