Diagnostic challenges in patients with temporal lobe seizures and features of autoimmune limbic encephalitis

Ismail FS, Spatola M, Wörmann FG, Popkirov S, Jungilligens J, Bien C, Wellmer J, Schlegel U (Accepted)
European journal of neurology.

Zeitschriftenaufsatz | Angenommen | Englisch
 
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Autor*in
Ismail, Fatme Seval; Spatola, Marianna; Wörmann, Friedrich G.; Popkirov, Stoyan; Jungilligens, Johannes; Bien, ChristianUniBi; Wellmer, Jorg; Schlegel, Uwe
Abstract / Bemerkung
BACKGROUND: Consensus criteria for autoimmune limbic encephalitis (ALE) allow for a diagnosis even without neural antibodies (Abs), but it remains unclear which clinical features should prompt neural Ab screening in temporal lobe epilepsy patients.; METHODS: We identified 47 patients from a tertiary epilepsy center with mediotemporal lobe seizures and additional features suggestive of limbic involvement, including either memory deficits, psychiatric symptoms, mediotemporal MRI hyperintensities or inflammatory CSF. Neuronal Ab testing was carried out at two independent reference laboratories (Bielefeld-Bethel, Germany, and Barcelona, Spain). All brain MRIs were assessed by two reviewers independently.; RESULTS: Temporal lobe seizures were accompanied by memory deficits in 35/46 (76%), by psychiatric symptoms in 27/42 (64%), and by both in 19/42 (45%). Limbic T2/FLAIR signal hyperintensities were found in 26/46 (57%) patients (unilateral n = 22, bilateral n = 4). Standard CSF studies were abnormal in 2/37 (5%). Neuronal Abs were confirmed in serum and/or CSF in 8/47 (17%) cases and were directed against neuronal cell-surface targets (LGI1 n = 1, CASPR2 n = 1, undetermined target n = 3) or GAD65 (n = 3, all with high titers). Compared to Ab-negative patients, those who harbored neuronal Abs were more likely to have uni- or bilateral mediotemporal MRI changes (8/8, 100% versus 18/38, 47%, p=0.01, Fisher exact test).; CONCLUSIONS: In patients with temporal lobe seizures and additional limbic signs, 17% had neuronal Abs affirming ALE diagnosis. Mediotemporal MRI changes were found in all Ab-positive cases and had a positive likelihood ratio of 2.11 (95% CI 1.51 - 2.95). This article is protected by copyright. All rights reserved.
Erscheinungsjahr
2021
Zeitschriftentitel
European journal of neurology
eISSN
1468-1331
Page URI
https://pub.uni-bielefeld.de/record/2956424

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Ismail FS, Spatola M, Wörmann FG, et al. Diagnostic challenges in patients with temporal lobe seizures and features of autoimmune limbic encephalitis. European journal of neurology. Accepted.
Ismail, F. S., Spatola, M., Wörmann, F. G., Popkirov, S., Jungilligens, J., Bien, C., Wellmer, J., et al. (Accepted). Diagnostic challenges in patients with temporal lobe seizures and features of autoimmune limbic encephalitis. European journal of neurology. https://doi.org/10.1111/ene.15026
Ismail, F. S., Spatola, M., Wörmann, F. G., Popkirov, S., Jungilligens, J., Bien, C., Wellmer, J., and Schlegel, U. (Accepted). Diagnostic challenges in patients with temporal lobe seizures and features of autoimmune limbic encephalitis. European journal of neurology.
Ismail, F.S., et al., Accepted. Diagnostic challenges in patients with temporal lobe seizures and features of autoimmune limbic encephalitis. European journal of neurology.
F.S. Ismail, et al., “Diagnostic challenges in patients with temporal lobe seizures and features of autoimmune limbic encephalitis”, European journal of neurology, Accepted.
Ismail, F.S., Spatola, M., Wörmann, F.G., Popkirov, S., Jungilligens, J., Bien, C., Wellmer, J., Schlegel, U.: Diagnostic challenges in patients with temporal lobe seizures and features of autoimmune limbic encephalitis. European journal of neurology. (Accepted).
Ismail, Fatme Seval, Spatola, Marianna, Wörmann, Friedrich G., Popkirov, Stoyan, Jungilligens, Johannes, Bien, Christian, Wellmer, Jorg, and Schlegel, Uwe. “Diagnostic challenges in patients with temporal lobe seizures and features of autoimmune limbic encephalitis”. European journal of neurology (Accepted).

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