caa a22 4500 463179692 CHVBK 20180406164835.0 cr unu---uuuuu 170326e20070701xx s 000 0 eng 10.1007/s00381-006-0271-8 doi (NATIONALLICENCE)springer-10.1007/s00381-006-0271-8 Lhermitte-Duclos disease [Elektronische Daten] [Raj Kumar, Vivek Vaid, Samir Kalra] Background: Lhermitte-Duclos disease (LDD; dysplastic gangliocytoma of the cerebellum) is a rare hamartoma of the cerebellum. It was first described by Lhermitte and Duclos in 1920 as "Sur un ganglioneurome diffus du cortex du cervelet” (Lhermitte and Duclos [Bull Assoc Fr Etude Cancer 9:99-107, 1920]). Since then, due to its rarity and the variability of its presentation, the diagnosis is often missed. It is not only important for the neurosurgeon and the pathologist to be aware of this entity so as to differentiate from malignant lesions but also to distinguish it from other cerebellar malformations, which may be hypoplastic or dysplastic, focal or diffuse in nature, with brain stem and cerebral involvement, and varied natural course. Illustrative case: We report a case of LDD in a 16-year-old boy. This case exemplifies the rarity of the disease leading to lack of awareness with which the diagnosis was easily missed. We also take this opportunity to discuss the clinical presentation, neuroradiological appearance, and a brief summary of histopathological findings, pathogenetic considerations, and neurogenetical aspects. Springer-Verlag, 2007 Lhermitte-Duclos disease (LDD) nationallicence Dysplastic gangliocytoma of the cerebellum nationallicence Kumar Raj Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, 226014, U.P., Lucknow, India aut Vaid Vivek Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, 226014, U.P., Lucknow, India aut Kalra Samir Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, 226014, U.P., Lucknow, India aut Child's Nervous System Springer-Verlag 23/7(2007-07-01), 729-732 0256-7040 23:7<729 2007 23 381 https://doi.org/10.1007/s00381-006-0271-8 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00381-006-0271-8 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Kumar Raj Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, 226014, U.P., Lucknow, India aut NATIONALLICENCE 700 1- Vaid Vivek Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, 226014, U.P., Lucknow, India aut NATIONALLICENCE 700 1- Kalra Samir Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, 226014, U.P., Lucknow, India aut NATIONALLICENCE 773 0- Child's Nervous System Springer-Verlag 23/7(2007-07-01), 729-732 0256-7040 23:7<729 2007 23 381 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer