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Romanian Journal of Neurology, Volume XIII, No. 4, 2014
ISSN 1843-8148  |  e-ISSN 2069-6094
ISSN-L 1843-8148
DOI: 10.37897/RJN

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LHERMITTE-DUCLOS DISEASE ASSOCIATED WITH MENINGIOMA – A RARE CASE

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ABSTRACT

Lhermitte-Duclos disease (LDD), also known as dysplastic gangliocytoma of the cerebellum is a benign disease, extremely rare, characterized by loss of normal cerebellar cortical architecture and focal thickening of the folia. Lhermitte-Duclos disease as a major CNS manifestation of Cowden disease is a rare condition. The association between Lhermitte-Duclos disease and meningioma is even more rare, only few cases having been documented in the literature. We present a very rare case of a 53-year-old woman who was admitted in our department for recurrent episodes of headache and epilepsy. Magnetic resonance imaging revealed the presence of a well-defined lesion with an abnormal laminated pattern of cortical architecture involving most of the right cerebellar hemisphere, compressing the fourth ventricle and also a meningioma. Complete removal of the meningioma and partial removal of the cerebellar lesion was performed and histopathological exam confirmed Lhermitte-Duclos disease.

Keywords: Lhermitte Duclos disease, meningioma

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