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CD34-immunoreactive balloon cells in cortical malformations

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Abstract

Balloon cells are histopathological hallmarks of various cortical malformations, i.e., focal cortical dysplasia (Taylor’s type, FCD IIb), hemimegalencephaly (HME) or cortical tubers (tuberous sclerosis, TSC). Whether this intriguing cell type results from similar pathogenetic pathways remains to be shown. Here, we analyzed the immunohistochemical distribution pattern of the CD34 epitope in surgical specimens from 34 patients with FCD IIb, compared to that of 6 patients with TSC and 3 patients with HME. In normal brain, CD34 occurs only transiently during neurulation, but cannot be detected in mature neuroectodermal cell progenies. In contrast, 58% of our patients showed CD34 immunoreactivity within a subpopulation of balloon cells. Interestingly, CD34-positive balloon cells were confined to the white matter, but never observed in neocortical layers. Furthermore, balloon cells expressing neurofilament protein were also restricted to white matter, whereas GFAP-positive balloon cells were observed either in white or gray matter location. Clinical characteristics did not significantly differ between patients with CD34-positive versus CD34-negative lesions. No significant correlation was found between CD34 expression and genetic alterations of the TSC1 gene, which is affected in many FCD and TSC patients and which plays a role in the regulation of cell size. Further studies are warranted to clarify the restricted expression of CD34 in balloon cells of the white matter.

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Authors and Affiliations

  1. Epilepsy Center, University of Freiburg, Breisacher Str. 64, 79106, Freiburg im Breisgau, Germany

    Susanne Fauser & Andreas Schulze-Bonhage

  2. Department of Neuropathology, University of Bonn, Sigmund-Freud Str. 25, 53105, Bonn, Germany

    Albert Becker

  3. Epilepsy Center, Krankenhaus Mara gGmbH, Karl-Siebold-Weg 11, 33617, Bielefeld, Germany

    Ingrid Tuxhorn, Heinz W. Pannek & Rainer Lahl

  4. Department of Neuropathology, University of Erlangen, Krankenhausstr. 8–10, 91054, Erlangen, Germany

    Michelle Hildebrandt & Ingmar Blümcke

  5. Neuropathological Reference Center for Epilepsy Surgery, University of Erlangen, Krankenhausstr. 8–10, 91054, Erlangen, Germany

    Michelle Hildebrandt & Ingmar Blümcke

  6. Department of Neurosurgery, University of Bonn, Sigmund-Freud Str. 25, 53105, Bonn, Germany

    Johannes Schramm

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  1. Susanne Fauser
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  2. Albert Becker
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  3. Andreas Schulze-Bonhage
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  4. Michelle Hildebrandt
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  5. Ingrid Tuxhorn
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  6. Heinz W. Pannek
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  7. Rainer Lahl
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  8. Johannes Schramm
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  9. Ingmar Blümcke
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Correspondence to Susanne Fauser.

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Fauser, S., Becker, A., Schulze-Bonhage, A. et al. CD34-immunoreactive balloon cells in cortical malformations. Acta Neuropathol 108, 272–278 (2004). https://doi.org/10.1007/s00401-004-0889-0

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  • DOI: https://doi.org/10.1007/s00401-004-0889-0

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