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Bulbar involvement in patients with antiganglioside antibodies against NeuNAc(α2–3)Gal
  1. Ricard Rojas-Garcia1,2,
  2. Eduard Gallardo2,3,
  3. Noemi De Luna2,3,
  4. Candido Juarez4,
  5. Eugenia Martinez-Hernandez1,2,
  6. Alejandra Carvajal5,
  7. Carlos Casasnovas6,
  8. Eva Fages7,
  9. Pablo Davila-González8,
  10. Isabel Illa1,2
  1. 1Department of Neurology, Neuromuscular Diseases Unit, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain
  2. 2Centro de Investigación Biomédica en Red de Neurodegeneración (CIBERNED), Seville, Spain
  3. 3Laboratory of Experimental Neurology, Institut de Recerca de l'Hospital de la Santa Creu i Sant Pau, Barcelona, Spain
  4. 4Department of Immunology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain
  5. 5Department of Neurology, Hospital Sierrallana, Torrelavega, Spain
  6. 6Department of Neurology, Hospital Universitari de Bellvitge, Institut d'Investigació Biomèdica de Bellvitge, Hospital de Llobregat, Spain
  7. 7Department of Neurology, Hospital Santa Maria del Rosell, Cartagena, Spain
  8. 8Department of Neurology, Hospital de Manacor, Manacor, Spain
  1. Correspondence to Dr Ricard Rojas-Garcia, Neuromuscular Diseases Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Sant Antoni Maria Claret 167, Barcelona 08025, Spain; rrojas{at}santpau.cat

Abstract

Background Reactivity against terminal NeuNAc(α2–3)Gal, common to several gangliosides such as GD1a, GT1b and GM3, has rarely been reported. The authors recently described a patient with a clinical picture of acute relapsing sensory ataxic neuropathy and bulbar involvement in whom they demonstrated concomitant reactivity against NeuNAc(α2–3)Gal and disialosyl epitopes. The authors suggested a correlation between NeuNAc(α2–3)Gal reactivity and bulbar involvement.

Aim To determine the frequency of reactivity against terminal NeuNAc(α2–3)Gal in acute and chronic immune-mediated disorders, and its possible correlation with bulbar involvement.

Methods The authors retrospectively reviewed reactivity in the serum of more than 3000 consecutive patients with acute and chronic disorders in which antiganglioside antibodies were studied. The authors selected those patients who were simultaneously positive, by ELISA or thin-layer chromatography, for IgG or IgM antibodies anti-GM3, GD1a and GT1b, and reviewed their clinical features.

Results Reactivity against NeuNAc(α2–3)Gal, shared by GM3, GD1a and GT1b gangliosides, was detected in 10 patients: isolated in one patient, and concomitant with reactivity against other gangliosides in the remaining patients. Reactivity against NeuNAc(α2–3)Gal was frequently associated (8/10) with symptoms suggestive of bulbar involvement, such as dysphagia, dysarthria or dysphonia. Severe respiratory failure requiring mechanical ventilation was observed in four patients.

Conclusions Reactivity against the NeuNAc(α2–3)Gal epitope is rare and is generally found in association with reactivity against the disialosyl epitope. It can be detected in patients with acute or chronic disorders and could be a serological marker of clinical bulbar involvement and, to a lesser extent, associated with the development of severe respiratory failure.

  • Gangliosides
  • dysimmune neuropathies
  • GM3
  • GD1a
  • bulbar palsy
  • neuropathy

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Footnotes

  • Funding This work was supported by research grant FIS 06/0427 from the Fondo de Investigación Sanitaria, Instituto Carlos III, Spanish Ministry of Health.

  • Competing interests None.

  • Ethics approval Ethics approval was provided by the Ethics Committee of Santa Creu i Sant Pau Hospital.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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