Sanna Suoranta MD, Presenter: Nothing to Disclose

Kirsi Katariina Holi MSc, PhD, Abstract Co-Author: Nothing to Disclose

Eini Irene Niskanen PhD, Abstract Co-Author: Nothing to Disclose

Paivi Koskenkorva MD, Abstract Co-Author: Nothing to Disclose

Reetta Kalviainen, Abstract Co-Author: Nothing to Disclose

Ritva Liisa Vanninen MD, Abstract Co-Author: Nothing to Disclose

To investigate the feasibility of three-dimensional MRI texture analysis (3DTA) in the detection of subtle white matter (WM) and deep gray matter (GM) changes in EPM1. EPM1 is a rare neurodegenerative disorder caused by the mutations in the Cystatin B gene (CSTB). Despite the severe neurological symptoms no focal MR changes of the brain are found in visual assessment. Diffusion tensor imaging (DTI) in humans and mice has indicated widespread WM degeneration, and voxel-based morphometry (VBM) has revealed GM atrophy in EPM1.

Sixteen genetically verified patients with EPM1 and 16 healthy controls underwent MRI (MPRage, 1.5 T, Siemens Avanto) and 3DTA (MaZda software). Volumes of interest (VOIs) were placed manually in WM and deep GM covering as large volumes of the anatomical structures as possible. Altogether 223 different texture parameters per each VOI were computed. Textural differences between EPM1 patients and healthy controls were analyzed by the Mann-Whitney U test.

Visual assessment revealed no focal signal changes. Compared to the healthy controls, EPM1 patients showed statistically significant textural differences both in WM and GM. Compared to the WM VOIs, textural differences predominated in the deep GM. In right thalamus 28 %, left thalamus 37 %, and right putamen 26 % of the textural parameters differed amongst the 223 parameters analyzed. In WM, numbers of differing parameters were less frequent; left pons 19 %,corpus callosum genu 12 %, corpus 10 % and splenium 18 %; left corona radiate 14 %; right centrum semiovale 14 %. The number of differing parameters was less than 10 % in the remaining VOIs. The differing textural features included parameters based on histogram, gradient, co-occurrence matrix and run-length matrix.

WM textural alterations are widespread but less obvious than the deep GM findings. The 3DTA findings indicate that the texture of WM and deep GM in EPM1 patients is more coarse, complex and heterogeneous than in controls supporting widespread WM pathology in line with the previous DTI findings.

3DTA is able to reveal subtle morphological changes in MR images that cannot be detected by visual inspection. In patients with EPM1 3DTA is more sensitive to show alterations in deep GM than in WM.

Suoranta, S, Holi, K, Niskanen, E, Koskenkorva, P, Kalviainen, R, Vanninen, R, Three-dimensional MRI Texture Analysis Reveals Subtle Textural Alterations in the White Matter and Deep Gray Matter in Progressive Myoclonic Epilepsy Type 1 or Unverricht-Lundborg Disease (EPM1).  Radiological Society of North America 2013 Scientific Assembly and Annual Meeting, December 1 - December 6, 2013 ,Chicago IL. http://archive.rsna.org/2013/13015961.html