Preoperative MRI diagnosis of Lhermitte-Duclos disease: Case report with associated enlarged vessel and syrinx

Leo J. Wolansky, Grace P. Malantic, Robert Heary, Allen H. Maniker, Huey Jen Lee, Leroy R. Sharer, Upen J. Patel

Research output: Contribution to journalArticlepeer-review

40 Scopus citations

Abstract

BACKGROUND: The most common primary cerebellar tumor is hemangioblastoma, a lesion which is associated with magnetic resonance imaging (MRI)-detectable vascularity in over 60%. Lhermitte-Duclos disease is an uncommon cause of a cerebellar mass that is not typically vascular. METHODS: Computed tomography (CT), MRI with and without contrast, and magnetic resonance venography was performed in a patient with a cerebellar mass. RESULTS: The cerebellar mass was noted to have a prominent vessel, as well as an associated syrinx. In spite of MRI-detectable vascularity, the striped appearance of the lesion was felt to be typical of Lhermitte-Duclos disease. At surgery, the mass was resected and the diagnosis of Lhermitte-Duclos disease was confirmed. CONCLUSIONS: The diagnosis of Lhermitte-Duclos disease should be made when MRI shows a parallel linear 'tiger-striped' lesion of the cerebellum. The presence of an enlarged vessel and/or syrinx should not deter one from making the preoperative diagnosis.

Original languageEnglish (US)
Pages (from-to)470-476
Number of pages7
JournalSurgical Neurology
Volume45
Issue number5
DOIs
StatePublished - May 1996

All Science Journal Classification (ASJC) codes

  • Surgery
  • Clinical Neurology

Keywords

  • Lhermitte-Duclos disease
  • MRI diagnosis
  • dysplastic gangliocytoma
  • gangliocytoma
  • syringohydromyelia
  • venous angioma

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