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Lhermitte-Duclos disease

Case contributed by Alessandro Spano Mello
Diagnosis almost certain

Presentation

Headache and ataxia.

Patient Data

Age: 35 years
Gender: Female
mri

Expansive lesion at right cerebellar hemisphere and vermis, hyperintense in T2, isointense in T1, without enhancement or restricted diffusion in DWI (but shows T2 shine through effect), with mass effect and fourth ventricle shift and effacement.

Case Discussion

MRI of a 35-year-old female with dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease), which is a rare posterior fossa disease that results in an expansive appearance of the cerebellum with thickened folia. The lesion is isointense in T1 and hyperintense in T2, without enhancement. There is posterior fossa mass effect with fourth ventricle shift and effacement.  A ventricular diversion was performed before the MRI scan. 

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