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A Simple Cell-based Immunofluorescence Assay to Detect Autoantibody Against the N-Methyl-D-Aspartate NMDA Receptor in Blood
Published on: January 9, 2018
Precocious puberty as a consequence of anti-NMDA receptor encephalitis in children
Po-Ming Wu1, Chao-Ku Teng1, Yen-Yin Chou1
1Department of Pediatrics, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, 70403, Taiwan.
Insights
Anti-NMDA receptor encephalitis can lead to long-term neurological issues in children, including epilepsy, cognitive deficits, and notably, central precocious puberty in pre-pubertal patients. Pediatricians must monitor for these consequences during follow-up.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Endocrinology
Background:
- Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a common autoimmune condition in children, with most experiencing good initial recovery.
- However, the critical role of NMDA receptor network function in pediatric brain development necessitates understanding long-term consequences.
- Long-term outcomes in pediatric anti-NMDA receptor encephalitis survivors are infrequently reported.
Purpose of the Study:
- To investigate the long-term neurological and endocrine consequences of anti-NMDA receptor encephalitis in pediatric patients.
- To identify specific sequelae in children following treatment for this autoimmune encephalitis.
Main Methods:
- A retrospective case series study involving 10 children under 18 with antibody-proven anti-NMDA receptor encephalitis.
- Patients were treated at a tertiary medical center between 2010 and 2019, with follow-up for long-term neurological outcomes.
Main Results:
- The study included 1 boy and 9 girls, with a median onset age of 3.6 years. Common initial symptoms included verbal decline and psychiatric issues, followed by decreased consciousness, seizures, and orofacial-lingual dyskinesia.
- All patients survived immunotherapy. Long-term sequelae included epilepsy (4 patients), cognitive deficits (4 patients), and mild psychiatric symptoms (1 patient).
- Notably, two pre-pubertal children developed central precocious puberty approximately 3 years post-encephalitis, with one requiring treatment.
Conclusions:
- Central precocious puberty is a potential long-term consequence of anti-NMDA receptor encephalitis in pre-pubertal children.
- Pediatricians should be vigilant in monitoring for central precocious puberty during follow-up evaluations of children who have had anti-NMDA receptor encephalitis.
Background:
Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is one of the most common autoimmune encephalitis in children. Most children recovered well after anti-NMDA receptor encephalitis. However, the NMDA receptor network functions are critical for the developing brain in children. The long-term consequences in pediatric patients of anti-NMDA receptor encephalitis are very infrequently reported.
Methods:
This case series study retrospectively enrolled 10 children aged below 18 years old with antibody-proved anti-NMDA receptor encephalitis in a tertiary medical center from 2010 to 2019. Long-term neurological consequences of anti-NMDA receptor encephalitis in children were followed.
Results:
One boy and nine girls were enrolled with a median onset age of 3.6 years. The most common initial presentation was verbal reduction and psychiatric symptoms soon after some flu-like prodromal symptoms. Nearly all patients then developed decreased level of consciousness, mutism, seizures and orofacial-lingual dyskinesia. Autonomic instability occurred in 5 patients, particularly in pre-pubertal children. Only one adolescent patient had ovarian teratoma. All patients survived after immunotherapy and were followed for 5.8 ± 3.3 years after discharge. Four had epilepsy within 2 years after encephalitis, four had a cognitive deficit, one had mild psychiatric symptoms of hallucination, and none had residual involuntary movements. Moreover, two pre-pubertal children developed central precocious puberty about 3 years after encephalitis, and one required gonadotropin-releasing hormone agonist treatment.
Conclusion:
Central precocious puberty could be a consequence of anti-NMDA receptor encephalitis in the pre-pubertal children. The pediatrician should pay attention to its occurrence at follow-up.
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