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Epilepsia. 2017 Dec;58(12):2098-2103. doi: 10.1111/epi.13937. Epub 2017 Nov 3.

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

Author information

1
Departments of Pediatrics and Neurology, School of Medicine, University of Colorado Anschutz Medical Campus, Aurora, Colorado, U.S.A.
2
Departments of Pediatrics & Communicable Diseases (Division of Pediatric Neurology), University of Michigan, Ann Arbor, Michigan, U.S.A.
3
Center for Neuroscience, Children's National Health System, Washington, District of Columbia, U.S.A.
4
Jane and John Justin Neurosciences Department, Cook Children's Hospital, Fort Worth, Texas, U.S.A.
5
Departments of Neurology and Pediatrics, Mayo Clinic, Rochester, Minnesota, U.S.A.
6
Department of Pediatric Neurology, Mattel Children's Hospital at UCLA, Los Angeles, California, U.S.A.
7
Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Boston, Massachusetts, U.S.A.
8
Departments of Neurology and Pediatrics, Nationwide Children's Hospital and the Ohio State University College of Medicine, Columbus, Ohio, U.S.A.
9
Department of Neurology/Division of Pediatric Neurology, Seattle Children's Hospital University of Washington, Seattle, Washington, U.S.A.
10
Department of Neurology, Massachusetts General Hospital/Harvard Medical School, Boston, Massachusetts, U.S.A.
11
Departments of Healthcare Policy & Research and Department of Pediatrics, Weill Cornell Medical Center, New York, New York, U.S.A.
12
Division of Child Neurology, Stanford University, Palo Alto, California, U.S.A.
13
Division of Neurology, Department of Pediatrics, University of Alabama, Birmingham, Alabama, U.S.A.
14
Departments of Neurology and Pediatrics, Johns Hopkins Hospital, Baltimore, Maryland, U.S.A.
15
Departments of Pediatrics and Neurology, Division of Child Neurology, Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, U.S.A.
16
Departments of Neurology and Pediatrics, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York, U.S.A.
17
Departments of Neurology and Pediatrics, NYU School of Medicine, New York, New York, U.S.A.
18
Department of Neurology, Carolinas Healthcare System, Charlotte, North Carolina, U.S.A.

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.

KEYWORDS:

Adrenocorticotropic hormone; Epilepsy; Prednisolone; Vigabatrin

PMID:
29105055
PMCID:
PMC5863227
[Available on 2018-12-01]
DOI:
10.1111/epi.13937
[Indexed for MEDLINE]

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