Original ArticlePediatric Anti-N-methyl-D-Aspartate Receptor Encephalitis—Clinical Analysis and Novel Findings in a Series of 20 Patients
Section snippets
Methods
From January 2008 until February 2012, we identified 61 patients (median age 22 years; range 8 months-76 years) with anti-NMDAR encephalitis whose serum and CSF were referred for antibody testing to Hospital Clinic, University of Barcelona. All patients were suspected of having autoimmune encephalitis after being extensively studied by their physicians. Twenty patients (33%) were younger than 19 years and are the focus of this study. Analysis of serum and CSF for NMDAR antibodies was performed
Results
The median age of the patients was 13 years (8 months-18 years); 14 were Caucasian, 5 Hispanic, and 1 Asian. NMDAR antibodies were identified in the CSF of all patients and serum of 9; the serum of 2 patients was negative and was not available from the other 9. Seventy percent of the patients were female. The ratio female/male varied according to age, so that 33% of patients younger than 12 years and all above this age were female.
Discussion
Our study suggests that the presentation of anti-NMDAR encephalitis in children can be different from that reported in adults. Although 60% of children presented with seizures, abnormal movements, and focal neurologic deficits, previous experience with large series of predominantly young adults demonstrated that 70% presented with psychosis and other psychiatric symptoms.14 In the current study, patients older than age 18 years were excluded, but a similar trend was noted comparing the symptom
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2022, Rheumatic Disease Clinics of North AmericaCitation Excerpt :AE also must be included, however, on the differential diagnosis for ABD presenting with acute focal neurologic deficits, especially early in the disease course. Children may present with 1 prominent symptom, such as dyskinesias or choreoathetoid movements, focal loss of language, or psychosis.9,16,18,19 Although typically AE progresses to additional symptoms spanning multiple domains over the first few weeks to months, if there is prompt diagnosis and early effective treatment, it is possible for the disease to remain isolated to 1 domain.
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.
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A list of members of the Spanish Anti-NMDAR Encephalitis Work Group is available at www.jpeds.com (Appendix 1).