Lhermitte-Duclos disease: Difference between revisions
Jump to navigation
Jump to search
No edit summary |
|||
| Line 19: | Line 19: | ||
{{SK}} Dysplastic cerebellar gangliocytoma | {{SK}} Dysplastic cerebellar gangliocytoma | ||
== Overview == | |||
'''Lhermitte-Duclos disease''' (dysplastic gangliocytoma of the cerebellum, LDD) is a [[rare diseases|rare]], slowly growing [[tumor]] of [[cerebellum]], sometimes considered as [[hamartoma]], characterized by diffuse [[hypertrophy]] of the [[stratum granulosum]] of the cerebellum. It is often associated with [[Cowden syndrome]] and is [[pathognomonic]] for this disease. | |||
== Historical Perspective == | |||
It was described by Jacques Jean Lhermitte and P. Duclos in 1920. | |||
It was described by Jacques Jean Lhermitte and P. Duclos in 1920 | |||
==Epidemiology== | ==Epidemiology== | ||
[[Lhermitte-Duclos disease]] is a rare entity; approximately 220 cases of LDD have been reported in medical literature | [[Lhermitte-Duclos disease]] is a rare entity; approximately 220 cases of LDD have been reported in medical literature. It's most common in 3. and 4. decade of life. | ||
==Etiology== | ==Etiology== | ||
| Line 42: | Line 41: | ||
==References== | ==References== | ||
<references/> | <references /> | ||
==External links== | ==External links== | ||
Revision as of 15:29, 15 April 2019
| Lhermitte-Duclos disease | |
 | |
|---|---|
| OMIM | 158350 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Dysplastic cerebellar gangliocytoma
Overview
Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum, LDD) is a rare, slowly growing tumor of cerebellum, sometimes considered as hamartoma, characterized by diffuse hypertrophy of the stratum granulosum of the cerebellum. It is often associated with Cowden syndrome and is pathognomonic for this disease.
Historical Perspective
It was described by Jacques Jean Lhermitte and P. Duclos in 1920.
Epidemiology
Lhermitte-Duclos disease is a rare entity; approximately 220 cases of LDD have been reported in medical literature. It's most common in 3. and 4. decade of life.
Etiology
Cowden disease is caused by mutations of PTEN gene.
Clinical signs
Main clinical signs are:
References
External links