Category: Choreas (Non-Huntington's Disease)
Objective: The spectrum of movement disorders (MDs) associated with anti N-methyl-D-aspartate (NMDAR) receptor encephalitis is vast and heterogenous, particularly in children, possibilities of which were investigated from two tertiary care centres in India
Background: Anti NMDAR encephalitis is a multi-staged, progressive, immune mediated disorder predominantly presenting with MDs, behavioural and language perturbations, seizures and dysautonomia. Extra-pyramidal MDs are believed to be one of the hallmarks of the disease, particularly in paediatric population, in whom neuropsychiatric manifestations might be difficult to appreciate.
Method: A retrospective study was conducted in two tertiary care referral centres in India, analysing the data of eight (n=8) paediatric patients (age <18 years) diagnosed as anti NMDAR encephalitis, presenting with one or more MDs
Results: All the patients were of Bengali ethnicity with a median age of 9 years (3-16 years) and female predilection (62.5%). Cerebrospinal fluid (CSF) pleocytosis was a common feature in all. Seizures were described sin 62.5% of patients, with a solitary patient having abnormalities on brain imaging. 3 out of 8 (37.5%) patients presented with a single MD while the others had combination of more than one type. Oro-linguo-facial dyskinesias and dystopias (37.5%) were the most common movement type, followed by chorea (12.5%). Complex motor stereotypes, myoclonus, and facial tics were noted in one patient each. All patients received pulse methyl prednisolone therapy. 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. A mortality of 37.5% was noted. Median duration of follow up was 26 months, during which none of the patients had evidence of neoplasm.
Conclusion: MDs are a core feature of anti NDMAR encephalitis, particularly in children and adolescents. Understanding and characterisation of these movement semiology’s in paramount in order to enable early diagnosis and prompt and effective therapy.
To cite this abstract in AMA style:A. Datta, A. Mukherjee, A. Pandit. Spectrum of movement disorders in paediatric anti NMDAR encephalitis [abstract]. Mov Disord. 2022; 37 (suppl 2). https://www.mdsabstracts.org/abstract/spectrum-of-movement-disorders-in-paediatric-anti-nmdar-encephalitis/. Accessed December 7, 2023.
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MDS Abstracts - https://www.mdsabstracts.org/abstract/spectrum-of-movement-disorders-in-paediatric-anti-nmdar-encephalitis/