TY - JOUR
T1 - Seizure characteristics and outcomes in patients with neurological conditions related to high-risk paraneoplastic antibodies
AU - Smith, Kelsey M.
AU - Britton, Jeffrey W.
AU - Thakolwiboon, Smathorn
AU - Chia, Nicholas H.
AU - Gupta, Pranjal
AU - Flanagan, Eoin P.
AU - Zekeridou, Anastasia
AU - Lopez Chiriboga, Alfonso S.
AU - Valencia Sanchez, Cristina
AU - McKeon, Andrew
AU - Pittock, Sean J.
AU - Dubey, Divyanshu
N1 - Publisher Copyright:
© 2023 International League Against Epilepsy.
PY - 2023/9
Y1 - 2023/9
N2 - Objective: Seizures are a common manifestation of paraneoplastic neurologic syndromes. The objective of this study was to describe the seizure characteristics and outcomes in patients with high-risk paraneoplastic autoantibodies (>70% cancer association) and to determine factors associated with ongoing seizures. Methods: Patients from 2000 to 2020 with seizures and high-risk paraneoplastic autoantibodies were retrospectively identified. Factors associated with ongoing seizures at last follow-up were evaluated. Results: Sixty patients were identified (34 males, median age at presentation = 52 years). ANNA1-IgG (Hu; n = 24, 39%), Ma2-IgG (n = 14, 23%), and CRMP5-IgG (CV2; n = 11, 18%) were the most common underlying antibodies. Seizures were the initial presenting symptom in 26 (43%), and malignancy was present in 38 (63%). Seizures persisted for >1 month in 83%, and 60% had ongoing seizures, with almost all patients (55/60, 92%) still being on antiseizure medications at last follow-up a median of 25 months after seizure onset. Ongoing seizures at last follow-up were associated with Ma2-IgG or ANNA1-IgG compared to other antibodies (p =.04), highest seizure frequency being at least daily (p =.0002), seizures on electroencephalogram (EEG; p =.03), and imaging evidence of limbic encephalitis (LE; p =.03). Death occurred in 48% throughout the course of follow-up, with a higher mortality in patients with LE than in those without LE (p =.04). Of 31 surviving patients at last follow-up, 55% continued to have intermittent seizures. Significance: Seizures in the setting of high-risk paraneoplastic antibodies are frequently resistant to treatment. Ongoing seizures are associated with ANNA1-IgG and Ma2-IgG, high seizure frequency, and EEG and imaging abnormalities. Although a subset of patients may respond to immunotherapy and achieve seizure freedom, poor outcomes are frequently encountered. Death was more common among patients with LE.
AB - Objective: Seizures are a common manifestation of paraneoplastic neurologic syndromes. The objective of this study was to describe the seizure characteristics and outcomes in patients with high-risk paraneoplastic autoantibodies (>70% cancer association) and to determine factors associated with ongoing seizures. Methods: Patients from 2000 to 2020 with seizures and high-risk paraneoplastic autoantibodies were retrospectively identified. Factors associated with ongoing seizures at last follow-up were evaluated. Results: Sixty patients were identified (34 males, median age at presentation = 52 years). ANNA1-IgG (Hu; n = 24, 39%), Ma2-IgG (n = 14, 23%), and CRMP5-IgG (CV2; n = 11, 18%) were the most common underlying antibodies. Seizures were the initial presenting symptom in 26 (43%), and malignancy was present in 38 (63%). Seizures persisted for >1 month in 83%, and 60% had ongoing seizures, with almost all patients (55/60, 92%) still being on antiseizure medications at last follow-up a median of 25 months after seizure onset. Ongoing seizures at last follow-up were associated with Ma2-IgG or ANNA1-IgG compared to other antibodies (p =.04), highest seizure frequency being at least daily (p =.0002), seizures on electroencephalogram (EEG; p =.03), and imaging evidence of limbic encephalitis (LE; p =.03). Death occurred in 48% throughout the course of follow-up, with a higher mortality in patients with LE than in those without LE (p =.04). Of 31 surviving patients at last follow-up, 55% continued to have intermittent seizures. Significance: Seizures in the setting of high-risk paraneoplastic antibodies are frequently resistant to treatment. Ongoing seizures are associated with ANNA1-IgG and Ma2-IgG, high seizure frequency, and EEG and imaging abnormalities. Although a subset of patients may respond to immunotherapy and achieve seizure freedom, poor outcomes are frequently encountered. Death was more common among patients with LE.
KW - autoimmune-associated epilepsy
KW - paraneoplastic antibodies
KW - seizures
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U2 - 10.1111/epi.17695
DO - 10.1111/epi.17695
M3 - Article
C2 - 37366270
AN - SCOPUS:85164355530
SN - 0013-9580
VL - 64
SP - 2385
EP - 2398
JO - Epilepsia
JF - Epilepsia
IS - 9
ER -