Category: Pediatrics
Keywords: pediatrics, seizure, infant, epilepsy, spams, infantile spasms (PubMed Search)
Posted: 12/13/2024 by Kathleen Stephanos, MD
(Updated: 12/26/2024)
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Infantile Spasms (now known as Infantile Epileptic Spasms Syndrome- IESS) is a subtle, can't miss diagnosis occurring in children under the age of 2, with 90% presenting before 1 year of age.
History:
Presentation for these patients can be sporadic, recurrent, flexion or extension of the limbs or head. The patient typically remains alert during an episode, but they can be startled or appear uncomfortable from the sudden movement.
With smart phone use, parents often have videos of the events that a provider can see as they are often no events in the ED.
Prognosis is very poor with up to 50% having long-term neuro-cognitive complications or regression.
Examination:
A complete physical exam should be performed with particular attention to focal neurologic deficits which would require emergent imaging, signs of electrolyte abnormalities (eg. Chvostek's sign in hypocalcemia), and evidence of neurocutenous syndromes (eg. Neurofibromatosis, tuberous sclerosis).
Testing:
Diagnosis is made with EEG showing hypsarrhythmia. This should be done as soon as possible, most often requiring an inpatient admission.
Lab work can be done to exclude other possible causes of abnormal movements including assessing for electrolyte abnormalities.
Imaging in the ED is not typically indicated unless there is concern based on exam. MRI is the imaging modality of choice, and is often completed after EEG confirmed diagnosis.
Treatment:
Management is initiated in conjunction with a pediatric neurologist with most common therapies being corticotropin (ACTH) and vigabatrin.
Smith MS, Matthews R, Rajnik M, Mukherji P. Infantile Epileptic Spasms Syndrome (West Syndrome). 2024 Feb 1. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan–. PMID: 30725936.
