Assessing a dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) with 7T MR imaging

Abstract

Lhermitte-Duclos disease (LDD; dysplastic cerebellar gangliocytoma) is a rare hamartomatous lesion of the cerebellar cortex and this was first described in 1920. LDD is considered to be part of the autosomal-dominant phacomatosis and cancer syndrome Cowden disease (CS). We examined the brain of a 46-year-old man, who displayed the manifestations of CS, with 7 Tesla (T) and 1.5 T MRI and 1.5 T MR spectroscopy (1H-MRS). We discuss the possible benefits of employing ultrahigh-field MRI for making the diagnosis of this rare lesion.

Keywords: 7 Tesla; Dysplastic cerebellar gangliocytoma; Lhermitte-Duclos disease; Magnetic resonance (MR); Susceptibility-weighted imaging.

Fig. 1

Dysplastic cerebellar gangliocytoma in 46-year-old man. A. 1.5T T2-weighted axial image reveals left cerebellar mass with typical 'tiger striped' pattern of alternating inner hyperintense and outer hypointense layers (black arrows). B. Proton-MR spectroscopy (TE 135 ms) shows inverted lactate peak and reduction in choline (white arrows). C, D. 1.5T susceptibility weighted minimal intensity projection image depicts single veins running deep between thick folia (arrowheads), whereas 7T susceptibility weighted image demonstrates compression of dentate nucleus (open arrow in D) and multiple, draining veins in greater detail. E, F. Due to increased sensitivity for susceptibility effects, 7T T2^* weighted image (F) shows more peritumoral veins (white arrows) than corresponding 1.5T T2^* image (E).