Lhermitte-Duclos disease

Case contributed by Dr Y. Amy Chen

Presentation

Ataxia

Patient Data

Age: 25 years
Gender: Female
Modality: CT

Predominantly nonenhancing hypodense mass in the right cerebellum causing mass effect on the posterior fossa structures, with effacement of the 4th ventricle.

Modality: MRI

MRI demonstrates a T1 hypointense T2 hyperintense nonenhancing lesion in the right cerebellum. T2-characteristics are classic, and show widened cerebellar folia with striated, "corduroy" appearance. The mass causes mass effect on the posterior fossa structures and effaces the 4th ventricle. There is no associated restricted diffusion nor increased perfusion.

Case Discussion

Lhermitte-Duclos (dysplastic gangliocytoma of the cerebellum) is a rare hamartomatous tumour of the cerebellum. It usually presents in young adults and is associated with Cowden's disease. MRI appearance is classic, with striated, "corduroy" appearance of widened cerebellar folia. 

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Case Information

rID: 51350
Case created: 12th Feb 2017
Last edited: 13th Feb 2017
Inclusion in quiz mode: Included

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