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Facilitation of infantile spasms by partial seizures
E J Carrazana1, C T Lombroso, M Mikati
1Department of Neurology, Children's Hospital, Harvard Medical School, Boston, Massachusetts.
Insights
Infantile spasms in infants may be triggered by preceding partial seizures. This condition often shows poor response to standard treatments but may improve with surgery.
Area of Science:
- Pediatric Neurology
- Epileptology
- Neuroscience
Background:
- Infantile spasms are a severe epilepsy syndrome in infants.
- The precise triggers and underlying mechanisms of infantile spasms remain under investigation.
- Focal cerebral lesions are frequently observed in infants with infantile spasms.
Purpose of the Study:
- To investigate the relationship between partial seizures and the onset of infantile spasms.
- To characterize clinical and EEG features of infantile spasms associated with partial seizures.
- To evaluate treatment responses and outcomes in this specific patient cohort.
Main Methods:
- Retrospective analysis of 16 patients diagnosed with infantile spasms.
- Detailed review of seizure semiology, EEG findings, neuroimaging, and treatment history.
- Correlation of partial seizure occurrence with the onset of infantile spasms.
- Assessment of response to adrenocorticotropic hormone (ACTH), antiepileptic drugs (AEDs), and surgical interventions.
Main Results:
- 16 infants presented with infantile spasms triggered by preceding partial seizures.
- Focal cerebral lesions were present in 12 infants; 3 had cryptogenic epilepsy.
- Clusters of bilateral spasms consistently followed partial seizures with EEG localization.
- Response to ACTH and traditional AEDs was generally poor.
- Surgical cortical resections in 6 infants resulted in good outcomes.
- Complete agenesis of the corpus callosum in 3 infants suggested spasms were not solely due to interhemispheric spread.
Conclusions:
- Partial seizures may facilitate or induce infantile spasms, likely involving subcortical generation modulated by focal cortical discharges.
- The findings support a model where focal cortical pathology plays a role in the pathogenesis of these spasms.
- Surgical intervention can be an effective treatment option for selected infants with this specific presentation.
Abstract:
We report 16 patients with infantile spasms in whom onset of the clusters of spasms appeared to be triggered by close temporal association with partial seizures. Common features included the presence of focal cerebral lesions in 12 infants (3 were classifiable as cryptogenic); all had partial seizures with EEG localization, clusters of bilateral spasms always preceded by partial seizures, and response to adrenocorticotropic hormone (ACTH) and traditional antiepileptic drugs (AEDs) generally was poor. Three had complete agenesis of the corpus callosum, which argues against interhemispheric callosal spread of focal discharges resulting in the generalized spasms. Surgical cortical resections were performed in 6 of the infants, with good outcomes. This group of patients supports a model in which the spasms, although probably generated at a subcortical level, are facilitated or possibly induced by focal discharges from cortical pathology.