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A video-based discussion of movement disorders in paediatric anti NMDAR encephalitis: A case series from Eastern
Amlan Kusum Datta1, Prakash Chandra Ghosh2, Mitali Bera2
1Department of Neurology, Bangur Institute of Neurology, IPGMER SSKM Hospital, Kolkata, West Bengal, India.
Insights
Movement disorders are a key feature of anti-NMDAR encephalitis in children. Early diagnosis and characterization of these movement disorders (MDs) are crucial for effective treatment and improved outcomes.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Anti-N-Methyl-d-Aspartate-Receptor (NMDAR) encephalitis is a severe autoimmune neurological disorder.
- Movement disorders (MDs) are a prominent and diverse clinical manifestation, especially in pediatric cases.
Purpose of the Study:
- To investigate the spectrum of movement disorders in children diagnosed with anti-NMDAR encephalitis.
- To analyze the clinical characteristics, treatment response, and outcomes in pediatric patients with anti-NMDAR encephalitis and MDs.
Main Methods:
- A retrospective study was conducted across two tertiary care centers in Eastern India.
- Data from 8 pediatric patients diagnosed with anti-NMDAR encephalitis and presenting with movement disorders were analyzed.
Main Results:
- The study included 8 pediatric patients, with a median age of 9 years and a female predilection.
- Oro-linguo-facial dyskinesias and dystonia were the most common movement disorders (37.5% each).
- Immunotherapy led to resolution of hyperkinetic movements in 50% of patients, with a 37.5% mortality rate.
Conclusions:
- Movement disorders are a core feature of anti-NMDAR encephalitis in children.
- Understanding and characterizing these movement disorders are essential for early diagnosis and effective therapeutic strategies.
- Despite immunotherapy, a significant portion of patients experienced persistent movements or mortality.
Purpose:
The spectrum of movement disorders associated with anti N-Methyl-d-Aspartate-Receptor (NMDAR) encephalitis is myriad, particularly in children, possibilities of which were investigated from two tertiary care centres.
Methods:
A retrospective study was conducted in two tertiary referral centres in Eastern India, analysing data of 8 paediatric patients diagnosed as anti NMDAR encephalitis, presenting with one or more movement disorders (MDs).
Results:
All the patients were of Bengali ethnicity with a median age of 9 years (3-16 years) and with female predilection (62.5%). CSF pleocytosis was a common feature in all. Seizures were described in 62.5%% of patients with a solitary patient exhibiting abnormalities on brain imaging. 3 out of 8 (37.5%) of patients presented with a single MD while the remaining had more than one type. Oro-linguo-facial dyskinesias and dystonia (37.5% each) were the most common movement type followed by chorea (12.5%). Complex stereotypies, myoclonus and facial tics were noted in one patient each. All patients received pulse methyl prednisolone. Escalation to second line therapy in form of rituximab was done for 5 patients (62.5%). Following immunotherapy, hyperkinetic movements resolved in 50% of patients, with persistence of movements in one (12.5%). A mortality of 37.5% was noted. Median duration of follow up was 26 months, during which none of the patients had evidence of systemic neoplasm.
Conclusion:
MDs are a core feature of anti NMDAR encephalitis, particularly in the paediatric age group, understanding and characterization of which, is the key to early diagnosis and effective therapy.
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