Miller Fisher Syndrome - A Case Report

Introduction Miller Fisher Syndrome (MFS) is a rare variant of GBS (Guillain-Barre Syndrome). It involves both adults and children and its mostly a clinical diagnosis. It comprises a classic triad of ophthalmoplegia, ataxia and areflexia which all develop over a period of few days. This triad was first described by James Collier in 1932 and it was subsequently reported as a variant of GBS by Charles Miller Fisher in three clinical cases in 1956(1). MFS is a geographically variable variant of GBS observed in about 1% to 5% of all GBS cases in Western countries, yet up to 19% and 25% in Taiwan and Japan respectively(2). There is a male predominance at a ratio of 2:1 and a mean age of 43.6 years, although cases of MFS have been reported in all age groups(2). Unlike the classic ascending weakness/paralysis that is characteristic of GBS, neurological deficits follow a top down pattern in MFS, starting with diplopia in the eyes; caused by external ophthalmoplegia-the most common presenting symptoms(2,4). In a clinical series of 50 consecutive cases of MFS in Japan it was discovered that 78% of cases presented initially with diplopia, 46% with ataxia, and 34% with both. Other abnormalities reported, albeit less frequently, were limb dysesthesia; blepharoptosis; face, bulbar, and pupillary palsies; mild (grade 4) motor weakness; and micturition disturbance(2). As in GBS an antecedent infectious illness can be identified. Upper respiratory infection is the most commonly described prodromal entity followed by gastrointestinal illness(3). Campylobacter jejuni and Haemophilus influenza have been the most commonly implicated pathogens. Other pathogens, including Mycoplasma pneumonia and Cytomegalovirus, have been associated too. An acute onset is typical of MFS, beginning with neurologic symptoms approximately 8-10 days (range of 1-30) following the antecedent illness(2,5). The disease then progresses until a clinical nadir is reached approximately 6 days (range of 2-21) after the initial neurologic symptoms(2). The recovery period is marked by gradual improvement and often resolution of symptoms; although rarely, serious complications such as respiratory failure or cardiac arrhythmia (that are common in GBS, with 30% of cases requiring ventilator support) have been reported(5). The following case presents key clinical features of MFS. Familiarity with this rare syndrome will clue the clinician to consider MFS in patients presenting with areflexia, ataxia, and ophthalmic symptom.