PMID- 37217310
OWN - NLM
STAT- MEDLINE
DCOM- 20230524
LR  - 20230605
IS  - 2332-7812 (Electronic)
IS  - 2332-7812 (Linking)
VI  - 10
IP  - 4
DP  - 2023 Jul
TI  - Anti-LGI1 Encephalitis With Co-occurring IgLON5 Antibodies: Clinical Features and 
      Human Leukocyte Antigen Haplotypes.
LID - 10.1212/NXI.0000000000200126 [doi]
LID - e200126
AB  - OBJECTIVES: Autoimmune encephalitis (AE) with antibodies against LGI1 and IgLON5 
      are clinically distinctive but share some particularities such as a strong 
      association with specific human leukocyte antigen (HLA) class II alleles. 
      METHODS: We clinically describe a patient with double positivity for LGI1 and 
      IgLON5 antibodies. In addition, we conducted specific immunodepletion with the 
      patient's serum and HLA typing and investigated the presence of serum IgLON5 
      antibodies in a cohort of 23 anti-LGI1 patients carrying the HLA predisposing for 
      anti-IgLON5 encephalitis. RESULTS: A 70-year-old woman with a history of 
      lymphoepithelial thymoma presented with subacute cognitive impairment and 
      seizures. Investigations included MRI and EEG showing medial temporal 
      involvement, increased CSF protein content, and polysomnography with REM and 
      non-REM motor activity, along with obstructive apnea. Neural antibody testing 
      revealed both LGI1 and IgLON5 antibodies in serum and CSF, and serum 
      immunodepletion ruled out cross-reactivity. The patient carried DRB1*07:01 and 
      DQA1*01:01 approximately DQB1*05:01, but no other IgLON5-positive case was identified in a 
      cohort of anti-LGI1 patients carrying DQA1*01 approximately DQB1*05. Nearly full therapeutic 
      response was obtained after intensified immunosuppressive treatment. DISCUSSION: 
      We present a case of anti-LGI1 encephalitis with concomitant IgLON5 antibodies. 
      Co-occurring IgLON5 antibodies in anti-LGI1 encephalitis are exceptional, but may 
      appear in genetically predisposed individuals.
CI  - Copyright (c) 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on 
      behalf of the American Academy of Neurology.
FAU - Schiff, Pierre
AU  - Schiff P
AD  - From the Neurology Department (P.S., M.L.F., E.C., L.P.-M.), University Hospital 
      of Clermont-Ferrand; French Reference Center for Paraneoplastic Neurological 
      Syndromes and Autoimmune Encephalitis (S.M.-C., L.D.D., V.R., J.H.), Hospices 
      Civils de Lyon; MeLiS - UCBL-CNRS UMR 5284 - INSERM U1314 (S.M.-C., L.D.D., V.R., 
      J.H.), Universite Claude Bernard Lyon 1, France; Stanford Center for Sleep 
      Sciences and Medicine (S.M.-C.), Stanford University, Palo Alto, CA; Sleep and 
      EEG (M.L.F.), Neurophysiology Unit, University Hospital and UMR 6602- Universite 
      Clermont Auvergne, CNRS, Institute Pascal; and UMR 1107 Inserm (L.P.-M.), 
      Neuro-Dol, Clermont-Ferrand, France.
FAU - Muniz-Castrillo, Sergio
AU  - Muniz-Castrillo S
AUID- ORCID: 0000-0001-5958-3288
AD  - From the Neurology Department (P.S., M.L.F., E.C., L.P.-M.), University Hospital 
      of Clermont-Ferrand; French Reference Center for Paraneoplastic Neurological 
      Syndromes and Autoimmune Encephalitis (S.M.-C., L.D.D., V.R., J.H.), Hospices 
      Civils de Lyon; MeLiS - UCBL-CNRS UMR 5284 - INSERM U1314 (S.M.-C., L.D.D., V.R., 
      J.H.), Universite Claude Bernard Lyon 1, France; Stanford Center for Sleep 
      Sciences and Medicine (S.M.-C.), Stanford University, Palo Alto, CA; Sleep and 
      EEG (M.L.F.), Neurophysiology Unit, University Hospital and UMR 6602- Universite 
      Clermont Auvergne, CNRS, Institute Pascal; and UMR 1107 Inserm (L.P.-M.), 
      Neuro-Dol, Clermont-Ferrand, France.
FAU - Do, Le Duy
AU  - Do LD
AUID- ORCID: 0000-0001-7664-533X
AD  - From the Neurology Department (P.S., M.L.F., E.C., L.P.-M.), University Hospital 
      of Clermont-Ferrand; French Reference Center for Paraneoplastic Neurological 
      Syndromes and Autoimmune Encephalitis (S.M.-C., L.D.D., V.R., J.H.), Hospices 
      Civils de Lyon; MeLiS - UCBL-CNRS UMR 5284 - INSERM U1314 (S.M.-C., L.D.D., V.R., 
      J.H.), Universite Claude Bernard Lyon 1, France; Stanford Center for Sleep 
      Sciences and Medicine (S.M.-C.), Stanford University, Palo Alto, CA; Sleep and 
      EEG (M.L.F.), Neurophysiology Unit, University Hospital and UMR 6602- Universite 
      Clermont Auvergne, CNRS, Institute Pascal; and UMR 1107 Inserm (L.P.-M.), 
      Neuro-Dol, Clermont-Ferrand, France.
FAU - Fantini, Maria Livia
AU  - Fantini ML
AD  - From the Neurology Department (P.S., M.L.F., E.C., L.P.-M.), University Hospital 
      of Clermont-Ferrand; French Reference Center for Paraneoplastic Neurological 
      Syndromes and Autoimmune Encephalitis (S.M.-C., L.D.D., V.R., J.H.), Hospices 
      Civils de Lyon; MeLiS - UCBL-CNRS UMR 5284 - INSERM U1314 (S.M.-C., L.D.D., V.R., 
      J.H.), Universite Claude Bernard Lyon 1, France; Stanford Center for Sleep 
      Sciences and Medicine (S.M.-C.), Stanford University, Palo Alto, CA; Sleep and 
      EEG (M.L.F.), Neurophysiology Unit, University Hospital and UMR 6602- Universite 
      Clermont Auvergne, CNRS, Institute Pascal; and UMR 1107 Inserm (L.P.-M.), 
      Neuro-Dol, Clermont-Ferrand, France.
FAU - Chanson, Eve
AU  - Chanson E
AD  - From the Neurology Department (P.S., M.L.F., E.C., L.P.-M.), University Hospital 
      of Clermont-Ferrand; French Reference Center for Paraneoplastic Neurological 
      Syndromes and Autoimmune Encephalitis (S.M.-C., L.D.D., V.R., J.H.), Hospices 
      Civils de Lyon; MeLiS - UCBL-CNRS UMR 5284 - INSERM U1314 (S.M.-C., L.D.D., V.R., 
      J.H.), Universite Claude Bernard Lyon 1, France; Stanford Center for Sleep 
      Sciences and Medicine (S.M.-C.), Stanford University, Palo Alto, CA; Sleep and 
      EEG (M.L.F.), Neurophysiology Unit, University Hospital and UMR 6602- Universite 
      Clermont Auvergne, CNRS, Institute Pascal; and UMR 1107 Inserm (L.P.-M.), 
      Neuro-Dol, Clermont-Ferrand, France.
FAU - Rogemond, Veronique
AU  - Rogemond V
AUID- ORCID: 0000-0003-2098-920X
AD  - From the Neurology Department (P.S., M.L.F., E.C., L.P.-M.), University Hospital 
      of Clermont-Ferrand; French Reference Center for Paraneoplastic Neurological 
      Syndromes and Autoimmune Encephalitis (S.M.-C., L.D.D., V.R., J.H.), Hospices 
      Civils de Lyon; MeLiS - UCBL-CNRS UMR 5284 - INSERM U1314 (S.M.-C., L.D.D., V.R., 
      J.H.), Universite Claude Bernard Lyon 1, France; Stanford Center for Sleep 
      Sciences and Medicine (S.M.-C.), Stanford University, Palo Alto, CA; Sleep and 
      EEG (M.L.F.), Neurophysiology Unit, University Hospital and UMR 6602- Universite 
      Clermont Auvergne, CNRS, Institute Pascal; and UMR 1107 Inserm (L.P.-M.), 
      Neuro-Dol, Clermont-Ferrand, France.
FAU - Honnorat, Jerome
AU  - Honnorat J
AD  - From the Neurology Department (P.S., M.L.F., E.C., L.P.-M.), University Hospital 
      of Clermont-Ferrand; French Reference Center for Paraneoplastic Neurological 
      Syndromes and Autoimmune Encephalitis (S.M.-C., L.D.D., V.R., J.H.), Hospices 
      Civils de Lyon; MeLiS - UCBL-CNRS UMR 5284 - INSERM U1314 (S.M.-C., L.D.D., V.R., 
      J.H.), Universite Claude Bernard Lyon 1, France; Stanford Center for Sleep 
      Sciences and Medicine (S.M.-C.), Stanford University, Palo Alto, CA; Sleep and 
      EEG (M.L.F.), Neurophysiology Unit, University Hospital and UMR 6602- Universite 
      Clermont Auvergne, CNRS, Institute Pascal; and UMR 1107 Inserm (L.P.-M.), 
      Neuro-Dol, Clermont-Ferrand, France.
FAU - Poncet-Megemont, Louis
AU  - Poncet-Megemont L
AD  - From the Neurology Department (P.S., M.L.F., E.C., L.P.-M.), University Hospital 
      of Clermont-Ferrand; French Reference Center for Paraneoplastic Neurological 
      Syndromes and Autoimmune Encephalitis (S.M.-C., L.D.D., V.R., J.H.), Hospices 
      Civils de Lyon; MeLiS - UCBL-CNRS UMR 5284 - INSERM U1314 (S.M.-C., L.D.D., V.R., 
      J.H.), Universite Claude Bernard Lyon 1, France; Stanford Center for Sleep 
      Sciences and Medicine (S.M.-C.), Stanford University, Palo Alto, CA; Sleep and 
      EEG (M.L.F.), Neurophysiology Unit, University Hospital and UMR 6602- Universite 
      Clermont Auvergne, CNRS, Institute Pascal; and UMR 1107 Inserm (L.P.-M.), 
      Neuro-Dol, Clermont-Ferrand, France. lponcet-megemont@chu-clermontferrand.fr.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20230522
PL  - United States
TA  - Neurol Neuroimmunol Neuroinflamm
JT  - Neurology(R) neuroimmunology & neuroinflammation
JID - 101636388
RN  - 0 (Autoantibodies)
RN  - 0 (Proteins)
RN  - 0 (HLA Antigens)
RN  - 0 (Histocompatibility Antigens Class II)
SB  - IM
MH  - Female
MH  - Humans
MH  - Aged
MH  - Haplotypes
MH  - *Autoantibodies
MH  - Proteins
MH  - *Encephalitis/diagnosis
MH  - HLA Antigens
MH  - Histocompatibility Antigens Class II
PMC - PMC10238150
COIS- The authors report no disclosures relevant to the manuscript. Go to 
      Neurology.org/NN for full disclosures.
EDAT- 2023/05/23 01:06
MHDA- 2023/05/24 06:42
PMCR- 2023/05/22
CRDT- 2023/05/22 21:43
PHST- 2023/01/27 00:00 [received]
PHST- 2023/03/31 00:00 [accepted]
PHST- 2023/05/24 06:42 [medline]
PHST- 2023/05/23 01:06 [pubmed]
PHST- 2023/05/22 21:43 [entrez]
PHST- 2023/05/22 00:00 [pmc-release]
AID - 10/4/e200126 [pii]
AID - NXI-2023-000031 [pii]
AID - 10.1212/NXI.0000000000200126 [doi]
PST - epublish
SO  - Neurol Neuroimmunol Neuroinflamm. 2023 May 22;10(4):e200126. doi: 
      10.1212/NXI.0000000000200126. Print 2023 Jul.