Klisch J, Juengling F, Spreer J, Koch D, Thiel T, Büchert M, Arnold S, Feuerhake F, Schumacher M
Department of Neuroradiology, University of Freiburg, Breisacher Str. 64, D-79106 Freiburg, Germany.
AJNR Am J Neuroradiol. 2001 May;22(5):824-30.
Lhermitte-Duclos disease (LDD) is a rare cerebellar lesion with features of both malformation and benign neoplasm. However, the fundamental nature of the entity, its pathogenesis, and the exact genetic alterations remain unknown. We describe MR findings (including perfusion- and diffusion-weighted images) in two patients with LDD, as well as findings from single-photon emission CT (SPECT), MR spectroscopy (MRS), and fluorodeoxyglucose (FDG) positron emission tomography (PET) that give additional information about tumor pathophysiology. MR imaging usually distinguishes the LDD by its characteristic "tiger-striped" appearance. The regions of increased regional cerebral blood volume (rCBV) within the lesion correlated closely to the regions of FDG-hypermetabolism and high thallium (201-Tl) uptake. Proton MRS revealed an increased level of lactate and decreased level of myo-inositiol and N-acetyl-aspartate, as observed in low-grade gliomas, but decreased levels of choline. Our cases indicate that the functional investigations give additional information about tumor pathophysiology and reflect the histopathologic controversial entity with both characteristics found in low-grade gliomas and characteristics not typical for tumors.
Lhermitte-Duclos病（LDD）是一种罕见的小脑病变，具有畸形和良性肿瘤的特征。然而，该病变的基本性质、发病机制以及确切的基因改变仍不清楚。我们描述了两名LDD患者的磁共振成像（MR）表现（包括灌注加权成像和扩散加权成像），以及单光子发射计算机断层扫描（SPECT）、磁共振波谱（MRS）和氟脱氧葡萄糖（FDG）正电子发射断层扫描（PET）的表现，这些检查提供了有关肿瘤病理生理学的额外信息。MR成像通常通过其特征性的“虎纹状”外观来鉴别LDD。病变内局部脑血容量（rCBV）增加的区域与FDG高代谢区域和高铊（201-Tl）摄取区域密切相关。质子MRS显示乳酸水平升高，肌醇和N-乙酰天门冬氨酸水平降低，这与低级别胶质瘤中观察到的情况相似，但胆碱水平降低。我们的病例表明，功能检查提供了有关肿瘤病理生理学的额外信息，并反映了具有低级别胶质瘤特征和非典型肿瘤特征的组织病理学争议性病变。
