Abstract
Purpose :
Miller Fisher Syndrome (MFS) is a rare variant of Guillan-Barre Syndrome characterized by the triad of ataxia, ophthalmoplegia, and hyporeflexia. Prompt diagnosis and aggressive treatment is necessary to prevent potentially fatal deterioration. Very few studies describing MFS and how it manifests in the pediatric population exist.
Methods :
Retrospective review of patients <18 y.o. diagnosed with MFS that were treated at _ Hospital between 2010-2022.
Results :
A total of 12 pediatric patients with MFS were identified. The mean age was 6.7 y.o. (range 0.3-17.0), 6/12 (50%) were male, and 5/12 (42%) were white. The average length of hospital stay was 17 days (range 1-61). A preceding infection was present in 9/12 (75%) patients and the median length of time between the infectious symptoms and the onset of MFS was 14 days (range 1-21). All patients presented with both ophthalmoplegia and hyporeflexia. Other commonly reported symptoms were ataxia (82%), lower extremity weakness (75%), diplopia (58%), facial paralysis (45%), extremity paralysis (42%), and ptosis (27%). Intubation due to paralysis and respiratory decline was required for 3/12 (25%) of patients. Anti-GQ1b antibodies were present in 1 patient and GM1 antibodies were present in one patient and equivocal in another. Th majority of patients (67%) had elevated protein in their cerebrospinal fluid (CSF) with an average CSF protein of 117.3 mg (range 17.8-599.9). 10/12 patients were treated with intravenous immunoglobulin (IVIG). 2/12 were also treated with plasmapheresis (PLEX). 1 patient that did not improve clinically with IVIG improved with prednisone. A complete recovery was seen in 5/12 patients, 4/12 had mild persisting symptoms, 2/12 were lost-to-follow-up after discharge, and 1/12 had a chronic, recurrent course of MFS.
Conclusions :
Here we report the clinical presentations of 12 patients with MFS. Though most adults with MFS have anti-GQ1b antibodies, only 1/12 child in our cohort was positive for anti-GQ1b antibodies. IVIG was an effective treatment for most patients. In one case, where IVIG was ineffective, the patient had clinical improvement with steroids. In a different patient that did not improve with IVIG, there was improvement with PLEX.
This abstract was presented at the 2024 ARVO Annual Meeting, held in Seattle, WA, May 5-9, 2024.