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Management and prognosis of infantile spasms

Author
Daniel G Glaze, MD
Section Editor
Douglas R Nordli, Jr, MD
Deputy Editor
April F Eichler, MD, MPH

INTRODUCTION

Infantile spasms (IS) is an age-specific epileptic disorder of infancy and early childhood. Children with IS typically exhibit epileptic spasms along with the electroencephalographic pattern known as hypsarrhythmia.

The major options for treatment of IS include hormonal therapy, mainly corticotropin (ACTH), and antiseizure medication, mainly vigabatrin. Pyridoxine is often used as first line therapy for IS in Japan, although there are no randomized controlled trials of this agent as treatment for IS.

Treatment of IS has been evaluated in a 2004 and a 2012 American Academy of Neurology (AAN) practice parameter [1,2], a 2013 Cochrane systemic review [3], and a 2010 United States consensus report [4]. Conclusions were limited by the overall poor methodology of the available studies. Lack of adherence to standardized case definitions and outcome measures is one problem with many studies. Another is that inclusion of a control group is critical, as the natural history of the disease is that clinical spasms subside and electroencephalogram patterns evolve without therapy, yet many clinicians would be reluctant not to treat as there is some observational data that delayed therapy may worsen prognosis. As a result, many questions still remain regarding the mechanism, optimal drug, dose, duration of therapy, and the importance of prompt initiation of treatment after the appearance of spasms.

The management and prognosis of IS are reviewed here. The etiology and pathogenesis, clinical features, and diagnosis of this disorder are discussed separately. (See "Etiology and pathogenesis of infantile spasms" and "Clinical features and diagnosis of infantile spasms".)

MONITORING TREATMENT RESPONSE

The consensus of experts in a 2010 review was that effective treatment of IS is defined by complete cessation of spasms and resolution of hypsarrhythmia on electroencephalography (EEG) [4]. Both parents and trained observers may miss the occurrence of spasms, especially if they are subtle. (See "Clinical features and diagnosis of infantile spasms", section on 'Spasms'.) Less commonly, they may “over count” imitators of spasms, especially in patients in the symptomatic group. A standard EEG to evaluate interictal activity may miss the hypsarrhythmia pattern, which can be variably present in an awake child, but is detected more sensitively in sleep. (See "Clinical features and diagnosis of infantile spasms", section on 'Hypsarrhythmia'.) As a result, video-EEG monitoring is ideally used to assess treatment response in children with IS.

                                

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Literature review current through: Nov 2016. | This topic last updated: Wed Sep 07 00:00:00 GMT+00:00 2016.
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