Advanced Imaging Techniques in Lhermitte-Duclos Disease (LDD): A Case Report and Brief Literature Review

Abstract

Lhermitte-Duclos disease (LDD), also called dysplastic gangliocytoma of the cerebellum, is a rare condition described in 1920. It represents a disorganization of cerebellar architecture with overgrowth of cerebellar ganglion cells which replace granular cells and Purkinje cells. In this report we present the case of a 62-year-old male affected by this disease, as well as literature review of the clinical, morphological and functional radiological findings.

Lhermitte-Duclos disease in a 51-year old patient

“…The low Cho/NAA ratio has been linked to the diagnosis of LDD [6] . However, non-significant MRS findings in LDD have been reported [16] , just as in our patient.…”

“…DWI and ADC images in our patient showed no diffusion restriction. Some LDD cases showed diffusion restriction, likely due to hypercellularity and dense collection of axons [16] . While cases of LDD showing increased cerebral blood flow on perfusion studies have been reported [11] , our patient perfusion studies showed no significant elevation in cerebral blood flow.…”

Lhermitte-Duclos Disease and Cowden Syndrome: A Case Report and Literature Review

“…Of these, 5 reports are Brazilian. [9][10][11][12][13][14] Therefore, this article is the 6th report of LDD and the first to report the association with CS in Brazil.…”