Anti-N-methyl-D-aspartate Receptor (NMDAR) Encephalitis: Neuronal Burden of a Comorbid Ovarian Teratoma.
- Author:
Jihye HWANG
1
;
Jung Hye BYEON
;
Gun Ha KIM
;
So Hee EUN
;
Baik Lin EUN
Author Information
1. Department of Pediatrics, College of Medicine, Korea University, Seoul, Korea. byeonagnes@naver.com
- Publication Type:Case Report
- Keywords:
Anti-N-Methyl-D-Aspartate Receptor Encephalitis;
Epilepsy;
Children;
Teratoma
- MeSH:
Anti-N-Methyl-D-Aspartate Receptor Encephalitis;
Antibodies;
Cerebrospinal Fluid;
Child;
Encephalitis*;
Epilepsy;
Female;
Fever;
Humans;
Hypoventilation;
Immunoglobulins, Intravenous;
Movement Disorders;
Neurologic Manifestations;
Neurons*;
Plasmapheresis;
Rituximab;
Seizures;
Sleep Initiation and Maintenance Disorders;
Teratoma*
- From:
Journal of the Korean Child Neurology Society
2017;25(1):62-65
- CountryRepublic of Korea
- Language:English
-
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.
