MRI in Lhermitte-Duclos disease

Abstract

Lhermitte-Duclos disease (LDD) is a disorder sometimes referred to as a dysplastic gangliocytoma of the cerebellum. This is a focally indolent growth of the cerebellar cortex in which the folia enlarge due to a profusion of dysplastic cortical neurons and a thickening of the molecular layer. Loss of Purkinje cells and thinning of medullary white matter results. The enlarged folia lose their secondary foldings and asymmetrically expand the cerebellar hemisphere. These morphologic features produce a characteristic pattern on some CTs and all MRIs, affording an opportunity for a preoperative diagnosis. MRI of a pathologic specimen suggested that the abnormal T1 and T2 signals corresponded to the atrophic folial white matter, thickened granule cell layer, and outer molecular layer. Because of inherent Hounsfield artifact in posterior fossa with CT, MRI is the imaging modality of choice, with better visualized striated pattern. The uniqueness of these imaging features obviates the need for an obligatory biopsy for asymptomatic patients and either permits more definitive planning for surgical decompression or, in restricted lesions, guides more assuredly complete excision of the cerebellar mass.