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Anti-NMDA receptor encephalitis in Japan: long-term outcome without tumor removal.
Neurology. 2008 Feb 12; 70(7):504-11.Neur

Abstract

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.

Authors+Show Affiliations

Department of Neurology, School of Medicine, Kitasato University, 1-15-1 Kitasato, Sagamihara, Kanagawa, 228-8555, Japan. takahiro@med.kitasato-u.ac.jpNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, N.I.H., Extramural

Language

eng

PubMed ID

17898324

Citation

Iizuka, T, et al. "Anti-NMDA Receptor Encephalitis in Japan: Long-term Outcome Without Tumor Removal." Neurology, vol. 70, no. 7, 2008, pp. 504-11.
Iizuka T, Sakai F, Ide T, et al. Anti-NMDA receptor encephalitis in Japan: long-term outcome without tumor removal. Neurology. 2008;70(7):504-11.
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, 70(7), 504-11.
Iizuka T, et al. Anti-NMDA Receptor Encephalitis in Japan: Long-term Outcome Without Tumor Removal. Neurology. 2008 Feb 12;70(7):504-11. PubMed PMID: 17898324.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Anti-NMDA receptor encephalitis in Japan: long-term outcome without tumor removal. AU - Iizuka,T, AU - Sakai,F, AU - Ide,T, AU - Monzen,T, AU - Yoshii,S, AU - Iigaya,M, AU - Suzuki,K, AU - Lynch,D R, AU - Suzuki,N, AU - Hata,T, AU - Dalmau,J, Y1 - 2007/09/26/ PY - 2007/9/28/pubmed PY - 2008/3/7/medline PY - 2007/9/28/entrez SP - 504 EP - 11 JF - Neurology JO - Neurology VL - 70 IS - 7 N2 - 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. SN - 1526-632X UR - https://www.unboundmedicine.com/medline/citation/17898324/Anti_NMDA_receptor_encephalitis_in_Japan:_long_term_outcome_without_tumor_removal_ L2 - http://www.neurology.org/cgi/pmidlookup?view=long&pmid=17898324 DB - PRIME DP - Unbound Medicine ER -