Lhermitte-Duclos disease

Case contributed by Alexandra Stanislavsky


Three week history of headaches.

Patient Data

Age: 40 years
Gender: Female

CT brain


A large, heterogeneous, non enhancing and non-calcified right cerebellar mass compresses and distorts the fourth ventricle with resultant dilatation of the third and lateral ventricles and tonsillar herniation.There is no appreciable contrast enhancement following administration of contrast.

MRI brain


MRI confirms the abnormality. The mass is striated and hyperintense on T2 and grey matter isointense on T1, with no restricted diffusion and no contrast enhancement.

Case Discussion

This patient went on to have a posterior fossa craniotomy and biopsy, confiming the diagnosis of Lhermitte-Duclos disease. Further investigations for possible Cowden syndrome did not demonstrate any hamartomatous lesions or tumors elsewhere.

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