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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

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


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. 


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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:

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.