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A Simple Cell-based Immunofluorescence Assay to Detect Autoantibody Against the N-Methyl-D-Aspartate (NMDA) Receptor in Blood
Published on: January 9, 2018
[Associated tumors in patients with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis]
Makoto Hara1, Akihiko Morita, Satoshi Kamei
1Division of Neurology, Department of Medicine, Nihon University School of Medicine.
Abstract:
In 2007, Dalmau and colleagues described anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis associated with ovarian teratoma. As the numbers of patients with anti-NMDAR encephalitis increased, the frequency of paraneoplastic findings declined. The frequency of anti-NMDAR encephalitis with the tumor declined to 60% of a total of 100 patients in 2008, and 42% of a total of 400 patients in 2011. This 42% was the similar value as the tumor frequency in Acute Juvenile Female Non-Herpetic Encephalitis in Japan. It was also revealed that the discrepancies in tumor frequency of anti-NMDAR encephalitis between investigations existed. The results of stratification analyses of anti-NMDAR encephalitis revealed that patients who were younger than 18 years old and male patients with anti-NMDAR encephalitis were less likely to have associated tumors. Dalmau also reported tumors other than ovarian tumors were associated with 2% (9/400) of patients in 2011. These patients included breast cancer, neuroendocrine tumors, pancreatic carcinoma, sex cord stromal tumors, testicular germ-cell tumors and small-cell lung carcinoma. We encountered a 65-year-old female affected by anti-NMDAR encephalitis with carcinosarcoma with neuroendocrine differentiation of the uterus. The prognosis of anti-NMDAR encephalitis with malignant tumor could be dependent on the prognosis of the associated tumor.
Insights
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is increasingly diagnosed, with a declining frequency of associated tumors over time. However, tumor prevalence varies, and certain patient groups are less likely to have tumors.
Area of Science:
- Neurology
- Immunology
- Oncology
Background:
- Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis was first described in 2007, often associated with ovarian teratomas.
- The reported frequency of associated tumors in NMDAR encephalitis has decreased over time, from 60% in 2008 to 42% in 2011.
- Discrepancies in tumor frequency across studies suggest variability in patient populations and diagnostic criteria.
Purpose of the Study:
- To analyze the changing frequency of paraneoplastic findings in anti-NMDAR encephalitis.
- To investigate factors influencing the likelihood of tumor association in NMDAR encephalitis patients.
- To report on non-ovarian tumors associated with NMDAR encephalitis and present a unique case.
Main Methods:
- Review of published data on anti-NMDAR encephalitis and associated tumors.
- Stratification analysis based on patient demographics (age, sex).
- Case report of a patient with uterine carcinosarcoma and NMDAR encephalitis.
Main Results:
- The frequency of tumor association in NMDAR encephalitis decreased from 60% (100 patients, 2008) to 42% (400 patients, 2011).
- Younger patients (<18 years) and male patients were less likely to have associated tumors.
- Non-ovarian tumors (e.g., breast cancer, small-cell lung carcinoma) were found in 2% of patients; a uterine carcinosarcoma case was identified.
Conclusions:
- The prevalence of tumors in anti-NMDAR encephalitis has declined, though variations exist.
- Patient age and sex are significant factors in the likelihood of tumor association.
- The prognosis of NMDAR encephalitis may depend on the underlying malignancy.
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