Epileptic spasms

Shaun Hussain MD MS (Dr. Hussain of David Geffen School of Medicine at UCLA received research support from Insys, Lundbeck, Gw Pharma, and Eisai and honoraria for his services as an advisor from Upsher-Smith and Mallinckrodt.)
Jerome Engel Jr MD PhD, editor. (Dr. Engel of the David Geffen School of Medicine at the University of California, Los Angeles, has no relevant financial relationships to disclose.)
Originally released September 8, 2003; last updated March 28, 2016; expires March 28, 2019

This article includes discussion of epileptic spasms and epileptic spasms including infantile spasms. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Epileptic spasms are a peculiar seizure-type that usually manifest in infants as West syndrome. These seizures characteristically occur in daily clusters on awakening, and individual spasms typically manifest with a brief axial contraction (ie, head-drop), with simultaneous extension of the arms, and intermixed limbic phenomena, such as fear and crying. Epileptic spasms are often accompanied by severely abnormal interictal epileptiform patterns, such as hypsarrhythmia, and they demand prompt diagnosis and treatment given the potential for enduring intellectual devastation as a result of inadequate or late treatment. The author details contemporary treatment protocols.

Key points

 

• Video-EEG is critical to confirm epileptic spasms and distinguish it from mimics.

 

• Prompt diagnosis of epileptic spasms is essential as delays in treatment are associated with treatment failure and diminished neurodevelopmental outcomes.

 

• Epileptic spasms may be exacerbated by traditional antiseizure drugs that target voltage-gated sodium channels (eg, carbamazepine, oxcarbazepine).

Historical note and terminology

Having observed epileptic spasms in his own son, Dr. William West first provided a terrifying account of the phenomena in an 1841 letter to the editor of The Lancet (West 1894). Accordingly, West syndrome has come to denote the triad of epileptic spasms, intellectual impairment, and hypsarrhythmia—the classic interictal electroencephalography (EEG) pattern often accompanying epileptic spasms (Gibbs and Gibbs 1952). As it pertains to epileptic spasms, nomenclature continues to be a challenge. A great variety of terms have been used to describe epileptic spasms, including salaam convulsions, komplimentierkrämpfe, spasmes salutatoires, lightning major seizures, startle seizures, and others (Shields 2006). West syndrome is problematic because a principle goal of care is the prevention of intellectual disability, and epileptic spasms often occur without hypsarrhythmia. Furthermore, although the most popular contemporary descriptor is infantile spasms, “epileptic spasms” is the favored descriptor because this seizure type can be present in infants, as well as in older children, and, occasionally, in adults (Berg et al 2010).

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