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Emergency: requires immediate attention
Infantile spasms
Other Resources UpToDate PubMed
Emergency: requires immediate attention

Infantile spasms

Contributors: Jamie Adams MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed

Synopsis

Infantile spasms (West syndrome) is an epilepsy syndrome that starts in infancy, characterized by epileptic spasms and associated with hypsarrhythmia on electroencephalogram (EEG) and developmental regression. Onset of disease is typically between 3 and 12 months of age.

The seizures are characterized by sudden, typically bilateral, spasms of the neck, trunk, and/or extremities with associated brief loss of consciousness. Spasms can be flexor, extensor, or mixed. Most seizures last seconds and occur in clusters with up to hundreds of spasms in a day.

Infantile spasms can be associated with many neurologic conditions including tuberous sclerosis, neuronal migration disorders, hypoxic-ischemic injury, cerebral palsy, or Down syndrome. Up to one-third of cases have no obvious underlying abnormality. Prognosis is typically poor (although somewhat better in the cryptogenic group); while spasms usually remit by age 3, most children go on to develop other seizure types, with up to 50% developing Lennox-Gastaut syndrome. Developmental delay and cognitive impairment is common.

Codes

ICD10CM:
G40.822 – Epileptic spasms, not intractable, without status epilepticus

SNOMEDCT:
28055006 – West syndrome

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Therapy

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References

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Last Reviewed:05/21/2017
Last Updated:06/19/2022
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Emergency: requires immediate attention
Infantile spasms
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A medical illustration showing key findings of Infantile spasms : Seizures, Loss of consciousness
Copyright © 2024 VisualDx®. All rights reserved.