Preoperative MRI diagnosis of lhermitte-duclos disease: Case report with associated enlarged vessel and syrinx

Abstract

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. Computed tomography (CT), MRI with and without contrast, and magnetic resonance venography was performed in a patient with a cerebellar mass. 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. 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.