Management of unusual dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) in a developing country: Case report and review of the literature

Dysplastic cerebellar gangliocytoma or Lhermitte-Duclos disease (LDD) especially in children are extremely rare. In this report we add one further case to this rare entity. A three year old boy with a history of cerebellar and brain stem compression signs was presented in unconscious condition. Comp...

Full description

Saved in:
Bibliographic Details
Published in:Asian journal of neurosurgery 2016-04, Vol.11 (2), p.170-170
Main Authors: Golden, Nyoman, Tjokorda, Mahadewa, Sri, Maliawan, Niryana, Wayan, Herman, Saputra
Format: Article
Language:English
Subjects:
Care and treatment
CASE REPORT
CAT scans
Cerebrospinal fluid shunts
Diagnosis
Multiple hamartoma syndrome
Citations: Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Dysplastic cerebellar gangliocytoma or Lhermitte-Duclos disease (LDD) especially in children are extremely rare. In this report we add one further case to this rare entity. A three year old boy with a history of cerebellar and brain stem compression signs was presented in unconscious condition. Computerized tomography (CT) scan revealed a well defined lesion mixed with area of calcification in the right cerebellum and severe obstructive hydrocephalus. Ventricular shunting was performed followed by gross total tumor removal three days after shunting. In the follow up period, the patient showed almost total resolution of all neurological deficits. MRI has been an imaging tool to preoperatively diagnose this disease; otherwise it would be uncertain preoperative diagnosis. However, in this report we would like to emphasize that not all uncertain diagnosis of LDD leads to palliative treatment. Well demarcated lesion may account for the safe surgical resection of this disease.
ISSN:1793-5482
2248-9614
DOI:10.4103/1793-5482.145091