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CC BY-NC-ND 4.0 · Journal of Pediatric Epilepsy 2023; 12(02): 076-083
DOI: 10.1055/s-0042-1756717
Original Article

Cerebellar Tubers in Tuberous Sclerosis Complex Patients: New Imaging Characteristics and the Relationship with Cerebral Tubers

Akira Yogi 
1   Department of Radiology, University of the Ryukyus Hospital, Okinawa, Japan
2   Department of Radiological Science, David Geffen School of Medicine, University of California, Los Angeles, California, United States
,
Yoko Hirata
2   Department of Radiological Science, David Geffen School of Medicine, University of California, Los Angeles, California, United States
3   Department of Neurosurgery, Toho University Ohashi Medical Center, Tokyo, Japan
,
Michael Linetsky
2   Department of Radiological Science, David Geffen School of Medicine, University of California, Los Angeles, California, United States
,
Benjamin M. Ellingson
2   Department of Radiological Science, David Geffen School of Medicine, University of California, Los Angeles, California, United States
,
Noriko Salamon
2   Department of Radiological Science, David Geffen School of Medicine, University of California, Los Angeles, California, United States
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Abstract

Objective The imaging characteristics, evolution, and clinical features of cerebellar tubers in tuberous sclerosis complex (TSC) patients have not been well described. The purpose of this study is to investigate the imaging characteristics of cerebellar tubers, including their dynamic changes, and to evaluate the relationship with cerebral tubers in TSC patients.

Materials and Methods Two observers retrospectively reviewed 75 consecutive TSC patients to identify cerebellar tubers and to evaluate their imaging characteristics, including location, presence of retraction change, calcification, contrast enhancement, and the presence of an associated vascular anomaly, as well as dynamic changes in these characteristics. The number of cerebral tubers was compared between TSC patients with and without cerebellar tubers.

Results Twenty-five TSC patients with 28 cerebellar tubers were identified. All cerebellar tubers occurred within the lateral portions of the cerebellar hemispheres. Thirteen cerebellar tubers demonstrated calcification. Ten cerebellar tubers showed contrast enhancement, half of which demonstrated a zebra-like appearance. A vascular anomaly was associated with 12 tubers, one of which subsequently developed parenchymal hemorrhage. Fifteen cerebellar tubers demonstrated complex dynamic changes in size and contrast enhancement. Patients with cerebellar tubers had more cerebral tubers (p = 0.001).

Conclusion Cerebellar tubers demonstrate a specific distribution, suggesting a possible influence on higher brain function. The presence of an associated vascular anomaly may be an important imaging characteristic. Cerebellar tubers may be associated with a more severe manifestation of TSC, given their association with increased numbers of cerebral tubers. These findings may provide insights into the pathogenesis and clinical manifestations of cerebellar tubers in TSC patients.

Keywords

cerebellar tuber - tuberous sclerosis complex - associated vascular anomaly - dynamic change - anatomical location

Authors' Contributions

1. Guarantor of integrity of the entire study: A.Y. and N.S.


2. Study concepts: A.Y. and N.S.


3. Study design: A.Y. and N.S.


4. Data acquisition: A.Y., Y.H., B.M.E., and N.S.


5. Data analysis: A.Y., Y.H., B.M.E., and N.S.


6. Statistical analysis: A.Y. and N.S.


7. Manuscript preparation: A.Y.


8. Manuscript editing: A.Y., Y.H., M.L., B.M.E., and N.S.


9. Manuscript review: A.Y., Y.H., M.L., B.M.E., and N.S.




Publication History

Received: 14 May 2022

Accepted: 20 August 2022

Article published online:
07 October 2022

© 2022. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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