Diagnostic value of N-methyl-D-aspartate receptor antibodies in women with new-onset epilepsy.
BACKGROUND
In women younger than 45 years, a new form of encephalitis associated with ovarian teratoma and presenting with seizures and psychiatric symptoms has been described. Most patients have antibodies to NR1/NR2 heteromers of the N-methyl-D-aspartate receptor (NMDAR).


OBJECTIVE
To assess the frequency and significance of antibodies to NMDAR in otherwise unexplained new-onset epilepsies in young women.


METHODS
Prospective cohort study.


METHODS
University department of epileptology.


METHODS
From January 1, 2005, to June 30, 2007, we identified 19 female patients aged 15 to 45 years with unexplained new-onset epilepsy. In addition, we studied 61 cerebrospinal fluid-serum sample pairs from patients with other cryptogenic epilepsies and 11 cerebrospinal fluid-serum sample pairs from surgically treated patients with epilepsy with no evident encephalitic abnormalities.


METHODS
Antibodies to NMDAR and characteristics of affected patients.


RESULTS
Five of the 19 patients had antibodies against NMDAR. These patients had diffuse cerebral dysfunction and seizure origins. Psychiatric symptoms and pleocytosis were significantly associated with this group of patients. The disease course was episodic, in part relapsing-remitting, with full recoveries either spontaneously or after corticosteroid or intravenous immunoglobulin treatments. Only 1 patient had a neoplasm (multiple neuroendocrine tumors that included the ovaries) identified to date. In the control series, one 22-year-old man with a cryptogenic, severely encephalopathic seizure disorder was NMDAR antibody positive, and he also recovered fully.


CONCLUSIONS
Anti-NMDAR encephalitis accounts for a relevant proportion of otherwise unexplained new-onset epilepsies. Patients harboring NMDAR antibodies usually have prominent psychiatric symptoms and pleocytosis, and they may develop hypoventilation. Anti-NMDAR encephalitis is not always paraneoplastic.
Department of Epileptology, University of Bonn, Bonn, Germany.
Pitt
P
Niehusmann
Josep
J
Dalmau
Christian
C
Rudlowski
Angela
A
Vincent
Christian E
CE
Elger
Jeffrey E
JE
Rossi
Christian G
CG
Bien
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000818:Animals; D001323:Autoantibodies / Q000378:metabolism*; D016022:Case-Control Studies; D003937:Diagnosis, Differential; D004827:Epilepsy / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology / Q000601:surgery; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology; D006624:Hippocampus / Q000276:immunology / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D019581:Neuropil / Q000276:immunology / Q000473:pathology; D010257:Paraneoplastic Syndromes / Q000175:diagnosis / Q000276:immunology / Q000473:pathology; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology; D051381:Rats; D017208:Rats, Wistar; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology*; D012016:Reference Values
D001323:Autoantibodies; D024642:Potassium Channels, Voltage-Gated; D016194:Receptors, N-Methyl-D-Aspartate; D005968:Glutamate Decarboxylase