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Clinical spectrum of the anti-GQ1b antibody syndrome: a case series of eight patients

  • Alexander de Bruyn
  • Koen Poesen
  • Xavier Bossuyt
  • Isaac P. Heremans
  • Thomas Claeys
  • Christophe E. Depuydt
  • Philip Van Damme
  • Kristl G. ClaeysEmail author
Original article

Abstract

Anti-GQ1b antibodies can be detected in the serum of patients with Miller Fisher syndrome (MFS) and its incomplete forms such as acute ophthalmoparesis (AO), acute ptosis, acute mydriasis, acute oropharyngeal palsy and acute ataxic neuropathy (AAN), as well as in pharyngeal–cervical–brachial weakness, Bickerstaff brainstem encephalitis (BBE) and in overlap syndromes with Guillain–Barré syndrome (MFS–GBS, BBE–GBS). We searched the laboratory medicine database at University Hospitals Leuven between 2002 and 2017 for serum samples with anti-GQ1b IgG antibodies. We identified eight patients with anti-GQ1b antibodies: 4 MFS, 2 AO, 1 MFS–GBS and 1 AAN. Mean age was 57 years and five patients were males. Preceding illness was present in all patients. At nadir, we observed most frequently gait disturbance, external ophthalmoplegia and absent/decreased reflexes. Albumino-cytological dissociation was present in four patients. Mean time between onset and nadir was 4 days, between onset and recovery 2.5 months. Five patients recovered completely and three had minor residual symptoms. Interestingly, one patient with AO experienced a second identical episode, approximately 1 year after the first one. Our data confirm the broad clinical spectrum associated with the presence of anti-GQ1b IgG antibodies. Incomplete MFS subtypes such as AO are a challenge for diagnosis, because of the limited (though invalidating) clinical presentation and the lack of confirming ancillary tests. Subacute onset of ophthalmoplegia and/or ataxia should urge the clinician to include the anti-GQ1b antibody syndrome in the differential diagnosis.

Keywords

Miller Fisher syndrome Incomplete MFS Acute ophthalmoparesis Guillain–Barré syndrome GQ1b Anti-gangliosides 

Notes

Acknowledgements

We would like to thank the technical personnel of the Laboratory Medicine department at UZ Leuven for their technical support. PVD holds a senior clinical investigatorship of FWO-Vlaanderen.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© Belgian Neurological Society 2019

Authors and Affiliations

  1. 1.Department of NeurologyUniversity Hospitals LeuvenLeuvenBelgium
  2. 2.Laboratory for Molecular Neurobiomarker Research, Department of NeurosciencesKU LeuvenLeuvenBelgium
  3. 3.Laboratory MedicineUniversity Hospitals LeuvenLeuvenBelgium
  4. 4.Department of Microbiology and ImmunologyKU LeuvenLeuvenBelgium
  5. 5.Laboratory for Muscle Diseases and Neuropathies, Department of NeurosciencesKU LeuvenLeuvenBelgium
  6. 6.Laboratory of Neurobiology, Department of NeurosciencesKU Leuven and Center for Brain and Disease Research VIB LeuvenLeuvenBelgium

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