Infantile spasms

Hancock EC, Osborne JP, Edwards SW. Treatment of infantile spasms. Cochrane
Database Syst Rev. 2013 Jun 5;6:CD001770.

To date, few well-designed RCTs have considered the treatment of infantile spasms, and the numbers of patients enrolled have been small. In the majority, methodology has been poor, hence it is not clear which treatment is optimal in the treatment of this epilepsy syndrome. Hormonal treatment resolves spasms in more infants than vigabatrin, but this may or may not translate into better long-term outcomes. If prednisolone or vigabatrin is used, high dosage is recommended. Vigabatrin may be the treatment of choice in tuberous sclerosis. Resolution of the EEG features may be important, but this has not been proven. Further research using large studies with robust methodology is required.

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Wheless JW, Gibson PA, Rosbeck KL, et al. Infantile spasms (West syndrome): update and resources for pediatricians and providers to share with parents. BMC Pediatr. 2012 Jul 25;12:108.

Infantile spasms (IS; West syndrome) is a severe form of epilepsy of early infancy [1]. Although IS was first described over 160 years ago [1], its diagnosis, evaluation, and management continue to pose many challenges to health care professionals and affected families. Educating pediatricians and general pediatric health care providers about IS may be especially important as a recent IS working group (ISWG) of pediatric neurologists reviewed the literature and determined that early recognition of IS and prompt treatment are mandatory and may improve developmental and cognitive outcomes in some patients [2]. IS presents with myoclonic-tonic seizures (spasms) that may be characterized by flexor, extensor, or mixed movements, a distinct electroencephalogram (EEG) pattern of hypsarrhythmia, and psychomotor delay/arrest [3]. The incidence of IS ranges from 2 to 3.5/10,000 live births, with onset during the first year of life in 90% of those affected. This translates to approximately 2,000 to 2,500 new cases per year in the United States. The peak age of onset is between 3 and 7 months; onset after 18 months is rare, though onset up to 4 years of age has been reported [4]. Spasms usually cease by 5 years of age, but other seizure types are reported in as many as 60% of children with IS even after cessation of spasms [5]. IS occurs in children from all ethnic groups, and boys are affected slightly more often than girls (ratio of 60:40) [6-8].

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Kossoff EH. Infantile spasms. Neurologist. 2010 Mar;16(2):69-75.

In this review, the clinical features, electroencephalogram (EEG) findings (hypsarrhythmia), prognostic factors, and myriad of treatment options for this condition will be discussed. Guidelines, surveys, and practice parameters have judged adrenocorticotropin hormone and vigabatrin to be the most proven treatments, with the latter indicated for tuberous sclerosis. However, potentially helpful therapies with fewer side effects have recently emerged including high-dose oral prednisolone, ketogenic diet, and topiramate. Additionally, advances in the past several years include the creation of viable animal models for testing new treatments.

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Fois A. Infantile spasms: review of the literature and personal experience. Ital J Pediatr. 2010 Feb 8;36:15.

This epileptic disorder has become a classic topic for neuropediatricians and the interest is documented by the large number of publications on this subject.The relative frequency among the epileptic syndromes is an another reason why not only neuropediatricians but also general pediatricians must be fully informed about diagnostic, clinical, imaging and genetic aspects.Early diagnosis is of paramount importance in order to obtain even complete results in patients with so called idiopathic situations. A number of problems are still to be solved. There is no agreement on the type and the schedule of treatment. A common denominator about this problem is not jet available even if some advances in this regard have been accomplished. Of paramount importance is an accurate clinical and laboratory examination as a prerequisite regarding prognosis and results of therapy in every single case.However, even if more than 170 years have elapsed since the first communication of Dr. West on the peculiar syndrome that his child was suffering of, the interest of scientists on this subject has now been enriched and rewarded.

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More PubMed results on infantile spasms.

One thought on “Infantile spasms

  1. Pingback: Adrenocorticotrophic hormone (ACTH) therapy in infantile spasms | Pediatric Focus

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