Background and objective: Miller-Fisher syndrome (MFS) is considered the most common variant of Guillain-Barré syndrome (GBS) and is characterized by the clinical triad of ophthalmoplegia, ataxia and areflexia. Respiratory involvement and relapses are unusual. Patients with MFS usually have a good recovery and no residual deficits. We describe the clinical features, associated infections and evolution in eight patients with MFS.
Patients and method: Eight cases of MFS and sixty-one of GBS were studied between 1994 and 2003. All cases showed the clinical triad of MFS without major limb weakness or other signs suggestive of CNS involvement.
Results: The proportion of MFS with respect to GBS during the same period was 13.1%. Four had a positive serology for Epstein-Barr virus, Salmonella enteritidis, Chlamydia pneumoniae and Mycoplasma pneumoniae. Our cases showed facial palsy (75%), dysphagia (75%), pupillary abnormalities (37.5%) and ventilation support (37.5%). Antiganglioside antibodies, determined in three cases (4 episodes), were positive [GQ1b (50%) and GD1b (50%)]. In all cases, there was a markedly reduced amplitude of the distal sensory as well as frequent axonal degeneration signs. The oldest three patients relapsed and required ventilation support.
Conclusions: We report for the first time an association between S. enteritidis and C. pneumoniae and MFS. Older patients in our series suffered a faster disease progression with need of ventilation support. We conclude that an older age correlates with poor prognosis and relapses.