Abstract

LARA-TORRES, Héctor Ricardo et al. Lhermitte-Duclos disease: Case report and review of literature. Bol. Med. Hosp. Infant. Mex. [online]. 2006, vol.63, n.6, pp.402-407. ISSN 1665-1146.

Introduction. Dysplastic cerebellar gangliocytoma was first described in 1920 by Lhermitte and Duelos, authors who gave their name to the entity. It is a rare condition, which is characterized by a very slowly growing unilateral tumor mass of the cerebellar cortex, producing increased intracranial pressure. In 1991 Padberg et al. described the association of Lhermitte-Duclos disease and Cowden syndrome. The relationship between these 2 entities has been associated with mutations of the phosphatase and tensin homolog deleted on chromosome (PTEN) gene. Magnetic resonance imaging findings are the hallmark of this entity, and the diagnosis is established without hystopathologic studies. Case report. We inform a case of a 17 years old girl with Lhermitte-Duclos of the right cerebellar hemisphere without Cowden syndrome.

Keywords : Lhermitte-Duclos disease; cerebellar dysplastic gangliocytoma; Cowden's syndrome.

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