J Korean Neurol Assoc.  2001 Nov;19(6):614-618.

Focal Transmantle Dysplasia: MRI and Clinical Manifestations in 5 Cases

• Kim CH
• Choi CG
• Lee SA

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.

Keyword

Neuronal migration; Cortical dysplasia; Epilepsy; Magnetic resonance imaging; Septum pellucidum

MeSH Terms

Epilepsies, Partial
Epilepsy
Female
Humans
Lateral Ventricles
Magnetic Resonance Imaging*
Male
Malformations of Cortical Development
Neurologic Examination
Nystagmus, Pathologic
Seizures
Septo-Optic Dysplasia
Septum Pellucidum
Stem Cells