Delayed diagnosis of extraovarian teratoma in relapsing anti-NMDA receptor encephalitis

Kümpfel, Tania; Gerdes, Lisa Ann; Heck, Clarissa; Prüß, Harald

doi:10.1212/nxi.0000000000000250

Cited by 8 publications

(6 citation statements)

References 7 publications

(12 reference statements)

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“… 3 5 The largest cohort study of patients with anti-NMDAR encephalitis (n = 577) reported that associated tumors were found in 38% of overall patients and 46% of female patients; among them 94% had ovarian teratomas, 2% had extraovarian teratomas and 4% had other tumors types. 5 A few anti-NMDAR encephalitis cases with extraovarian teratomas have been reported in the anterior mediastinum 6 8 9 10 11 and thyroid, 12 suggesting that rapid teratoma detection is key because surgical removal of the primary tumor along with prompt initiation of immunotherapy can achieve improvement and prevent relapse.…”

Section: Discussionmentioning

confidence: 99%

Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis Associated With Mediastinal and Ovarian Teratomas: A Case Report

Lee

Park

et al. 2023

J Korean Med Sci

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Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most common type of autoimmune encephalitis. Approximately 80% of patients with anti-NMDAR encephalitis are women. Tumors are detected in approximately 50% of female patients with anti-NMDAR encephalitis, of which 96% are ovarian teratomas. We describe the case of a 28-year-old woman diagnosed with anti-NMDAR encephalitis with mediastinal and bilateral ovarian teratomas in July 2019. The patient recovered following surgical management of the mediastinal mass and both ovarian teratomas, and immunotherapy. This case shows that teratomas can be found at multiple sites other than ovaries. Therefore, detecting teratomas using whole-body evaluation may be helpful for diagnosis and treatment.

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Section: Discussionmentioning

confidence: 99%

Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis Associated With Mediastinal and Ovarian Teratomas: A Case Report

Lee

Park

et al. 2023

J Korean Med Sci

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“…Anti-NMDAR encephalitis triggered by extra-ovarian teratoma, especially mediastinum teratoma, was detected in 2/21 patients with teratoma in our cohort. Overlooked extra-ovarian teratoma may result in delayed diagnosis in some cases ( 14 ). In addition, the teratoma cohort presented with more severe neurological sequelae, with greater disturbance of consciousness and central hypoventilation, and higher anti-NMDAR antibody titer in the CSF, which is similar to the report by Gresa-Arribas et al ( 15 ).…”

Section: Discussionmentioning

confidence: 99%

Long-Term Prognosis of Patients With Anti-N-Methyl-D-Aspartate Receptor Encephalitis Who Underwent Teratoma Removal: An Observational Study

et al. 2022

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ObjectiveThis study aimed to evaluate the clinical characteristics and long-term surgical outcomes of patients with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis with teratoma.MethodsPatients who were admitted to West China Hospital from June 2012 to June 2019 and diagnosed with anti-NMDAR encephalitis were enrolled in the study. Medical records were reviewed prospectively to gather clinical characteristic data. Patients were followed up at long-term every 3 months.ResultsThis study included 192 patients, among whom 21 (10.9%) were detected with having a teratoma. Patients included 20 women, with a mean age of 24.62 ± 7.61 years. Seizure and psychiatric symptoms were the most dominant symptoms in both groups, followed by memory deficits. Central hypoventilation (52.4 vs. 17%, p < 0.001) and decreased consciousness (71.4 vs. 31.3%, p = 0.002) were significantly more frequent in patients with teratoma than in those without. Moreover, the anti-NMDAR antibody titer was higher (p = 0.021) and the baseline modified Rankin scale score was lower (p = 0.004) in patients with teratoma than in those without. First-line immunotherapy was performed in 21 (100%) patients with teratoma and 167 (97.7%) patients without teratoma. All patients with teratoma had the tumor removed. During follow-up, two (9.5%) patients with teratoma and 11 (6.4%) patients without teratoma died, whereas 1 (4.8%) patient with teratoma and 37 (21.6%) patients without teratoma had relapses. Overall, 19 (90.5%) patients with teratoma and 151 (88.3%) patients without teratoma achieved favorable clinical outcomes at the final follow-up.ConclusionsWith early detection and removal of teratoma, most patients with anti-NMDAR encephalitis and teratoma achieved a favorable long-term prognosis.

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“…Changes in the antibody levels in the CSF and serum have implications for relapse [ 9 , 16 ]. Teratomas that contain neural tissue could trigger an immune response resulting in the overproduction of anti-NMDAR antibodies [ 17 ], and when these titers are persistent in patients with a prolonged relapsing disease course, the presence of tumors should be considered [ 18 ]. Thus, periodic screening of serum and CSF is useful to assess both therapeutic effects and possible relapse.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics and long-term prognosis of relapsing anti-N-methyl-d-aspartate receptor encephalitis: a retrospective, multicenter, self-controlled study

Zeng

Zheng

2020

Neurol Sci

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Objective To analyze the clinical profile and long-term prognosis of relapsing anti- N -methyl- d -aspartate receptor (NMDAR) encephalitis. Method This is a retrospective, multicenter, self-controlled study of 10 patients with relapsing anti-NMDAR encephalitis. Relapse was defined as new psychiatric or neurologic syndrome unexplainable by other causes that improved after immunotherapy. Results The main symptoms at first onset and relapse included psychiatric symptoms, cognitive impairment, speech dysfunction, seizures, consciousness disturbance, movement disorders, central hypoventilation, and autonomic dysfunction. There were significantly fewer seizures and consciousness disturbances at relapse. At the first onset, the antibody positivity rate was significantly higher in the cerebrospinal fluid (CSF) than in the serum, and abnormal electroencephalograms results were noted in all patients. The relapse rate was 12.2%. After first-onset discharge, the duration of medication intake was 3.10 ± 2.69 months; the relapse time was 18.3 ± 16.5 months. The Modified Rankin Scale (MRS) score at relapse was significantly lower than that at the first onset. The MRS scores at relapse and first onset after immunotherapy were significantly lower than those before immunotherapy. At follow-up, the average duration of antiepileptic drug (AED) intake was < 1 year; the relapse rate was low. Conclusions Patients have fewer symptoms and better quality of life at relapse than at the first onset. Active immunotherapy can significantly improve the quality of life during first onset and relapse. Encephalitis antibody testing in the CSF is preferred at first onset and relapse. Increasing antibody titers suggest clinical relapse. Prematurely stopping immunotherapy may lead to relapse, but prolonged AED intake is unnecessary.

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Delayed diagnosis of extraovarian teratoma in relapsing anti-NMDA receptor encephalitis

Cited by 8 publications

References 7 publications

Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis Associated With Mediastinal and Ovarian Teratomas: A Case Report

Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis Associated With Mediastinal and Ovarian Teratomas: A Case Report

Long-Term Prognosis of Patients With Anti-N-Methyl-D-Aspartate Receptor Encephalitis Who Underwent Teratoma Removal: An Observational Study

Clinical characteristics and long-term prognosis of relapsing anti-N-methyl-d-aspartate receptor encephalitis: a retrospective, multicenter, self-controlled study

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