Epileptic spasm
- Article
- 2021-03-14
An epileptic spasm is a specific type of seizure seen in childhood and childhood epilepsy syndrome, often referred to as West syndrome. These are more commonly referred to as infantile spasms (IS), as they are most often seen in the first year of life. West syndrome / IS is characterized by epileptic spasms, developmental problems and a specific brain wave pattern on electroencephalography (EEG) tests called hypsarrhythmia. The onset is usually in the first year of life, usually between 4 and 8 months. The attacks often appear as a sudden bend of the body with stiffening of the arms and legs that lasts for 1-2 seconds; some children arch their backs while stretching their arms and legs. Spasms usually occur on awakening and often occur in multiple clusters and hundreds of attacks per day. Most, but not all, children will have EEG measurements of hypsarrhythmia. Infantile spasms usually stop by age five, but can be replaced by other types of seizures. Many underlying conditions, such as birth damage, metabolic disorders, and genetic disorders, can give rise to IS, making it important to identify the underlying cause. No cause can be found in some children.
Therapy
Standard first-line treatments for IS include various forms of hormone therapy (including adrenocorticotropic hormone [ACTH] or prednisolone) or the anticonvulsant vigabatrin. These treatments are very effective, but have serious side effects and must be administered in consultation with a pediatric neurologist. The goal of treating infantile spasms is to stop the seizures and improve the EEG (hypsarrhythmia should disappear). Some children have spasms due to brain injuries, and surgical removal of these lesions may lead to improvement. When standard treatments fail to improve, other options such as the ketogenic diet and anti-seizure medications are considered. Regardless of the specific treatment chosen, it is critical to start therapy as soon as possible.
Prognosis
The prognosis for children with IS largely depends on the underlying cause. The intellectual prognosis for children with IS is generally poor because many babies with IS have neurological impairment before the spasms occur. Children with a quick start of treatment, normal development prior to infantile spasms, and no identifiable cause may do well. IS usually resolves in mid-childhood, but more than half of children with IS will have other types of seizures, such as Lennox-Gastaut syndrome, a later childhood epileptic condition. In addition, children with IS have a higher risk of autism. A shorter time between the start of IS and the start of standard treatment seems to lead to a better outcome; therefore early recognition of the attacks and early treatment are essential.
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