Treatment of infantile spasms with very high dose prednisolone before high dose adrenocorticotropic hormone

Authors

  • Shaun A. Hussain,

    Corresponding author
    1. Division of Neurology, Department of Pediatrics, Mattel Children's Hospital and David Geffen School of Medicine at UCLA, Los Angeles, California, U.S.A
    • Address correspondence to Shaun A. Hussain, Division of Pediatric Neurology, Mattel Children's Hospital, David Geffen School of Medicine at UCLA, 22-474 Marion Davies Children's Center, Box 951752, Los Angeles, CA 90095-1752, U.S.A. E-mail: shussain@mednet.ucla.edu

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  • Shlomo Shinnar,

    1. Departments of Neurology, Pediatrics, and Epidemiology and Population Health, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York, U.S.A
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  • Grace Kwong,

    1. Division of Neurology, Department of Pediatrics, Mattel Children's Hospital and David Geffen School of Medicine at UCLA, Los Angeles, California, U.S.A
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  • Jason T. Lerner,

    1. Division of Neurology, Department of Pediatrics, Mattel Children's Hospital and David Geffen School of Medicine at UCLA, Los Angeles, California, U.S.A
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  • Joyce H. Matsumoto,

    1. Division of Neurology, Department of Pediatrics, Mattel Children's Hospital and David Geffen School of Medicine at UCLA, Los Angeles, California, U.S.A
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  • Joyce Y. Wu,

    1. Division of Neurology, Department of Pediatrics, Mattel Children's Hospital and David Geffen School of Medicine at UCLA, Los Angeles, California, U.S.A
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  • W. Donald Shields,

    1. Division of Neurology, Department of Pediatrics, Mattel Children's Hospital and David Geffen School of Medicine at UCLA, Los Angeles, California, U.S.A
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  • Raman Sankar

    1. Division of Neurology, Department of Pediatrics, Mattel Children's Hospital and David Geffen School of Medicine at UCLA, Los Angeles, California, U.S.A
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Summary

Purpose

This study investigated the short-term response to a standardized hormonal therapy protocol for treatment of infantile spasms.

Methods

Twenty-seven children with video electroencephalography (EEG)–confirmed infantile spasms received very high dose (8 mg/kg/day, max 60 mg/day) oral prednisolone for 2 weeks. Response (absence of both hypsarrhythmia and spasms) to prednisolone was ascertained by repeat overnight video-EEG. Responders were tapered over 2 weeks and nonresponders were immediately transitioned to high dose (150 IU/m2/day) intramuscular adrenocorticotropic hormone (ACTH) for two additional weeks. Response was again determined by overnight video-EEG after ACTH therapy.

Key Findings

Sixty-three percent (17/27) of patients responded completely to prednisolone. Subsequently, 40% (4/10) of prednisolone nonresponders exhibited a complete response after an additional 2-week course with ACTH. Among 27 subjects with median follow-up of 13.5 months (interquartile range [IQR] 4.8–25.9), 12% (2/17) of prednisolone responders and 50% (2/4) of ACTH responders experienced a relapse between 2 and 9 months after initial response.

Significance

Very high dose prednisolone demonstrated significantly higher efficacy than previously reported for lower doses in prior studies. High dose ACTH may be superior to very high dose prednisolone, and in lieu of a definitive clinical trial, the choice between prednisolone and ACTH for initial treatment of infantile spasms remains controversial.

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