Symptomatic treatment of children with anti-NMDAR encephalitis
Shekeeb S Mohammad1,2, Hannah Jones3, Martin Hong2
1Neuroimmunology Group, Institute for Neuroscience and Muscle Research, The Children's Hospital at Westmead, Sydney, Australia.
Insights
Symptomatic management for anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis in children shows varied effectiveness. Sedatives and sleep medications were most beneficial, while antipsychotics carried significant adverse effects, particularly in Pacific Islander children.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
Background:
- Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a severe autoimmune neurological disorder.
- Symptomatic management is crucial for addressing diverse clinical manifestations in affected children.
Purpose of the Study:
- To evaluate the perceived benefits and adverse effects of symptomatic treatments in pediatric anti-NMDAR encephalitis.
- To identify effective and safe medication strategies for managing symptoms in this population.
Main Methods:
- Retrospective chart review of 27 children diagnosed with anti-NMDAR encephalitis at two tertiary pediatric hospitals.
- Analysis of medication efficacy and adverse effects for symptoms including agitation, seizures, movement disorders, and sleep disruption.
Main Results:
- Sedative and sleep medications (excluding benzodiazepines) demonstrated the highest benefit-to-adverse effect ratio (0.04).
- Antipsychotic medications showed the lowest mean benefit (35.4%) and a high adverse effect-benefit ratio (2.0).
- Pacific Islander children represented a significant proportion of the study cohort and may exhibit increased vulnerability.
Conclusions:
- Long-acting benzodiazepines, anticonvulsants, and clonidine are effective for managing multiple symptoms.
- Children with anti-NMDAR encephalitis are particularly susceptible to adverse effects from antipsychotic medications.
- A notable prevalence of anti-NMDAR encephalitis was observed in Pacific Islander populations within the study region.
Aim:
We performed the first study on the perceived benefit and adverse effects of symptomatic management in children with anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis.
Method:
A retrospective chart review was undertaken at two tertiary paediatric hospitals in Australia and New Zealand. We included 27 children (12 males, 15 females; mean age at admission 7y 1mo) with anti-NMDAR antibodies in serum or cerebrospinal fluid with a typical clinical syndrome.
Results:
Only two out of 27 patients were white, whereas 16 out of 27 patients were from the Pacific Islands/New Zealand Maori. The mean duration of admission was 69 days (10-224d) and 48% of patients (13/27) needed treatment in an intensive care setting. A mean of eight medications per patient was used for symptomatic management. Symptoms treated were agitation (n=25), seizures (n=24), movement disorders (n=23), sleep disruption (n=17), psychiatric symptoms (n=10), and dysautonomia (n=four). The medications used included five different benzodiazepines (n=25), seven anticonvulsants (n=25), eight sedatives and sleep medications (n=23), five antipsychotics (n=12), and five medications for movement disorders (n=10). Sedative and sleep medications other than benzodiazepines were the most effective, with a mean benefit of 67.4% per medication and a mean adverse effect-benefit ratio of 0.04 per medication. Antipsychotic drugs were used for a short duration (median 9d), and had the poorest mean benefit per medication of 35.4% and an adverse effect-benefit ratio of 2.0 per medication.
Interpretation:
Long-acting benzodiazepines, anticonvulsants, and clonidine can treat multiple symptoms. Patients with anti-NMDAR encephalitis appear vulnerable to antipsychotic-related adverse effects. Pacific Islanders appear to have a vulnerability to anti-NMDAR encephalitis in our region.
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