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Published on: September 20, 2024
Dysplasia: a common finding in intractable pediatric temporal lobe epilepsy
B E Porter1, A R Judkins, R R Clancy
1Pediatric Regional Epilepsy Program, Children's Hospital of Philadelphia, and Department of Pediatrics and Neurology, University of Pennsylvania School of Medicine, Philadelphia 19104, USA. Porterb@email.chop.edu
Insights
Cortical dysplasia (CD) is common in children with refractory temporal lobe epilepsy (TLE), regardless of other epilepsy risk factors. This neuropathologic finding was present in 64% of surgical cases studied.
Area of Science:
- Neuropathology
- Pediatric Neurology
- Epilepsy Research
Background:
- Refractory temporal lobe epilepsy (TLE) in children can be associated with various risk factors, including central nervous system (CNS) infections, family history of epilepsy, and febrile convulsions (FC).
- Pre-existing cortical dysplasia (CD) is increasingly recognized as a potential independent risk factor for developing refractory TLE.
- Understanding the prevalence and characteristics of CD in pediatric TLE is crucial for improving diagnosis and treatment.
Purpose of the Study:
- To investigate the neuropathologic features of temporal lobe epilepsy (TLE) in pediatric patients.
- To determine the prevalence of cortical dysplasia (CD) in surgically resected temporal lobe tissue from children with refractory TLE.
- To assess the association between CD and known epilepsy risk factors in this cohort.
Main Methods:
- A review of neuropathologic findings from surgical temporal lobectomy specimens was conducted.
- The study included 33 pediatric patients diagnosed with refractory TLE.
- Patients' clinical histories regarding epilepsy risk factors (CNS infection, family history, febrile convulsions) were analyzed in conjunction with neuropathologic results.
Main Results:
- Cortical dysplasia (CD) was identified in 64% (21 out of 33) of pediatric patients with refractory TLE.
- CD was prevalent across subgroups with epilepsy risk factors: 73% in those with a history of febrile convulsions (FC), 66% with CNS infections, and 83% with a family history of epilepsy.
- Characteristic features of CD included disrupted cortical lamination, dystrophic and maloriented neurons, and balloon cells in the temporal neocortex.
Conclusions:
- Cortical dysplasia (CD) is a significant neuropathologic finding in pediatric refractory temporal lobe epilepsy (TLE), observed in 21 of 33 surgical cases.
- The presence of CD in refractory TLE was noted in children both with and without other established epilepsy risk factors.
- These findings underscore the importance of evaluating for CD in pediatric TLE, irrespective of other contributing factors.
Background:
Risk factors for temporal lobe epilepsy (TLE) include history of CNS infection, family history of epilepsy, and history of febrile convulsions (FC). Pre-existing cortical dysplasia (CD) may also predispose to refractory TLE, independent of other risk factors for epilepsy.
Methods:
The authors reviewed the neuropathologic features of surgical tissue from temporal lobectomies of 33 pediatric patients with refractory TLE, with and without a history of epilepsy risk factors.
Results:
CD was found in 64% (21/33) of all patients with refractory TLE, including 73% (11/15) patients with a history of FC, 66% (2/3) patients with CNS infections, and 83% (5/6) patients with a family history of epilepsy. Disrupted cortical lamination, dystrophic and maloriented neurons, and balloon cells characterized the CD found in the temporal neocortex.
Conclusion:
CD was seen in 21 of 33 surgical specimens from children with refractory TLE, including those with and without other epilepsy risk factors.
