Which medications in the drug class Corticosteroids are used in the treatment of Infantile Spasm (West Syndrome)?

Updated: Jan 11, 2019
  • Author: Tracy A Glauser, MD; Chief Editor: Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP  more...
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Answer

Corticosteroids

These agents cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

Corticotropin (Acthar, ACTH)

A 2004 American Academy of Neurology and Child Neurology Society practice parameter concluded that (1) "ACTH is probably effective for the short-term treatment of infantile spasms and in resolution of hypsarrhythmia (Level B)" and (2) "[t]here is insufficient evidence to recommend the optimum dosage and duration of treatment with ACTH for the treatment of infantile spasms (Level U)."

One study found that after approximately 2 weeks, hormonal therapy provided better relief from spasm than did vigabatrin. The 2004 multicenter, randomized, controlled trial compared hormonal therapy (either oral prednisolone or intramuscular [IM] tetracosactide depot, a synthetic analogue of ACTH) with vigabatrin in 107 infants with infantile spasms. More infants assigned hormonal treatments (73%) had no spasms on days 13 and 14 than did infants assigned vigabatrin (54%). [42]

However, a follow-up study demonstrated that, although hormonal treatment initially controlled spasms better than vigabatrin did, by age 12-14 months, infants in the hormonal and vigabatrin groups had similar seizure-free rates. [43]

Older studies have suggested that ACTH's efficacy (percentage of infants with West syndrome reaching seizure freedom) is between 50% and 67%.

Corticotropin is associated with serious, potentially life-threatening adverse effects. It must be administered intramuscularly, and such injections are painful for the infant to receive and are unpleasant for the parent to perform.

A prospective, single-blind study demonstrated no difference in effectiveness between high-dose, long-duration corticotropin (150 U/m2/day for 3 wk, tapering over 9 wk) and low-dose, short-duration corticotropin (20-30 U/day for 2-6 wk, tapering over 1 wk with respect to spasm cessation and improvement in the patient's EEG. Hypertension was more common with larger doses.

Prednisone

A 2004 American Academy of Neurology and Child Neurology Society practice parameter concluded that "there is insufficient evidence that oral corticosteroids are effective in the treatment of infantile spasms (Level U)."

Few comparative studies between ACTH and prednisone have been performed. One double-blind, placebo-controlled, crossover study demonstrated no difference between low-dose ACTH (20-30 U/day) and prednisone (2 mg/kg/day). However, a prospective, randomized, single-blinded study demonstrated high-dose ACTH at 150 U/m2/day to be superior to prednisone (2 mg/kg/day) in suppressing clinical spasms and hypsarrhythmic EEG in infants with infantile spasms.

One study found that after approximately 2 weeks, hormonal therapy provided better relief from spasm than did vigabatrin. The 2004 multicenter, randomized, controlled trial compared hormonal therapy (either oral prednisolone or IM tetracosactide depot) with vigabatrin in 107 infants with infantile spasms. More infants assigned hormonal treatments (73%) had no spasms on days 13 and 14 than did infants assigned vigabatrin (54%). [42]

However, a follow-up study demonstrated that, although hormonal treatment initially controlled spasms better than vigabatrin did, by age 12-14 months, infants in the hormonal and vigabatrin groups had similar seizure-free rates. [43]

Findings from a multicenter prospective database of infants with new diagnosis of infantile spasms compared “standard therapy” with ACTH, oral steroids, or vigabatrin to all other medications, and found that 55% of patients treated with ACTH had remission of spasms and resolution of hypsarrhythmia sustained at 3 months after initiation of the treatment, compared to 39% treated with oral steroids, 36% treated with vigabatrin, and 9% of patients treated with “nonstandard” therapy. [15]


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