The impact of hypsarrhythmia on infantile spasms treatment response: Observational cohort study from the National Infantile Spasms Consortium

Scott T. Demarest, Rene´e A. Shellhaas, William D. Gaillard, Cynthia Keator, Katherine C. Nickels, Shaun A. Hussain, Tobias Loddenkemper, Anup D. Patel, Russell P. Saneto, Elaine Wirrell, Iván Sánchez Fernández, Catherine J. Chu, Zachary Grinspan, Courtney J. Wusthoff, Sucheta Joshi, Ismail S. Mohamed, Carl E. Stafstrom, Cynthia V. Stack, Elissa G. Yozawitz, Judith S. BluvsteinRani K. Singh, Kelly G. Knupp

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

Objective: The multicenter National Infantile Spasms Consortium prospective cohort was used to compare outcomes and phenotypic features of patients with infantile spasms with and without hypsarrhythmia. Methods: Patients aged 2 months to 2 years were enrolled prospectively with new-onset infantile spasms. Treatment choice and categorization of hypsarrhythmia were determined clinically at each site. Response to therapy was defined as resolution of clinical spasms (and hypsarrhythmia if present) without relapse 3 months after initiation. Results: Eighty-two percent of patients had hypsarrhythmia, but this was not associated with gender, mean age, preexisting developmental delay or epilepsy, etiology, or response to first-line therapy. Infants with hypsarrhythmia were more likely to receive standard treatment (adrenocorticotropic hormone, prednisolone, or vigabatrin [odds ratio (OR) 2.6, 95% confidence interval (CI) 1.4-4.7] and preexisting epilepsy reduced the likelihood of standard treatment (OR 3.2, 95% CI 1.9-5.4). Hypsarrhythmia was not a determinant of response to treatment. A logistic regression model demonstrated that later age of onset (OR 1.09 per month, 95% CI 1.03-1.15) and absence of preexisting epilepsy (OR 1.7, 95% CI 1.06-2.81) had a small impact on the likelihood of responding to the first-line treatment. However, receiving standard first-line treatment increased the likelihood of responding dramatically: vigabatrin (OR 5.2 ,95% CI 2-13.7), prednisolone (OR 8, 95% CI 3.1-20.6), and adrenocorticotropic hormone (ACTH; OR 10.2, 95% CI 4.1-25.8) . Significance: First-line treatment with standard therapy was by far the most important variable in determining likelihood of response to treatment of infantile spasms with or without hypsarrhythmia.

Original languageEnglish (US)
JournalEpilepsia
DOIs
StateAccepted/In press - 2017

Fingerprint

Infantile Spasms
Observational Studies
Cohort Studies
Odds Ratio
Confidence Intervals
Therapeutics
Adrenocorticotropic Hormone
Vigabatrin
Prednisolone
Epilepsy
Logistic Models
Absence Epilepsy
Spasm
Age of Onset

Keywords

  • Adrenocorticotropic hormone
  • Epilepsy
  • Prednisolone
  • Vigabatrin

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Demarest, S. T., Shellhaas, R. A., Gaillard, W. D., Keator, C., Nickels, K. C., Hussain, S. A., ... Knupp, K. G. (Accepted/In press). The impact of hypsarrhythmia on infantile spasms treatment response: Observational cohort study from the National Infantile Spasms Consortium. Epilepsia. https://doi.org/10.1111/epi.13937

The impact of hypsarrhythmia on infantile spasms treatment response : Observational cohort study from the National Infantile Spasms Consortium. / Demarest, Scott T.; Shellhaas, Rene´e A.; Gaillard, William D.; Keator, Cynthia; Nickels, Katherine C.; Hussain, Shaun A.; Loddenkemper, Tobias; Patel, Anup D.; Saneto, Russell P.; Wirrell, Elaine; Sánchez Fernández, Iván; Chu, Catherine J.; Grinspan, Zachary; Wusthoff, Courtney J.; Joshi, Sucheta; Mohamed, Ismail S.; Stafstrom, Carl E.; Stack, Cynthia V.; Yozawitz, Elissa G.; Bluvstein, Judith S.; Singh, Rani K.; Knupp, Kelly G.

In: Epilepsia, 2017.

Research output: Contribution to journalArticle

Demarest, ST, Shellhaas, RA, Gaillard, WD, Keator, C, Nickels, KC, Hussain, SA, Loddenkemper, T, Patel, AD, Saneto, RP, Wirrell, E, Sánchez Fernández, I, Chu, CJ, Grinspan, Z, Wusthoff, CJ, Joshi, S, Mohamed, IS, Stafstrom, CE, Stack, CV, Yozawitz, EG, Bluvstein, JS, Singh, RK & Knupp, KG 2017, 'The impact of hypsarrhythmia on infantile spasms treatment response: Observational cohort study from the National Infantile Spasms Consortium', Epilepsia. https://doi.org/10.1111/epi.13937
Demarest, Scott T. ; Shellhaas, Rene´e A. ; Gaillard, William D. ; Keator, Cynthia ; Nickels, Katherine C. ; Hussain, Shaun A. ; Loddenkemper, Tobias ; Patel, Anup D. ; Saneto, Russell P. ; Wirrell, Elaine ; Sánchez Fernández, Iván ; Chu, Catherine J. ; Grinspan, Zachary ; Wusthoff, Courtney J. ; Joshi, Sucheta ; Mohamed, Ismail S. ; Stafstrom, Carl E. ; Stack, Cynthia V. ; Yozawitz, Elissa G. ; Bluvstein, Judith S. ; Singh, Rani K. ; Knupp, Kelly G. / The impact of hypsarrhythmia on infantile spasms treatment response : Observational cohort study from the National Infantile Spasms Consortium. In: Epilepsia. 2017.
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abstract = "Objective: The multicenter National Infantile Spasms Consortium prospective cohort was used to compare outcomes and phenotypic features of patients with infantile spasms with and without hypsarrhythmia. Methods: Patients aged 2 months to 2 years were enrolled prospectively with new-onset infantile spasms. Treatment choice and categorization of hypsarrhythmia were determined clinically at each site. Response to therapy was defined as resolution of clinical spasms (and hypsarrhythmia if present) without relapse 3 months after initiation. Results: Eighty-two percent of patients had hypsarrhythmia, but this was not associated with gender, mean age, preexisting developmental delay or epilepsy, etiology, or response to first-line therapy. Infants with hypsarrhythmia were more likely to receive standard treatment (adrenocorticotropic hormone, prednisolone, or vigabatrin [odds ratio (OR) 2.6, 95{\%} confidence interval (CI) 1.4-4.7] and preexisting epilepsy reduced the likelihood of standard treatment (OR 3.2, 95{\%} CI 1.9-5.4). Hypsarrhythmia was not a determinant of response to treatment. A logistic regression model demonstrated that later age of onset (OR 1.09 per month, 95{\%} CI 1.03-1.15) and absence of preexisting epilepsy (OR 1.7, 95{\%} CI 1.06-2.81) had a small impact on the likelihood of responding to the first-line treatment. However, receiving standard first-line treatment increased the likelihood of responding dramatically: vigabatrin (OR 5.2 ,95{\%} CI 2-13.7), prednisolone (OR 8, 95{\%} CI 3.1-20.6), and adrenocorticotropic hormone (ACTH; OR 10.2, 95{\%} CI 4.1-25.8) . Significance: First-line treatment with standard therapy was by far the most important variable in determining likelihood of response to treatment of infantile spasms with or without hypsarrhythmia.",
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T1 - The impact of hypsarrhythmia on infantile spasms treatment response

T2 - Observational cohort study from the National Infantile Spasms Consortium

AU - Demarest, Scott T.

AU - Shellhaas, Rene´e A.

AU - Gaillard, William D.

AU - Keator, Cynthia

AU - Nickels, Katherine C.

AU - Hussain, Shaun A.

AU - Loddenkemper, Tobias

AU - Patel, Anup D.

AU - Saneto, Russell P.

AU - Wirrell, Elaine

AU - Sánchez Fernández, Iván

AU - Chu, Catherine J.

AU - Grinspan, Zachary

AU - Wusthoff, Courtney J.

AU - Joshi, Sucheta

AU - Mohamed, Ismail S.

AU - Stafstrom, Carl E.

AU - Stack, Cynthia V.

AU - Yozawitz, Elissa G.

AU - Bluvstein, Judith S.

AU - Singh, Rani K.

AU - Knupp, Kelly G.

PY - 2017

Y1 - 2017

N2 - Objective: The multicenter National Infantile Spasms Consortium prospective cohort was used to compare outcomes and phenotypic features of patients with infantile spasms with and without hypsarrhythmia. Methods: Patients aged 2 months to 2 years were enrolled prospectively with new-onset infantile spasms. Treatment choice and categorization of hypsarrhythmia were determined clinically at each site. Response to therapy was defined as resolution of clinical spasms (and hypsarrhythmia if present) without relapse 3 months after initiation. Results: Eighty-two percent of patients had hypsarrhythmia, but this was not associated with gender, mean age, preexisting developmental delay or epilepsy, etiology, or response to first-line therapy. Infants with hypsarrhythmia were more likely to receive standard treatment (adrenocorticotropic hormone, prednisolone, or vigabatrin [odds ratio (OR) 2.6, 95% confidence interval (CI) 1.4-4.7] and preexisting epilepsy reduced the likelihood of standard treatment (OR 3.2, 95% CI 1.9-5.4). Hypsarrhythmia was not a determinant of response to treatment. A logistic regression model demonstrated that later age of onset (OR 1.09 per month, 95% CI 1.03-1.15) and absence of preexisting epilepsy (OR 1.7, 95% CI 1.06-2.81) had a small impact on the likelihood of responding to the first-line treatment. However, receiving standard first-line treatment increased the likelihood of responding dramatically: vigabatrin (OR 5.2 ,95% CI 2-13.7), prednisolone (OR 8, 95% CI 3.1-20.6), and adrenocorticotropic hormone (ACTH; OR 10.2, 95% CI 4.1-25.8) . Significance: First-line treatment with standard therapy was by far the most important variable in determining likelihood of response to treatment of infantile spasms with or without hypsarrhythmia.

AB - Objective: The multicenter National Infantile Spasms Consortium prospective cohort was used to compare outcomes and phenotypic features of patients with infantile spasms with and without hypsarrhythmia. Methods: Patients aged 2 months to 2 years were enrolled prospectively with new-onset infantile spasms. Treatment choice and categorization of hypsarrhythmia were determined clinically at each site. Response to therapy was defined as resolution of clinical spasms (and hypsarrhythmia if present) without relapse 3 months after initiation. Results: Eighty-two percent of patients had hypsarrhythmia, but this was not associated with gender, mean age, preexisting developmental delay or epilepsy, etiology, or response to first-line therapy. Infants with hypsarrhythmia were more likely to receive standard treatment (adrenocorticotropic hormone, prednisolone, or vigabatrin [odds ratio (OR) 2.6, 95% confidence interval (CI) 1.4-4.7] and preexisting epilepsy reduced the likelihood of standard treatment (OR 3.2, 95% CI 1.9-5.4). Hypsarrhythmia was not a determinant of response to treatment. A logistic regression model demonstrated that later age of onset (OR 1.09 per month, 95% CI 1.03-1.15) and absence of preexisting epilepsy (OR 1.7, 95% CI 1.06-2.81) had a small impact on the likelihood of responding to the first-line treatment. However, receiving standard first-line treatment increased the likelihood of responding dramatically: vigabatrin (OR 5.2 ,95% CI 2-13.7), prednisolone (OR 8, 95% CI 3.1-20.6), and adrenocorticotropic hormone (ACTH; OR 10.2, 95% CI 4.1-25.8) . Significance: First-line treatment with standard therapy was by far the most important variable in determining likelihood of response to treatment of infantile spasms with or without hypsarrhythmia.

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KW - Vigabatrin

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