Preoperative diagnosis of Lhermitte-Duclos disease: MRI and 1H-MR spectroscopy findings

Abstract

Lhermitte-Duclos disease (LDD), also known as dysplastic gangliocytoma of the cerebellum, is a rare cerebellar disorder of uncertain etiology. LDD presents with highly typical findings on MRI. Furthermore, 1H-MR spectroscopy can provide additional information supporting the diagnosis. Herein, we present the MRI and 1H-MR spectroscopy findings of a 46-year-old LDD patient with a brief review of the relevant literature. The patient had a unilateral, cerebellar hemispheric mass with a layered appearance, resembling “tiger stripes”. On single-voxel 1H-MR spectroscopy, N-acetyl aspartate (NAA), cholin (Cho), creatinine (Cr) levels and NAA/Cr, Cho/Cr ratios were found to be within reference values, however, lactate level was elevated. In conclusion, although conventional MRI characteristics of LDD are typical, 1H-MR spectroscopy can provide additional and supportive information, revealing the benign nature of this rare lesion. Hence, preoperative diagnosis of LDD can be made with high accuracy using MRI and 1H-MR spectroscopy.