Cortical Tubers, Cognition, and Epilepsy in Tuberous Sclerosis

Comprehensive Epilepsy Program, University of Alberta Hospital, Edmonton, Alberta, Canada.
Pediatric Neurology (Impact Factor: 1.7). 05/2011; 44(5):328-32. DOI: 10.1016/j.pediatrneurol.2011.01.001
Source: PubMed


Tuberous sclerosis complex is an autosomal-dominant genetic disorder characterized by hamartomatous growth in various organs. Patients who have this disorder exhibit a high rate of epilepsy and cognitive problems. We investigated number of tubers, location, seizure types, and cognitive outcome, and we analyzed the relationships among them in our tuberous sclerosis patients in the Comprehensive Epilepsy Program at the University of Alberta. We also examined the seizure outcome after tuber resection. Our study cohort included 24 patients with tuberous sclerosis complex. We obtained seizure history, electroencephalogram, and neuropsychologic parameters. Magnetic resonance imaging was used to examine tuber numbers and locations. Ten patients underwent surgical removal of tubers responsible for intractable epilepsy. A negative correlation was found between the number of tubers and intelligent quotient score. Epilepsy surgery led to freedom from seizures in this patient group. We demonstrated that the total number and location of cortical tubers play a significant role in the extent of mental retardation in patients with tuberous sclerosis complex. In addition, patients with intractable seizures and well-defined epileptic focus had excellent surgical outcome.

25 Reads
    • "Cortical tubers are mostly supratentorial, in the frontal lobes, although infratentorial cerebellar tubers have been described as well.[25] The number and location of these tubers is associated with the degree of cerebral dysfunction including cognitive impairment, seizures, and autism.[26–28] There have been attempts to describe clinical severity based on MRI imaging characteristics [T1, T2, and fluid attenuated inversion recovery (FLAIR) signal] of cortical tubers,[29] but larger studies are needed to establish reliable criteria. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Tuberous sclerosis (TS), also known as Bourneville disease or Bourneville-Pringle disease, is an autosomal dominant genetic disorder classically characterized by the presence of hamartomatous growths in multiple organs. TS and tuberous sclerosis complex (TSC) are different terms for the same genetic condition. Both terms describe clinical changes due to mutations involving either of the two genes named TSC1 and TSC2, which regulate cell growth. The diagnosis of TSC is established using diagnostic criteria based on clinical and imaging findings. Routine screening and surveillance of patients with TSC is needed to determine the presence and extent of organ involvement, especially the brain, kidneys, and lungs, and identify the development of associated complications. As the treatment is organ specific, imaging plays a crucial role in the management of patients with TSC.
    Journal of Clinical Imaging Science 07/2011; 1(1):39. DOI:10.4103/2156-7514.83230
  • [Show abstract] [Hide abstract]
    ABSTRACT: The shortest common superstring (SCS) problem requires that the maximal overlays between a given set of strings be found. An efficient VLSI design of a systolic array for finding maximal overlays of strings is described. The systolic array consists of two types of cells, comparator and accumulator. The comparator cell is a combinational circuit, and the accumulator cell is sequential. Inverting half shift register stages are used to move the streams. Therefore two versions (positive and negative) of each of the two cells are needed. The positive accumulator cell circuit and cell layout are illustrated. Alternate cells in the systolic array are clocked by a different phase of a two-phase nonoverlapping clock
    Circuits and Systems, 1992., Proceedings of the 35th Midwest Symposium on; 01/1992
  • Journal of Medical Ultrasound 06/2011; 19(2):68-70. DOI:10.1016/j.jmu.2011.05.008
Show more