Ernesto Gonzalez-Giraldo1, Carl E Stafstrom1, Anthony C Stanfield2, Eric H Kossoff3. 1. Departments of Neurology and Pediatrics, The Johns Hopkins Hospital, Baltimore, Baltimore, Maryland. 2. Department of Neurology, The Johns Hopkins Hospital, Baltimore, MD, Baltimore, Maryland. 3. Departments of Neurology and Pediatrics, The Johns Hopkins Hospital, Baltimore, Baltimore, Maryland. Electronic address: ekossoff@jhmi.edu.
Abstract
BACKGROUND: Hormonal therapy is the treatment of choice in most patients with infantile spasms, but the optimal way to provide this therapy is unclear. Intramuscular adrenocorticotropic hormone (ACTH) has historically used first-line; however, there are significant logistical and financial issues. Our institution has used high-dose prednisolone as the first-line hormonal treatment of infantile spasms since 2006 and published our early experience with 15 infants in 2009. This study updates our institutional experience over more than 10 years of continuous use. METHODS: Charts of infants who presented to the Johns Hopkins Hospital with infantile spasms and were treated with high-dose oral prednisolone (40-60 mg/day) from January, 2006 through December, 2016 were reviewed. Electroclinical response was defined as clinical spasm-freedom and resolution of hypsarrhythmia within two weeks of initiation of therapy. Presence of infantile spasms at three months and adverse effects throughout treatment were evaluated. RESULTS: Over the 10-year period, 87 infants with new-onset infantile spasms were treated. Electroclinical response occurred in 64% infants within two weeks; 62% were spasm-free at three months. Fifty-two percent had side effects, primarily irritability, weight gain, and gastroesophageal reflux. Five percent had major adverse events, including gastrointestinal bleeding (n = 2), herpes simplex virus reactivation (n = 1), and necrotizing enterocolitis (n = 1). CONCLUSIONS: Our results continue to demonstrate that high-dose oral prednisolone is very effective for the treatment of new-onset infantile spasms, with few major adverse effects. Oral prednisolone represents a less expensive, readily available alternative to adrenocorticotropic hormone injections.
BACKGROUND: Hormonal therapy is the treatment of choice in most patients with infantile spasms, but the optimal way to provide this therapy is unclear. Intramuscular adrenocorticotropic hormone (ACTH) has historically used first-line; however, there are significant logistical and financial issues. Our institution has used high-dose prednisolone as the first-line hormonal treatment of infantile spasms since 2006 and published our early experience with 15 infants in 2009. This study updates our institutional experience over more than 10 years of continuous use. METHODS: Charts of infants who presented to the Johns Hopkins Hospital with infantile spasms and were treated with high-dose oral prednisolone (40-60 mg/day) from January, 2006 through December, 2016 were reviewed. Electroclinical response was defined as clinical spasm-freedom and resolution of hypsarrhythmia within two weeks of initiation of therapy. Presence of infantile spasms at three months and adverse effects throughout treatment were evaluated. RESULTS: Over the 10-year period, 87 infants with new-onset infantile spasms were treated. Electroclinical response occurred in 64% infants within two weeks; 62% were spasm-free at three months. Fifty-two percent had side effects, primarily irritability, weight gain, and gastroesophageal reflux. Five percent had major adverse events, including gastrointestinal bleeding (n = 2), herpes simplex virus reactivation (n = 1), and necrotizing enterocolitis (n = 1). CONCLUSIONS: Our results continue to demonstrate that high-dose oral prednisolone is very effective for the treatment of new-onset infantile spasms, with few major adverse effects. Oral prednisolone represents a less expensive, readily available alternative to adrenocorticotropic hormone injections.
Authors: Alexander B Ramos; Roberto A Cruz; Nicole R Villemarette-Pittman; Piotr W Olejniczak; Edward C Mader Journal: J Investig Med High Impact Case Rep Date: 2019 Jan-Dec