Magazine article The Exceptional Parent

Infantile Spasms: The New Consensus

Magazine article The Exceptional Parent

Infantile Spasms: The New Consensus

Article excerpt

Therapy for Infantile Spasms:

In 2004 the American Academy of Neurology associated with the Child Neurology Society published a Practice Parameter regarding the medical treatment of infantile spasms. Their recommendation was that ACTH is probably effective (the highest rating given with the available data) and vigabatrin was possibly effective. The Practice Parameter further stated that vigabatrin was possibly effective for children with IS secondary to tuberous sclerosis and infantile spasms. At the time of publication, there was insufficient evidence to recommend oral corticosteroids, high dose prednisolone, valproic acid, other AEDs, or the ketogenic diet as first line therapies. It was noted that surgery may also be an option in some cases.

This panel carefully argued the pros and cons of ACTH versus other steroid therapies, and examined all available information. The goal for improving outcomes in infantile spasms include early recognition and diagnosis, short duration of therapy and first line treatment with timely EEG done for diagnosis and when indicated for follow up. The effective treatment should not only include cessation or the stopping of observed IS but also improvement of the hypsarrhythmic EEG. Thus, unlike all treatments for other seizure types, success in the treatment of IS requires an "all or nothing" approach, that is, no spasms and resolution of hypsarrhythmia. This all or none treatment and plan has been associated with the best outcome both cognitively and developmentally and reduced progression to other types of epilepsy.

When using ACTH it is recommended that high dose, 150 units/m2/day be given in twice daily dosage for approximately two weeks, followed by taper as treatment response is evaluated. Longer periods of treatment have been used with prednisone and with vigabatrin and with these treatments ongoing assessment of benefits versus risk is required. Specifically for ACTH, the high dose, short duration protocol is recommended based on existing studies that tend to demonstrate that high dose is superior to low dose and that short duration results in fewer adverse effects. In those in whom ACTH is successful, spasms usually stop within the first week. Existing studies suggest approximately 80-90% of infants with spasms will respond to high dose ACTH. In some cases, ACTH may be contraindicated or not acceptable to the family, who must administer the dose twice daily by injection. Acthar gel, the ACTH preparation available in the United States, is now FDA approved for IS. Although used for over 50 years, formal FDA approval in fact, came after our consensus meeting and publication.

Vigabtrin has also been approved for use in infantile spasms in the United States over the last few years. It has been used for treatment of IS in Europe and in other countries for many years. It is regarded as the drug of first choice in children with associated tuberous sclerosis. Typical dosing schedule begins initially with 50mg/kg/day, which is then increased to 100 to 150 mg/kg/day over a few weeks. As opposed to ACTH where the main risks are hypertension and increased risk of infection, vigabatrin has been associated with a direct effect on the retina of the eye causing a decrease in peripheral vision. This adverse effect tends to come on after six months of therapy and thus, limiting its treatment in those who do not respond, is recommended. In patients who do respond to vigabatrin, the length of treatment continuation and absolute dose is unclear. An abnormality on MRI was noted in some children with vigabatrin treatment but further studies indicate that these changes have been noted in children with IS not treated with vigabatrin, and they may remain or seemingly resolve without change in vigabatrin therapy. Continued evaluation of benefit versus risk is recommended.

Other therapies for infantile spasms include oral corticosteroids, ketogenic diet, other anticonvulsants and in those with a focal area of the brain responsible for spasms, surgery has been performed successfully. …

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