AOCCN2017

Presentation information

Poster Presentation

[P3-147~204] Poster Presentation 3

Sat. May 13, 2017 10:00 AM - 3:40 PM Poster Room B (1F Argos F)

[P3-195] Microstructural changes of white matter of brain in Rett syndrome

Wang-Tso LEE (Graduate Institute of Brain and Mind Sciences, College of Medicine, National Taiwan University, Taiwan)

Background
To understand the disease-specific pathologic changes of each tract bundle in white matter in stable stage of Rett syndrome (RTT), we applied tract-specific analysis of the whole brain to investigate the alteration of white matter tracts by diffusion spectrum image.

Methods:
Subjects: 12 patients (age: 19.8± 4.6 1 males and 11 females) and 14 age- and sex-matched healthy controls (age: 19.4± 4.9, 1 male and 13 females) were recruited. The connectogram provides generalized fractional anisotropy (GFA) profiles of 76 white matter tract bundles.

Results: Comparing with healthy controls, GFAs in 23 of 76 tract bundles in RTT were significantly reduced after correction (p<0.0006), including bilateral arcuate fasciculus (AF), cingulum of hippocampus part, inferior longitudinal fasciculus(ILF), perpendicular fasciculus, and uncinated fasciculus(UF); left inferior frontal occipital fasciculus (IFOF), and cingulum of body part; right frontal aslent tract, superior longitudinal fasciculus II (SLF II), and SLF III. The callosal fibers in commissure system of genu, DLPFC part, VLPFC part, inferior parietal part, superior parietal part, and precuneus were significantly reduced. In RTT group, the value of GFA in anterior commissure (AC) showed higher than (RTT: 0.253 ±0.02; Control: 0.208 ±0.02, p=0.0004)
Conclusion: The altered microstructural integrity of white matter tracts in RTT were covered in whole brain, however, in systematically view, most of them were located in associative and commissure tract bundles. Surprisingly, the GFA value of anterior commissure in RTT was higher than controls, which might explain the well-preserved visual perception clinically.