Does MAP2 have a Role in Predicting the Development of Anti-NMDAR Encephalitis Associated with Benign Ovarian Teratoma? A Report of Six New Pediatric Cases

• Published in: Pediatric and developmental pathology Vol. 18; no. 2; pp. 122 - 126
• Main Authors: Cundiff, Caitlin A., Elawabdeh, Nancy, Naguib, Mina M., Jactel, Samuel N., El Demellawy, Dina, Abramowsky, Carlos R., Durham, Megan M., Youssef, Lara, Wittkamp, Michael L., Shehata, Bahig M.
• Format: Journal Article
• Language: English
• Published: Los Angeles, CA SAGE Publications 01-03-2015; SAGE PUBLICATIONS, INC
• Subjects: Adolescent; Anti-N-Methyl-D-Aspartate Receptor Encephalitis - blood; Anti-N-Methyl-D-Aspartate Receptor Encephalitis - cerebrospinal fluid; Anti-N-Methyl-D-Aspartate Receptor Encephalitis - immunology; Anti-N-Methyl-D-Aspartate Receptor Encephalitis - pathology; Anti-N-Methyl-D-Aspartate Receptor Encephalitis - therapy; Autoantibodies - blood; Autoantibodies - cerebrospinal fluid; Biomarkers, Tumor - analysis; Case-Control Studies; Child; Child, Preschool; Female; Humans; Immunohistochemistry; Microtubule-Associated Proteins - analysis; Ovarian Neoplasms - chemistry; Ovarian Neoplasms - complications; Ovarian Neoplasms - pathology; Ovarian Neoplasms - therapy; Predictive Value of Tests; Prognosis; Receptors, N-Methyl-D-Aspartate - immunology; Risk Factors; Teratoma - chemistry; Teratoma - complications; Teratoma - pathology; Teratoma - therapy; anti-NMDAR; ovarian teratoma; neuronal markers; NeuN; encephalitis; MAP2
• ISSN: 1093-5266; 1615-5742
• DOI: 10.2350/14-09-1554-OA.1

Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is a potentially fatal neurologic syndrome in which patients present with a spectrum of central nervous system deficits. Sixty percent of the cases can be attributed to the presence of tumors, most often ovarian teratomas. This report examines 6 pediatric patients who presented with neurologic deficits associated with the presence of such tumors. These cases illustrate a perplexing phenomenon, where benign teratomas could have a possible association with anti-NMDAR encephalitis. The purpose of this study was to compare the histology and immunohistochemistry of tumors associated with this syndrome to ovarian teratomas found in patients presenting with no neurologic symptoms. After obtaining institutional review board approval, 57 cases of ovarian teratomas were identified at our institution over 12 years. Six patients were identified with anti-NMDAR encephalitis. A panel of immunostains, including S100, GFAP, MAP2, and NeuN was applied to patients' tumor sections as well as the 6 controls from age-matched patients. No qualitative histologic or immunohistochemical differences were seen between the study cases and control group. Because no qualitative differences were identified between the study cases and the control group, testing of paired serum and cerebrospinal fluid remains the best method for diagnosis of anti-NMDAR encephalitis. Tumor banking with molecular analysis of ovarian teratomas, including whole-genome sequencing and comparative genomic hybridization between ovarian tissue saved from patients with and without anti-NMDAR encephalitis, is necessary to fully understand the etiopathogenesis of anti-NMDAR encephalitis.