Abstracts

MRI CHARACTERIZATION OF CEREBELLAR TUBERS IN TUBEROUS SCLEROSIS COMPLEX: LONGITUDINAL FOLLOW UP AND ASSOCIATIONS WITH CLINICAL MANIFESTATIONS

Abstract number : 3.255
Submission category : 5. Neuro Imaging
Year : 2014
Submission ID : 1868703
Source : www.aesnet.org
Presentation date : 12/6/2014 12:00:00 AM
Published date : Sep 29, 2014, 05:33 AM

Authors :
Akira Yogi, Yoko Hirata, Joyce Wu, Patrick Pan, Gary Mathern and Noriko Salamon

Rationale: The purpose of this study is to review interval MRI feature change of cerebellar tubers in tuberous sclerosis complex (TSC) patients, and to demonstrate the relationship between imaging findings and clinical features. Methods: Twenty-five patients (0.4-23.8 year old, mean age 7.3; 33 female, 17 male) with 28 cerebellar tubers were enrolled from UCLA TSC cohort out of 75 TSC patients. Two observers retrospectively reviewed the imaging characteristics of cerebellar tubers as following: number, location, signal characteristics on T1WI and T2WI, the presence of contour deformity, the presence of contrast enhancement, associated vascular anomaly, presence of SEGAs, internal cystic degeneration, autism, and infantile spasms using Chi-square test. In order to evaluate the size of abnormal vessels, mean diameters of these vessels were measured and compared with the mean diameters of normal vessels at the contralateral side using unpaired t test. Cerebellar tubers were also assessed if they showed an interval change on follow-up MRI. Results: Twenty-two patients had one cerebellar tuber and 3 patients had two. 14 tubers were seen in the right hemisphere. 9 tubers were seen in superior semilunar lobule, 4 tubers were seen in inferior semilunar lobule, and 15 tubers were seen in superior and inferior semilunar lobule. All tubers showed hyperintensity on T2WI, and showed isointensity or hypointensity on T1WI. Three tubers showed hypointensity on T2WI due to calcification. All tubers showed contour retraction change, 9 tubers (32%) showed contrast enhancement, and 11 tubers (39%) showed enlarged vessels running through the contour retraction. Mean diameter of the vessels running through the tuber was significantly larger than the diameter of control vessels (p<0.01) (Fig. 1). Furthermore, one case developed a parenchymal hemorrhage adjacent to the abnormal vessel (Fig. 2). 21 patients with 24 tubers had follow-up MR, and 15 (63%) showed interval changes. Cerebellar tubers with interval change showed significant relationship with the presence of SEGA (p<0.01). No statistical significance was observed between cerebellar tubers and presence of autism or infantile spasm. Conclusions: 33 % of TSC patient showed cerebellar tubers and 63% of cerebellar tubers demonstrate interval change in imaging features. The development of "dilated" vessels running through the contour deformity of the tuber was seen in 39 % of the cases and this may have risk of hemorrhage. SEGA may be related to change of cerebellar tubers, and genetic correlation may be assessed in the future research.
Neuroimaging