Lhermitte-Duclos disease

is also known as "Dysplastic gangliocytoma of the cerebellum".

Incidence

This tumor is extremely rare.

Origin

Lhermitte-Duclos disease refers to a pseudotumoral dysplastic neuronal lesion of the cerebellum.

Macroscopic appearance and general characteristics

Macroscopically, the lesion is well defined and mimics normal cerebellum but with enlargement of the folia. It corresponds to Grade I.

Microscopic appearance (images 2 - 3)

There is a diffuse cortical accumulation of abnormally large dysplastic granular cells.

Imaging features (see case reports)

- On CT the involved cerebellar hemisphere is enlarged and somewhat hypodense. There is no contrast enhancement.

- The MR image is pathognomonic with diffuse T2 hyperintensitivity of the involved hemisphere, in which both on T1 and T2 weighted images, the hypertrophic cerebellar folia can be recognized (image 4).

Case reports

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Lhermitte-Duclos disease

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