Lhermitte-Duclos disease: A case report with radiologic-pathologic correlation

Lhermitte-Duclos disease (LDD) stems from the development of a rare benign lesion of uncertain pathogenesis that distorts the normal cerebellar laminar cytoarchitecture. We explored the lesion's appearance on conventional magnetic resonance imaging (MRI) combined with susceptibility-weighted imaging, diffusion-weighted imaging, perfusion imaging, or arterial spin labeling. Although many cases of LDD have been previously reported in the literature, the radiologic-pathologic correlation has been described in only a few of these cases. To the best of our knowledge, this is the first case report to provide detailed information about the radiologic-pathologic correlation of LDD. A 48-year-old woman presented with left facial tics, occipital headache, and dizziness for 1 month. MRI revealed a left cerebellar lesion with hypointensity on T1-weighted images. On T2-weighted images, the mass was hyperintense with tigroid appearance due to alternating high and normal signal intensities. High signal intensity was noted on fluid-attenuated inversion recovery images. Magnetic resonance spectroscopy indicated decreased level of choline (Cho), -acetyl aspartate, and myoinositol with elevated level of lactate on the affected side. The lesion showed a bright signal on diffusion-weighted images, whereas apparent diffusion coefficient mapping revealed no disturbance of diffusion. The pathology of the excised lesion was consistent with LDD. MRI with advanced techniques can provide not only preoperative diagnosis but also better pathologic correlation.