Lhermitte-Duclos disease

Case contributed by Frank Gaillard
Diagnosis almost certain

Patient Data

Age: 5 years
Gender: Male

Brain

mri

Marked enlargement of the right cerebellar hemisphere without a focal mass or cystic change. The hemisphere is isointense on T1 and hyperintense on T2 weighted images with no abnormal enhancement. There is marked positive mass effect in the posterior fossa which is enlarged. The fourth ventricle is effaced and there is prominence of the lateral ventricles (best seen on coronal T2).

Case Discussion

These findings are characteristic of Lhermitte-Duclos-Cowden syndrome.

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