Reversal of abnormalities of myelination by thyroxine therapy in congenital hypothyroidism: localized in vivo proton magnetic resonance spectroscopy (MRS) study
Reversal of abnormalities of myelination by thyroxine therapy in congenital hypothyroidism: localized in vivo proton magnetic resonance spectroscopy (MRS) study
复制标题
DOI：
10.1016/s0165-3806(98)00081-9
复制
发表时间：
1998-08-08
期刊：
影响因子：
--
通讯作者：
Kochupillai, N
中科院分区：
文献类型：
--
作者：
Jagannathan, NR;Tandon, N;Kochupillai, N
中枢神经系统发育过程中甲状腺激素的缺乏会导致多种临床、解剖和生化缺陷。先天性甲状腺功能减退症新生儿延迟甲状腺素治疗可导致不可逆的脑损伤。我们已经使用本地化在体内质子磁共振波谱（MRS），以评估在不同地区的大脑在甲状腺素治疗前后的先天性甲状腺功能减退症的三名患者的生化变化。1例患者小脑和额叶出现异常脂质峰，经甲状腺素治疗后消失。与对照组相比，所有3例患者的NAA/（Cr + PCr）比值均显著降低[P < 0.009]，Cho/（Cr + PCr）比值显著升高[P < 0.008]，甲状腺素治疗后趋于正常。记录了与髓鞘成熟相关的各种生化异常，并发现这些异常在甲状腺素治疗中是可逆的。即使甲状腺素治疗开始于髓鞘形成异常被认为不可逆的年龄，也有可逆性的记录。此外，质子MRS显示先天性甲状腺功能减退症患者之间的生化异质性。(C)1998 Elsevier Science B. V.保留所有权利。
Deficiency of thyroid hormone during central nervous system ontogeny results in a variety of clinical, anatomical and biochemical defects. Delay in thyroxine therapy in newborns with congenital hypothyroidism leads to irreversible brain damage. We have used localized in vivo proton magnetic resonance spectroscopy (MRS) to assess biochemical changes in different regions of brain in three patients with congenital hypothyroidism before and after thyroxine therapy. An abnormal lipid peak which disappeared with thyroxine therapy was observed in cerebellum and frontal lobe in one patient. Statistically significant reduction of NAA/(Cr + PCr) [P < 0.009] and elevation of Cho/(Cr + PCr) [P < 0.008] ratios in comparison to controls were documented in all three patients which tended to normalise with thyroxine therapy. A variety of biochemical abnormalities relatable to myelin maturation were documented and these were found to be reversible on thyroxine therapy. Reversibility was documented even though thyroxine therapy was initiated at ages beyond which abnormalities in myelinogenesis are considered irreversible. Also, proton MRS revealed biochemical heterogeneity between patients with congenital hypothyroidism. (C) 1998 Elsevier Science B.V. All rights reserved.