Department of Pediatric Radiology, Necker Children's Hospital, Assistance Publique-Hôpitaux de Paris, University of Paris cité, 149 rue de Sèvres, 75015, Paris, France.
Imagine Institute for Genetic Diseases, INSERM U1163, Paris, France.
Eur Radiol. 2023 Jan;33(1):196-206. doi: 10.1007/s00330-022-09036-3. Epub 2022 Sep 6.
To study longitudinal changes in tuber and whole-brain perfusion in children with tuberous sclerosis complex (TSC) using arterial spin labeling (ASL) perfusion MRI and correlate them with pathological EEG slow wave activity and neurodevelopmental outcomes.
Retrospective longitudinal cohort study of 13 children with TSC, 3 to 6 serial ASL-MRI scans between 2 months and 7 years of age (53 scans in total), and an EEG examination performed within 2 months of the last MRI. Tuber cerebral blood flow (CBF) values were calculated in tuber segmentation masks, and tuber:cortical CBF ratios were used to study tuber perfusion. Logistic regression analysis was performed to identify which initial tuber characteristics (CBF value, volume, location) in the first MRI predicted tubers subsequently associated with EEG slow waves. Whole-brain and lobar CBF values were extracted for all patient scans and age-matched controls. CBF ratios were compared in patients and controls to study longitudinal changes in whole-brain CBF.
Perfusion was reduced in tubers associated with EEG slow waves compared with other tubers. Low tuber CBF values around 6 months of age and large tuber volumes were predictive of tubers subsequently associated with EEG slow waves. Patients with severe developmental delay had more severe whole-brain hypoperfusion than those with no/mild delay, which became apparent after 2 years of age and were not associated with a higher tuber load.
Dynamic changes in tuber and brain perfusion occur over time. Perfusion is significantly reduced in tubers associated with EEG slow waves. Whole-brain perfusion is significantly reduced in patients with severe delay.
• Tubers associated with EEG slow wave activity were significantly more hypoperfused than other tubers, especially after 1 year of age. • Larger and more hypoperfused tubers at 6 months of age were more likely to subsequently be associated with pathological EEG slow wave activity. • Patients with severe developmental delay had more extensive and severe global hypoperfusion than those without developmental delay.
使用动脉自旋标记（ASL）灌注 MRI 研究结节性硬化症（TSC）儿童结节和全脑灌注的纵向变化，并将其与病理 EEG 慢波活动和神经发育结果相关联。
对 13 名 TSC 儿童进行回顾性纵向队列研究，在 2 个月至 7 岁之间进行了 3 至 6 次连续 ASL-MRI 扫描（共 53 次扫描），并在最后一次 MRI 检查后 2 个月内进行了 EEG 检查。在结节分割掩模中计算结节脑血流（CBF）值，并使用结节：皮质 CBF 比值来研究结节灌注。进行逻辑回归分析，以确定首次 MRI 中哪些初始结节特征（CBF 值、体积、位置）预测随后与 EEG 慢波相关的结节。从所有患者扫描和年龄匹配的对照组中提取全脑和脑叶 CBF 值。比较患者和对照组之间的 CBF 比值，以研究全脑 CBF 的纵向变化。
与其他结节相比，与 EEG 慢波相关的结节灌注减少。6 个月左右时低的结节 CBF 值和大的结节体积是随后与 EEG 慢波相关的结节的预测指标。严重发育迟缓的患者比无/轻度迟缓的患者全脑灌注更严重，这种情况在 2 岁后变得明显，与更高的结节负荷无关。
随着时间的推移，结节和脑灌注的动态变化会发生。与 EEG 慢波活动相关的结节灌注明显减少。严重发育迟缓的患者全脑灌注明显减少。
与 EEG 慢波活动相关的结节比其他结节明显灌注不足，尤其是在 1 岁以后。
6 个月时更大和灌注不足的结节更有可能随后与病理性 EEG 慢波活动相关联。
严重发育迟缓的患者比无发育迟缓的患者具有更广泛和严重的全脑灌注不足。
