A case of psychosis with lymphocytic pleocytosis in CSF… Anti-NMDA Receptor Encephalitis!

Today we discussed a very interesting case of a young female who presented with 3 months of progressive behavioral disturbances, hallucinations, headache, insomnia, and a seizure. In young females with progressive symptoms and seizures, anti-NMDA receptor encephalitis is an important diagnostic consideration, although it is quite rare.

Illness Script for NMDA encephalitis:

  • occurs in young females with ovarian teratomas, but can be associated with other tumors as well and is sometimes seen in men and children
  • proposed association with a history of HSV encephalitis (in one study 20-30% of HSV encephalitis patients who didn’t have anti-NMDAR antibodies during their episode of encephalitis developed them later)
  • Clinical presentation: headache, fever, viral-like prodrome followed by multistage progressive symptoms:
    • psychiatric manifestations: anxiety, agitation, bizarre behavior, hallucinations, delusions, disorganized thinking
    • insomnia
    • memory deficits
    • language dysfunction: diminished language output, mutism, echolalia
    • seizures
    • decreased level of consciousness, stupor with catatonic features
    • dyskinesias: orofacial, choreoathetoid movements, dystonia, rigidity, opisthotonic postures
    • autonomic instability: hyperthermia, fluctuations of BP, tachycardia, bradycardia, cardiac pauses, hypoventilation
  • LP: can be initially negative or have lymphocytic pleocytosis or oligoclonal bands
  • EEG: Can have infrequent epileptiform activity, but frequently slow, disordganized activity that doesn’t correlate with patient’s abnormal movements
    • Unique pattern associated with prolonged illness: extreme delta brush
  • MRI: Usually normal, but can have transient FLAIR abnormalities
  • Diagnosis: CSF anti-GluN1 antibodies + clinical symptoms, abnormal EEG + oligoclonal bands, and reasonable exclusion of other disorders
  • Treatment:
    • Tumor resection when possible
    • Methylprednisolone 1g IV daily x5d
    • IVIG 400mg/kg IV daily x5d OR plasma exchange
    • If sypmtoms very severe, consider Rituximab

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