Abstract | OBJECTIVE: METHODS: Information was obtained by the authors or referring physicians. Antibodies were determined by immunocytochemistry and enzyme-linked immunosorbent assay (ELISA) using HEK293 cells ectopically expressing NR1. RESULTS: Over an 8-month period, 81 patients (12 male) with anti-NMDAR encephalitis were identified. Thirty-two (40%) were < or =18 years old (youngest 23 months, median 14 years); 6 were male. The frequency of ovarian teratomas was 56% in women >18 years old, 31% in girls < or =18 years old (p = 0.05), and 9% in girls < or =14 years old (p = 0.008). None of the male patients had tumors. Of 32 patients < or =18 years old, 87.5% presented with behavioral or personality change, sometimes associated with seizures and frequent sleep dysfunction; 9.5% with dyskinesias or dystonia; and 3% with speech reduction. On admission, 53% had severe speech deficits. Eventually, 77% developed seizures, 84% stereotyped movements, 86% autonomic instability, and 23% hypoventilation. Responses to immunotherapy were slow and variable. Overall, 74% had full or substantial recovery after immunotherapy or tumor removal. Neurological relapses occurred in 25%. At the last follow-up, full recovery occurred more frequently in patients who had a teratoma that was removed (5/8) than in those without a teratoma (4/23; p = 0.03). INTERPRETATION:
|
Authors | Nicole R Florance, Rebecca L Davis, Christopher Lam, Christina Szperka, Lei Zhou, Saba Ahmad, Cynthia J Campen, Heather Moss, Nadja Peter, Amy J Gleichman, Carol A Glaser, David R Lynch, Myrna R Rosenfeld, Josep Dalmau |
Journal | Annals of neurology
(Ann Neurol)
Vol. 66
Issue 1
Pg. 11-8
(Jul 2009)
ISSN: 1531-8249 [Electronic] United States |
PMID | 19670433
(Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
|
Chemical References |
- Antibodies
- NR1 NMDA receptor
- Receptors, N-Methyl-D-Aspartate
|
Topics |
- Adolescent
- Antibodies
(metabolism)
- Cell Line, Transformed
- Child
- Child, Preschool
- Encephalitis
(immunology, metabolism, physiopathology, therapy)
- Enzyme-Linked Immunosorbent Assay
(methods)
- Female
- Follow-Up Studies
- Humans
- Immunotherapy
(methods)
- Infant
- Male
- Postural Balance
(physiology)
- Receptors, N-Methyl-D-Aspartate
(genetics, immunology)
- Retrospective Studies
- Statistics, Nonparametric
- Transfection
(methods)
|