J Korean Child Neurol Soc.  2017 Mar;25(1):62-65. 10.0000/jkcns.2017.25.1.62.

Anti-N-methyl-D-aspartate Receptor (NMDAR) Encephalitis: Neuronal Burden of a Comorbid Ovarian Teratoma

• Hwang J
• Byeon JH
• Kim GH
• Eun SH
• Eun BL

• KMID: 2385217
• DOI: http://doi.org/10.0000/jkcns.2017.25.1.62

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.

Keyword

Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Epilepsy; Children; Teratoma

MeSH Terms

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*
Antibodies
Immunoglobulins, Intravenous
Rituximab