Surgical treatment of pediatric focal cortical dysplasia: Clinical spectrum and surgical outcome

Neurology. 2016 Aug 30;87(9):945-51. doi: 10.1212/WNL.0000000000003042. Epub 2016 Jul 27.

Abstract

Objective: To analyze the clinical presentation and outcomes of surgically treated focal cortical dysplasia (FCD) in children.

Methods: We reviewed 75 cases of confirmed FCD by pathology after resective surgery. We used the pathologic classification proposed by the International League Against Epilepsy and included clinical profile and seizure and neurodevelopmental outcomes in analyses.

Results: There were 11 cases of FCD type I, 34 of type IIa, 19 of type IIb, and 11 of type III. Fifty-one cases (68.0%) presented clinically as focal epilepsy (FE) and 24 (32.0%) as epileptic encephalopathy (EE), including 16 with Lennox-Gastaut syndrome and 8 with West syndrome. We observed EE in 7 cases (63.6%) in FCD type I, 14 (41.2%) in type IIa, 2 (10.5%) in type IIb, and 1 (9.1%) in type III. We found the following more frequently in EE: seizure onset occurring at younger than 2 years (EE: 20 [83.3%], FE: 19 [37.3%]; p < 0.001), presence of intellectual disability before surgery (EE: 22 [91.7%], FE: 29 [56.9%]; p = 0.003), and multilobar resections (EE: 19 [79.2%], FE: 15 [29.4%]; p < 0.001). Forty-eight cases (64.0%) were seizure-free; 66.7% (34/51) in FE, 58.3% (14/24) in EE. Neurodevelopmental level showed more improvement (11/48 vs 0/27, p = 0.011) and less deterioration (2/48 vs 9/27, p = 0.001) in the seizure-free group compared to the non-seizure-free group.

Conclusions: FCD can cause FE and EE in pediatric age, and resective surgery should be considered as a treatment option for both types of epilepsy.

MeSH terms

  • Adolescent
  • Anticonvulsants / therapeutic use
  • Child
  • Child, Preschool
  • Electroencephalography
  • Female
  • Humans
  • Kaplan-Meier Estimate
  • Longitudinal Studies
  • Male
  • Malformations of Cortical Development / drug therapy
  • Malformations of Cortical Development / surgery*
  • Neuropsychological Tests
  • Neurosurgical Procedures / methods*
  • Retrospective Studies
  • Treatment Outcome*

Substances

  • Anticonvulsants