Academic Commons

Articles

Epileptogenic but MRI-normal perituberal tissue in Tuberous Sclerosis Complex contains tuber-specific abnormalities

Sosunov, Alexandre A.; McGovern III, Robert Allen; Mikell, Charles B.; Wu, Xiaoping; Coughlin, David G.; Crino, Peter B.; Weiner, Howard L.; Ghatan, Saadi; Goldman, James E.; McKhann II, Guy M.

Introduction: Recent evidence has implicated perituberal, MRI-normal brain tissue as a possible source of seizures in tuberous sclerosis complex (TSC). Data on aberrant structural features in this area that may predispose to the initiation or progression of seizures are very limited. We used immunohistochemistry and confocal microscopy to compare epileptogenic, perituberal, MRI-normal tissue with cortical tubers. Results: In every sample of epileptogenic, perituberal tissue, we found many abnormal cell types, including giant cells and cytomegalic neurons. The majority of giant cells were surrounded by morphologically abnormal astrocytes with long processes typical of interlaminar astrocytes. Perituberal giant cells and astrocytes together formed characteristic “microtubers”. A parallel analysis of tubers showed that many contained astrocytes with features of both protoplasmic and gliotic cells. Conclusions: Microtubers represent a novel pathognomonic finding in TSC and may represent an elementary unit of cortical tubers. Microtubers and cytomegalic neurons in perituberal parenchyma may serve as the source of seizures in TSC and provide potential targets for therapeutic and surgical interventions in TSC.

Files

  • thumnail for 40478_2015_Article_191.pdf 40478_2015_Article_191.pdf binary/octet-stream 3.27 MB Download File
  • thumnail for art_9069316334067008113.zip art_9069316334067008113.zip binary/octet-stream 3.16 MB Download File

More Information

Published In
Acta Neuropathologica Communications
Publisher DOI
https://doi.org/10.1186/s40478-015-0191-5
Volume
3
Issue
17
Pages
1 - 15
Publisher
BioMed Central
Publication Origin
London
Academic Units
Neurological Surgery
Pathology and Cell Biology
Academic Commons provides global access to research and scholarship produced at Columbia University, Barnard College, Teachers College, Union Theological Seminary and Jewish Theological Seminary. Academic Commons is managed by the Columbia University Libraries.