Long-Term Clinical and Biological Prognostic Factors of Anti-NMDA Receptor Encephalitis in Children
Neurol Neuroimmunol Neuroinflamm. 2025-03-01; 12(2):
DOI: 10.1212/NXI.0000000000200346
Lire sur PubMed
1. Neurol Neuroimmunol Neuroinflamm. 2025 Mar;12(2):e200346. doi:
10.1212/NXI.0000000000200346. Epub 2024 Dec 23.
Long-Term Clinical and Biological Prognostic Factors of Anti-NMDA Receptor
Encephalitis in Children.
Mazowiecki M(1), Flet-Berliac L(1), Roux J(1), Lépine A(2), Chretien P(3)(4),
Hacein-Bey-Abina S(3)(4), Giorgi L(1)(5), Villega F(6)(7), Cheuret E(8),
Benaiteau M(9), Rogemond V(9), Picard G(9), Baer S(10)(11), Cleuziou P(12),
Lametery E(13), Desguerre I(14), Aubart M(14), Chevignard M(15)(16)(17), Le
Grand R(18), Horellou P(18)(5), Leroy C(18)(5), Joubert B(9), Honnorat J(9),
Deiva K(1)(18)(5)(19).
Author information:
(1)Pediatric Neurology Departement, Assistance Publique-Hôpitaux de Paris,
Paris-Saclay University Hospitals, Bicêtre Hospital, and Paris-Saclay
University, Le Kremlin-Bicêtre.
(2)Pediatric Neurology Department, Assistance Publique des Hôpitaux de
Marseille, Hôpital Universitaire, Marseille.
(3)Clinical Immunology Laboratory, Assistance Publique des Hôpitaux de Paris,
Hôpitaux Universitaires Paris-Saclay, Bicêtre Hospital, and Paris-Saclay
University, Le Kremlin-Bicêtre.
(4)UTCBS, UMR8258 CNRS-U1267 INSERM, Faculté de Pharmacie de Paris, Université
de Paris.
(5)National Referral Center for Rare Inflammatory Brain and Spinal Diseases, Le
Kremlin-Bicêtre; and.
(6)Pediatric Neurology Department, CICp-1401, University Children Hospital,
Bordeaux.
(7)Interdisciplinary Institute for Neurosciences, CNRS UMR 5297.
(8)Pediatric Neurology Department, Purpan University Hospital, Toulouse.
(9)Reference Center on autoimmune encephalitis, Hospices Civils de Lyon,
Institut MELIS, Inserm U1314/CNRS UMR 5284, Université Claude Bernard Lyon 1.
(10)Department of Neuropediatrics, ERN EpiCare, Hôpitaux Universitaires de
Strasbourg.
(11)Institute for Genetics and Molecular and Cellular Biology (IGBMC),
University of Strasbourg, CNRS UMR7104, INSERM U1258, Illkirch, France.
(12)Department of Pediatric Neurology, Lille University Hospital.
(13)Pediatric Department, Grenoble Alpes University Hospital, Hôpital Albert
Michallon.
(14)Pediatric Neurology Department Necker-Enfants Malades Hospital, University
of Paris, AP-HP.
(15)Rehabilitation Department for Children with Acquired Neurological Injury,
Saint-Maurice Hospitals (M.C.); Saint Maurice Hospitals.
(16)Sorbonne Université, CNRS, INSERM, Laboratoire d’Imagerie Biomédicale (LIB).
(17)Sorbonne Université, GRC 24 Handicap Moteur Cognitif et Réadaptation
(HaMCRe), Paris.
(18)Université Paris-Saclay, CEA, INSERM Center for Immunology of Viral,
Auto-Immune, Hematological and Bacterial diseases (IMVA-HB/IDMIT).
(19)Institut Universitaire de France, France.
BACKGROUND AND OBJECTIVES: Anti-NMDAR encephalitis (NMDARE) is a severe
neurologic condition, and recently, the NMDAR Encephalitis One-Year Functional
Status (NEOS) score has emerged as a 1-year prognostic tool. This study aimed to
evaluate NEOS score and biomarker (neurofilament light chains [NfL], total-Tau
protein, glial fibrillary acidic protein, and serum cytokines) correlation with
modified Rankin Scale (mRS), cognitive impairment, and clinical recovery in
pediatric NMDARE over 2 years.
METHODS: In this French multicenter observational study, 104 pediatric patients
with NMDARE were followed for a minimum of 2 years. Clinical data and
serum/plasma samples were collected. Biomarker levels, measured using
electroluminescence mesoscale discovery (MSD) S-PLEX, were compared between
patients and controls and assessed for correlations with disease activity, mRS,
cognitive/language impairment, and recovery status at 2 years.
RESULTS: At a median follow-up of 39.5 months, 68 percent of patients had
unfavorable recovery and 54% had significant cognitive impairment. Both outcomes
were strongly associated with younger age at diagnosis (OR 6.10 [1.91-27.3] p <
0.01 and 5.69 [1.46-27.7] p = 0.02, respectively). A higher NEOS score was
significantly correlated with increased cognitive impairment (OR 2.53
[1.52-4.21], p < 0.001), higher mRS scores (OR 2.12 [1.34-3.57], p < 0.01), and
unfavorable recovery at 2 years (OR 2.00 [1.30-3.06], p = 0.015). Elevated NfL
levels were significantly associated with unfavorable recovery (OR 3.62
[1.29-10.9] p = 0.012) and severe cognitive impairment (OR 3.77 [1.38-10.9] p =
0.012) at 2 years. The combined area under the curve (AUC) for NfL and NEOS was
significantly higher than the AUCs of NEOS and NfL alone (p = 0.01).
DISCUSSION: The NEOS score strongly predicts long-term outcomes in NMDARE, with
its predictive value extending beyond the first-year mR prediction. NfL levels
at disease onset seem to improve accuracy in predicting poor outcomes, providing
valuable information for treatment decisions and future clinical trials.
DOI: 10.1212/NXI.0000000000200346
PMID: 39715492 [Indexed for MEDLINE]