(591) Guillain-Barre Miller-Fisher Variant With Bilateral Severe Ptosis on Presentation

Introduction: Guillain-Barré syndrome (GBS) is the umbrella term for various types of acute immune-mediated polyneuropathies. The rare variant of GBS is Miller Fisher Syndrome (MFS). Ophthalmoplegia, areflexia, and ataxia are the clinical triad of MFS. Other associated symptoms include respiratory failure, limb weakness, distal paresthesia, diplopia, dysarthria, and dysautonomia. The following case presents the Miller Fisher variant with the unusual symptom of severe bilateral ptosis.

Description: 39-year-old with PTSD and TBI presented with progressive proximal muscle weakness, visual disturbance, and shortness of breath. Patient had a gastrointestinal illness two weeks prior to presentation. On examination severe bilateral ptosis, dysarthria, ophthalmoplegia, diffuse proximal muscle weakness, areflexia, and ataxia were present. CT and MRI brain imaging ruled out cerebrovascular disease. CSF studies showed mild cytoalbuminologic dissociation. EMG/NCS showed normal amplitude but prolonged F-responses consistent with demyelinating neuropathy. Patient received plasmapheresis. Disease plateaued following treatment and started improving. Acetylcholine and muscle specific kinase (MuSK) antibodies were negative effectively ruling out myasthenia gravis (MG). Ganglioside antibody panel was positive for G1M and GQ1B antibodies, consistent with Miller-Fisher variant.

Discussion: The patient in this case presented with symptoms of MFS: acute ophthalmoplegia, areflexia, and ataxia preceded by a viral illness. Studies have suggested that molecular mimicry between peripheral nerve gangliosides and bacterial or viral antigens is the culprit of neurological symptoms. MFS patients have antibodies against the ganglioside GQ1b. High ganglioside content can be found in oculomotor and bulbar nerves and extra-ocular muscle motor end plate, resulting in ophthalmoplegia. Due to the presentation of severe bilateral ptosis, ocular MG was considered until MuSK antibodies were negative and the G1M/GQ1B antibodies were positive. Randomized controlled trials showed that PLEX or intravenous immunoglobulin (IVIG) are standards of care for GBS. There are only a handful of case reports showing patients with atypical Miller Fisher variant presenting with ptosis. It is crucial to test for ganglioside antibodies to rule out MFS.