Evidence map›Paper›PMID 36288995›Full record

ArticleNeurology(R) neuroimmunology & neuroinflammation2023

Early-Stage Contactin-Associated Protein-like 2 Limbic Encephalitis: Clues for Diagnosis.

Jeanne Benoit, Sergio Muñiz-Castrillo, Alberto Vogrig, Antonio Farina, Anne-Laurie Pinto, Geraldine Picard, Veronique Rogemond, Deborah Guery, Agusti Alentorn, Dimitri Psimaras and 3 more

Open access · goldAbstract read
In one paragraph

Article in Neurology(R) neuroimmunology & neuroinflammation, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
17citing papers in PubMed, 2 pooled it
2.7field-weighted citation impact, top 8% of its field
1 · What the graph read from it

What it found

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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

17 citing papers in PubMed, 2 syntheses or guidelines pooled it, 24 citations in OpenAlex.

  1. Pooled it
  2. Pooled it
  3. Article
  4. Review
  5. High Relapse Rate in Anti-CASPR2 Disease: Implications for Prolonged Immunotherapy.Neurology(R) neuroimmunology & neuroinflammation · 2026
    Observational
  6. Review
  7. Review
  8. Article
  9. Autoimmune encephalitis.Nature reviews. Disease primers · 2025
    Review
  10. Review
  11. Review
  12. Recent advances in autoimmune encephalitis.Arquivos de neuro-psiquiatria · 2024
    Review
  13. Article
  14. Review
  15. Outcome and Sequelae of Autoimmune Encephalitis.Journal of clinical neurology (Seoul, Korea) · 2024
    Review
  16. Review
  17. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

13 authors at 5 institutions in 1 country.

Jeanne BenoitFrom the French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Hospices Civils de Lyon; Synaptopathies and Autoantibodies (SynatAc) Team (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Institut NeuroMyoGène, MELIS, INSERM U1314/CNRS UMR 5284, Université Claude Bernard Lyon 1; Epileptology-EEG Department (J.B.), Neurology, Pasteur2 Hospital, University Hospitals of Nice; Côte d'Azur University (J.B.), UR2CA, URRIS, Pasteur2 Hospital, University Hospitals of Nice; Department of Functional Neurology and Epileptology (D.G., S.R.), Hospices Civils de Lyon, France; European Network for Rare and Complex Epilepsies (EPICARE) (D.G., S.R.); Service de Neurologie 2-Mazarin (A.A., D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière-Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (A.A., D.P.), Institut Du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris; and Lyon's Neuroscience Research Center (S.R.), INSERM U1028/CNRS UMR 5292; Université Lyon 1 Claude Bernard, France.
Sergio Muñiz-CastrilloFrom the French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Hospices Civils de Lyon; Synaptopathies and Autoantibodies (SynatAc) Team (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Institut NeuroMyoGène, MELIS, INSERM U1314/CNRS UMR 5284, Université Claude Bernard Lyon 1; Epileptology-EEG Department (J.B.), Neurology, Pasteur2 Hospital, University Hospitals of Nice; Côte d'Azur University (J.B.), UR2CA, URRIS, Pasteur2 Hospital, University Hospitals of Nice; Department of Functional Neurology and Epileptology (D.G., S.R.), Hospices Civils de Lyon, France; European Network for Rare and Complex Epilepsies (EPICARE) (D.G., S.R.); Service de Neurologie 2-Mazarin (A.A., D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière-Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (A.A., D.P.), Institut Du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris; and Lyon's Neuroscience Research Center (S.R.), INSERM U1028/CNRS UMR 5292; Université Lyon 1 Claude Bernard, France.
Alberto VogrigFrom the French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Hospices Civils de Lyon; Synaptopathies and Autoantibodies (SynatAc) Team (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Institut NeuroMyoGène, MELIS, INSERM U1314/CNRS UMR 5284, Université Claude Bernard Lyon 1; Epileptology-EEG Department (J.B.), Neurology, Pasteur2 Hospital, University Hospitals of Nice; Côte d'Azur University (J.B.), UR2CA, URRIS, Pasteur2 Hospital, University Hospitals of Nice; Department of Functional Neurology and Epileptology (D.G., S.R.), Hospices Civils de Lyon, France; European Network for Rare and Complex Epilepsies (EPICARE) (D.G., S.R.); Service de Neurologie 2-Mazarin (A.A., D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière-Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (A.A., D.P.), Institut Du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris; and Lyon's Neuroscience Research Center (S.R.), INSERM U1028/CNRS UMR 5292; Université Lyon 1 Claude Bernard, France.
Antonio FarinaFrom the French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Hospices Civils de Lyon; Synaptopathies and Autoantibodies (SynatAc) Team (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Institut NeuroMyoGène, MELIS, INSERM U1314/CNRS UMR 5284, Université Claude Bernard Lyon 1; Epileptology-EEG Department (J.B.), Neurology, Pasteur2 Hospital, University Hospitals of Nice; Côte d'Azur University (J.B.), UR2CA, URRIS, Pasteur2 Hospital, University Hospitals of Nice; Department of Functional Neurology and Epileptology (D.G., S.R.), Hospices Civils de Lyon, France; European Network for Rare and Complex Epilepsies (EPICARE) (D.G., S.R.); Service de Neurologie 2-Mazarin (A.A., D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière-Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (A.A., D.P.), Institut Du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris; and Lyon's Neuroscience Research Center (S.R.), INSERM U1028/CNRS UMR 5292; Université Lyon 1 Claude Bernard, France.
Anne-Laurie PintoFrom the French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Hospices Civils de Lyon; Synaptopathies and Autoantibodies (SynatAc) Team (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Institut NeuroMyoGène, MELIS, INSERM U1314/CNRS UMR 5284, Université Claude Bernard Lyon 1; Epileptology-EEG Department (J.B.), Neurology, Pasteur2 Hospital, University Hospitals of Nice; Côte d'Azur University (J.B.), UR2CA, URRIS, Pasteur2 Hospital, University Hospitals of Nice; Department of Functional Neurology and Epileptology (D.G., S.R.), Hospices Civils de Lyon, France; European Network for Rare and Complex Epilepsies (EPICARE) (D.G., S.R.); Service de Neurologie 2-Mazarin (A.A., D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière-Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (A.A., D.P.), Institut Du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris; and Lyon's Neuroscience Research Center (S.R.), INSERM U1028/CNRS UMR 5292; Université Lyon 1 Claude Bernard, France.
Geraldine PicardFrom the French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Hospices Civils de Lyon; Synaptopathies and Autoantibodies (SynatAc) Team (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Institut NeuroMyoGène, MELIS, INSERM U1314/CNRS UMR 5284, Université Claude Bernard Lyon 1; Epileptology-EEG Department (J.B.), Neurology, Pasteur2 Hospital, University Hospitals of Nice; Côte d'Azur University (J.B.), UR2CA, URRIS, Pasteur2 Hospital, University Hospitals of Nice; Department of Functional Neurology and Epileptology (D.G., S.R.), Hospices Civils de Lyon, France; European Network for Rare and Complex Epilepsies (EPICARE) (D.G., S.R.); Service de Neurologie 2-Mazarin (A.A., D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière-Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (A.A., D.P.), Institut Du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris; and Lyon's Neuroscience Research Center (S.R.), INSERM U1028/CNRS UMR 5292; Université Lyon 1 Claude Bernard, France.
Veronique RogemondFrom the French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Hospices Civils de Lyon; Synaptopathies and Autoantibodies (SynatAc) Team (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Institut NeuroMyoGène, MELIS, INSERM U1314/CNRS UMR 5284, Université Claude Bernard Lyon 1; Epileptology-EEG Department (J.B.), Neurology, Pasteur2 Hospital, University Hospitals of Nice; Côte d'Azur University (J.B.), UR2CA, URRIS, Pasteur2 Hospital, University Hospitals of Nice; Department of Functional Neurology and Epileptology (D.G., S.R.), Hospices Civils de Lyon, France; European Network for Rare and Complex Epilepsies (EPICARE) (D.G., S.R.); Service de Neurologie 2-Mazarin (A.A., D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière-Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (A.A., D.P.), Institut Du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris; and Lyon's Neuroscience Research Center (S.R.), INSERM U1028/CNRS UMR 5292; Université Lyon 1 Claude Bernard, France.
Deborah GueryFrom the French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Hospices Civils de Lyon; Synaptopathies and Autoantibodies (SynatAc) Team (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Institut NeuroMyoGène, MELIS, INSERM U1314/CNRS UMR 5284, Université Claude Bernard Lyon 1; Epileptology-EEG Department (J.B.), Neurology, Pasteur2 Hospital, University Hospitals of Nice; Côte d'Azur University (J.B.), UR2CA, URRIS, Pasteur2 Hospital, University Hospitals of Nice; Department of Functional Neurology and Epileptology (D.G., S.R.), Hospices Civils de Lyon, France; European Network for Rare and Complex Epilepsies (EPICARE) (D.G., S.R.); Service de Neurologie 2-Mazarin (A.A., D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière-Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (A.A., D.P.), Institut Du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris; and Lyon's Neuroscience Research Center (S.R.), INSERM U1028/CNRS UMR 5292; Université Lyon 1 Claude Bernard, France.
Agusti AlentornFrom the French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Hospices Civils de Lyon; Synaptopathies and Autoantibodies (SynatAc) Team (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Institut NeuroMyoGène, MELIS, INSERM U1314/CNRS UMR 5284, Université Claude Bernard Lyon 1; Epileptology-EEG Department (J.B.), Neurology, Pasteur2 Hospital, University Hospitals of Nice; Côte d'Azur University (J.B.), UR2CA, URRIS, Pasteur2 Hospital, University Hospitals of Nice; Department of Functional Neurology and Epileptology (D.G., S.R.), Hospices Civils de Lyon, France; European Network for Rare and Complex Epilepsies (EPICARE) (D.G., S.R.); Service de Neurologie 2-Mazarin (A.A., D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière-Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (A.A., D.P.), Institut Du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris; and Lyon's Neuroscience Research Center (S.R.), INSERM U1028/CNRS UMR 5292; Université Lyon 1 Claude Bernard, France.
Dimitri PsimarasFrom the French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Hospices Civils de Lyon; Synaptopathies and Autoantibodies (SynatAc) Team (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Institut NeuroMyoGène, MELIS, INSERM U1314/CNRS UMR 5284, Université Claude Bernard Lyon 1; Epileptology-EEG Department (J.B.), Neurology, Pasteur2 Hospital, University Hospitals of Nice; Côte d'Azur University (J.B.), UR2CA, URRIS, Pasteur2 Hospital, University Hospitals of Nice; Department of Functional Neurology and Epileptology (D.G., S.R.), Hospices Civils de Lyon, France; European Network for Rare and Complex Epilepsies (EPICARE) (D.G., S.R.); Service de Neurologie 2-Mazarin (A.A., D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière-Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (A.A., D.P.), Institut Du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris; and Lyon's Neuroscience Research Center (S.R.), INSERM U1028/CNRS UMR 5292; Université Lyon 1 Claude Bernard, France.
Sylvain RheimsFrom the French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Hospices Civils de Lyon; Synaptopathies and Autoantibodies (SynatAc) Team (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Institut NeuroMyoGène, MELIS, INSERM U1314/CNRS UMR 5284, Université Claude Bernard Lyon 1; Epileptology-EEG Department (J.B.), Neurology, Pasteur2 Hospital, University Hospitals of Nice; Côte d'Azur University (J.B.), UR2CA, URRIS, Pasteur2 Hospital, University Hospitals of Nice; Department of Functional Neurology and Epileptology (D.G., S.R.), Hospices Civils de Lyon, France; European Network for Rare and Complex Epilepsies (EPICARE) (D.G., S.R.); Service de Neurologie 2-Mazarin (A.A., D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière-Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (A.A., D.P.), Institut Du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris; and Lyon's Neuroscience Research Center (S.R.), INSERM U1028/CNRS UMR 5292; Université Lyon 1 Claude Bernard, France.
Jérôme HonnoratFrom the French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Hospices Civils de Lyon; Synaptopathies and Autoantibodies (SynatAc) Team (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Institut NeuroMyoGène, MELIS, INSERM U1314/CNRS UMR 5284, Université Claude Bernard Lyon 1; Epileptology-EEG Department (J.B.), Neurology, Pasteur2 Hospital, University Hospitals of Nice; Côte d'Azur University (J.B.), UR2CA, URRIS, Pasteur2 Hospital, University Hospitals of Nice; Department of Functional Neurology and Epileptology (D.G., S.R.), Hospices Civils de Lyon, France; European Network for Rare and Complex Epilepsies (EPICARE) (D.G., S.R.); Service de Neurologie 2-Mazarin (A.A., D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière-Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (A.A., D.P.), Institut Du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris; and Lyon's Neuroscience Research Center (S.R.), INSERM U1028/CNRS UMR 5292; Université Lyon 1 Claude Bernard, France.
Bastien JoubertFrom the French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Hospices Civils de Lyon; Synaptopathies and Autoantibodies (SynatAc) Team (J.B., S.M.-C., A.V., A.F., A.-L.P., G.P., V.R., J.H., B.J.), Institut NeuroMyoGène, MELIS, INSERM U1314/CNRS UMR 5284, Université Claude Bernard Lyon 1; Epileptology-EEG Department (J.B.), Neurology, Pasteur2 Hospital, University Hospitals of Nice; Côte d'Azur University (J.B.), UR2CA, URRIS, Pasteur2 Hospital, University Hospitals of Nice; Department of Functional Neurology and Epileptology (D.G., S.R.), Hospices Civils de Lyon, France; European Network for Rare and Complex Epilepsies (EPICARE) (D.G., S.R.); Service de Neurologie 2-Mazarin (A.A., D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière-Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (A.A., D.P.), Institut Du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris; and Lyon's Neuroscience Research Center (S.R.), INSERM U1028/CNRS UMR 5292; Université Lyon 1 Claude Bernard, France. bastien.joubert@chu-lyon.fr.
Hôpital Pasteur · FRCentre de Recherche en Neurosciences de Lyon · FRHospices Civils de Lyon · FRInserm · FRCentre National de la Recherche Scientifique · FR

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

BACKGROUND AND

objectivesPrevious studies suggested that autoimmune limbic encephalitis with antibodies against contactin-associated protein-like 2 (CASPR2-encephalitis) is clinically heterogeneous and progresses slowly, preventing its early recognition. We aimed to describe the onset and progression of CASPR2-encephalitis and to assess long-term outcomes.

methodsWe retrospectively analyzed the medical records of all patients whose CSF tested positive for anti-CASPR2 antibodies in our center between 2006 and 2020. Standardized telephone interviews of all available patients and relatives were conducted, assessing long-term functional independence using the Functional Activity Questionnaire (FAQ) and quality of life using the 36-Item Short-Form Survey (SF36).

resultsForty-eight patients were included (98% males; median age 64 years), and 35 participated in telephone interviews (73%). At onset, 81% had at least 1 neurologic symptom among the following: limbic (54%), peripheral nerve hyperexcitability (PNH; 21%), and/or cerebellar symptoms (17%). Most of the patients (75%) had initially symptoms of only one of these categories. Limbic symptoms at onset included mostly seizures (33%), while memory disturbances were less frequent (10%). PNH signs were mostly neuropathic pain (9/10 patients). Other symptoms seen at onset included asthenia (33%), mood disorders (25%), and insomnia (21%); 19% of patients did not show any limbic, peripheral, or cerebellar symptom at onset but only asthenia (15%), mood disorders (6%), weight loss (8%), dysautonomia (4%), and/or insomnia (2%). The peak of the disease was attained in median 16.7 months after onset. Over the study period (median follow-up, 58.8 months, range 10.6-189.1), 77% of patients developed ≥3 core CASPR2 symptoms and 42% fulfilled the diagnostic criteria for autoimmune limbic encephalitis, although all patients ultimately developed limbic symptoms. At the last visit, most interviewed patients (28/35 patients, 80%; median, 5 years after onset) had recovered functional independence (FAQ <9) while only the vitality subscore of the SF36 was lower than normative data (mean 49.9 vs 58.0, DISCUSSION: CASPR2-encephalitis has a progressive course and is highly heterogeneous at the early stage. In men older than 50 years, otherwise unexplained seizures, cerebellar ataxia, and/or neuropathic pain are suggestive of early-stage CASPR2-encephalitis, especially if they coincide with recent asthenia, mood disorders, or insomnia.

Indexed as

EncephalitisLimbic EncephalitisNeuralgiaSleep Initiation and Maintenance DisordersAstheniaAutoantibodiesAutoimmune DiseasesContactinsFemaleHumansMaleMembrane ProteinsMiddle AgedNerve Tissue ProteinsQuality of LifeRetrospective StudiesAutoantibodiesContactinsMembrane ProteinsNerve Tissue Proteins

Identifiers

PMID36288995
PMCPMC9608385
OpenAlexW4307502386

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