LHERMITTE-DUCLOS DISEASE-ASSOCIATED WITH SYRINGOMYELIA

A 23-year-old man presented with a 2-week history of intracranial hype rtension. CT showed a large, nonenhancing cerebellar mass with surroun ding calcification and displacement of the fourth ventricle. MRI revea led a septate lesion, with low signal on T1-weighted and high signal o n T2-weighted images. The cerebellar tonsils were displaced below the foramen magnum and there was associated syringomyelia. The MRI feature s were characteristic of Lhermitte-Duclos (LD) disease (dysplastic gan gliocytoma) and the diagnosis was confirmed following surgery. In this case, we emphasise the usefulness of MRI in the diagnosis of LD disea se and consider the possible pathogenesis of the associated syringomye lia.