Related Experiment Videos
Treatment of infantile spasms: medical or surgical?
W D Shields1, D A Shewmon, H T Chugani
1Department of Neurology, University of California, School of Medicine, Los Angeles 90024-1752.
Insights
Infantile spasms, a challenging seizure disorder, may stem from localized brain abnormalities. Identifying and removing these "zones of cortical abnormality" offers new treatment hope for medically refractory cases.
Area of Science:
- Pediatric Neurology
- Epileptology
- Neuroscience
Background:
- Infantile spasms (IS) are a severe epilepsy syndrome with poorly understood pathophysiology, limiting treatment efficacy.
- IS are classified as generalized seizures, implying diffuse brain abnormalities, yet focal lesions have been implicated.
- Existing treatments for IS are often ineffective, necessitating novel therapeutic strategies.
Purpose of the Study:
- To explore the hypothesis that localized cortical abnormalities can cause infantile spasms.
- To introduce a new surgical assessment approach focusing on identifying the 'zone of cortical abnormality' in medically refractory IS.
- To improve treatment outcomes for children with infantile spasms.
Main Methods:
- Review of cases with medically refractory infantile spasms and localized brain abnormalities.
- Development of surgical assessment strategies targeting the zone of cortical abnormality, not just seizure onset.
- Analysis of treatment outcomes following surgical intervention for identified cortical abnormalities.
Main Results:
- Evidence suggests that focal cortical abnormalities can indeed cause generalized infantile spasms.
- Surgical removal of localized abnormalities has, in some cases, led to the cessation of infantile spasms.
- The identification of a 'zone of cortical abnormality' is a key factor in successful surgical management.
Conclusions:
- Infantile spasms may arise from focal cortical defects, challenging the traditional 'generalized' classification.
- A paradigm shift in surgical assessment for refractory infantile spasms is proposed, focusing on abnormality zones.
- This approach holds promise for improving seizure control in difficult-to-treat infantile spasms.
Abstract:
Although infantile spasms were initially described in 1841, remarkably little progress has been made in understanding the pathophysiology of this "peculiar form of infantile convulsions." Consequently, our ability to treat infantile spasms is limited. Infantile spasms are classified as a "generalized" seizure disorder in the international classification system, which suggests that the underlying brain abnormality causing the seizures also must be diffuse or generalized. As the classification suggests, there are many diffuse, or multifocal, brain disorders related to infantile spasms, e.g., inborn errors of metabolism, hypoxic-ischemic brain injury, and developmental brain defects such as tuberous sclerosis or Aicardi's syndrome. On the other hand, infantile spasms have been reported in which a localized brain abnormality was present, e.g., tumor, stroke, and trauma. On rare occasions, removal of a tumor has resulted in cessation of the generalized infantile spasms. This finding suggests that focal cortical abnormalities can cause infantile spasms and that removing the abnormality can stop the seizures. At University of California, Los Angeles, the Pediatric Epilepsy Surgery Program has developed new approaches to the treatment of infantile spasms. The principal underlying concepts are (a) children with medically refractory infantile spasms may have an area of cortical defect (called the zone of cortical abnormality) that causes the seizures and (b) infantile spasms are usually generalized seizures. Thus, the goal of the surgical assessment is not the identification of the focus of seizure onset but rather the identification of the zone of cortical abnormality.