Abstract
Hormone therapies and vigabatrin are first-line agents in infantile spasms, but more than one-third of patients fail to respond to these treatments. This was a retrospective study of patients with infantile spasms who were treated between January 2005 and December 2017. We analyzed the response rates of initial treatment and second-line treatment. Responders were defined as those in whom cessation of spasms was observed for a period of at least one month, within 2 weeks of treatment initiation. Regarding the response rate to initial treatment, combination therapy of vigabatrin with prednisolone showed a significantly better response than that of vigabatrin monotherapy (55.3% vs. 39.1%, p = 0.037). Many drugs, such as clobazam, topiramate, and levetiracetam, were used as second-line agents after the failure of vigabatrin. Among these, no antiepileptic drug showed as good a response as prednisolone. For patients who used prednisolone, the proportion of responders was significantly higher in the higher-dose group (≥40 mg/day) than in the lower-dose group (66.7% vs. 12.5%, p = 0.028). Further studies of combination therapy to assess dosage protocols and long-term outcomes are needed.
Highlights
Infantile spasms is a rare and severe seizure disorder that is difficult to treat [1]
In the United Kingdom Infantile Spasms Study (UKISS) trial, hormonal therapies showed a higher rate of cessation of spasms at 2 weeks than vigabatrin (73% vs. 54%, p = 0·043) [8]
We considered vigabatrin and oral corticosteroids as the standard therapies in this study
Summary
Infantile spasms is a rare and severe seizure disorder that is difficult to treat [1]. Hormonal treatment was first used in the treatment of this disorder in 1958, and has been shown to be effective in the cessation of spasms and resolution of EEG abnormalities [6]. In the United Kingdom Infantile Spasms Study (UKISS) trial, hormonal therapies showed a higher rate of cessation of spasms at 2 weeks than vigabatrin (73% vs 54%, p = 0·043) [8]. Some studies have shown a short-term response rate that is significantly higher with combination treatment than with steroids alone [18,19], but such advantages are still unclear. We aimed to assess the efficacy of vigabatrin and steroid combination treatment in comparison with standard monotherapy as first-line treatments for infantile spasms. We evaluated the response rates of various secondary medications in patients in whom these first-line treatments were ineffective
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