Retrospective analysis of NMDA receptor antibodies in encephalitis of unknown origin.

Abstract

BACKGROUND:

Anti-NMDA-receptor (NMDAR) encephalitis is a severe disorder that occurs in association with antibodies to the NR1 subunit of the NMDAR and results in a characteristic syndrome.

OBJECTIVE:

To determine in a single institution setting whether patients previously diagnosed with encephalitis of unknown origin had anti-NMDAR encephalitis.

METHODS:

Charts of 505 patients aged 18 to 35 years admitted to the intensive care unit (ICU) during a 5-year period were retrospectively reviewed for criteria of encephalitis of unknown etiology. These included encephalitic signs with psychiatric symptoms (agitation, paranoid thoughts, irritability, or hallucinations); seizures; CSF inflammation; and exclusion of viral or bacterial infection. Archived serum and CSF samples of patients fulfilling these criteria were examined for NMDAR antibodies. Follow-up visits allowed the analysis of the natural disease course and estimation of prognosis.

RESULTS:

Seven patients (all women) fulfilled the indicated criteria; 6 of them had NMDAR antibodies. Ovarian teratomas were detected in 2 patients, in one 3 years after the onset of encephalitis. Outcome was favorable in all patients. One patient without teratoma improved spontaneously along with disappearance of NMDAR antibodies.

CONCLUSIONS:

Anti-NMDAR encephalitis represented 1% of all young patients' admissions to the ICU. Six of 7 cases with the indicated clinical criteria had anti-NMDAR encephalitis. NMDAR antibodies should be tested in all patients with encephalitis who fulfill these criteria.

• Authors: H Prüss, J Dalmau, L Harms, M Höltje, G Ahnert-Hilger, K Borowski, W Stoecker, K P Wandinger
• Journal: Neurology (Neurology) Vol. 75 Issue 19 Pg. 1735-9 (Nov 09 2010) ISSN: 1526-632X [Electronic] United States
• PMID: 21060097 (Publication Type: Comparative Study, Journal Article)

Chemical References

• Autoantibodies
• NR1 NMDA receptor
• NR2A NMDA receptor
• Receptors, N-Methyl-D-Aspartate

Topics

• Adolescent
• Adult
• Autoantibodies (biosynthesis, blood)
• Cell Line
• Encephalitis (diagnosis, immunology, metabolism)
• Female
• Follow-Up Studies
• Humans
• Receptors, N-Methyl-D-Aspartate (immunology)
• Retrospective Studies
• Young Adult