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Distinct clinicopathologic subtypes of cortical dysplasia of Taylor

From the Comprehensive Epilepsy Program (Drs. Lawson, Jayakar, Resnick, Dean, and Duchowny) and Radiology Department (Drs. Birchansky and Pacheco), Miami Children’s Hospital, FL.

Address correspondence and reprint requests to Dr. J.A. Lawson, Neurology Department, Sydney Children’s Hospital, Level 4 Emergency Wing, Randwick 2031, Australia; e-mail: john.lawson{at}unsw.edu.au

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.

Received December 3, 2003. Accepted in final form August 5, 2004.

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