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

Surg Neurol. 1996 May;45(5):470-5; discussion 475-6. doi: 10.1016/0090-3019(95)00244-8.

Abstract

Background: The most common primary cerebellar tumor is hemangioblastoma, a lesion which is associated with magnetic resonance imaging (MR)-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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Cerebellar Neoplasms / diagnostic imaging
  • Cerebellar Neoplasms / pathology*
  • Cerebellum / diagnostic imaging
  • Cerebellum / pathology*
  • Female
  • Ganglioneuroma / pathology
  • Humans
  • Magnetic Resonance Imaging
  • Spinal Cord / pathology
  • Tomography, X-Ray Computed