Inpatient Pathway for Evaluation and Treatment of Children
with Anti-NMDA Receptor Encephalitis (Anti-NMDARE)
Criteria for Suspected anti-NMDARE
- Rapid onset, < 3 mos, of at least 4 of the 6 following major groups of symptoms:
- Abnormal psychiatric behavior or cognitive dysfunction
- Speech dysfunction
- Seizures
- Movement disorder, dyskinesias, or rigidity/abnormal postures
- Decreased level of consciousness
- Autonomic dysfunction or central hypoventilation
- At least 1 of the following laboratory results:
- Abnormal EEG with focal or diffuse slow or disorganized activity, epileptic activity, or extreme delta brush
- CSF with pleocytosis or oligoclonal bands
- Diagnosis Confirmed
When anti-NMDA receptor antibodies are present
Consult Multidisciplinary Care Team
Children with anti-NMDARE present with a wide array of symptoms and severity and require the expertise of a multidisciplinary team to optimize treatment, symptom management, recovery strategies. This multidisciplinary team includes clinicians from the following areas:
- Neurology
- Physical Medicine and Rehabilitation
- Behavioral Health
- Speech Therapy
- Occupational Therapy
- Physical Therapy
- Neuropsychology
- Social Work
- Child Life
- Board Certified Behavioral Analyst
- History and Physical Exam
- Neurologic symptoms, infectious symptoms
- Neurologic exam
- Results of evaluation, treatment to date
- Differential Diagnosis for Autoimmune Encephalitis
Management
- First Line: Methylprednisolone
- Discuss subsequent therapies with Neuroimmunology team:
- Second Line: IVIG or plasma exchange
- Third Line: Rituximab
- Fourth Line: Cyclophosphamide
Screen for Malignancy
Testicular/ovarian ultrasound, MRI pelvis
Testicular/ovarian ultrasound, MRI pelvis
Posted: January 2023
Authors: S. Hopkins, MD; B. Banwell, MD; J. Mcguire, MD; M. Kirschen, MD; R. Prasad, MD; M. Mircetic, MD; L. Simon, MD; B. Blowey, PharmD; D. Deike, MD; A. Colin, CCC-SLP; C. Black PhD;
K. Young, CRNP; P. DiDonato, RN
Evidence
- Treatment and Prognostic Factors for Long-term Outcome in Patients with Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis: a Cohort Study
- Diagnosis and Significance of Antibody Titers in Anti-NMDA Receptor Encephalitis, a Retrospective Study
- A Clinical Approach to Diagnosis of Autoimmune Encephalitis
- An Update on Anti-NMDA Receptor Encephalitis for Neurologists and Psychiatrists: Mechanisms and Models
- Clinical Approach to the Diagnosis of Autoimmune Encephalitis in the Pediatric Patient
- International Consensus Recommendations for the Treatment of Pediatric NMDAR Antibody Encephalitis
- Neuroleptic Intolerance in Patients with Anti-NMDAR Encephalitis
- Autoimmune Psychosis: an International Consensus on an Approach to the Diagnosis and Management of Psychosis of Suspected Autoimmune Origin
- Agitation Management in Pediatric Males with Anti-N-Methyl-D-Aspartate Receptor Encephalitis
- Refining the Psychiatric Syndrome of Anti-N-Methyl-D-Aspartate Receptor Encephalitis
- Best Practices for Evaluation and Treatment of Agitated Children and Adolescents (BETA) in the Emergency Department: Consensus Statement of the American Association for Emergency Psychiatry