Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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A young child of anti-NMDA receptor encephalitis presenting with epilepsia partialis continua: the first pediatric case in Korea

  • Kim, Eun-Hee (Department of Pediatrics, CHA Gangnam Medical Center, CHA University) ;
  • Kim, Yeo Jin (Department of Pediatrics, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine) ;
  • Ko, Tae-Sung (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Yum, Mi-Sun (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Lee, Jun Hwa (Department of Pediatrics, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine)
  • 투고 : 2014.09.30
  • 심사 : 2015.01.23
  • 발행 : 2016.11.15
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초록

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.

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참고문헌

  1. Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 2008;7:1091-8. https://doi.org/10.1016/S1474-4422(08)70224-2
  2. Armangue T, Titulaer MJ, Malaga I, Bataller L, Gabilondo I, Graus F, et al. Pediatric anti-N-methyl-D-aspartate receptor encephalitis-clinical analysis and novel findings in a series of 20 patients. J Pediatr 2013;162:850-6.e2. https://doi.org/10.1016/j.jpeds.2012.10.011
  3. Florance NR, Davis RL, Lam C, Szperka C, Zhou L, Ahmad S, et al. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents. Ann Neurol 2009;66:11-8. https://doi.org/10.1002/ana.21756
  4. Titulaer MJ, McCracken L, Gabilondo I, Armangue T, Glaser C, Iizuka T, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol 2013;12:157-65. https://doi.org/10.1016/S1474-4422(12)70310-1
  5. Irani SR, Bera K, Waters P, Zuliani L, Maxwell S, Zandi MS, et al. N-methyl-D-aspartate antibody encephalitis: temporal progression of clinical and paraclinical observations in a predominantly non-paraneoplastic disorder of both sexes. Brain 2010;133(Pt 6):1655-67. https://doi.org/10.1093/brain/awq113
  6. Lim JA, Lee ST, Jung KH, Kim S, Shin JW, Moon J, et al. Anti-N-methyl-d-aspartate receptor encephalitis in Korea: clinical features, treatment, and outcome. J Clin Neurol 2014;10:157-61. https://doi.org/10.3988/jcn.2014.10.2.157
  7. Gable MS, Sheriff H, Dalmau J, Tilley DH, Glaser CA. The frequency of autoimmune N-methyl-D-aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the California Encephalitis Project. Clin Infect Dis 2012;54:899-904. https://doi.org/10.1093/cid/cir1038
  8. Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011;10:63-74. https://doi.org/10.1016/S1474-4422(10)70253-2
  9. Goldberg EM, Taub KS, Kessler SK, Abend NS. Anti-NMDA receptor encephalitis presenting with focal non-convulsive status epilepticus in a child. Neuropediatrics 2011;42:188-90. https://doi.org/10.1055/s-0031-1295408
  10. Finne Lenoir X, Sindic C, van Pesch V, El Sankari S, de Tourtchaninoff M, Denays R, et al. Anti-N-methyl-D-aspartate receptor encephalitis with favorable outcome despite prolonged status epilepticus. Neurocrit Care 2013;18:89-92. https://doi.org/10.1007/s12028-012-9788-8
  11. Barros P, Brito H, Ferreira PC, Ramalheira J, Lopes J, Rangel R, et al. Resective surgery in the treatment of super-refractory partial status epilepticus secondary to NMDAR antibody encephalitis. Eur J Paediatr Neurol 2014;18:449-52. https://doi.org/10.1016/j.ejpn.2014.01.013
  12. Florance-Ryan N, Dalmau J. Update on anti-N-methyl-D-aspartate receptor encephalitis in children and adolescents. Curr Opin Pediatr 2010;22:739-44. https://doi.org/10.1097/MOP.0b013e3283402d2f
  13. Zhang Q, Tanaka K, Sun P, Nakata M, Yamamoto R, Sakimura K, et al. Suppression of synaptic plasticity by cerebrospinal fluid from anti-NMDA receptor encephalitis patients. Neurobiol Dis 2012;45:610-5. https://doi.org/10.1016/j.nbd.2011.09.019
  14. Kashyape P, Taylor E, Ng J, Krishnakumar D, Kirkham F, Whitney A. Successful treatment of two paediatric cases of anti-NMDA receptor encephalitis with cyclophosphamide: the need for early aggressive immunotherapy in tumour negative paediatric patients. Eur J Paediatr Neurol 2012;16:74-8. https://doi.org/10.1016/j.ejpn.2011.07.005
  15. Dale RC, Brilot F, Duffy LV, Twilt M, Waldman AT, Narula S, et al. Utility and safety of rituximab in pediatric autoimmune and inflammatory CNS disease. Neurology 2014;83:142-50. https://doi.org/10.1212/WNL.0000000000000570