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Case of the Week

Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada

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May 19, 2008

Lhermitte-Duclos Disease

  • AKA: dysplastic gangliocytoma (thickened folia give rise to 'layered' or 'striped' appearance, WHO grade 1)
  • CNS manifestation of Cowden syndrome (multiple hamartomas of skin & mucosas, mutations in PTEN gene)
  • May show high perfusion (cRBV) & hypermetabolism on PET.
  • No significant diffusion restriction on ADC
  • Some contrast enhancement occasionally
  • MRS: high lactate, low NAA & Cho
  • SWI: prominent blood vessels (veins?)

Suggested Reading

Klisch J, Juengling F, Spreer J, et al. Lhermitte-Duclos disease: Assessment with MR Imaging, Positron Emission Tomography, Single-photon Emission CT, and MR Spectroscopy. AJNR AM J Neuroradiol 2001;22:824-30.

Awwad EE, Levy E, Martin DS, et al. Atypical MR appearance of Lhermitte-Duclos disease with contrast enhancement. AJNR Am J Neuroradiol 1995;16:1719-20.

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