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Published on: January 9, 2018
Pharmacologic Treatment and Early Rehabilitation Outcomes in Pediatric Patients With Anti-NMDA Receptor Encephalitis
Gabrielle Alvarez1, Alyssa Krentzel2, Joshua Vova3
1Division of Rehabilitation Psychology, Seattle Children's Hospital.
Insights
Pediatric N-methyl-D-aspartate receptor encephalitis (NMDARE) patients received extensive immunotherapy and polypharmacy during rehabilitation. Younger age and specific symptoms like seizures were linked to unfavorable early functional outcomes.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Rehabilitation Medicine
Background:
- N-methyl-D-aspartate receptor encephalitis (NMDARE) is a severe autoimmune neurological disorder affecting children.
- Understanding treatment patterns and early outcomes in pediatric NMDARE is crucial for optimizing care.
- Inpatient rehabilitation plays a key role in managing the complex needs of these patients.
Purpose of the Study:
- To describe immunotherapy and pharmacologic treatments for pediatric NMDARE during inpatient rehabilitation.
- To identify clinical and demographic factors associated with early functional outcomes in these patients.
Main Methods:
- Retrospective chart review and post hoc analysis of 26 pediatric patients with NMDARE.
- Utilized the FIM for Children (WeeFIM) Developmental Functional Quotient (DFQ) to assess functional outcomes.
- Examined demographic data and prescribed medications for symptom management.
Main Results:
- All patients received first-line immunotherapy, with 69% receiving second-line treatments.
- Average of 8 medications prescribed per patient, primarily for agitation, psychiatric symptoms, and seizures.
- 65% showed improved Total WeeFIM DFQ; 35% had unfavorable early outcomes.
- Younger age, seizures, movement disorders, and decreased consciousness were associated with unfavorable outcomes.
Conclusions:
- Pediatric NMDARE care involves significant symptomatic heterogeneity and polypharmacy.
- Seizures, movement disorders, and neurological status impact early functional outcomes.
- Further research is needed to refine classification and understand long-term outcomes.
Objectives:
To describe the immunotherapy and pharmacologic treatments administered to pediatric patients with N-methyl-D-aspartate receptor encephalitis (NMDARE) during inpatient rehabilitation as well as to examine clinical and demographic variables associated with early functional outcomes.
Design:
Retrospective chart review and post hoc analysis.
Setting:
Pediatric inpatient rehabilitation unit.
Participants:
Pediatric patients (N=26; mean age, 10.79±5.17y) admitted to an inpatient rehabilitation unit with a confirmed diagnosis of NMDARE.
Interventions:
Inpatient rehabilitation; pharmacologic treatments.
Main Outcome Measure:
FIM for Children (WeeFIM) Developmental Functional Quotient (DFQ).
Results:
All patients received first-line immunotherapies to treat NMDARE, and 69% also received second-line immunotherapies. Patients were prescribed an average of 8 medications for symptom management (range, 3-15 per patient), most often for the treatment of agitation (100%), psychiatric symptoms (92%), and seizures (65%). Sixty-five percent of patients demonstrated an improvement in Total WeeFIM DFQ over the course of inpatient rehabilitation, with 35% demonstrating limited to no change in Total WeeFIM DFQ ("unfavorable early outcome"). Those with unfavorable early outcome were significantly younger than those showing more favorable outcome. Pharmacologic treatment for seizures, movement disorders, and decreased arousal or level of consciousness were each associated with unfavorable early outcome independent of age differences.
Conclusion:
Findings highlight the symptomatic heterogeneity and polypharmacy involved in the care and treatment of patients with NMDARE, with patients receiving a variety of immunotherapies and medications for symptom management. The presence of (and treatment for) seizures, movement disorders, and deteriorated neurologic status may each be associated with poor early outcomes in this population. Further investigation is needed to better classify presentations and treatments for this disease and to determine how differences are associated with long-term outcomes.
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