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Ann Child Neurol > Volume 25(1); 2017 > Article
Journal of the Korean Child Neurology Society 2017;25(1):62-65.
DOI: https://doi.org/10.26815/jkcns.2017.25.1.62    Published online March 30, 2017.
Anti-N-methyl-D-aspartate Receptor (NMDAR) Encephalitis: Neuronal Burden of a Comorbid Ovarian Teratoma.
Jihye Hwang, Jung Hye Byeon, Gun Ha Kim, So Hee Eun, Baik Lin Eun
Department of Pediatrics, College of Medicine, Korea University, Seoul, Korea. byeonagnes@naver.com
Abstract
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a disease that is characterized by acute psychiatric symptoms, seizures, and central hypoventilation. Patients with anti-NMDAR encephalitis exhibit speech alterations, insomnia, seizures, and movement disorders. We describe a previously healthy 6-year-old girl who presented with seizures, disorientation, and fever. Over the five weeks of treatment, the patient exhibited progressive neurologic symptoms, including a change in mental status. Her serum and cerebrospinal fluid contained high titers of antibodies against the NMDAR, and she was diagnosed with anti-NMDAR encephalitis. She was treated with plasmapheresis, steroid pulse therapy, intravenous immunoglobulins, and repeated doses of rituximab. After the patient was diagnosed with a concomitant ovarian teratoma, a unilateral salpingo-oophorectomy was performed. A histopathologic examination revealed that neuronal elements accounted for 60% of the resected ovarian teratoma. The patient's clinical symptoms and antibody titers improved after the surgical treatment and rituximab therapy. These observations suggested that patients with high titers of anti-NMDAR antibodies should be examined for the presence and quantity of neuronal components in concurrent ovarian teratomas.
Key Words: Anti-N-Methyl-D-Aspartate Receptor Encephalitis, Epilepsy, Children, Teratoma
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