T2 hyperintense signal of the central tegmental tracts in children: Disease or normal maturational process?

Introduction Cerebral central tegmental tract hyperintense signal on T2-weighted MRI (CTTH) is known from various clinical conditions, including children treated with vigabatrin (VGB) for West syndrome (WS), with hypoxicischemic brain injury, and metabolic diseases. Considering this clinical diversity, we hypothesized that CTTH might primarily mirror a physiologic process. Methods We retrospectively analysed brain MRI data of the central tegmental tracts deriving from four different groups: (1) children with WS and VGB therapy (WS+VGB+), (2) children with WS but without VGB therapy (WS+VGB?), (3) children with different neurological diseases (WS?VGB?; maximum age 15 years), and (4) controls younger than 25 months of age (this age includes the peak age of WS). Results CTTH were detected in 4/17 WS+VGB+ children (24%), 4/34 WS+VGB? children (12%), 18/296 WS-VGBchildren (6%), and 8/112 controls (7%). Independently from the underlying diagnosis, CTTH showed a peak age during early infancy and were not found before 4 months and after 7 years of life. The rate of CTTH among WS children ± VGB therapy was similar so that VGB therapy seems of minor etiological impact. However, comparison ofWS patients younger than 25 months of age (CTTH present in 7/40) with agematched controls (CTTH present in 8/112) revealed that CTTH tend to be more frequent among WS patients in general. Conclusions Our study suggests that CTTH represents a physiological maturation-related process. The high prevalence of CTTH among patients with WS indicates that this physiological process may be modified by additional endoor exogeneous factors.