An unusual case of Lhermitte-Duclos disease presenting with intratumoral hemorrhage: a case report.

An unusual case of Lhermitte-Duclos disease presenting with intratumoral hemorrhage: a case report.

World Neurosurg. 2018 Apr 03;:

Authors: Uchida D, Nakatogawa H, Inenaga C, Tanaka T

Abstract
BACKGROUND: Lhermitte-Duclos disease (LDD) is a rare neurological disease that causes a hamartomatous lesion in the cerebellum. LDD is usually associated with mass lesion effects, but with appropriate surgical treatments the clinical course is usually benign. We report a rare case of a patient with LDD with contrast enhancement on MRI who died of intratumoral hemorrhage.
CASE DESCRIPTION: We describe a 59-year-old woman with LDD, which was discovered after a routine MRI. She did not complain of any symptoms neither initially, nor after follow-up MRI suggested minor hemorrhage in the tumor. Eleven months after her first visit to our department, she suddenly lost consciousness and CT revealed massive intratumoral hemorrhage. Surgical decompression was done, but she died 17 days after the operation. Histopathological findings were consistent with LDD.
CONCLUSION: Hemorrhagic events can occur with LDD, even though the growth of the lesion is slow. We stress the need of long-term careful follow-up of LDD, especially for patients who present atypical enhancement on MRI. To avoid possible hemorrhagic events, surgical treatment should be considered even if only subtle changes are seen on neuroimaging.

PMID: 29625313 [PubMed - as supplied by publisher]

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