Distinct clinicopathologic subtypes of cortical dysplasia of Taylor

J. A. Lawson, S. Birchansky, E. Pacheco, P. Jayakar, T. J. Resnick, P. Dean, M. S. Duchowny

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71 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)55-61
Number of pages7
JournalNeurology
Volume64
Issue number1
DOIs
StatePublished - Jan 11 2005

ASJC Scopus subject areas

  • Clinical Neurology

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    Lawson, J. A., Birchansky, S., Pacheco, E., Jayakar, P., Resnick, T. J., Dean, P., & Duchowny, M. S. (2005). Distinct clinicopathologic subtypes of cortical dysplasia of Taylor. Neurology, 64(1), 55-61. https://doi.org/10.1212/01.WNL.0000148647.55705.A3