LIFE-THREATENING FOCAL STATUS EPILEPTICUS DUE TO OCCULT CORTICAL DYSPLASIA

Objective.-Neuronal migration disorders are usually, but not necessari ly, demonstrated by magnetic resonance imaging. Preoperative suspicion of these anomalies in the presence of normal magnetic resonance studi es has important practical implications. This study delineates some cl inical features that permit early suspicion of focal cortical dysplasi a localized in the central and precentral regions. Design.-In a retros pective case series, we studied the clinical presentation of four cons ecutive patients with normal preoperative magnetic resonance images in whom focal cortical dysplasia was found in the surgical specimen. Set ting.-Patients were seen in three referral centers specializing in epi lepsy surgery. Patients.-Four patients (three female), between the age s of 4 and 21 years, had intractable partial seizures leading to resec tive brain surgery. Intervention.-Three patients had corticectomies in the central (two patients) or frontal (one patient) regions. One unde rwent an en bloc resection of the central area after two unsuccessful corticectomies and cortical transcetion. Results.-Three patients prese nted with life-threatening focal motor status epilepticus necessitatin g intubation, and one had epilepsia partialis continua. All had had se izures previously, and the attacks progressed to intractability after 1 1/2 to 3 years. Surgery led to control of the seizures, but only two patients became seizure free (mean follow-up, 15.7 months). All but o ne developed a postoperative deficit, which eventually improved. Concl usions.-Focal cortical dysplasia should be suspected when life-threate ning focal motor status epilepticus or epilepsia partialis continua oc cur in children or young persons without another obvious cause. Normal magnetic resonance studies do not exclude neuronal migration disorder s.