Lhermitte-Duclos Disease with Syringomyelia

Abstract

Dysplastic gangliocytoma is defined as a rare cerebellar mass lesion with malformative, hamartomatous and neoplastic features, arising from cerebellar neurons and affecting the population in early adulthood. Common clinical presentation is in the form of headache, features of raised intracranial tension or hydrocephalus. It may also be found as an incidental lesion. Even though many cases of this disease entity have been reported in literature, its association with syrinx is less commonly highlighted and hence we are reporting these two cases which we encountered at our institute. Presence of an associated syrinx should not deter one to make a preoperative diagnosis of this condition once the striking imaging findings are visualized.