Practice Parameter: Medical Treatment of Infantile Spasms

Abstract

Abstract-Objective:To determine the current best practice for treatment of infantile spasms in children. Methods: Database searches of MEDLINE from 1966 and EMBASE from 1980 and searches of reference lists of retrieved articles were performed. Inclusion criteria were the documented presence of infantile spasms and hypsarrhythmia. Outcome measures included complete cessation of spasms, resolution of hypsarrhythmia, relapse rate, developmental outcome, and presence or absence of epilepsy or an epileptiform EEG. One hundred fifty-nine articles were selected for detailed review. Recommendations were based on a four-tiered classification scheme. Results: Adrenocorticotropic hormone (ACTH) is probably effective for the short-term treatment of infantile spasms, but there is insufficient evidence to recommend the optimum dosage and duration of treatment. There is insufficient evidence to determine whether oral corticosteroids are effective. Vigabatrin is possibly effective for the short-term treatment of infantile spasm and is possibly also effective for children with tuberous sclerosis. Concerns about retinal toxicity suggest that serial ophthalmologic screening is required in patients on vigabatrin; however, the data are insufficient to make recommendations regarding the frequency or type of screening. There is insufficient evidence to recommend any other treatment of infantile spasms. There is insufficient evidence to conclude that successful treatment of infantile spasms improves the long-term prognosis. Conclusions: ACTH is probably an effective agent in the short-term treatment of infantile spasms. Vigabatrin is possibly effective. NEUROLOGY 2004;62:1668 -1681 West syndrome 1 is a unique, age-specific epilepsy of early infancy. Infantile spasms are distinct from myoclonic and tonic seizures.2 They are characterized by an initial contraction phase followed by a more sustained tonic phase. They can be divided into three types (flexor, extensor, and mixed flexor-extensor spasms), and they can also be asymmetrical.2 The EEG characteristically demonstrates hypsarrhythmia, and onset of spasms is frequently associated with neurodevelopmental regression. The incidence of infantile spasms is

Cardiac Vagal Activation by Adrenocorticotropic Hormone Treatment in Infants with West Syndrome

“…Most protocols of ACTH treatment for WS indicate that ACTH should be administered for more than 14 days and then tapered (Yanagaki et al 1999;Mackay et al 2004). Because longer ACTH treatment is associated with more side effects including hypertension, infection, subdural hemorrhage and brain atrophy, some investigators have recommended a shorter duration of ACTH treatment (Ito et al 1990;Hrachovy et al 1994;Kondo et al 2005;Oguni et al 2006).…”

Choosing the Correct Antiepileptic Drugs: From Animal Studies to the Clinic

“…AEDs are typically screened in mature rats that have acute seizures rather than epilepsy and the limited number of animal models for the severe epileptic encephalopathies in children has also been a major hindrance in developing new AEDs [62]. An example of this situation is the case of vigabatrin where it has been shown that the drug is quite successful in the treatment of infantile spasms [63]. This was not predicted by animal models.…”

“…This was not predicted by animal models. Likewise, adrenocorticotrophin hormone (ACTH) has little role in treating seizures in adults, but is quiet effective in the treatment of infantile spasms, an epilepsy syndrome confined to children [63,64].…”

Síndrome de West, experiencia con una serie de casos con acceso al tratamiento de primera línea, en Lima

“…No existe tratamiento específico para el SW; sin embargo, el ACTH y la Vigabatrina son las drogas que han dado los mejores resultados (15)(16)(17)(18)(19)(20)(21)(22)(23)(24)34). Lamentablemente estos medicamentos no se encuentran en el petitorio nacional.…”