Brain MRI features in Lhermitte-Duclos disease

Abstract

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 allele 3,4 .

MR imaging features of Lhermitte–Duclos disease

“…All 3 patients with cerebellar dysplastic gangliocytoma in our center had typical “tiger striped” manifestations on MRI [10] . Moreover, on T2WI and FLAIR, this tumor has very unique features.…”

“…All 3 patients with cerebellar dysplastic gangliocytoma in our center had typical “tiger striped” manifestations on MRI. [ 10 ] Moreover, on T2WI and FLAIR, this tumor has very unique features. The high-signal areas on T2WI and FLAIR are equivalent to the center of the diseased cerebellar lobe, including the atrophic white matter, a hyperplastic granular cell layer, and a thickened inner molecular layer.…”

Lhermitte-Duclos Disease and Cowden Syndrome: A Case Report and Literature Review

“…Of these, 5 reports are Brazilian. [9][10][11][12][13][14] Therefore, this article is the 6th report of LDD and the first to report the association with CS in Brazil.…”