Thomas B, Krishnamoorthy T, Radhakrishnan V V, Kesavadas C
Department of Imaging Sciences and Interventional Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.
Neuroradiology. 2007 Sep;49(9):733-8. doi: 10.1007/s00234-007-0241-1. Epub 2007 Jun 5.
Lhermitte-Duclos disease (LDD, dysplastic gangliocytoma) is an extremely rare cerebellar lesion of uncertain etiology. The debate as to whether it constitutes a neoplastic, malformative, or hamartomatous lesion is still continuing. In this report we explore the usefulness of susceptibility-weighted imaging (SWI), diffusion weighted imaging (DWI), perfusion imaging, and chemical shift imaging (CSI) in demonstrating the pathology and pathophysiology in two patients with LDD.
MR imaging of the brain and the cervicodorsal spine was performed on a 1.5-T scanner in a 47-year-old woman presenting with numbness and paresthesia of both upper and lower limbs, and in a 17-year-old male with right frontal headache associated with neck pain.
Routine imaging in the first patient showed a left-side cerebellar mass with characteristic 'tiger-striped' thick folia associated with Chiari I malformation, tonsillar herniation and cervicodorsal syringomyelia and in the second patient a right cerebellar mass with similar findings. The SWI demonstrated the characteristic deep running veins between the folia, which is thought to be the cause for vascular contrast enhancement. Diffusion showed a T2 shine-through effect with mild increased diffusivity, and perfusion showed increase in relative cerebral blood volume, relative cerebral blood flow, and mean transit time in the lesion. MR spectroscopy demonstrated reduction in metabolites and a prominent lactate peak in both the patients. The pathological and pathophysiological significance of these findings is discussed.
MRI with the newer imaging capabilities can demonstrate the pathology and pathophysiology in Lhermitte-Duclos disease better. SWI helps in detecting the veins around the thickened folia.
Lhermitte-Duclos病（LDD，发育异常性神经节细胞瘤）是一种病因不明的极为罕见的小脑病变。关于它是肿瘤性、发育异常性还是错构瘤性病变的争论仍在继续。在本报告中，我们探讨了磁敏感加权成像（SWI）、扩散加权成像（DWI）、灌注成像和化学位移成像（CSI）在两名LDD患者中显示病理和病理生理情况的作用。
对一名47岁出现双上肢和双下肢麻木及感觉异常的女性患者以及一名17岁伴有与颈部疼痛相关的右额部头痛的男性患者，在1.5-T扫描仪上进行脑部和颈胸段脊柱的磁共振成像检查。
首例患者的常规成像显示左侧小脑肿块，伴有特征性的“虎纹”状增厚小叶，合并Chiari I畸形、扁桃体疝和颈胸段脊髓空洞症；第二例患者为右侧小脑肿块，有类似表现。SWI显示小叶间特征性的深部走行静脉，这被认为是血管对比增强的原因。扩散成像显示T2透射效应，扩散率轻度增加，灌注成像显示病变处相对脑血容量、相对脑血流量和平均通过时间增加。磁共振波谱显示两名患者的代谢物减少且乳酸峰明显。讨论了这些发现的病理和病理生理意义。
具备更新成像能力的MRI能更好地显示Lhermitte-Duclos病的病理和病理生理情况。SWI有助于检测增厚小叶周围的静脉。
