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 language | English |
|---|---|
| Pages (from-to) | 55-61 |
| Number of pages | 7 |
| Journal | Neurology |
| Volume | 64 |
| Issue number | 1 |
| State | Published - Jan 11 2005 |
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ASJC Scopus subject areas
- Neuroscience(all)
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Distinct clinicopathologic subtypes of cortical dysplasia of Taylor. / Lawson, J. A.; Birchansky, S.; Pacheco, E.; Jayakar, P.; Resnick, T. J.; Dean, P.; Duchowny, M. S.
In: Neurology, Vol. 64, No. 1, 11.01.2005, p. 55-61.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Distinct clinicopathologic subtypes of cortical dysplasia of Taylor
AU - Lawson, J. A.
AU - Birchansky, S.
AU - Pacheco, E.
AU - Jayakar, P.
AU - Resnick, T. J.
AU - Dean, P.
AU - Duchowny, M. S.
PY - 2005/1/11
Y1 - 2005/1/11
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=11444268528&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=11444268528&partnerID=8YFLogxK
M3 - Article
C2 - 15642904
AN - SCOPUS:11444268528
VL - 64
SP - 55
EP - 61
JO - Neurology
JF - Neurology
SN - 0028-3878
IS - 1
ER -