Course and clinical impact of magnetic resonance imaging findings in diabetes insipidus associated with Langerhans cell histiocytosis
Course and clinical impact of magnetic resonance imaging findings in diabetes insipidus associated with Langerhans cell histiocytosis
复制标题
DOI：
10.1002/pbc.20003
复制
发表时间：
2004-07-01
影响因子：
3.2
通讯作者：
Gadner, H
中科院分区：
文献类型：
--
作者：
Grois, N;Prayer, D;Gadner, H
背景尿崩症（DI）是朗格汉斯细胞组织细胞增生症（LCH）最常见的后遗症。LCH患者病程中脑磁共振成像（MRI）结果的临床相关性和治疗影响尚不清楚。Procedure.在这项回顾性研究中，我们回顾了59例DI患者的113次脑MRI，其中17例连续随访的MRI检查结果与临床病程和治疗相关。结果在DI诊断时，71%的患者显示出柄部增厚，24%的患者在DI发作后5年以上进行的MRI上柄部仍然增厚，2例患者在DI发作前几个月柄部已经增厚。垂体柄厚度的变化是高度可变的，并没有明显的相关性与治疗。MRI上垂体厚度的回归与DI的临床恢复并不一致，除了一名最初部分DI的患者外，所有患者的DI均持续存在。垂体前叶激素缺乏的发生似乎与DI诊断时的柄增厚有关。在75%的DI患者中观察到颅面骨中的LCH病变，并且在DI诊断后5年或更长时间进行随访MRI的DI患者中，76%的DI患者具有脑实质神经退行性改变。结论.我们的研究表明，重复MR检查DI患者的评估垂体柄治疗的反应是有限的价值，但重要的颅面骨病变的监测和实质性CNS疾病的检测。(C)2004 Wiley-Liss，Inc.
Background. Diabetes insipidus (DI) is the most frequent sequela in Langerhans cell histiocytosis (LCH). The clinical relevance and therapeutic impact of brain magnetic resonance imaging (MRI) findings in LCH patients with LCH during the disease course is unclear. Procedure. In this retrospective study, we reviewed 113 brain MRIs from 59 DI patients, in 17 of these serial follow up MR-examination findings were correlated to the clinical course and therapy. Results. At DI diagnosis, 71% patients showed a thickened stalk, in 24% the stalk was still thickened on MRIs done more than 5 years after DI onset, and in two patients the stalk was already thickened several months before DI onset. The changes of the pituitary stalk thickness were highly variable and did not clearly correlate with the treatment. Regression of pituitary thickness on MRI did not concur with clinical recovery of DI, which persisted in all but one patient with initially partial DI. The occurrence of anterior pituitary hormone deficiencies appeared to be linked to a thickening of the stalk at DI diagnosis. LCH-lesions in the craniofacial bones were seen in 75% DI patients, and 76 % of DI patients with follow up MRIs done 5 years or longer after DI diagnosis had parenchymal neurodegenerative brain changes. Conclusions. Our study indicates that repeated MR-examinations in DI patients are of limited value for assessing a response to therapy in the pituitary stalk, but are important for the monitoring of craniofacial bone lesions and for the detection of parenchymal CNS disease. (C) 2004 Wiley-Liss, Inc.