title: Epileptogenic but MRI-normal perituberal tissue in Tuberous Sclerosis Complex contains tuber-specific abnormalities.
abstract: BACKGROUND
  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.
authors: Alexander A
authors: AA
authors: Sosunov
authors: Robert A
authors: RA
authors: McGovern
authors: Charles B
authors: CB
authors: Mikell
authors: Xiaoping
authors: X
authors: Wu
authors: David G
authors: DG
authors: Coughlin
authors: Peter B
authors: PB
authors: Crino
authors: Howard L
authors: HL
authors: Weiner
authors: Saadi
authors: S
authors: Ghatan
authors: James E
authors: JE
authors: Goldman
authors: Guy M
authors: GM
authors: McKhann
mesh_terms: D000293:Adolescent; D001253:Astrocytes / Q000473:pathology*; D001921:Brain / Q000002:abnormalities / Q000473:pathology*; D002540:Cerebral Cortex / Q000002:abnormalities / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005911:Gliosis / Q000150:complications / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D018613:Microscopy, Confocal; D009474:Neurons / Q000473:pathology*; D012640:Seizures / Q000473:pathology*; D014402:Tuberous Sclerosis / Q000473:pathology*