Lhermitte-Duclos Disease.

Abstract

HomeRadiologyVol. 307, No. 1 PreviousNext Reviews and CommentaryFree AccessImages in RadiologyLhermitte-Duclos DiseaseDhairya A. Lakhani , SoHyun BooDhairya A. Lakhani , SoHyun BooAuthor AffiliationsFrom the Department of Radiology, West Virginia University, Ruby Memorial Hospital, 1 Medical Center Dr, Morgantown, WV 26506 (D.A.L., S.H.B.); and Department of Neuroradiology, Rockefeller Neuroscience Institute, West Virginia University, Morgantown, WV (S.H.B.).Address correspondence to D.A.L. (email: [email protected]).Dhairya A. Lakhani SoHyun BooPublished Online:Dec 13 2022https://doi.org/10.1148/radiol.221979MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In An 18-year-old woman with no past medical history presented with chronic headache and new onset cerebellar ataxia. MRI of the brain (Figure, A–D) demonstrated a mass in the left cerebellar hemisphere. The mass was predominantly hyperintense with T2-weighted images with intrinsic hyper- to isointense striations relative to gray matter (Figure, A–B). The mass was predominantly hypointense with T1-weighted images with striations hypo- to isointense relative to gray matter (Figure, C). The mass showed no enhancement with contrast-enhanced images (Figure, D) and did not restrict diffusion with diffusion-weighted images (not shown). Mild obstructive-pattern hydrocephalus was present from compression of the fourth ventricle.Images in an 18-year-old woman who presented with chronic headache and cerebellar ataxia. (A, B) Axial T2-weighted, (C) precontrast T1-weighted, and (D) postcontrast T1-weighted MRI scans of the brain demonstrate a mass in the left cerebellar hemisphere. The mass was predominantly hyperintense with (A, B) T2-weighted images with intrinsic hyper- to isointense striations relative to gray matter and predominantly hypointense with (C) T1-weighted images with hypo- to isointense striations relative to gray matter. No intrinsic contrast enhancement was seen with (D) postcontrast images.Download as PowerPointOpen in Image Viewer The typical striated, corduroy, or tiger-striped folial pattern consisting of alternating bands with T2-weighted and T1-weighted images is characteristic of Lhermitte-Duclos disease or dysplastic cerebellar gangliocytoma, a rare hamartomatous disorder (1,2). It is an Aunt Minnie diagnosis and has close association with Cowden syndrome. Clinically, patients may be asymptomatic or present from symptoms related to mass effect (1).Disclosures of conflicts of interest: D.A.L. RSNA William W. Olmsted Trainee Editorial Fellow (2022). S.H.B. No relevant relationships.