155 A case series; a Miller Fisher Syndrome epidemic. Issue 3 (9th February 2012)

• Record Type: Journal Article
• Authors: Banik, S; Johnston, J A; Hinds, N P; Walters, J; Hill, M
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 83:Issue 3(2012)
• Page Start: e1
• Page End: e1
• Publication Date: 2012-02-09
• Subjects: Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp-2011-301993.197
• Languages: English
• ISSNs: 0022-3050

Abstract

Abstract : Miller Fisher Syndrome (MFS) classically consists of a triad of ataxia, areflexia and ophthalmoplegia. Facial nerve involvement has also been reported in up to 45.7% of patients and antiganglioside antibodies (GQ1b Ab) are frequently positive. MFS annual incidence has been estimated to be 0.09 per 100 000. In 2010 we managed four suspected cases of MFS in 1 month. Here we present this case series and outline the clinical features, Ab titres and radiological findings of each case. All cases presented with bilateral facial weakness; three were within the MFS clinical spectrum and demonstrated the classical clinical triad with positive GQ1b Ab. Of these three, two were treated with intravenous immunoglobulins (IVIg). The third case made a spontaneous recovery. However the fourth case challenged the original diagnosis given its initial poor response to treatment and the evolving clinical and radiological findings. This case despite treatment with IVIg failed to improve and at 6 weeks follow-up had developed bilateral sensorineural deafness. A repeat MRI scan demonstrated diffuse left-sided lesion in the pontine region extending into cerebellum. The admission of three patients in a month to our neurology department with MFS, challenges the incidence figures. In all the three patients we report bilateral facial weakness in addition to the recognised clinical triad. The fourth case serves to illustrate, that atypical cases, those which evolve or whom fail to respond to IVIg should be re-evaluated.