title: Higher frequencies of HLA DQB1*05:01 and anti-glycosphingolipid antibodies in a cluster of severe Guillain-Barré syndrome.
abstract: Few regional and seasonal Guillain-Barré syndrome (GBS) clusters have been reported so far. It is unknown whether patients suffering from sporadic GBS differ from GBS clusters with respect to clinical and paraclinical parameters, HLA association and antibody response to glycosphingolipids and Campylobacter jejuni (Cj). We examined 40 consecutive patients with GBS from the greater Munich area in Germany with 14 of those admitted within a period of 3 months in fall 2010 defining a cluster of GBS. Sequencing-based HLA typing of the HLA genes DRB1, DQB1, and DPB1 was performed, and ELISA for anti-glycosphingolipid antibodies was carried out. Clinical and paraclinical findings (Cj seroreactivity, cerebrospinal fluid parameters, and electrophysiology) were obtained and analyzed. GBS cluster patients were characterized by a more severe clinical phenotype with more patients requiring mechanical ventilation and higher frequencies of autoantibodies against sulfatide, GalC and certain ganglioside epitopes (54 %) as compared to sporadic GBS cases (13 %, p = 0.017). Cj seropositivity tended to be higher within GBS cluster patients (69 %) as compared to sporadic cases (46 %, p = 0.155). We noted higher frequencies of HLA class II allele DQB1*05:01 in the cluster cohort (23 %) as compared to sporadic GBS patients (3 %, p = 0.019). Cluster of severe GBS was defined by higher frequencies of autoantibodies against glycosphingolipids. HLA class II allele DQB1*05:01 might contribute to clinical worsening in the cluster patients.
authors.affiliation: Department of Neurology, Klinikum rechts der Isar, Technical University of Munich, Munich, Germany.
authors.forename: L
authors.initials: L
authors.lastname: Schirmer
authors.affiliation: MRC Centre for Neuromuscular Diseases and Neuroimmunology and CSF Laboratory, National Hospital for Neurology, London, UK.
authors.forename: V
authors.initials: V
authors.lastname: Worthington
authors.affiliation: DKMS German Bone Marrow Center, Tübingen, Germany.
authors.forename: U
authors.initials: U
authors.lastname: Solloch
authors.affiliation: Department of Neurology, Klinikum rechts der Isar, Technical University of Munich, Munich, Germany.
authors.forename: V
authors.initials: V
authors.lastname: Loleit
authors.affiliation: Department of Neurology, Klinikum rechts der Isar, Technical University of Munich, Munich, Germany.
authors.forename: V
authors.initials: V
authors.lastname: Grummel
authors.affiliation: MRC Centre for Neuromuscular Diseases and Neuroimmunology and CSF Laboratory, National Hospital for Neurology, London, UK.
authors.forename: N
authors.initials: N
authors.lastname: Lakdawala
authors.affiliation: MRC Centre for Neuromuscular Diseases and Neuroimmunology and CSF Laboratory, National Hospital for Neurology, London, UK.
authors.forename: D
authors.initials: D
authors.lastname: Grant
authors.affiliation: DKMS Life Science Lab, Dresden, Germany.|Department of Quality Management, University Hospital Düsseldorf, Düsseldorf, Germany.
authors.forename: R
authors.initials: R
authors.lastname: Wassmuth
authors.affiliation: DKMS German Bone Marrow Center, Tübingen, Germany.|DKMS Life Science Lab, Dresden, Germany.
authors.forename: A H
authors.initials: AH
authors.lastname: Schmidt
authors.affiliation: Institute for Medical Microbiology, Klinikum rechts der Isar, Technical University of Munich, Munich, Germany.
authors.forename: F
authors.initials: F
authors.lastname: Gebhardt
authors.affiliation: Max Planck Institute of Psychiatry, Munich, Germany.|Munich Cluster for Systems Neurology (SyNergy), Munich, Germany.
authors.forename: T F M
authors.initials: TF
authors.lastname: Andlauer
authors.affiliation: DKMS German Bone Marrow Center, Tübingen, Germany.
authors.forename: J
authors.initials: J
authors.lastname: Sauter
authors.affiliation: Department of Neurology, Klinikum rechts der Isar, Technical University of Munich, Munich, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Berthele
authors.affiliation: MRC Centre for Neuromuscular Diseases and Neuroimmunology and CSF Laboratory, National Hospital for Neurology, London, UK.
authors.forename: M P
authors.initials: MP
authors.lastname: Lunn
authors.affiliation: Department of Neurology, Klinikum rechts der Isar, Technical University of Munich, Munich, Germany. hemmer@tum.de.|Munich Cluster for Systems Neurology (SyNergy), Munich, Germany. hemmer@tum.de.
authors.forename: Bernhard
authors.initials: B
authors.lastname: Hemmer
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000483:Alleles; D001323:Autoantibodies / Q000134:cerebrospinal fluid*; D002169:Campylobacter Infections / Q000276:immunology; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005500:Follow-Up Studies; D005787:Gene Frequency; D020022:Genetic Predisposition to Disease / Q000235:genetics; D005858:Germany; D006028:Glycosphingolipids / Q000276:immunology*; D020275:Guillain-Barre Syndrome / Q000134:cerebrospinal fluid* / Q000235:genetics* / Q000503:physiopathology; D059866:HLA-DQ beta-Chains / Q000235:genetics*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009431:Neural Conduction / Q000235:genetics; D012720:Severity of Illness Index; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D006028:Glycosphingolipids; D059866:HLA-DQ beta-Chains; C069051:HLA-DQB1 antigen
keywords: Autoantibodies; Campylobacter jejuni; Glycosphingolipids; Guillain–Barré syndrome (GBS); HLA class II alleles