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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.72 no.8 São Paulo Aug. 2014

http://dx.doi.org/10.1590/0004-282X20140091 

IMAGES IN NEUROLOGY

Brain MRI features in Lhermitte-Duclos disease

Achados de RM cerebral na doença de Lhermitte-Duclos

Wladimir Bocca Vieira de Rezende Pinto

Paulo Victor Sgobbi de Souza

1Divisão de Neurologia Geral, Departamento de Neurologia, Universidade Federal de São Paulo, Sao Paulo SP, Brazil.

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)1,2 (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 allele3,4. Cranial nerve palsies, gait ataxia and obstructive hydrocephalus secondary to a slowly progressive unilateral cortical cerebellar tumor represents the most common clinical findings5.

Figure 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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. Meltzer CC, Smirniotopoulos JG, Jones RV. The striated cerebellum: an MR imaging sign in Lhermitte-Duclos disease (dysplastic gangliocytoma). Radiology 1995;194:699-703. [ Links ]

. Nowak DA, Trost HA. Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma): a malformation, hamartoma or neoplasm? Acta Neurol Scand 2002;105:137-145. [ Links ]

. Shinagare AB, Patil NK, Sorte SZ. Case 144: Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease). Radiology 2009;251:298-303. [ Links ]

. 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. [ Links ]

Received: March 11, 2014; Revised: May 08, 2014; Accepted: May 28, 2014

Correspondence: Wladimir Bocca Vieira de Rezende Pinto; Rua Botucatu, 740; 04023-900 São Paulo SP, Brasil; E-mail: wladimirbvrpinto@gmail.com

Conflict of interest: There is no conflict of interest to declare.

Creative Commons License This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.