J Clin Neurol.  2014 Apr;10(2):157-161. 10.3988/jcn.2014.10.2.157.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis in Korea: Clinical Features, Treatment, and Outcome

• Lim JA
• Lee ST
• Jung KH
• Kim S
• Shin JW
• Moon J
• Byun JI
• Kim TJ
• Shin YW
• Lee KJ
• Kim YS
• Park KI
• Lee SK
• Chu K

• KMID: 2287534
• DOI: http://doi.org/10.3988/jcn.2014.10.2.157

Abstract

BACKGROUND AND PURPOSE
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common type of autoimmune synaptic encephalitis and it often responds to treatment. We analyzed the clinical characteristics of anti-NMDAR encephalitis in Korea.
METHODS
Serum and/or cerebrospinal fluid (CSF) of adult patients (aged > or =18 years) with encephalitis of undetermined cause were screened for anti-NMDAR antibodies using a cell-based indirect immunofluorescence assay. The patients came from 41 university hospitals.
RESULTS
Of the 721 patients screened, 40 were identified with anti-NMDAR antibodies and clinical details of 32 patients were obtained (median age, 41.5 years; 15 females). Twenty-two patients (68.8%) presented with psychiatric symptoms, 16 (50%) with seizures, 13 (40.6%) with movement disorders, 15 (46.9%) with dysautonomia, 11 (34.4%) with memory disturbance, and 11 (34.4%) with speech disturbance. Magnetic resonance imaging, electroencephalography, and CSF examinations yielded nonspecific findings. Tumor information was only available for 22 patients: 5 patients had tumors, and 2 of these patients had ovarian teratomas. Twenty-two patients received immunotherapy and/or surgery, and therapeutic responses were analyzed in 21 patients, of which 14 (66.7%) achieved favorable functional outcomes (score on the modified Rankin Scale of 0-2).
CONCLUSIONS
This study investigated the clinical characteristics of adult anti-NMDAR encephalitis in Korea. Currently, elderly patients who do not have tumors are commonly diagnosed with this condition. Understanding the detailed clinical characteristics of this disease will improve the early detection of anti-NMDAR encephalitis in patients both young and old.

Keyword

encephalitis; N-methyl-D-aspartate receptor antibody; autoimmune encephalitis

MeSH Terms

Adult
Aged
Anti-N-Methyl-D-Aspartate Receptor Encephalitis*
Antibodies
Cerebrospinal Fluid
Electroencephalography
Encephalitis
Fluorescent Antibody Technique, Indirect
Hospitals, University
Humans
Immunotherapy
Korea
Magnetic Resonance Imaging
Memory
Movement Disorders
Primary Dysautonomias
Seizures
Teratoma
Antibodies

Reference

1. Vitaliani R, Mason W, Ances B, Zwerdling T, Jiang Z, Dalmau J. Paraneoplastic encephalitis, psychiatric symptoms, and hypoventilation in ovarian teratoma. Ann Neurol. 2005; 58:594–604.

2. Dalmau J, Tüzün E, Wu HY, Masjuan J, Rossi JE, Voloschin A, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007; 61:25–36.

3. 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; 7:1091–1098.

4. Titulaer MJ, McCracken L, Gabilondo I, Armangué 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; 12:157–165.

5. 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.

6. Kayser MS, Titulaer MJ, Gresa-Arribas N, Dalmau J. Frequency and characteristics of isolated psychiatric episodes in anti-N-methyl-d-aspartate receptor encephalitis. JAMA Neurol. 2013; 70:1133–1139.

7. Quek AM, Britton JW, McKeon A, So E, Lennon VA, Shin C, et al. Autoimmune epilepsy: clinical characteristics and response to immunotherapy. Arch Neurol. 2012; 69:582–593.

8. Prüss H, Finke C, Höltje M, Hofmann J, Klingbeil C, Probst C, et al. N-methyl-D-aspartate receptor antibodies in herpes simplex encephalitis. Ann Neurol. 2012; 72:902–911.

9. Verhelst H, Verloo P, Dhondt K, De Paepe B, Menten B, Dalmau J, et al. Anti-NMDA-receptor encephalitis in a 3 year old patient with chromosome 6p21.32 microdeletion including the HLA cluster. Eur J Paediatr Neurol. 2011; 15:163–166.

10. Kang JH, Lee SH, Shin JW, Ahn MY, Baek SH, Lee HS, et al. Anti-NMDA receptor encephalitis. J Korean Neurol Assoc. 2011; 29:339–342.

11. Lee HS, Kim SW, Chung SJ, Yoo HS, Lee PH, Choi SA. Anti-NMDA receptor encephalitis which has shown clinical symptoms of schizophrenia. J Korean Neurol Assoc. 2013; 31:115–117.

12. 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):1655–1667.

13. Titulaer MJ, McCracken L, Gabilondo I, Iizuka T, Kawachi I, Bataller L, et al. Late-onset anti-NMDA receptor encephalitis. Neurology. 2013; 81:1058–1063.

14. Leypoldt F, Titulaer MJ, Aguilar E, Walther J, Bönstrup M, Havemeister S, et al. Herpes simplex virus-1 encephalitis can trigger anti-NMDA receptor encephalitis: case report. Neurology. 2013; 81:1637–1639.