The DOI matches its Crossref record; 18 of the 18 references it cites were found in a registry or an index.
Anti-NMDA receptor encephalitis in Japan: Long-term outcome without tumor removal
Open accessAbstract
OBJECTIVE: To report the definitive diagnosis of anti-NMDA receptor (NMDAR) encephalitis in four Japanese women previously diagnosed with "juvenile acute nonherpetic encephalitis" of unclear etiology, and to describe their long-term follow-up in the absence of tumor resection. METHODS: We extensively reviewed the case histories with current clinical and laboratory evaluations that include testing for antibodies to NR1/NR2 heteromers of the NMDAR in serum/CSF available from the time of symptom onset (4 to 7 years ago) and the present. RESULTS: All patients sequentially developed prodromal symptoms, psychosis, hypoventilation, severe orofacial dyskinesias, and bizarre immunotherapy-resistant involuntary movements that lasted 1 to 12 months. Two patients required mechanical ventilation for 6 and 9 months. Initial tests were normal or unrevealing, including the presence of nonspecific CSF pleocytosis, and normal or mild changes in brain MRI. Eventually, all patients had dramatic recovery of cognitive functions, although one had bilateral leg amputation due to systemic complications. Antibodies to NR1/NR2 heteromers were found in archived serum or CSF but not in long-term follow-up samples. An ovarian teratoma was subsequently demonstrated in three patients (all confirmed pathologically). CONCLUSION: 1) These findings indicate that "juvenile acute nonherpetic encephalitis" or a subset of this disorder is mediated by an antibody-associated immune response against NR1/NR2 heteromers of the NMDA receptor (NMDAR). 2) Our patients' clinical features emphasize that anti-NMDAR encephalitis is severe but potentially reversible and may precede by years the detection of an ovarian teratoma. 3) Although recovery may occur without tumor removal, the severity and extended duration of symptoms support tumor removal.
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Citations
Cites
Paraneoplastic anti– N ‐methyl‐ D ‐aspartate receptor encephalitis associated with ovarian teratoma
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Self-citationTargeted disruption of NMDA receptor 1 gene abolishes NMDA response and results in neonatal death
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Cited by
Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies
Dalmau, J., Gleichman, A. J., Hughes, E. G., Rossi, J. E., Peng, X., Lai, M., Dessain, S. K., Rosenfeld, M. R., Balice-Gordon, R., & Lynch, D. R. (2008) · The Lancet Neurology · doi:10.1016/s1474-4422(08)70224-2 · Cited by 3,488
Self-citationGresa-Arribas, N., Titulaer, M. J., Torrents, A., Aguilar, E., McCracken, L., Leypoldt, F., Gleichman, A. J., Balice-Gordon, R., Rosenfeld, M. R., Lynch, D., et al. (2014) · The Lancet Neurology · doi:10.1016/s1474-4422(13)70282-5 · Cited by 1,306
Self-citationCellular and synaptic mechanisms of anti-NMDA receptor encephalitis
Hughes, E. G., Peng, X., Gleichman, A. J., Lai, M., Zhou, L., Tsou, R., Parsons, T. D., Lynch, D. R., Dalmau, J., & Balice-Gordon, R. J. (2010) · The Journal of Neuroscience · doi:10.1523/jneurosci.0167-10.2010 · Cited by 1,143
Self-citationAnti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents
Florance, N. R., Davis, R. L., Lam, C., Szperka, C., Zhou, L., Ahmad, S., Campen, C. J., Moss, H., Peter, N., Gleichman, A. J., et al. (2009) · Annals of Neurology · doi:10.1002/ana.21756 · Cited by 1,090
Self-citationIrani, S. R., Bera, K., Waters, P., Zuliani, L., Maxwell, S., Zandi, M. S., Friese, M. A., Galea, I., Kullmann, D. M., Beeson, D., et al. (2010) · Brain · doi:10.1093/brain/awq113 · Cited by 1,035
Metadata
- Authors
- Iizuka, T., Sakai, F., Ide, T., Monzen, T., Yoshii, S., Iigaya, M., Suzuki, K., Lynch, D. R., Suzuki, N., Hata, T., & Dalmau, J.
- Venue
- Neurology
- Published
- 2008-02-12
- Volume
- 70
- Issue
- 7
- Pages
- 504-511
- ISSN
- 0028-3878 (ISSN-L)
- 1526-632X
- OpenAlex
- W2133423973
- Cited by
- 517
- References
- 18
- FWCI
- 17.20
- Type
- journal article
- Language
- en
- Open access
- green
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- Iizuka, T., Sakai, F., Ide, T., Monzen, T., Yoshii, S., Iigaya, M., Suzuki, K., Lynch, D. R., Suzuki, N., Hata, T., & Dalmau, J. (2008). Anti-NMDA receptor encephalitis in Japan: Long-term outcome without tumor removal. Neurology. https://doi.org/10.1212/01.wnl.0000278388.90370.c3
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