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Outcomes of epileptic spasms in patients aged less than 3 years: single-center United States experience
Martina Vendrame1, Laura M F F Guilhoto, Tobias Loddenkemper
1Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA.
Insights
Adrenocorticotropic hormone and vigabatrin showed short-term efficacy for infantile epileptic spasms. However, most children experienced ongoing seizures and developmental delays, with etiology and initial symptoms predicting outcomes.
Area of Science:
- Pediatric Neurology
- Epileptology
Background:
- Infantile epileptic spasms are a severe epilepsy syndrome in young children.
- Understanding treatment efficacy and long-term outcomes is crucial for managing this condition.
Purpose of the Study:
- To evaluate the short-term and long-term outcomes of epileptic spasms in children under 3 years old.
- To identify predictors of treatment response and neurodevelopmental outcomes.
Main Methods:
- Retrospective review of 173 children diagnosed with epileptic spasms between 2004 and 2010.
- Analysis of treatment modalities including adrenocorticotropic hormone, vigabatrin, and phenobarbital.
- Assessment of short-term seizure control and long-term outcomes including seizures and developmental delay.
Main Results:
- Adrenocorticotropic hormone and vigabatrin demonstrated superior short-term epileptic spasm control compared to other agents.
- At follow-up, 54% of children had ongoing seizures and 83% had developmental delay.
- Known etiology, bilateral/diffuse brain lesions, and initial presentation with hypotonia or developmental delay predicted poor outcomes.
Conclusions:
- While certain treatments offer short-term benefits for infantile epileptic spasms, long-term neurodevelopmental outcomes remain a significant concern.
- Early identification of prognostic factors like etiology and initial symptoms is vital for predicting patient trajectories.
- The presence of developmental delay or hypotonia at onset may indicate a poorer prognosis regardless of treatment choice.
Abstract:
Retrospective review was performed of children aged <3 years with epileptic spasms at our center from 2004-2010. Short-term (<6 months) and long-term (≥6 months) outcomes were assessed. We included 173 children (104 boys; median age of onset, 6.8 months) with epileptic spasms of known (62%) and unknown (38%) etiology. Treatments included adrenocorticotropic hormone (n = 103), vigabatrin (n = 82), phenobarbital (n = 34), and other agents (n = 121). Short-term treatment with adrenocorticotropic hormone and vigabatrin provided better epileptic spasm control in groups with known and unknown etiology than other agents. At follow-up (6-27 months), 54% of children manifested seizures, and 83% manifested developmental delay. Known etiology was a predictor of poor developmental outcome (P = 0.006), whereas bilateral/diffuse brain lesions predicted both poor development and seizures (P = 0.001 and 0.005, respectively). Initial presentations of epileptic spasms with hypotonia or developmental delay most strongly predicted both seizures and neurodevelopmental outcomes (P < 0.001). In a child presenting with epileptic spasms with developmental delay or hypotonia, no specific treatment may offer superior benefit.
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