A Rare Guillain-Barré Syndrome Variant with Multi-Ganglioside Reactivity: A Case of Severe Cranial Nerve Involvement
Authors:
Open Access
- 12 February 2025
- journal article
- case report
- Published by IMR Press in Revista De Neurologia
- Vol. 80 (1) , 37744
Abstract
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.Keywords
This publication has 12 references indexed in Scilit:
- Guillain-Barré syndromeThe Lancet, 2021
- Optimizing the electrodiagnostic accuracy in Guillain-Barré syndrome subtypes: Criteria sets and sparse linear discriminant analysisClinical Neurophysiology, 2017
- Antiganglioside, antiganglioside-complex, and antiglycolipid-complex antibodies in immune-mediated neuropathiesCurrent Opinion in Neurology, 2016
- Prevalence of Anti-Ganglioside Antibodies and Their Clinical Correlates with Guillain-Barré Syndrome in Korea: A Nationwide Multicenter StudyJournal of Clinical Neurology, 2014
- Diagnosis of Guillain-Barre syndrome and validation of Brighton criteriaBrain, 2013
- Bulbar involvement in patients with antiganglioside antibodies against NeuNAc( 2-3)GalJournal of Neurology, Neurosurgery & Psychiatry, 2009
- A novel antiganglioside specificity against terminal NeuNAc(alfa 2–3)Gal in acute bulbar palsyJournal of Neuroimmunology, 2006
- Peripheral neuropathies and anti-glycolipid antibodiesBrain, 2002
- A Randomized Trial Comparing Intravenous Immune Globulin and Plasma Exchange in Guillain–Barré SyndromeNew England Journal of Medicine, 1992
- Plasmapheresis and guillain‐barré syndrome: Analysis of prognostic factors and the effect of plasmapheresisAnnals of Neurology, 1988