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Anti-NMDAR Encephalitis in a 13-Year-Old Female: A 24-Month Clinical Follow-Up.
J Epilepsy Res. 2018 Jun; 8(1):41-48.JE

Abstract

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a rare autoimmune disorder manifesting as seizures, movement disorders, and psychiatric changes. However, there have been few case reports concerning this disorder in South Korean children. The current case report describes a pediatric patient with anti-NMDAR encephalitis. A 13-year-old female patient developed clonic movements of the right arm followed by aphasia, paresthesia, and right-sided hemiparesis. The electroencephalogram (EEG) results indicated electroclinical seizures arising from the left temporal area. Brain magnetic resonance imaging (MRI) revealed high signal intensity and cortical swelling in left temporal lobe. Anti-NMDAR antibodies were detected in the cerebrospinal fluid (CSF). The patient was treated with intravenous immunoglobulin and high-dose methylprednisolone and showed partial improvement in language skills, paresthesia, and motor power. The brain MRI and EEG results also indicated improvement. However, anti-NMDAR antibodies persisted in the CSF. After four doses of rituximab, the patient exhibited complete recovery of language and motor skills, and was seizure free under treatment with antiepileptic medication. There were no residual anti-NMDAR antibodies in the CSF at her 24-month follow-up visit. This case report elucidates the benefits of early intervention using rituximab to improve neurological deficits and achieve baseline recovery in patients with anti-NMDAR encephalitis.

Authors+Show Affiliations

Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.Department of Pediatrics, Uijeongbu St. Mary's Hospital, The Catholic University School of Medicine, Seoul, Korea.Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Pub Type(s)

Case Reports

Language

eng

PubMed ID

30090761

Citation

Kim, Eunsil, et al. "Anti-NMDAR Encephalitis in a 13-Year-Old Female: a 24-Month Clinical Follow-Up." Journal of Epilepsy Research, vol. 8, no. 1, 2018, pp. 41-48.
Kim E, Park EG, Lee J, et al. Anti-NMDAR Encephalitis in a 13-Year-Old Female: A 24-Month Clinical Follow-Up. J Epilepsy Res. 2018;8(1):41-48.
Kim, E., Park, E. G., Lee, J., Lee, M., Kim, J., & Lee, J. (2018). Anti-NMDAR Encephalitis in a 13-Year-Old Female: A 24-Month Clinical Follow-Up. Journal of Epilepsy Research, 8(1), 41-48. https://doi.org/10.14581/jer.18007
Kim E, et al. Anti-NMDAR Encephalitis in a 13-Year-Old Female: a 24-Month Clinical Follow-Up. J Epilepsy Res. 2018;8(1):41-48. PubMed PMID: 30090761.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Anti-NMDAR Encephalitis in a 13-Year-Old Female: A 24-Month Clinical Follow-Up. AU - Kim,Eunsil, AU - Park,Eu Gene, AU - Lee,Jiwon, AU - Lee,Munhyang, AU - Kim,Jihye, AU - Lee,Jeehun, Y1 - 2018/06/30/ PY - 2018/04/13/received PY - 2018/06/25/accepted PY - 2018/8/10/entrez PY - 2018/8/10/pubmed PY - 2018/8/10/medline KW - Autoimmune KW - Child KW - Encephalitis KW - NMDA receptor antibody SP - 41 EP - 48 JF - Journal of epilepsy research JO - J Epilepsy Res VL - 8 IS - 1 N2 - Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a rare autoimmune disorder manifesting as seizures, movement disorders, and psychiatric changes. However, there have been few case reports concerning this disorder in South Korean children. The current case report describes a pediatric patient with anti-NMDAR encephalitis. A 13-year-old female patient developed clonic movements of the right arm followed by aphasia, paresthesia, and right-sided hemiparesis. The electroencephalogram (EEG) results indicated electroclinical seizures arising from the left temporal area. Brain magnetic resonance imaging (MRI) revealed high signal intensity and cortical swelling in left temporal lobe. Anti-NMDAR antibodies were detected in the cerebrospinal fluid (CSF). The patient was treated with intravenous immunoglobulin and high-dose methylprednisolone and showed partial improvement in language skills, paresthesia, and motor power. The brain MRI and EEG results also indicated improvement. However, anti-NMDAR antibodies persisted in the CSF. After four doses of rituximab, the patient exhibited complete recovery of language and motor skills, and was seizure free under treatment with antiepileptic medication. There were no residual anti-NMDAR antibodies in the CSF at her 24-month follow-up visit. This case report elucidates the benefits of early intervention using rituximab to improve neurological deficits and achieve baseline recovery in patients with anti-NMDAR encephalitis. SN - 2233-6249 UR - https://www.unboundmedicine.com/medline/citation/30090761/Anti_NMDAR_Encephalitis_in_a_13_Year_Old_Female:_A_24_Month_Clinical_Follow_Up_ L2 - http://www.j-epilepsy.org/journal/view.php?year=2018&vol=8&page=41 DB - PRIME DP - Unbound Medicine ER -
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