A video-based discussion of movement disorders in paediatric anti NMDAR encephalitis: A case series from Eastern India

Eur J Paediatr Neurol. 2022 Mar;37:40-45. doi: 10.1016/j.ejpn.2022.01.003. Epub 2022 Jan 13.

Abstract

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.

Keywords: Anti NMDAR; Hyperkinetic movements; Immunotherapy; Oro-facial dyskinesias; Rituximab.

MeSH terms

  • Adolescent
  • Anti-N-Methyl-D-Aspartate Receptor Encephalitis* / complications
  • Anti-N-Methyl-D-Aspartate Receptor Encephalitis* / diagnosis
  • Anti-N-Methyl-D-Aspartate Receptor Encephalitis* / drug therapy
  • Brain
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Male
  • Movement Disorders* / complications
  • Movement Disorders* / etiology
  • Receptors, N-Methyl-D-Aspartate
  • Retrospective Studies

Substances

  • Receptors, N-Methyl-D-Aspartate