Anti-NMDA receptor encephalitis due to large-cell neuroendocrine carcinoma of the uterus

Kobayashi, Manami; Nishioka, Kenya; Takanashi, Masashi; Hattori, Anri; Shojima, Yuri; Hayashida, Arisa; Sumii, Akiko; Ota, Tsuyoshi; Terao, Yasuhisa; Yokoyama, Kazumasa

doi:10.1016/j.jns.2017.10.024

Cited by 16 publications

(8 citation statements)

References 6 publications

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“…Tumors were detected in 26.7% of patients (8/30). The specific tumor types were as follows: ovarian teratoma (n=2), thymoma (n=2), brain tumor and nasopharyngeal cancer (n=1), nasopharyngeal cancer (n=1), testicular tumor (n=1), and uterus neuroendocrine tumor (n=1; previously reported by our group) (36). The prevalence of complicating tumors was significantly lower in comparison to previous reports (26.7%, 8/30 vs. 61.5%, 16/26; p=0.01).…”

Section: Resultsmentioning

confidence: 93%

Clinical Characterization of Definite Autoimmune Limbic Encephalitis: A 30-case Series

et al. 2019

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Objective Limbic encephalitis (LE) is an inflammatory condition of the limbic system that has an acute or subacute onset. Several types of antibodies are related to the onset of LE, including anti-N-methyl Daspartate receptor (NMDAR) antibodies and voltage-gated potassium channel (VGKC)-complex antibodies. However, the characteristics and prevalence of LE remain unclear, especially in Asian cohorts, due to the rarity. We aimed to survey their characteristics. Materials and Methods Data of 30 cases clinically defined as "definite autoimmune LE" (based on the standard criteria) were retrospectively collected. These patients were categorized into four subtypes: NMDAR (+) (n=8), VGKC (+) (n=2), antibodies related to paraneoplastic syndrome (n=2), and an antibody-negative group (uncategorized) (n=18). Results LE is rare in Japan, and affected only 30 of 16,759 hospital patients (0.2%) over a ten-year period. The NMDAR (+) group showed distinctive symptoms, while the other three groups had similar indications. Brain MRI indicated significant medial temporal lobe atrophy at one year follow up after discharge. The prevalence of cognitive dysfunction as a complication was 64% (9/14). First-line immunotherapy resulted in a good outcome. A drastic improvement was seen from 4.0±1.1 to 1.1+ on the modified Rankin Scale. A good treatment outcome was observed in all groups (NMDAR, VGKC, and uncategorized), suggesting the importance of an early clinical diagnosis and the early initiation of treatment. Furthermore, we reviewed 26 cases that were clinically diagnosed as definitive autoimmune LE in previous case reports. Conclusion Our findings show that the establishment of a clinical diagnosis based on the clinical criteria of definitive autoimmune LE is important for the initiation of immunotherapy.

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Section: Resultsmentioning

confidence: 93%

Clinical Characterization of Definite Autoimmune Limbic Encephalitis: A 30-case Series

et al. 2019

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“…1,5 Due to the frequent recovery of patients, neuropathological studies on NMDAR-encephalitis are rare and mainly based on isolated cases with overlapping pathologies or small biopsy specimens, lacking systematic assessment of immune cell infiltration and anatomic correlations. [6][7][8][9][10][11][12][13][14] This study aims to characterize the spectrum of inflammatory changes in different brain areas in patients who have or have not been treated with immunotherapy. Moreover, we describe the overlapping neuropathology of NMDAR-encephalitis with multiple sclerosis (MS)-type demyelination and post-transplant lymphoproliferative disease (PTLD).…”

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confidence: 99%

“…NMDAR‐encephalitis is characterized by prominent intrathecal production of pathogenic autoantibodies and the reversibility of neuronal dysfunction is reflected by good response to immunotherapy 1,5 . Due to the frequent recovery of patients, neuropathological studies on NMDAR‐encephalitis are rare and mainly based on isolated cases with overlapping pathologies or small biopsy specimens, lacking systematic assessment of immune cell infiltration and anatomic correlations 6–14 . This study aims to characterize the spectrum of inflammatory changes in different brain areas in patients who have or have not been treated with immunotherapy.…”

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confidence: 99%

Neuropathological Variability within a Spectrum of NMDAR‐Encephalitis

Zrzavy

Endmayr

Bauer

et al. 2021

Annals of Neurology

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Objective: To describe the neuropathological features of N-methyl-D-aspartate receptor (NMDAR)-encephalitis in an archival autopsy cohort. Methods: We examined four autopsies from patients with NMDAR-encephalitis; two patients were untreated, three had comorbidities: small cell lung cancer, brain post-transplant lymphoproliferative disease (PTLD), and overlapping demyelination. Results: The two untreated patients had inflammatory infiltrates predominantly composed of perivascular and parenchymal CD3 + /CD8 À T cells and CD79a + B cells/plasma cells in basal ganglia, amygdala, and hippocampus with surrounding white matter. The hippocampi showed a significant decrease of NMDAR-immunoreactivity that correlated with disease severity. The patient with NMDAR-encephalitis and immunosuppression for kidney transplantation developed a brain monomorphic PTLD. Inflammatory changes were compatible with NMDAR-encephalitis. Additionally, plasma cells accumulated in the vicinity of the necrotic tumor along with macrophages and activated microglia that strongly expressed pro-inflammatory activation markers HLA-DR, CD68, and IL18. The fourth patient developed demyelinating lesions in the setting of a relapse 4 years after NMDAR-encephalitis. These lesions exhibited the hallmarks of classic multiple sclerosis with radially expanding lesions and remyelinated shadow plaques without complement or immunoglobulin deposition, compatible with a pattern I demyelination. Interpretation: The topographic distribution of inflammation in patients with NMDAR-encephalitis reflects the clinical symptoms of movement disorders, abnormal behavior, and memory dysfunction with inflammation dominantly observed in basal ganglia, amygdala, and hippocampus, and loss of NMDAR-immunoreactivity correlates with disease severity. Co-occurring pathologies influence the spatial distribution, composition, and intensity of inflammation, which may modify patients' clinical presentation and outcome.

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“…Except for a patient with psychosis caused by anti-N-methyl-d-aspartate receptor encephalitis [20], none of them presented as paraneoplastic syndrome. This type of aggressive malignancy is often diagnosed at advanced stage, with more than 70% of patients suffering wide metastasis.…”

Section: Literature Reviewmentioning

confidence: 98%

Uterine Carcinoma With Complex Reasons for Hypercalcemia: a Case Report and Review of the Literature

Liu

et al. 2021

Preprint

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Introduction: Large-cell neuroendocrine carcinoma (LCNEC) is a seldom seen histological subtype of endometrial cancer with aggressive behavior and poor prognosis. Among current literatures, no one was found to be hormonally functional.Case presentation: We reported a rare case of endometrial LCNEC expressing both parathyroid hormone (PTH) and parathyroid hormone-related protein (PTH-rp). With ectopic PTH secreted into the blood stream, the hypercalcemia caused by malignant existence and osseous metastasis were concealed and misled initially. Literature review: Systematic literature search of previously reported uterine large cell neuroendocrine carcinomas and ectopic PTH-secreting neuroendocrine tumor (NET) cases were conducted in PubMed/MEDLINE databases respectively. We identified 55 cases of uterine LCNEC and 7 cases of PTH-secreting NET. Clinicopathologic characteristics, treatment and prognosis of all collected cases were summarized. Conclusion: Although quite rare, endometrial cancer can be functional and secret ectopic hormone, causing confusing clinical features. This case demonstrated the challenge in diagnosing malignancy-associated refractory hypercalcemia.

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Anti-NMDA receptor encephalitis due to large-cell neuroendocrine carcinoma of the uterus

Cited by 16 publications

References 6 publications

Clinical Characterization of Definite Autoimmune Limbic Encephalitis: A 30-case Series

Clinical Characterization of Definite Autoimmune Limbic Encephalitis: A 30-case Series

Neuropathological Variability within a Spectrum of NMDAR‐Encephalitis

Uterine Carcinoma With Complex Reasons for Hypercalcemia: a Case Report and Review of the Literature

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