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Anti-NMDAR Autoimmune Encephalitis

An autoimmune encephalitis caused by IgG antibodies against the NR1 subunit of the N-methyl-D-aspartate receptor, characterized by a multistage syndrome of psychiatric symptoms, seizures, abnormal movements, and dysautonomia, often associated with ovarian teratomas.

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.

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What is Anti-NMDAR Autoimmune Encephalitis?

Anti-NMDAR encephalitis is caused by IgG autoantibodies that bind to the N-terminal extracellular domain of the GluN1 (NR1) subunit of the NMDA receptor, leading to receptor internalization, glutamatergic dysfunction, and clinical syndrome dominated by limbic dysfunction.

The clinical course typically progresses through stages: prodromal viral-like illness, psychiatric symptoms (paranoia, hallucinations, behavioral change), neurologic deterioration with seizures and movement disorders (orofacial dyskinesia, dystonia), decreased consciousness, and autonomic instability with central hypoventilation.

Up to 50 percent of female patients aged 12 to 45 years have ovarian teratomas containing nervous tissue; tumor identification and removal is associated with better outcome, and immunotherapy with steroids, intravenous immunoglobulin, plasma exchange, and second-line agents (rituximab, cyclophosphamide) leads to substantial recovery in 75 to 80 percent of patients.

Symptoms

Subacute psychiatric symptoms: anxiety, depression, paranoia, hallucinations, mania, or atypical psychosis in a previously healthy patient
New-onset seizures, often focal evolving to generalized, and status epilepticus
Abnormal movements: orofacial dyskinesias, choreoathetosis, dystonia, opisthotonus
Speech dysfunction with mutism, perseveration, or echolalia
Decreased level of consciousness, catatonia, or coma in advanced disease
Autonomic instability: blood pressure fluctuations, tachycardia or bradycardia, hyperthermia, central hypoventilation

Risk Factors

Female sex (about 80 percent of cases) and age 12 to 45 years
Ovarian teratoma (present in up to 50 percent of female patients aged 12 to 45)
Recent herpes simplex virus encephalitis (post-herpetic anti-NMDAR encephalitis in 20 percent of survivors)
Children and adolescents have higher rates of seizures and movement disorders, lower rates of teratomas
Other autoimmune diseases or family history
Pregnancy and postpartum period

When to See a Doctor?

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

  • Subacute psychiatric symptoms with neurologic features (seizures, movement abnormalities, autonomic instability) — urgent neurology and psychiatry collaborative evaluation
  • First-onset psychosis in a young woman with associated neurologic findings — autoimmune encephalitis must be excluded
  • Acute encephalopathy following recent herpes simplex virus encephalitis
  • Refractory status epilepticus or super-refractory status — autoimmune workup including anti-NMDAR antibodies
  • Established anti-NMDAR encephalitis with relapse symptoms — restart immunotherapy and tumor screening

Treatment Methods

01
Diagnostic confirmation with serum and CSF anti-NMDAR antibodies (CSF more sensitive); CSF analysis showing pleocytosis, MRI features (limbic abnormalities or normal), and EEG findings (extreme delta brush)
02
Tumor screening: pelvic ultrasound, CT or MRI of chest/abdomen/pelvis, scrotal ultrasound in males; tumor removal when present
03
First-line immunotherapy: intravenous methylprednisolone 1 g daily for 5 days, intravenous immunoglobulin 2 g/kg over 5 days, or plasma exchange
04
Second-line immunotherapy in non-responders or severe disease: rituximab and/or cyclophosphamide
05
Long-term maintenance with rituximab or mycophenolate; psychiatric and neurorehabilitation support, monitoring for relapse (15 to 25 percent within 2 years), and management of seizures and movement disorders

Which Department to Visit?

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

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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.