Korean J Pediatr.  2016 Nov;59(Suppl 1):S133-S138. 10.3345/kjp.2016.59.11.S133.

A young child of anti-NMDA receptor encephalitis presenting with epilepsia partialis continua: the first pediatric case in Korea

• Kim EH
• Kim YJ
• Ko TS
• Yum MS
• Lee JH

• KMID: 2362275
• DOI: http://doi.org/10.3345/kjp.2016.59.11.S133

Abstract

Anti-N-methyl D-aspartate receptor (anti-NMDAR) encephalitis, recently recognized as a form of paraneoplastic encephalitis, is characterized by a prodromal phase of unspecific illness with fever that resembles a viral disease. The prodromal phase is followed by seizures, disturbed consciousness, psychiatric features, prominent abnormal movements, and autonomic imbalance. Here, we report a case of anti-NMDAR encephalitis with initial symptoms of epilepsia partialis continua in the absence of tumor. Briefly, a 3-year-old girl was admitted to the hospital due to right-sided, complex partial seizures without preceding febrile illness. The seizures evolved into epilepsia partialis continua and were accompanied by epileptiform discharges from the left frontal area. Three weeks after admission, the patient's seizures were reduced with antiepileptic drugs; however, she developed sleep disturbances, cognitive decline, noticeable oro-lingual-facial dyskinesia, and choreoathetoid movements. Anti-NMDAR encephalitis was confirmed by positive detection of NMDAR antibodies in the patient's serum and cerebrospinal fluid, and her condition slowly improved with immunoglobulin, methylprednisolone, and rituximab. At present, the patient is no longer taking multiple antiepileptic or antihypertensive drugs. Moreover, the patient showed gradual improvement of motor and cognitive function. This case serves as an example that a diagnosis of anti-NMDAR encephalitis should be considered when children with uncontrolled seizures develop dyskinesias without evidence of malignant tumor. In these cases, aggressive immunotherapies are needed to improve the outcome of anti-NMDAR encephalitis.

Keyword

Anti-N-methyl-D-aspartate receptor encephalitis; Epilepsia partialis continua; Child

MeSH Terms

Anti-N-Methyl-D-Aspartate Receptor Encephalitis*
Antibodies
Anticonvulsants
Antihypertensive Agents
Cerebrospinal Fluid
Child*
Child, Preschool
Cognition
Consciousness
D-Aspartic Acid
Diagnosis
Dyskinesias
Encephalitis
Epilepsia Partialis Continua*
Female
Fever
Humans
Immunoglobulins
Immunotherapy
Korea*
Methylprednisolone
Rituximab
Seizures
Virus Diseases
Antibodies
Anticonvulsants
Antihypertensive Agents
D-Aspartic Acid
Immunoglobulins
Methylprednisolone
Rituximab