Lhermitte Duclos disease: a rare cerebellar lesion with characteristic neuroimaging features
DOI:
https://doi.org/10.18203/2320-6012.ijrms20161836Keywords:
Lhermitte-Duclos disease/ dysplastic cerebellar gangliocytoma, Hamartomatous, Cowden syndromeAbstract
Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma) is a rare disorder of uncertain pathogenesis characterized by disarrangement of the normal cerebellar laminar cytoarchitecture. A case of histopathologically confirmed Lhermitte-Duclos disease with characteristic imaging findings and successful surgical management is reported and our findings are discussed.
References
Dietlein M, Schroder R, Widemann B, Benz-Bohm C. Dysplastic gangliocytoma of cerebellum in a newborn. Pediatr Radiol. 1992;22:131-3.
Vieco PT, O’Donovan RDC, Melanson D, Montes J, O’Gorman AM, Meagher-Villemure K. Dysplastic gangliocytoma (Lhermitte-Duclos disease): CT and MR imaging. Pediatr Radiol. 1992;22:366-9.
Marano SR, Johnson PC, Spetzler RF. Recurrent Lhermitte-Duclos disease in a child:case report. J Neurosurg. 1988;69:599-603.
Ambler M, Pogacar S, Sidman R. Lhermitte-Duclos disease (granular cell hypertrophy of the cerebellum): pathological analysis of the first familial cases. J Neuropathol Exp Neurol. 1969;28:622-47.
Di Lorenzo N, Lunardi P, Fortuna A. Granulomolecular hypertrophy of the cerebellum (Lhermitte-Duclos disease). J Neurosurg 1984;60:644-6.
Lhermitte J, Duclos P. Sur un ganglioneurome diffus du cortex du cervelet. Bull Assoc Fr Etude Cancer. 1920;9:99-107.
PTEN hamartoma tumor syndromes. Eur J Hum Genet. 2008;16:1289-300.
Milbouw C, Born JD, Martin D. Clinical and radiological aspects of dysplastic gangliocytoma (Lhermitte-Duclos disease): a report of two cases with review of the literature. Neurosurgery 1988;22:124-8.
Reznik M, Schoenen J. Lhermitte-Duclos disease. Acta Neuropathol. 1983;59:88-94.
Ashley DC, Zee CS, Chandrasoma PT, Segall HD. Lhermitte-Duclos disease: CT and MR findings. J Comput Assist Tomogr. 1990;14:984-7.
Meltzer CC, Smirniotopoulos JG, Jones RV. The striated cerebellum: an MR imaging sign in Lhermitte-Duclos disease (dysplastic gangliocytoma). Radiology 1995;194:699–703.
Kulkantrakorn K, Awwad EE, Levy B. MRI in Lhermitte-Duclos disease. Neurology. 1997;48:725-31.
Ortiz O, Bloomfield S, Schochet S. Vascular contrast enhancement in Lhermitte-Duclos disease: case report. Neuroradiology. 1995;37:545-8.
Awwad EE, Levy E, Martin DS, Merenda GO. Atypical MR appearance of Lhermitte- Duclos disease with contrast enhancement. AJNR Am J Neuroradiol. 1995;16:1719-20.
Nagaraja S, Powell T, Griffiths P, Wilkinson ID. MR imaging and spectroscopy in Lhermitte- Duclos disease. Neuroradiology. 2004;46:355-8.