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Epilepsy Res. 2018 Dec;148:1-7. doi: 10.1016/j.eplepsyres.2018.09.016. Epub 2018 Oct 2.

High vigabatrin dosage is associated with lower risk of infantile spasms relapse among children with tuberous sclerosis complex.

Author information

1
Division of Pediatric Neurology, UCLA Mattel Children's Hospital and David Geffen School of Medicine, Los Angeles, California, United States. Electronic address: shussain@mednet.ucla.edu.
2
Division of Pediatric Neurology, UCLA Mattel Children's Hospital and David Geffen School of Medicine, Los Angeles, California, United States.
3
Translational Neuroscience Center, Department of Neurology, Boston Children's Hospital, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts, United States.
4
Department of Neurology, University of Alabama at Birmingham, Birmingham, Alabama, United States.
5
University of Texas Houston, Houston, Texas, United States.
6
Department of Neurology, Cincinnati Children's Hospital, Cincinnati, Ohio, United States.
7
Department of Psychiatry and Behavioral Sciences, McGovern Medical School, University of Texas Health Science Center at Houston, 1941 East Road, 3.126 BBSB, Houston, Texas, 77054, United States. Electronic address: Deborah.A.Pearson@uth.tmc.edu.
8
Keck School of Medicine of USC, University of Southern California, Children's Hospital Los Angeles, 4650 Sunset Blvd., Mailstop #53, Los Angeles, CA, 90027, United States. Electronic address: mwilliams@chla.usc.edu.
9
Department of Developmental Medicine, Boston Children's Hospital, Boston, MA, 300 Longwood Ave, United States. Electronic address: Hanson@childrens.harvard.edu.
10
Department of Developmental and Behavioral Pediatrics, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, MLC 7004, Cincinnati, OH, 45229, United States. Electronic address: Nicole.Bing@cchmc.org.
11
Department of Developmental and Behavioral Pediatrics, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, MLC 7004, Cincinnati, OH, 45229, United States. Electronic address: Bridget.Kent@cchmc.org.
12
University of Alabama at Birmingham, 930 20th St S, Birmingham, AL, 35205, United States. Electronic address: sokelley@uab.edu.
13
Department of Neurology, Boston Children's Hospital, Boston, MA, 300 Longwood Ave, United States. Electronic address: Rajna.Filip-Dhima@childrens.harvard.edu.
14
Department of Neurology, Boston Children's Hospital, Boston, MA, 300 Longwood Ave, United States. Electronic address: Kira.Dies@childrens.harvard.edu.
15
Keck School of Medicine of USC, University of Southern California, Children's Hospital Los Angeles, 4650 Sunset Blvd., Mailstop #53, Los Angeles, CA, 90027, United States. Electronic address: Stephanie.Bruns@cchmc.org.
16
Department of Radiology, Boston Children's Hospital, Boston, MA, 300 Longwood Ave, United States. Electronic address: Benoit.Scherrer@childrens.harvard.edu.
17
University of Alabama at Birmingham, Data Coordinating Center, 665 University Boulevard, Birmingham, AL, 35294, United States. Electronic address: cutterg@uab.edu.
18
Autism Speaks, 85 Devonshire St., 9th Floor, United States. Electronic address: donna.murray@autismspeaks.org.
19
Tuberous Sclerosis Alliance, 801 Roeder Road, Suite 750, United States. Electronic address: sroberds@tsalliance.org.

Abstract

After initially successful treatment of infantile spasms, the long-term cumulative risk of relapse approaches 50%, and there is no established protocol to mitigate this risk. Although vigabatrin may be an effective means to prevent relapse, there is little guidance as to ideal duration and dosage. Using a cohort of children with infantile spasms and tuberous sclerosis complex (TSC), we evaluated the potential association of post-response VGB treatment and the rate of infantile spasms relapse. Patients with infantile spasms and clinical response to vigabatrin were identified among a multicenter prospective observational cohort of children with TSC. For each patient we recorded dates of infantile spasms onset, response to vigabatrin, relapse (if any), and quantified duration and dosage of vigabatrin after response. Time to relapse as a function of vigabatrin exposure was evaluated using survival analyses. We identified 50 children who responded to VGB. During a median follow-up of 16.6 months (IQR 10.3-22.9), 12 (24%) patients subsequently relapsed after a median of 7.8 months (IQR 3.1-9.6). Relapse occurred after VGB discontinuation in four patients, and during continued VGB treatment in the remaining eight cases. In survival analyses, risk of relapse was unaffected by the presence or absence of VGB treatment (HR 0.31, 95%CI 0.01-28.4, P =  0.61), but weighted-average dosage was associated with marked reduction in relapse risk: Each 50 mg/kg/d increment in dosage was associated with 61% reduction in risk (HR 0.39, 95%CI 0.17 - 0.90, P =  0.026). This study suggests that the risk of infantile spasms relapse in TSC may be reduced by high-dose vigabatrin treatment.

KEYWORDS:

Epileptic spasms; Secondary prevention; West syndrome

PMID:
30296632
PMCID:
PMC6347124
[Available on 2019-12-01]
DOI:
10.1016/j.eplepsyres.2018.09.016
[Indexed for MEDLINE]

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