Focal Transmantle Dysplasia : MRI and Clinical Manifestations in 5 Cases

J Korean Neurol Assoc > Volume 19(6); 2001 > Article

Journal of the Korean Neurological Association 2001;19(6): 614-618.

국소성 횡뇌개 이형성증 :5예의 MRI 및 임상적 소견

김철호, 최충곤*이상암

울산대학교 의과대학 서울중앙병원 신경과,방사선과*

Focal Transmantle Dysplasia : MRI and Clinical Manifestations in 5 Cases

Chul Ho Kim, M.D., Choong Gon Choi, M.D.*, Sang Ahm Lee, M.D.

Department of Neurology & Radiology,* Asan Medical Center, University of Ulsan College of Medicine

Abstract

"Background : Recently, focal transmantle dysplasia has been proposed to be a specific malformation of cortical development resulting from abnormal stem cell proliferation. Its clinical and imaging characteristics are not well known yet. Methods : Five patients (3 men and 2 women, mean age 29 years) with epilepsy diagnosed as focal transmantle dysplasia were included and reviewed with regard to the clinical and imaging features. Results : On the MRI, all patients had signal abnormality extending from the posterior frontal cortex to the wall of the lateral ventricle. The lesion signal was homogeneous and isointense to gray matter, which didn’t enhanced by contrast. The lesion was protruded into the ventricle in 3 cases. Agenesis of septum pellucidum was the most commonly associated anomaly in 3 cases. Neurological examination was normal except in one patient with septo-optic dysplasia showing pendular nystagmus. Simple partial seizures were in 2 patients and nocturnal seizures in 3. Seizure onset was different from the first decade to the fourth decade (mean 19 years). Interictal spikes were observed in 2 patients. Seizure outcome was variable. Conclusions : We found some characteristics of MR imaging including the protruded lesion into the ventricle and asso-ciation with agenesis of septum pellucidum, which have not been recognized previously. Key Words : Neuronal migration, Cortical dysplasia, Epilepsy, Magnetic resonance imaging, Septum pellucidum"