CELLULAR CHARACTERIZATION OF BALLOON CELLS IN PATIENTS WITH FOCAL CORTICAL DYSPLASIA
Abstract number :
1.089
Submission category :
Year :
2003
Submission ID :
656
Source :
www.aesnet.org
Presentation date :
12/6/2003 12:00:00 AM
Published date :
Dec 1, 2003, 06:00 AM
Authors :
Candice M. Burrier, Zhong Ying, Berit E. Jacobson, Alicia Paullin, Shinichi Watanabe, Richard Prayson, William Bingamam, Imad M. Najm Neurology, Cleveland Clinic Foundation, Cleveland, OH; Anatomic Pathology, Cleveland Clinic Foundation, Cleveland, OH; Ne
Focal cortical dysplasia (FCD) is one of the common pathological causes of medically intractable epilepsy. FCD is characterized by the presence of dysmorphic neurons, laminar and comlumnar disorganization. A small number of patients with FCD have balloon cells (BC) intermixed with dysmorphic neurons. The cellular and molecular characteristics of BC remain unknown. The objective of the study was to determine the cellular characteristics of balloon cells.
Neocortical tissues resected from 3 patients with medically intractable focal epilepsy due to CD who underwent focal resection were studied. The diagnosis of CD and the presence of BC (large opalescent cells with eccentric nuclei) were confirmed in all 3 patients using cresylecht violet (CV) staining. Immunocytochemical staining (ICC) was done using antibodies against markers of stem/progenitor cells (CD 131 and Nestin), immature neurons ([beta] tubulin III; TUJ1), immature glia (Vimentin), mature neurons (MAP and NeuN), and astrocytes (GFAP).
Balloon cells were identified in the deeper layers of the cortex and the subjacent white matter. Most BC present in the subcortical white matter were immunopositive to CD131 or nestin antibodies. Immunopositivity to TUJ1 or vimentin was seen in balloon cells located in the deeper cortical layers. A small numbner of BC were immunopositive to mature neuronal (MAP, NeuN) or astrocytic (GFAP) markers. Confocal staining showed the lack of neuronal and glial co-localization.
These results show that ballon cells are a heterogeneous population with protein characteristics of stem cells, immature neurons/glia or mature neurons/astrocytes. The presence of stem cell/progenitor markers could be due to the occurrence of postnatal neurogenesis or may be the result of early embryonic insult that resulted in arrest of proliferation at early stages. Studies to test for these possibilities are currently under way in our laboratory.
[Supported by: Grants from the National Institute of Neurological Disorders and Stroke (NINDS) to IN]