Iranian Journal of Pathology

Lhermitte – Duclos Disease in a Young Adult: Rare Entity

Authors

Vaishali Walke 1
Sanjay Bijwe 2
Grace D’ Costa 3
Aashish Jawarkar 1

1 Dept. of Pathology, Grant Medical College & Sir J. J. Group of Hospitals, Byculla, Mumbai MS – India

2 Dept. of Pathology, Grant Medical College & Sir J. J. Group of Hospitals, Byculla, Mumbai MS , India

3 Dept. of Pathology, Grant Medical College & Sir J. J. Group of Hospitals, Byculla, Mumbai MS – India

Abstract
Lhermitte - Duclos disease also called dysplastic gangliocytoma of cerebellum is an extremely rare cerebellar lesion which share features of both malformation and neoplasm. The usual presentation is of raised intracranial pressure along with cerebellar signs. We report a case of 23 year male who presented with headache & diplopia. MRI was suggestive of the diagnosis. Subtotal excision of the mass along with shunt drainage was performed. Post operative course was uneventful. Histopathological features confirmed the diagnosis.
 

Keywords

Lhermitte Duclos Disease
Case Report
India
  1. Louis DN,Ohgaki H, WiestlerOD, Cavenee WK. WHO classification of tumors of central nervous system. 4th ed. Lyon: IARC;2007.
  2.  Di Lorndzo N, Lunardi P, Fortuna A. Granulomolecular hypertrophy of the  Cerebellum (Lhermitte - Duclos disease): Case report. J Neurosurgery.1984;60:644-6.
  3. Bero V, Sajko T, Talen-Hranilovic J, Negovetic L, NegovetiÊ L. Intraventricular hypertension due to Lhermitte -Duclos disease: case report. Acta Clin Croat    2000;39:181-6.
  4. Berger PC, Scheithauer BW. Surgical pathology of nervous system and its Covering. 3rd ed. New York:Churchill Livingston;1991.
  5. Nayil K, Wani M, Ramzan A, Shaheen F, Lone I, Wani A. Lhermitte - Duclos Disease with Syrinx: Case Report & Literature Review. Turk Neurosurg 2011;21:651-4.
  6.  Shingare AB, Patil NK, Sorte SZ. Dysplastic gangliocytoma of the cerebellum. Radiology 2009;251:298-303.
  7. Klisch J, Juengling F, Spreen J, Koch D, Thiel T, Büchert M, et al. Lhermitte - Duclos Disease: Assessment with MR Imaging, Positron Emission Tomography, Single –photon Emission CT & M R Spectroscopy. Am J Neuroradiol 2001;22(5):824-30.
  8.  Ozek MM, Zirh TA, Pamir N, Sav A,Erzen C. Lhermitte - Duclos Disease In A Young Adult Case Report. Turk Neurosurg 1990;1:178-81.
  9.  Nowak DA, Trost HA. Lhermitte - Duclos Disease (Dysplastic cerebellar gangliocytoma of the cerebellum): a malformation, hamartoma or neoplasm? Acta Neurol Scand 2002;105:137-14
  10.  Govindan A, Premkumar S, Alapatta J. Lhermitte - Duclos Disease (dysplastic gangliocytoma of the cerebellum) as a component of cowdens Syndrome. Indian J Pathol Microbiol 2012;55(1):107-8.
  11.  Rosenblum MK, Bilbao JM, Ang LC. Rosai & Ackerman’s surgical Pathology. 9th ed. New York:Elsevier;2005.
Iranian Journal of Pathology
Volume 8, Issue 3 - Serial Number 3
Summer 2013
Pages 194-198

  • Receive Date 05 March 2012
  • Accept Date 25 March 2012

Home

Guide for Authors

Submit Manuscript

Contact Us