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Epileptic spasms in older children: persistence beyond infancy
D Talwar1, M A Baldwin, R Hutzler
1Department of Pediatrics, Steele Memorial Children's Research Center, University of Arizona, Tucson.
Insights
Infantile spasms (IS) can persist beyond infancy in children with intractable epilepsy. This study found that spasms, a distinct seizure type, occurred in older children, indicating a need for continued management.
Area of Science:
- Epilepsy and Neurological Disorders
- Pediatric Neurology
- Clinical Neurophysiology
Background:
- Infantile spasms (IS) are a recognized epileptic syndrome.
- Recent proposals suggest classifying spasms as a distinct seizure type characterized by axial jerks in clusters.
- The persistence of spasms beyond infancy in older children with intractable epilepsy is not well-documented.
Purpose of the Study:
- To investigate the occurrence and characteristics of spasms in older children with intractable mixed seizure disorders.
- To describe the clinical and electrophysiological features of spasms persisting beyond infancy.
- To evaluate treatment outcomes in children with persistent spasms.
Main Methods:
- Retrospective analysis of video-electroencephalography (EEG) monitoring data from five older children (4.5-14.2 years) with intractable mixed seizure disorder.
- Detailed review of seizure semiology, EEG findings (ictal and interictal), and treatment responses.
- Seizure onset and persistence from infancy were documented.
Main Results:
- All five children experienced spasms in infancy that continued into older childhood.
- Spasms were characterized by axial flexion/extension jerks in clusters, with specific ictal EEG patterns (delta burst followed by background attenuation/decrease with superimposed rhythmic activity).
- Multiple other seizure types were present, and interictal EEGs were markedly abnormal.
- Two children showed no response to ACTH treatment; three underwent corpus callosotomy.
Conclusions:
- Spasms can occur in older children with intractable mixed seizure disorders.
- Spasms may persist beyond infancy, presenting as a distinct seizure type.
- These findings highlight the importance of recognizing persistent spasms and considering appropriate management strategies.
Abstract:
Although infantile spasms (IS) constitute a well-recognized epileptic syndrome, only recently did investigators propose that spasms be classified as a distinct seizure type, characterized by axial flexion/extension jerks in clusters. Five older children (aged 4.5-14.2 years) who underwent video-EEG monitoring in 1992 in our epilepsy program for intractable mixed seizure disorder (cryptogenic 1, symptomatic 4) demonstrated flexor and extensor spasms in clusters. Seizure onset was from birth to 1.33 years. All 5 had spasms during infancy that continued as the children aged. Ictal EEG during spasms showed a brief high-amplitude delta burst followed by diffuse background attenuation or diffuse background decrease with superimposed rhythmic beta or alpha activity. Multiple other seizure types were present. Interictal EEGs were markedly abnormal and demonstrated slowing, multifocal spikes, generalized slow spike-wave, and polyspike-wave. Two children with spasms were unsuccessfully treated with ACTH, and 3 underwent corpus callosotomy. We conclude that spasms occur in older children with intractable mixed seizure disorders and may persist beyond infancy.