Lhermitte-Duclos disease: MR diffusion and spectroscopy

Abstract

Aim: To describe two cases of Lhermitte-Duclos disease studied with Diffusion MRI and MRi Spectroscopy.

Materials and methods: Two patients showing a space-occupying lesion in the cerebellar hemisphere were studied by conventional MRI associated with Diffusion MRI and Spectroscopy.

Results: Conventional MRI showed the typical morphological pattern of Lhermitte-Duclos disease with a slow-growing cerebellar mass mainly involving the cortex and non-enhancing thickened cerebellar folia. The mass exerted mild compression of the IV ventricle causing hydrocephalus On Diffusion imaging showed the lesion to be hyperintense due to residual T2-activity and hypointense on the ADC map. Spectroscopy was typical with an increased lactate peak and a reduced choline peak, indicating abnormal anaerobic glycolysis and demyelination, respectively.

Discussion: Lhermitte-Duclos disease is a neurological condition which presents several signs and symptoms related to the cerebellar mass. Cowden's syndrome, an autosomal dominant neoplastic disorder with malignant features is associated in 40% of cases. MRI associated with functional examinations such as Diffusion Imaging and Spectroscopy is the imaging modality of choice in LDD patients. They allow a definite diagnosis as the neuroradiological findings correlate well with molecular biology theories regarding the malformative nature of dysplastic cerebellar gangliocytoma.