Lhermitte-Duclos-Cowden syndrome

Case contributed by Dr Sinéad Culleton

Presentation

Acute ataxia, Cowden's syndrome.

Patient Data

Age: 70 years
Gender: Male
Modality: CT

Within the right cerebellar hemisphere  there is an area of low attenuation. The fourth ventricle is partially compressed by the displaced vermis with associated hydrocephalus.

Modality: MRI

 6 cm hyperintense mass in the right lobe of the cerebellum extending into the cerebellar vermis, causing slight mass effect on the left cerebellar hemisphere and compression of the fourth ventricle. There appears to be preservation of the cerebellar folia. DWI also showed hyperintensity due to T2 shine through. Generalised ventricular dilatation secondary to obstructive hydrocephalous resulting from compression of the fourth ventricle.

Case Discussion

This case illustrates the typical finding on CT and MRI of Lhermitte duclos in the clinical setting of Cowden's. Calcification is uncommon. Enhancement is rare and occasionally may occur due to anomalous veins. As this is a posterior fossa process typically there is compression of the fourth ventricle with resultant hydrocephalus 1.

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Case Information

rID: 40791
Case created: 3rd Nov 2015
Last edited: 21st Nov 2015
Inclusion in quiz mode: Excluded

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