Miller-Fisher syndrome at King Chulalongkorn Memorial Hospital.

BACKGROUND: Miller-Fisher syndrome (MFS), a variant of Guillain-Barré syndrome (GBS) is a self-limiting demyelinating disease of the peripheral nervous system. Clinical spectrum, natural history, prognosis, and pathogenesis of MFS are not fully documented. OBJECTIVE: Probe the clinical features of MFS in a tertiary center in Thailand and compare its pattern with other Asian countries. MATERIAL AND METHOD: The clinical recordings were searched from databases at King Chulalongkorn Memorial Hospital (KCMH) between 2002 and 2007. Keywords were "Miller-Fisher syndrome" and "Guillain-Barré syndrome". Cases with MFS were recruited The data regarding clinical features, course, treatments, and investigations were reviewed. Comparisons with other large Asian series were demonstrated. RESULTS: Six patients (male:female; 3:3) with MFS were analyzed The incident rate is 7.7% that of GBS. The median age was 54.3 years (range 28 to 73 years). MFS frequently started with diplopia followed by ophthalmoplegia and ataxia. Other clinical symptoms included limb dysesthesia and weakness, dysphagia, dysarthria, and diffuse headache. Spontaneous recovery occurred in three patients while two patients received plasmapheresis and one received intravenous immunoglobulin (IVIG). Six months after neurological deficits, all patients were almost free of symptoms and had returned to their normal activities. CONCLUSION: The incidence, clinical features, and prognosis of MFS in KCMH were comparable with the previous studies in other Asian countries. High percentage of limb dysesthesia and optic neuropathy were detected in the present series. Headache was also common among Thai MFS. Immuno-pathogenesis of MFS is well documented but immunomodulatory therapy should be considered only in some cases.

Optic neuritis in a patient with Miller-Fisher syndrome.

Miller-Fisher syndrome (MFS) is considered a variant of Guillain-Barrd syndrome (GBS). The syndrome is characterized by acute onset of gait ataxia, ophthalmoplegia, and areflexia. Conventionally, MFS has been considered exclusively a disease of the peripheral nervous system. However there are occasional reports of central nervous system involvement. Here, a 62 year-old woman who presented with classical features of MFS and progressive bilateral dyschromatopsia and reported visual impairment. Normal MRI of the brain and CSF albumino-cytologic dissociation were observed. CSF oligoclonal IgG pattern indicated a passive transfer of oligoclonal IgG from a systemic inflammatory response. Nerve conduction studies showed slow motor conduction velocity in her extremities. The optic fundi were normal. Visual evoked potentials (VEPs) revealed bilateral optic neuropathy. Marked spontaneous improvement of her syndrome was documented within six weeks. Optic neuritis may be a central nervous system feature that should be recognized as part of the MFS.