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

Abstract

Background: 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. Case Report: 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), N -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. Conclusion: MRI with advanced techniques can provide not only preoperative diagnosis but also better pathologic correlation.

MR imaging features of Lhermitte–Duclos disease

“…According to the literature, only 3 patients underwent SWI (1 from our center). [ 17 , 20 ] In fact, the application of SWI in LDD is mainly focused on the anatomical structure—the display of the veins running between the folia (enhancement can also be observed initially, and the interlobular veins are thickened in some cases; Fig. 5 ).…”

Lhermitte–Duclos Disease Mimicking Cerebellopontine Angle Tumor: A Case Report

“…There are reported cases of contract-enhanced images similar to our case, although LDD does not present any obvious contrasting pattern. 2,3 For the differential diagnosis of the cases with tumors located in the cerebellum, medulloblastoma, astrocytoma and cerebellar infarction should be considered. 10 Because the tumor was located at the cerebellopontine angle in our case, the tumors located in this region should be considered for the differential diagnosis.…”

“…1 LDD is generally located in the cerebellar hemispheres and vermis. 2,3 LDD can occur at any age; however, it is frequently diagnosed in people aged 20 to 40 years. 4,5 The most common symptoms are headache, dizziness, nausea, vomiting, loss of balance, and impaired vision.…”

Imaging of brain tumors in children: the basics—a narrative review

“…On MRI, the lesion appears as a nonenhancing mass affecting one, or less frequently, both cerebellar hemispheres, causing a mild mass effect, and has a typical striated pattern described as "corduroy/laminated" appearance (Figure 8) (3,71,72). The main differential diagnosis includes some cerebellar malformative features with a pseudotumoral appearance, and other low-grade gliomas arising from the cerebellar hemispheres.…”