Pediatric Anti-N-methyl-D-Aspartate Receptor Encephalitis—Clinical Analysis and Novel Findings in a Series of 20 Patients - 25/03/13
Spanish Anti-N-methyl-D-Aspartate Receptor (NMDAR) Encephalitis Work Group
Reprint requests: Josep Dalmau, MD, PhD, Service of Neurology, IDIBAPS-Hospital Clínic, University of Barcelona, Casanova, 143, Floor 5, Lab 503, Department 2, Barcelona 08036, Spain.Abstract |
Objective |
To report the clinical features of 20 pediatric patients with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.
Study design |
Review of clinical data, long-term follow-up, and immunologic studies performed in a single center in Spain in the last 4 years.
Results |
The median age of the patients was 13 years (range, 8 months-18 years), 70% were female. In 12 patients (60%), the initial symptoms were neurologic, usually dyskinesias or seizures, and in the other 40% psychiatric. One month into the disease, all patients had involuntary movements and alterations of behavior and speech. All patients received steroids, intravenous immunoglobulin or plasma exchange, and 7 rituximab or cyclophosphamide. With a median follow up of 17.5 months, 85% had substantial recovery, 10% moderate or severe deficits, and 1 died. Three patients had previous episodes compatible with anti-NMDAR encephalitis, 2 of them with additional relapses after the diagnosis of the disorder. Ovarian teratoma was identified in 2 patients, 1 at onset of encephalitis and the other 1 year later. Two novel observations (1 patient each) include, the identification of an electroencephalographic pattern (“extreme delta brush”) considered characteristic of this disorder, and the development of anti-NMDAR encephalitis as post herpes simplex encephalitis choreoathetosis.
Conclusions |
The initial symptoms of pediatric anti-NMDAR encephalitis vary from those of the adults (more neurologic and less psychiatric in children), the development of a mono-symptomatic illness is extremely rare (except in relapses), and most patients respond to treatment. Our study suggests a link between post herpes simplex encephalitis choreoathetosis and anti-NMDAR encephalitis.
Il testo completo di questo articolo è disponibile in PDF.Keyword : CSF, EEG, FLAIR, HSE, HSV, IgG, IVIG, MRI, NMDAR, PCPC, PCR
Mappa
| Supported by the National Institutes of Health (RO1NS077851 and RO1MH094741 to J.D.), the National Cancer Institute (RO1CA89054 to J.D.), Fundació la Marató de TV3 (to J.D.), Fondo de Investigaciones Sanitarias (PI11/01780 to J.D. and PS09/0193 to F.G.), a fellowship from the Dutch Cancer Society (KWF 2009-4451), and a McKnight Neuroscience of Brain Disorders award (to J.D.). J.D. has received a research grant from Euroimmun, and receives royalties from the Editorial Board of Up-To-Date and from patents for the use of Ma2 and NMDAR as autoantibody tests. The authors declare no conflicts of interest. |
Vol 162 - N° 4
P. 850 - Aprile 2013 Ritorno al numero
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