We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
Gray and white matter abnormalities in children with type 2 and 3 SMA: A morphological assessment.
- Authors
Shen, Wanqing; Yan, Zi; Su, Shu; Xiang, Pei; Zhou, Qin; Zou, Mengsha; Yang, Zhiyun; Tang, Wen; Liang, Yujian; Chen, Yingqian
- Abstract
This study investigated the changes in brain gray and white matter structure in SMA patients and their correlation with the severity of the disease. A total of 43 SMA patients (including 22 type II and 21 type III SMA patients) and 37 healthy controls were evaluated by MRI. The gray matter volume, gray matter thickness, gray matter surface area, and white matter volume of designated brain regions automatically segmented by FreeSurfer, were compared. We evaluate clinical characteristics of SMA and study the correlation between clinical characteristics and structural changes. SMA showed significant bilateral cortical superficial area loss in the frontal, parietal, and temporal lobes and global white matter volume decreases. Moreover, these patients were also found with an increased mean thickness of entire brain and right gray matter. An increased right postcentral gyrus superficial area, right central sulcus volume, and white matter volume of the right postcentral were associated with higher HFMSE scores. Conclusion: Type 2 and 3 children SMA had extensive, multifocal, symmetrical gray and white matter alterations. Postcentral gyrus degeneration of SMA was associated with the severity of muscular atrophy. The lack of SMN protein possibly interacted with cerebellar structural changes in somatosensory areas. What is Known: • MRI has found brain changes in SMA patients, however, there is no unified conclusion and lack of correlation with clinical degree in children SMA with type 2-3. What is New: • Type II and II children SMA had extensive, multifocal, symmetrical gray and white matter alterations. Postcentral gyrus degeneration of SMA was associated with the severity of muscular atrophy. Cerebellar structural changes in somatosensory areas may attribute to the lack of SMN protein.
- Subjects
GRAY matter (Nerve tissue); WHITE matter (Nerve tissue); BRAIN cortical thickness; MUSCULAR atrophy; SOMATOSENSORY cortex; LEUKOENCEPHALOPATHIES
- Publication
European Journal of Pediatrics, 2024, Vol 183, Issue 3, p1381
- ISSN
0340-6199
- Publication type
Article
- DOI
10.1007/s00431-023-05397-z