Lhermitte-Duclos disease associated with Cowden syndrome

Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia
Tze-Ching Tan, Luen-Cheung Ho

Abstract

Lhermitte-Duclos disease or dysplastic gangliocytoma of the cerebellum, is a rare cerebellar lesion, which can cause mass effects in the posterior fossa. It may occur sporadically, or in association with Cowden syndrome. Cowden syndrome or multiple hamartoma-neoplasia syndrome, is an uncommon autosomal dominant condition characterized by mucocutaneous lesions and systemic malignancies. We report two patients with Lhermitte-Duclos disease and associated Cowden syndrome. The clinical, radiological and histopathological features and management strategies of this rare disease complex are discussed.

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Citations

Jun 14, 2012·The Cerebellum·Omid R HaririJaved Siddiqi
May 10, 2011·Acta neurochirurgica·Maziar BehbahaniWilliam Stewart
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Dec 10, 2016·Journal of Child Neurology·Megan ZakPaul Maertens
Sep 18, 2015·Neurologia i neurochirurgia polska·I StępniakJ Zaremba

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