A 24-year-old woman presented with long-standing headache, blurred vision, and a 2-week-history of progressive ataxia and vomiting with papilledema and Parinaud syndrome, suggestive of raised intracranial pressure. Neuroimaging features were highly suggestive of dysplastic gangliocytoma of the cerebellum or Lhermitte-Duclos disease (LDD)11 . Kulkantrakorn K, Awwad EE, Levy B, et al. MRI in Lhermitte-Duclos disease. Neurology 1997;48:725-731.,22 . Meltzer CC, Smirniotopoulos JG, Jones RV. The striated cerebellum: an MR imaging sign in Lhermitte-Duclos disease (dysplastic gangliocytoma). Radiology 1995;194:699-703. (Figure), which was confirmed in postsurgical histopathological evaluation. LDD represents a rare harmatomatous disorder linked to germline loss of one allele of the PTEN gene with subsequent loss of the remaining allele33 . Nowak DA, Trost HA. Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma): a malformation, hamartoma or neoplasm? Acta Neurol Scand 2002;105:137-145.,44 . Shinagare AB, Patil NK, Sorte SZ. Case 144: Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease). Radiology 2009;251:298-303.. Cranial nerve palsies, gait ataxia and obstructive hydrocephalus secondary to a slowly progressive unilateral cortical cerebellar tumor represents the most common clinical findings55 . Nowak DA, Trost HA, Porr A, Stölzle A, Lumenta CB. Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum). Clin Neurol Neurosurg 2001;103:105-110..
Sagittal T1-weighted MRI showing a superior vermian hypointense mass with brainstem compression and cerebellar tonsil herniation (A). Axial contrast-enhanced T1-weighted MRI unvealing non-enhancing hypointense mass in the right cerebellar hemisphere and vermis with leptomeningeal vessels enhancement in sulci between cerebellar folia (B). Axial T2-weighted (C) and FLAIR MRI sequences (D) disclosing hyperintense gyriform pattern with enlargement of cerebellar folia and alternate high- and normal-signal bands.
References
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1Kulkantrakorn K, Awwad EE, Levy B, et al. MRI in Lhermitte-Duclos disease. Neurology 1997;48:725-731.
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2Meltzer CC, Smirniotopoulos JG, Jones RV. The striated cerebellum: an MR imaging sign in Lhermitte-Duclos disease (dysplastic gangliocytoma). Radiology 1995;194:699-703.
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3Nowak DA, Trost HA. Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma): a malformation, hamartoma or neoplasm? Acta Neurol Scand 2002;105:137-145.
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4Shinagare AB, Patil NK, Sorte SZ. Case 144: Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease). Radiology 2009;251:298-303.
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5Nowak DA, Trost HA, Porr A, Stölzle A, Lumenta CB. Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum). Clin Neurol Neurosurg 2001;103:105-110.
Publication Dates
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Publication in this collection
Aug 2014
History
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Received
11 Mar 2014 -
Reviewed
08 May 2014 -
Accepted
28 May 2014
