{
  "abstract": "Background Patients with leucine-rich glioma-inactivated 1 antibody (LGI1-Ab) encephalitis are typically elderly men that often carry human leucocyte antigen (HLA)- DRB1*07:01 (≈90%). Herein, we aimed to investigate whether patients with atypical demographic profiles or not carrying DRB1*07:01 have distinct clinical manifestations and outcome.Methods Retrospective chart review of LGI1-Ab patients diagnosed at the French Reference Centre and three other European centres.Results Among 238 patients included, median age at onset was 66 years (IQR: 60–73), 65% were male, 89% carried DRB1*07:01 and 10% DRB1*04:02, another known secondary HLA association. We identified three age groups (young, typical, old) based on percentiles of age distribution. Young (≤51 years) patients were less commonly male (35%, p=0.004), while faciobrachial dystonic seizures (FBDS; 65%, p=0.047) and hyponatraemia (64%, p=0.046) were more frequent in old (≥79 years) patients. Old patients experienced poor outcome (modified Rankin Scale [mRS] >2 at last follow-up) more frequently (64%, p<0.001). There were no significant differences between males and females. DRB1*07:01 non-carriers were younger (p=0.005) and less frequently male (47%, p=0.044), while non-carriers of both DRB1*07:01 and DRB1*04:02 experienced poor outcome more commonly (64%, p=0.005). Older age (adjusted OR: 1.08, 95% CI [1.02 to 1.14], p=0.008), higher mRS at nadir (4.22 [2.46–7.24], p<0.001) and DRB1*07:01 non-carrier status (8.39 [1.88–37.44], p=0.005) were independently associated with poor outcome. Moreover, older age (1.08 [1.04–1.11], p<0.001), FBDS (2.20 [1.17–4.13], p=0.014) and hyponatraemia (2.30 [1.22–4.34], p=0.010) were associated with severe encephalitis (mRS >3 at nadir).Conclusions Age appears to be the main driver of clinical presentation, severity and outcome in LGI1-Ab encephalitis. Remarkably, DRB1*07:01 non-carriers are younger, more commonly female and experience poorer prognosis, reflecting a distinct pathophysiology.",
  "authors": [
    {
      "affiliations": [
        "French National Reference Center for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, 69500 Bron, France",
        "Neurology Department, Hospital Universitario 12 de Octubre, Instituto de Investigación Sanitaria Hospital 12 de Octubre (imas12), 28041 Madrid, Spain"
      ],
      "name": "Lucia Campetella"
    },
    {
      "affiliations": [
        "French National Reference Center for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, 69500 Bron, France",
        "MeLiS – UCBL-CNRS UMR 5284 – INSERM U1314, Universite Claude Bernard Lyon 1, 69008 Lyon, France"
      ],
      "name": "Macarena Villagrán-García"
    },
    {
      "affiliations": [
        "French National Reference Center for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, 69500 Bron, France",
        "MeLiS – UCBL-CNRS UMR 5284 – INSERM U1314, Universite Claude Bernard Lyon 1, 69008 Lyon, France"
      ],
      "name": "Antonio Farina"
    },
    {
      "affiliations": [
        "French National Reference Center for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, 69500 Bron, France"
      ],
      "name": "Marine Villard"
    },
    {
      "affiliations": [
        "French National Reference Center for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, 69500 Bron, France",
        "MeLiS – UCBL-CNRS UMR 5284 – INSERM U1314, Universite Claude Bernard Lyon 1, 69008 Lyon, France"
      ],
      "name": "Marie Benaiteau"
    },
    {
      "affiliations": [
        "Clinical Neurology, Department of Head-Neck and Neuroscience, Azienda Sanitaria Universitaria Friuli Centrale (ASUFC), 33100 Udine, Italy",
        "Department of Medicine (DMED), University of Udine, 33100 Udine, Italy"
      ],
      "name": "Alberto Vogrig"
    },
    {
      "affiliations": [
        "Neuroscience Department, Catholic University of the Sacred Heart, 00168 Rome, Italy",
        "Clinical Neurology, Department of Ageing, Neurosciences, Head-neck and Orthopaedics Sciences, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy"
      ],
      "name": "Raffaele Iorio"
    },
    {
      "affiliations": [
        "Neurology Service, Regional University Hospital of Málaga, 29010 Málaga, Spain",
        "Biomedical Research Institute of Málaga and Nanomedicine Platform-IBIMA Platform BIONAND, 29590 Málaga, Spain",
        "Andalusian Network for Clinical and Translational Research in Neurology (NeuroRECA), 29010 Málaga, Spain"
      ],
      "name": "Nicolás Lundahl Ciano-Petersen"
    },
    {
      "affiliations": [
        "Neurology Department 2-Mazarin, Hôpitaux Universitaires La Pitié Salpêtrière-Charles Foix, APHP, 75013 Paris, France",
        "Paris Brain Institute, ICM, INSERM, CNRS, Sorbonne Université, 75013 Paris, France"
      ],
      "name": "Dimitri Psimaras"
    },
    {
      "affiliations": [
        "Paris Brain Institute, ICM, INSERM, CNRS, Sorbonne Université, 75013 Paris, France",
        "APHP, Epilepsy Unit, Reference Centre of Rare epilepsies, ERN-EPICARE, Pitié-Salpêtrière Hospital, 75013 Paris, France"
      ],
      "name": "Vincent Navarro"
    },
    {
      "affiliations": [
        "Stanford Centre for Sleep Science and Medicine, Stanford University, Palo Alto, CA, USA"
      ],
      "name": "Vicente Peris Sempere"
    },
    {
      "affiliations": [
        "Immunology Department, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, 69495 Oullins-Pierre-Bénite, France"
      ],
      "name": "David Gonçalves"
    },
    {
      "affiliations": [
        "French National Reference Center for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, 69500 Bron, France"
      ],
      "name": "Géraldine Picard"
    },
    {
      "affiliations": [
        "French National Reference Center for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, 69500 Bron, France"
      ],
      "name": "Véronique Rogemond"
    },
    {
      "affiliations": [
        "French National Reference Center for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, 69500 Bron, France",
        "Neurology Department, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, 69495 Oullins-Pierre-Bénite, France"
      ],
      "name": "Bastien Joubert"
    },
    {
      "affiliations": [
        "Stanford Centre for Sleep Science and Medicine, Stanford University, Palo Alto, CA, USA"
      ],
      "name": "Emmanuel Mignot"
    },
    {
      "affiliations": [
        "French National Reference Center for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, 69500 Bron, France",
        "MeLiS – UCBL-CNRS UMR 5284 – INSERM U1314, Universite Claude Bernard Lyon 1, 69008 Lyon, France"
      ],
      "name": "Jérôme Honnorat"
    },
    {
      "affiliations": [
        "French National Reference Center for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, 69500 Bron, France",
        "Neurology Department, Hospital Universitario 12 de Octubre, Instituto de Investigación Sanitaria Hospital 12 de Octubre (imas12), 28041 Madrid, Spain"
      ],
      "name": "Sergio Muñiz-Castrillo"
    }
  ],
  "title": "Clinical specificities and outcome of LGI1-antibody encephalitis according to age, sex and HLA",
  "uid": "fe4657d3-3451-531f-ad16-b9bfa48a8948"
}