Article type: Letter to Editor
Article
title: Lhermitte-duclos
disease (dysplastic cerebellar gangliocytoma): an overview
Journal: Formosan
Journal of Surgery
Year: 2020
Authors: Jamir Pitton Rissardo, Ana Letícia Fornari
Caprara
E-mail: jamirrissardo@gmail.com
ABSTRACT
Lhermitte-Duclos disease (LDD), also known as dysplastic cerebellar gangliocytoma, is a rare genetic disorder, which is characterized by abnormal development of the cerebellum that can increase intracranial pressure. In this context, it could occur isolated or associated with Cowden syndrome. Moreover, this disease has some histological hallmarks such as thickening and hypermyelination of the outer molecular layer, loss of Purkinje cells and white matter, dysplastic ganglion cells with rounded nuclei, and abundant mitochondria invading the inner granular layer. Here, we would like to highlight some important topics that together with the study of Che Chen et al. could lead to a better comprehension of LDD. In addition, we developed a mnemonic to remember the facts of LDD which is "DUCLOS."
Keywords: Lhermitte-duclos disease, gangliocytoma, cerebellum, mnemonic
Full text available at:
DOI
10.4103/fjs.fjs_96_19
Citation
Rissardo
JP, Caprara AL. Lhermitte-duclos disease (dysplastic cerebellar gangliocytoma): an overview. Formos J Surg 2020;53:79-80