Distinct clinicopathologic subtypes of cortical dysplasia of Taylor.

Abstract

BACKGROUND:

Two pathologic subtypes based on the presence or absence of balloon cells have been described in cortical dysplasia of Taylor (CDT).

OBJECTIVE:

To determine whether the pathologic subtype has any distinct clinical or MRI features that are relevant to management.

METHODS:

The histopathologic, clinical, and MRI features of 34 children with CDT who underwent epilepsy surgery at Miami Children's Hospital from 1990 to 2001 were investigated.

RESULTS:

Bizarre neuronal cytomegaly was the primary pathologic feature of 15 patients with the dysplasia-only subtype, and 19 cases showed additional characteristics including balloon cells and marked white matter abnormalities. Both groups presented with severe intractable epilepsy of very-early-onset, multiple daily seizures, cognitive disability, and focal neurologic deficits. The dysplasia-only subtype had higher rates of neonatal onset, hemiparesis, and severe mental retardation (p < 0.05). The MRI features of focal cortical thickening with associated cortical T2 signal change showed excellent sensitivity (94%) and reasonable specificity (73%) for the diagnosis of the balloon cell subtype. The overall surgical outcome was 59% seizure freedom at 2 years.

CONCLUSIONS:

Children with cortical dysplasia of Taylor type have in common a very-early-onset, severe epilepsy with neurologic co-morbidity. Patients with the non-balloon cell pathologic subtype have a more severe phenotype. A trend toward a better outcome in the balloon cell group suggests that preoperative identification of these subtypes may impact surgical planning.

• Authors: J A Lawson, S Birchansky, E Pacheco, P Jayakar, T J Resnick, P Dean, M S Duchowny
• Journal: Neurology (Neurology) Vol. 64 Issue 1 Pg. 55-61 (Jan 11 2005) ISSN: 1526-632X [Electronic] United States
• PMID: 15642904 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Topics

• Adolescent
• Adult
• Brain Diseases (classification, physiopathology, psychology)
• Cerebral Cortex (abnormalities, pathology, physiopathology)
• Child
• Child, Preschool
• Cytodiagnosis (methods)
• Epilepsy (diagnosis, pathology, surgery)
• Female
• Humans
• Infant
• Infant, Newborn
• Magnetic Resonance Imaging (methods)
• Male
• Neurons (pathology)
• Retrospective Studies