2025/02/12 by Laura Gómez-Dabó, Laura Gómez‐Dabó, Arnau Llauradó +16
Medicine · #Autoimmune Neurological Disorders and Treatments #Biology #Ganglioside #Genetics #Guillain-Barre syndrome #Medicine #Multiple Sclerosis Research Studies #Pathology #Pediatrics #Peripheral Neuropathies and Disorders #Reactivity (psychology)
paper · pdf · doi:10.31083/rn37744
crossref issued 2025/02/12 · crossref published 2025/02/12 · crossref published-online 2025/02/12 · openalex publication_date 2025/02/12 · crossref created 2025/03/07 · crossref deposited 2025/03/07 · openalex created_date 2025/10/10 · openalex updated_date 2026/08/06 · crossref indexed 2026/08/06
INTRODUCTION: We present a rare case of acute immune-mediated polyradiculoneuritis, a Guillain-Barré Syndrome (GBS) variant, manifesting as ophthalmoparesis-ataxia, facial diplegia, and acute bulbar palsy, accompanied by a unique autoimmune profile. CLINICAL CASE: A 75-year-old female developed rapidly progressive symptoms, including bilateral non-reactive mydriasis, ptosis, complete ophthalmoplegia, bilateral facial weakness, tongue immobility, palatal paralysis, limb dysmetria, ataxia, and brisk generalized tendon reflexes, all while maintaining a preserved mental state. Symptoms emerged 10 days after a probable gastrointestinal infection. Severe bulbar dysfunction necessitated orotracheal intubation and a tracheotomy. Extensive cranial nerve involvement initially suggested a brainstem lesion, with oculomotor and acute bulbar palsy as prominent signs. However, brainstem and spinal magnetic resonance imaging along with cerebrospinal fluid analysis yielded negative results. Electromyography reveled a sensorimotor demyelinating polyradiculoneuropathy, and serum testing identified IgG antibodies targeting multiple gangliosides, including the disialosyl group and terminal NeuNAc(α2-3)Gal. Treatment with intravenous immunoglobulin (IVIG) led to gradual clinical improvement. CONCLUSIONS: This case highlights a rare and severe GBS phenotype characterized by reactivity to multiple gangliosides. It highlights the role of shared ganglioside epitopes in antibody-mediated neurological damage and expands the clinical spectrum of GBS variants.