Clinical and Electrophysiologic Characteristics of Malformation of Cortical Development with Childhood Epilepsy

Purpose: To evaluate the clinical and electrophysiological characteristics of malformation of cortical development (MCD) with epilepsy. Method: We studied clinical and electroencephalographic (EEG) features of 54 childhood epilepsy patients with MCD diagnosed by magnetic resonance imaging (MRI) and pathologic examinations. Results: 1) Bilateral diffuse MCD’s were in 5 patients, bilateral focal MCD’s were in 8, unilateral diffuse MCD’s in 7, and unilateral focal MCD’s were noted in 34 patients. 2) Partial seizures were manifested in 35 patients, and 4 of them evolve to infantile spasm (IS), isolated IS was noted in 15 cases, and generalized seizures were noticed in 2 cases 3) Asymmetric EEG backgraound slowing was noted in 30 patients, and 29 patients (96.7%) had MCD’s in abnormal side of brain. 4) Polymorphic slowing was noted in 36 patients, and 28 cases (77.8%) had MCD’s in those area. 5) Sensitivity of partial epileptiform discharges (ED’s) for MCD was 79.6%, but specificity was 68.5%. 6) Localized paroxysmal fast activity was noted in 16 cases (29.6%), and specificity for MCD was 90.7%. 7) Spindle shaped fast activity was noted in 8 patients (14.8%), and its specificity was 100%. 8) Thirty-one cases (57.4%) were intractable to antiepileptic drugs (AED’s). Seventeen cases of them were treated by ketogenic diet, and 12 patients (66.7%) were completely controlled. Among 12 cases of surgical resection, 11 patients (91.7%) became seizure free for 6 months to 2 years. 9) In pathologically confirmed cases, EEG sensitivity for MCD lesion was 100%, but sensitivity of MRI was 69.2%. Conclusion: EEG is most sensitive diagnostic tool for MCD in childhood epilepsy. and many of intractable epilepsy could be controlled by ketogenic diet and surgery.ope