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Clinical Features and Biomarkers Associated With Intensive Care-Dependent Refractory NMDAR Encephalitis: A
Iker Elosua-Bayes1,2,3, Pauline Dumez1,2,4, Marie Benaiteau1,2
1Centre de Référence des Syndromes Neurologiques Paranéoplasiques et Encéphalites Auto-immunes, Hospices Civils de Lyon, Hôpital Neurologique, Bron, France.
Refractory, intensive care-dependent anti-N-methyl-D-aspartate receptor encephalitis (RI-NMDARE) is a distinct subgroup with severe symptoms and poorer outcomes. Early recognition and tailored therapies are crucial for this high-risk population.
Area of Science:
- Neurology
- Immunology
- Critical Care Medicine
Background:
- Anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) can be refractory to standard immunotherapy, necessitating prolonged intensive care.
- A clear clinical definition and understanding of refractory, intensive care-dependent NMDARE (RI-NMDARE) are currently lacking.
- The frequency, risk factors, and outcomes of RI-NMDARE remain unknown.
Purpose of the Study:
- To define refractory, intensive care-dependent NMDARE (RI-NMDARE).
- To compare the clinical and biomarker characteristics of RI-NMDARE with severe NMDARE.
- To identify risk factors and outcomes associated with RI-NMDARE.
Main Methods:
- Retrospective cohort study of 216 patients with nonherpetic NMDARE admitted to intensive care units (ICUs).
- Data collected from 2005 to 2023 at the French National Reference Center.
- Favorable outcome defined as modified Rankin Scale (mRS) score < 2 at 24 months.
Main Results:
- RI-NMDARE identified in 12% of patients (26/216), characterized by prolonged ICU stays (median 5.7 months).
- RI-NMDARE patients showed higher rates of non-White ethnicity, mechanical ventilation, ovarian teratoma, and the triad of seizures, movement disorders, and dysautonomia.
- Elevated CSF cell counts, antibody titers, and serum neurofilament levels were observed in RI-NMDARE patients, alongside poorer outcomes (mRS ≥ 2) and higher mortality.
Conclusions:
- RI-NMDARE represents a distinct, high-risk subgroup of NMDARE.
- This subgroup exhibits a severe clinical profile, including rapid progression, specific symptom clusters, and elevated biomarkers.
- The findings highlight the need for early identification and specialized treatment strategies for RI-NMDARE to improve patient outcomes.
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