Clinical outcomes of pediatric Anti-NMDA receptor encephalitis
YoungKyu Shim1, Soo Yeon Kim1, Hunmin Kim2
1Department of Pediatrics, Pediatric Clinical Neuroscience Center, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea.
Insights
Pediatric anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis often leads to favorable outcomes, but mild cognitive deficits like language and memory issues can persist. Early immunotherapy is crucial for managing this condition.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Autoimmune Disorders
Background:
- Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe autoimmune neurological disorder.
- Understanding the clinical spectrum and long-term prognosis in pediatric patients is essential for effective management.
Purpose of the Study:
- To investigate the clinical features and long-term outcomes of pediatric anti-NMDAR encephalitis.
- To evaluate the effectiveness of immunotherapy in this patient population.
Main Methods:
- Retrospective analysis of 32 pediatric patients with confirmed anti-NMDAR encephalitis.
- Clinical outcomes assessed using the Clinical Assessment Scale in Autoimmune Encephalitis (CASE) and modified Rankin Scale (mRS).
Main Results:
- The median age of onset was 9.0 years, with 75% of patients being female.
- 79.2% of patients achieved favorable outcomes (mRS ≤ 2) at 12-month follow-up.
- Mild cognitive deficits, particularly in language and memory, were observed in some patients, with younger children showing slower recovery.
Conclusions:
- Pediatric anti-NMDAR encephalitis generally has favorable outcomes with immunotherapy.
- Persistent mild cognitive impairments necessitate tailored outcome assessments and treatment strategies.
- The CASE scale is recommended for detailed outcome evaluation in pediatric anti-NMDAR encephalitis.
Objective:
To investigate the clinical features and long-term outcomes of pediatric Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.
Methods:
Thirty-two anti-NMDAR encephalitis patients with positive anti-NMDAR antibody test results were recruited. Clinical outcomes were evaluated using the Clinical Assessment Scale in Autoimmune Encephalitis (CASE) and the modified Rankin Scale (mRS).
Results:
The median age of onset was 9.0 years (range, 0.7-17.2 years). Twenty-four patients (75.0%) were female. All patients received first-line immunotherapy including intravenous immunoglobulin and/or steroid therapy. The second-line immunotherapy was administered to 22 patients (68.8%). Clinical outcomes were evaluated in 27 patients who were followed for longer than 6 months after onset, among whom the median follow-up duration was 31.2 months (range, 6.3-82.9 months). The proportion of patients with ≤2 points on the mRS at their 12-month follow-up was 79.2% (19/24). The CASE scores of these 19 patients ranged from 0 to 5, with language and memory deficits accounting for most of these disabilities. When the outcome was assessed according to onset age (<12 years or 12-18 years), the younger group tended to show a slower recovery over their clinical course.
Conclusions:
Despite overall favorable clinical outcomes, mild cognitive problems, including language and memory, may persist in pediatric anti-NMDAR encephalitis patients. A specific outcome measure, such as CASE, should be adopted to delineate clinical outcomes and aid the development of individualized treatment plans.
More Related Videos
07:20A Simple Cell-based Immunofluorescence Assay to Detect Autoantibody Against the N-Methyl-D-Aspartate NMDA Receptor in Blood
Published on: January 9, 2018
08:20Hippocampal Neuronal Cultures to Detect and Study New Pathogenic Antibodies Involved in Autoimmune Encephalitis
Published on: June 2, 2022