THERAPY AND OUTCOME OF INFANTS WITH INFANTILE SPASMS

More than fifty trials have carried out on the treatment of infantile spasms. There is still a lack of consensus about the treatment of first choice for infantile spasms. Up-to date information comparing hormonal therapy (ACTH and oral steroids) and vigabatrin show that hormonal therapy is more effective at day 14. Prednisolone is as effective as corticotrophin when used in maximal doses. This is in discrepancy with one recent theory, according to which the action of ACTH via steroids is likely to be of minor importance. Hormonal therapy might accelerate physiological events during critical stages of brain development.

Vigabatrin might be the choice of treatment for tuberous sclerosis.

Both, hormonal therapy and vigabatrin, have side effects. The side-effects of the hormonal therapy are well-known and treatable. The most serious concern of vigabatrin use is visual impairment which seems to be permanent. In addition, in animals, VGB can induce apoptosis in the developing brain.

Relapses after hormonal therapy occur in about 25%. The frequency after vigabatrin therapy is unknown. At the follow-up after 3–4 months, the numbers of seizure-free patients treated with different drugs (steroids or vigabatrin) from different series were disappointingly low, but the percentage who were free of seizure, was very similar (40–42%).

In the long-term follow-up (25–30 years) of patients, one third of the patients had died, one third was seizure-free, and a quarter was normal or subnormal. The remaining group was mentally retarded.

Patients should be treated at the minimal effective dose and duration (preferably with the most effective drug, ACTH). A short treatment lag is important.

Advancements in our understanding of brain maturation, etiologies, mechanisms and genetics underlying catastrophic epilepsy may facilitate more effective pharmacologic interventions.