PP08.12 – 2493: Atypical Miller Fisher syndrome presenting as acute isolated bilateral ophthalmoplegia: Report of two nex patients with serological specificity

Abstract

Objective Miller Fisher syndrome (MFS) is an acute polyradiculoneuritis regarded as an uncommon clinical variant of Guillain-Barre syndrome (GBS) asociated with antiGQ 1b positvie serology. MFS is characterized by the acute onset of a clinical triad of ophthalmoplegia, cereballar ataxia and areflexia. Atypical forms of MFS presenting as ophthalmoplegia without ataxia, have been scarcely described. Methods A detailed clinical and serological description of 2 young childhood cases of acute isolated bilateral ophthalmoplegia. Results Case 1 6-year-old boy presented with acute onset of diplopia, ten days after he experienced diarrhea and fever. Neurologic examination showed bilateral isolated sixth nerve palsy, more prominent on the left. Investigations including brain imaging, lumbar puncture and fundoscopic exam were normal. C. jejuni serology showed elevated IgG and IgA. Analysis of anti-ganglioside antibodies by ELISA in serum obtained immediately after admission revealed high levels of anti-GQ1b, anti-GT1a and anti-GD3 IgG and IgM. Case 2 4-year-old boy admitted with limited abduction of both eyes, as well as adduction paresis of the right eye. Two weeks before, the boy suffered from a febrile episode with diarrhea. All investigations carried out on admission were normal. ELISA for anti-ganglioside antibodies was performed on the serum obtained 2 weeks after onset and revealed elevated titers of anti-GQ1b and anti-GT1a IgG and IgM. Serology was positive to C. jejuni. We observed a complete spontaneous resolution 8 weeks after onset in both children. Conclusion The two patients we decribed confirm the rare but possible occurrence of atypical MFS in young children a few weeks following gastrointestinal infection. Identification of high titers of anti-GQ1b IgG associated with positive serology for C. jejuni enabled the diagnosis of atypical MFS. This paper highlights the relevance of measuring serum anti-GQ1b IgG level in the diagnostic work-up of acute isolated ophthalmoplegia of unknown aetiology in a child.