Pediatric Neurology Unit, Department of Pediatrics, Navarra Health Service, Pamplona, Spain.
Neuroradiology. 2012 Aug;54(8):863-71. doi: 10.1007/s00234-012-1006-z. Epub 2012 Jan 21.
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 hypoxic-ischemic brain injury, and metabolic diseases. Considering this clinical diversity, we hypothesized that CTTH might primarily mirror a physiologic process.
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).
CTTH were detected in 4/17 WS+VGB+ children (24%), 4/34 WS+VGB- children (12%), 18/296 WS-VGB- children (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 of WS patients younger than 25 months of age (CTTH present in 7/40) with age-matched controls (CTTH present in 8/112) revealed that CTTH tend to be more frequent among WS patients in general.
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 endo- or exogeneous factors.
在 T2 加权 MRI(CTTH)上观察到的脑中央被盖束高信号在各种临床情况下都可见,包括接受氨己烯酸(VGB)治疗的婴儿痉挛症(WS)患儿、缺氧缺血性脑损伤和代谢性疾病患儿。鉴于这种临床多样性,我们假设 CTTH 可能主要反映了一种生理过程。
我们回顾性分析了源自以下四个不同组的中央被盖束的脑 MRI 数据:(1)接受 WS 合并 VGB 治疗的患儿(WS+VGB+);(2)接受 WS 但未接受 VGB 治疗的患儿(WS+VGB-);(3)患有不同神经疾病的患儿(WS-VGB-;年龄最大 15 岁);(4)年龄小于 25 个月的对照组(这个年龄包括 WS 的发病高峰年龄)。
在 17 名 WS+VGB+患儿中有 4 名(24%)、34 名 WS+VGB-患儿中有 4 名(12%)、296 名 WS-VGB-患儿中有 18 名(6%)和 112 名对照组中有 8 名(7%)检测到 CTTH。独立于潜在的诊断,CTTH 在婴儿早期出现高峰年龄,在 4 个月前和 7 岁后均未发现。接受或未接受 VGB 治疗的 WS 患儿中 CTTH 的发生率相似,因此 VGB 治疗似乎对病因的影响较小。然而,将年龄小于 25 个月的 WS 患儿(7/40 例 CTTH 阳性)与年龄匹配的对照组(8/112 例 CTTH 阳性)进行比较,发现 WS 患儿 CTTH 的发生率普遍较高。
我们的研究表明 CTTH 代表了一种与成熟相关的生理过程。WS 患儿中 CTTH 的高发生率表明,这种生理过程可能受到内源性或外源性因素的影响。