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Autoimmune Encephalitis in Children

Anti-NMDAR and Other Antibody-Mediated Brain Inflammation

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Çocuk Sağlığı ve Hastalıkları department. Book Appointment →

What is Autoimmune Encephalitis in Children?

Autoimmune encephalitis (AE) in children encompasses a group of inflammatory brain disorders mediated by autoantibodies targeting neuronal cell-surface or synaptic proteins, intracellular antigens, or by suspected immune mechanisms without identified antibody.

Anti-NMDA receptor encephalitis is the most common pediatric AE, with up to 40% of cases occurring in children and adolescents; teratoma association is less frequent than in adults.

Other antibody-mediated forms include anti-MOG associated disorder, anti-LGI1, anti-GABA-B receptor, anti-GAD65, anti-CASPR2, anti-glycine receptor, and acute disseminated encephalomyelitis (ADEM) with brain involvement.

Diagnosis requires combination of clinical features, CSF analysis (lymphocytic pleocytosis, oligoclonal bands), MRI findings (often subtle), EEG (extreme delta brush in anti-NMDAR), and antibody testing in CSF and serum.

Symptoms

Subacute (days to weeks) progression of psychiatric symptoms: behavioral change, irritability, mania, psychosis, hallucinations
Cognitive dysfunction: memory loss, language regression, executive dysfunction, school decline
Movement disorders: orofacial dyskinesias, choreoathetosis, dystonia, stereotypies (especially in anti-NMDAR)
Seizures: focal or generalized, often refractory to standard antiepileptics
Autonomic instability: hypertension or hypotension, hyperthermia, tachycardia or bradycardia, hypoventilation requiring ICU support
Decreased level of consciousness ranging from somnolence to coma
Sleep disturbances, mutism, catatonia, and abnormal eye movements

Risk Factors

Female sex (anti-NMDAR shows female predominance, especially in adolescents)
Underlying ovarian teratoma (less common in pediatric cases than adults, but warrants screening in postpubertal girls)
Recent infection (particularly HSV encephalitis can trigger anti-NMDAR encephalitis)
Personal or family history of autoimmunity
Genetic predisposition (specific HLA haplotypes in some forms)
Demyelinating disorders for anti-MOG associated disorder

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Subacute new-onset psychiatric or behavioral symptoms in a previously healthy child
  • New-onset refractory seizures, especially with movement disorders or autonomic features
  • Acute cognitive decline, language regression, or unexplained encephalopathy
  • Movement disorders (orofacial dyskinesias, choreoathetosis) with altered mental status
  • Acute or subacute encephalopathy following infection (post-HSV encephalitis especially)
  • Suspected ADEM with neurologic deterioration

Treatment Methods

01
Diagnostic workup: lumbar puncture with CSF cell count, protein, glucose, oligoclonal bands, autoantibody panel; brain MRI; EEG (continuous monitoring if seizures); abdominal/pelvic ultrasound or MRI for tumor screening in postpubertal patients
02
First-line immunotherapy: IV methylprednisolone 30 mg/kg/day (max 1 g) for 5 days followed by oral taper, intravenous immunoglobulin (IVIG) 2 g/kg over 2–5 days, or plasmapheresis 5–7 sessions
03
Second-line immunotherapy for refractory or severe cases: rituximab 375 mg/m² weekly for 4 doses or 750 mg/m² for 2 doses, cyclophosphamide pulse therapy
04
Maintenance immunotherapy in relapsing or severe cases: mycophenolate mofetil, azathioprine, or rituximab cycles every 6 months
05
Symptomatic management: antiepileptic drugs (levetiracetam, lacosamide preferred; benzodiazepines for status), management of dyskinesias (clonidine, trihexyphenidyl), autonomic and ICU support
06
Tumor removal when teratoma identified — often dramatically improves outcome, especially for anti-NMDAR encephalitis
07
Multidisciplinary rehabilitation: physical, occupational, speech therapy, neuropsychological assessment, educational support during recovery (often prolonged 6–24 months)
08
Long-term follow-up: monitor for relapse (15–25% recurrence), cognitive sequelae, mood and behavior; gradual return to school and activities; patient and family education and support

Which Department to Visit?

You can visit our Çocuk Sağlığı ve Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Çocuk Sağlığı ve Hastalıkları Department

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.