Malformations of cortical development with balloon cells
| Autor: | Mark T Mackay, James T. Rutka, Laurence E. Becker, Bruria Ben-Zeev, Hiroshi Otsubo, N. A. Chuang, O. C. Snead, S. H. Chuang, Shelly K. Weiss |
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| Rok vydání: | 2003 |
| Předmět: |
Male
medicine.medical_specialty Nervous System Malformations Balloon Diagnosis Differential Central nervous system disease Tuberous sclerosis Epilepsy Tuberous Sclerosis medicine Humans Child Retrospective Studies Cerebral Cortex Neurons business.industry Infant Newborn Infant Electroencephalography Histology Cortical dysplasia medicine.disease Magnetic Resonance Imaging Surgery Child Preschool Female Neurology (clinical) business Complication Neuroglia Follow-Up Studies Calcification |
| Zdroj: | Neurology. 60:580-587 |
| ISSN: | 1526-632X 0028-3878 |
| DOI: | 10.1212/01.wnl.0000044053.09023.91 |
| Popis: | Background: Balloon cells are a key feature of tuberous sclerosis (TS) but are also seen in focal cortical dysplasia (FCD). The authors compare the clinical and MRI characteristics in children with medically refractory localization-related epilepsy who were found to have balloon cells on histology after cortical resections.Methods: A retrospective review of clinical and MRI data in cases ascertained from a search of pathology records from 1990 until 2000 for those with a diagnosis of FCD or TS. Seventeen patients were identified with malformations of cortical development with balloon cells on histology. Seven had clinical diagnosis of TS and the remaining 10, FCD with balloon cells (FCDBC).Results: Seventy percent of patients with FCDBC (mean follow-up 3.3 years) and 33% of patients with TS (mean follow-up 5.1 years) are seizure free after surgery. There was agreement between the diagnosis based on preoperative MR imaging and on histology in 60% of patients with FCDBC and 71% of patients with TS. Myelin depletion and calcification were noted more frequently in patients with TS.Conclusions: No significant differences were noted between patients with refractory epilepsy caused by TS or FCDBC. There was a trend toward better postoperative seizure control in the FCDBC group. These two conditions are difficult to distinguish on the basis of MR and histologic appearances. The authors conclude that FCDBC likely represents a phenotypic variation of TS, and as such, all patients with balloon cell dysplasias should be carefully screened for other features of TS to enable appropriate genetic counseling. |
| Databáze: | OpenAIRE |
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