Background/Purpose: NMDA receptor antibody encephalitis (NMDAR) is a potentially devastating isolated autoimmune condition affecting children and young adults that was mostly unrecognized prior to 2007. The clinical phenotype includes encephalopathy, neurocognitive deficits, seizures, and abnormal movements. The largest published observational study  to include children suggests that earlier diagnosis, and utilization of immunotherapy (rituximab or cyclophosphamide) in refractory cases may decrease relapse rates and neurologic morbidity (Titulaer MJ et al. Lancet neurology 2013: 12;157-165). Yet, best care practices regarding multi-disciplinary care, the role of pediatric rheumatologists, and timing of immunotherapy modalities have not been validated for children.

Methods: A standardized multidisciplinary approach to NMDAR care was designed at our institution after struggling with initial cases during 2009-2011. A task force composed of physicians from neurology, rheumatology, intensive care, and transfusion medicine services reviewed the literature and approaches of other pediatric hospitals. Institutional criteria were developed regarding the use of therapeutic plasma exchange (TPE) and early B-cell depletion with rituximab.  In our algorithm, patients remaining at a moderate disability level  one week after first-line therapy (corticosteroids + IVIG or TPE) received rituximab.  We assessed initial presentations, interventions, and neurologic outcomes for all children diagnosed with NMDAR during the period of 2009-2013 after obtaining IRB approval. Only descriptive statistics were analyzed due to the small sample size of our cohort.

Results:  We identified 13 patients with the appropriate clinical phenotype and NMDA receptor antibodies in serum and/or CSF. There were 9 females (70%), median age at presentation was 10 (range 3-17), and median time from symptom onset to diagnosis and initial treatment was 16 days (range 2-90 days). Of the first six children treated at our institution (median follow-up time 20.5 months) without a standardized approach (2009-July 2012) only 1 received rituximab, and 4 had relapses (66.6%; 3 children within the first year and one child at 19 months). Since July 2012, 7 children have been treated according to a standardized approach. Six of these children (86%) received rituximab and there have been no relapses during the first year of follow-up (median 10 months).  Median time to diagnosis was similar in both cohorts (15 vs. 24 days). A greater percentage of children in the standardized cohort (71% vs. 33%) achieved a modified Rankin score of 0 (no deficits) OR 1 (mild deficits, no impairment).

Conclusion:  Preliminary analyses of children presenting with NMDAR since 2009 reveal lower relapse rates and disability scores in the recent cohort receiving standardized care and early utilization of rituximab. It is not apparent whether low relapse rates status post rituximab will be sustained with longer follow up. The institutional task force aims to address best care practices for psychiatric, neuropsychological, and rehabilitation services.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/does-a-standardized-multidisciplinary-approach-improve-outcomes-for-children-with-nmda-receptor-antibody-encephalitis-a-preliminary-assessment-of-a-single-center-experience/