Lhermitte Duclos Disease in the Absence of Cowdens - A Varied Presentation
|Rajesh Parameshwaran Nair1, Vinod Kumar2, Deopujari CE3 and Bhavna Nayal4*|
|1Senior Registrar of Neurosurgery, KMC, Manipal University, Manipal, India|
|2Associate Professor of Neurosurgery, KMC, Manipal University, Manipal, India|
|3Professor and Head of Neurosurgery, Bombay Hospital and Medical Research Centre, India|
|4Associate Professor, Department of Pathology, KMC, Manipal University, Manipal, India|
|Corresponding Author :||Bhavna Nayal
Associate Professor, Department of Pathology, Kasturba Medical College
Manipal University, Manipal 576104, India.
E-mail: [email protected]
|Received: November 27, 2015; Accepted: January 27, 2016; Published: January 29, 2016|
|Citation: Nair RP, Kumar V, Deopujari CE, Nayal B (2016) Lhermitte Duclos Disease in the Absence of Cowdens - A Varied Presentation. J Neurol Disord 4:260. doi:10.4172/2329-6895.1000260|
|Copyright: © 2016 Nair RP, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.|
Background: Lhermitte-Duclos is a rare hamartomatous lesion of the cerebellar cortex with both neoplastic and hamartomatous features. Also known as dysplastic cerebellar gangliocytoma, they usually occur in the setting of Cowden’s syndrome, an autosomal dominant condition characterized by multiple hamartomas and neoplastic lesions in skin and internal organs. Although enlargement of the internal granular layer of the cerebellum is a consistent finding in our case, the background setting of Cowden’s syndrome was not present in our case.
Case description: We present a unique case of LDD in the absence of Cowdens syndrome in a 58-year old gentleman who presented with cerebellar signs, a first to be reported in literature. Here we describe our clinical vignette. C
onclusion: Most of the cases of LDD, reported in literature, were associated with Cowdens disease, however our case is the first to present without Cowdens disease. To date no other similar cases have been reported in literature.