49. Lhermitte-duclos disease (dysplastic cerebellar gangliocytoma): an overview

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:

https://www.e-fjs.org/article.asp?issn=1682-606X;year=2020;volume=53;issue=2;spage=79;epage=80;aulast=Rissardo

 

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