Efficacy and safety of corticosteroids and ACTH in epileptic syndromes beyond Infantile Epileptic Spasms Syndrome (IESS): A systematic review and meta‐analysis.

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Title: Efficacy and safety of corticosteroids and ACTH in epileptic syndromes beyond Infantile Epileptic Spasms Syndrome (IESS): A systematic review and meta‐analysis.
Authors: Korinthenberg, Rudolf (AUTHOR), Bast, Thomas (AUTHOR), Haberlandt, Edda (AUTHOR), Stephani, Ulrich (AUTHOR), Strzelczyk, Adam (AUTHOR), Rücker, Gerta (AUTHOR)
Source: Epilepsia (Series 4). May2024, Vol. 65 Issue 5, p1155-1175. 21p.
Subjects: Epilepsy, Infantile spasms, Adrenocorticotropic hormone, Cushing's syndrome, Oral drug administration, Corticosteroids
Abstract: We conducted a systematic review investigating the efficacy and tolerability of adrenocorticotropic hormone (ACTH) and corticosteroids in children with epilepsies other than infantile epileptic spasm syndrome (IESS) that are resistant to anti‐seizure medication (ASM). We included retrospective and prospective studies reporting on more than five patients and with clear case definitions and descriptions of treatment and outcome measures. We searched multiple databases and registries, and we assessed the risk of bias in the selected studies using a questionnaire based on published templates. Results were summarized with meta‐analyses that pooled logit‐transformed proportions or rates. Subgroup analyses and univariable and multivariable meta‐regressions were performed to examine the influence of covariates. We included 38 studies (2 controlled and 5 uncontrolled prospective; 31 retrospective) involving 1152 patients. Meta‐analysis of aggregate data for the primary outcomes of seizure response and reduction of electroencephalography (EEG) spikes at the end of treatment yielded pooled proportions (PPs) of 0.60 (95% confidence interval [CI] 0.52–0.67) and 0.56 (95% CI 0.43–0.68). The relapse rate was high (PP 0.33, 95% CI 0.27–0.40). Group analyses and meta‐regression showed a small benefit of ACTH and no difference between all other corticosteroids, a slightly better effect in electric status epilepticus in slow sleep (ESES) and a weaker effect in patients with cognitive impairment and "symptomatic" etiology. Obesity and Cushing's syndrome were the most common adverse effects, occurring more frequently in trials addressing continuous ACTH (PP 0.73, 95% CI 0.48–0.89) or corticosteroids (PP 0.72, 95% CI 0.54–0.85) than intermittent intravenous or oral corticosteroid administration (PP 0.05, 95% CI 0.02–0.10). The validity of these results is limited by the high risk of bias in most included studies and large heterogeneity among study results. This report was registered under International Prospective Register of Systematic Reviews (PROSPERO) number CRD42022313846. We received no financial support. [ABSTRACT FROM AUTHOR]
Copyright of Epilepsia (Series 4) is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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  Label: Title
  Group: Ti
  Data: Efficacy and safety of corticosteroids and ACTH in epileptic syndromes beyond Infantile Epileptic Spasms Syndrome (IESS): A systematic review and meta‐analysis.
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  Data: <searchLink fieldCode="AR" term="%22Korinthenberg%2C+Rudolf%22">Korinthenberg, Rudolf</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Bast%2C+Thomas%22">Bast, Thomas</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Haberlandt%2C+Edda%22">Haberlandt, Edda</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Stephani%2C+Ulrich%22">Stephani, Ulrich</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Strzelczyk%2C+Adam%22">Strzelczyk, Adam</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Rücker%2C+Gerta%22">Rücker, Gerta</searchLink> (AUTHOR)
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  Data: <searchLink fieldCode="JN" term="%22Epilepsia+%28Series+4%29%22">Epilepsia (Series 4)</searchLink>. May2024, Vol. 65 Issue 5, p1155-1175. 21p.
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  Data: <searchLink fieldCode="DE" term="%22Epilepsy%22">Epilepsy</searchLink><br /><searchLink fieldCode="DE" term="%22Infantile+spasms%22">Infantile spasms</searchLink><br /><searchLink fieldCode="DE" term="%22Adrenocorticotropic+hormone%22">Adrenocorticotropic hormone</searchLink><br /><searchLink fieldCode="DE" term="%22Cushing's+syndrome%22">Cushing's syndrome</searchLink><br /><searchLink fieldCode="DE" term="%22Oral+drug+administration%22">Oral drug administration</searchLink><br /><searchLink fieldCode="DE" term="%22Corticosteroids%22">Corticosteroids</searchLink>
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: We conducted a systematic review investigating the efficacy and tolerability of adrenocorticotropic hormone (ACTH) and corticosteroids in children with epilepsies other than infantile epileptic spasm syndrome (IESS) that are resistant to anti‐seizure medication (ASM). We included retrospective and prospective studies reporting on more than five patients and with clear case definitions and descriptions of treatment and outcome measures. We searched multiple databases and registries, and we assessed the risk of bias in the selected studies using a questionnaire based on published templates. Results were summarized with meta‐analyses that pooled logit‐transformed proportions or rates. Subgroup analyses and univariable and multivariable meta‐regressions were performed to examine the influence of covariates. We included 38 studies (2 controlled and 5 uncontrolled prospective; 31 retrospective) involving 1152 patients. Meta‐analysis of aggregate data for the primary outcomes of seizure response and reduction of electroencephalography (EEG) spikes at the end of treatment yielded pooled proportions (PPs) of 0.60 (95% confidence interval [CI] 0.52–0.67) and 0.56 (95% CI 0.43–0.68). The relapse rate was high (PP 0.33, 95% CI 0.27–0.40). Group analyses and meta‐regression showed a small benefit of ACTH and no difference between all other corticosteroids, a slightly better effect in electric status epilepticus in slow sleep (ESES) and a weaker effect in patients with cognitive impairment and "symptomatic" etiology. Obesity and Cushing's syndrome were the most common adverse effects, occurring more frequently in trials addressing continuous ACTH (PP 0.73, 95% CI 0.48–0.89) or corticosteroids (PP 0.72, 95% CI 0.54–0.85) than intermittent intravenous or oral corticosteroid administration (PP 0.05, 95% CI 0.02–0.10). The validity of these results is limited by the high risk of bias in most included studies and large heterogeneity among study results. This report was registered under International Prospective Register of Systematic Reviews (PROSPERO) number CRD42022313846. We received no financial support. [ABSTRACT FROM AUTHOR]
– Name: AbstractSuppliedCopyright
  Label:
  Group: Ab
  Data: <i>Copyright of Epilepsia (Series 4) is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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RecordInfo BibRecord:
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      – Type: doi
        Value: 10.1111/epi.17918
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      – Code: eng
        Text: English
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      Pagination:
        PageCount: 21
        StartPage: 1155
    Subjects:
      – SubjectFull: Epilepsy
        Type: general
      – SubjectFull: Infantile spasms
        Type: general
      – SubjectFull: Adrenocorticotropic hormone
        Type: general
      – SubjectFull: Cushing's syndrome
        Type: general
      – SubjectFull: Oral drug administration
        Type: general
      – SubjectFull: Corticosteroids
        Type: general
    Titles:
      – TitleFull: Efficacy and safety of corticosteroids and ACTH in epileptic syndromes beyond Infantile Epileptic Spasms Syndrome (IESS): A systematic review and meta‐analysis.
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            NameFull: Korinthenberg, Rudolf
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            NameFull: Bast, Thomas
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            NameFull: Haberlandt, Edda
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            NameFull: Strzelczyk, Adam
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            – D: 01
              M: 05
              Text: May2024
              Type: published
              Y: 2024
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