Lhermitte-Duclos disease

Case contributed by Tariq Walizai
Diagnosis probable

Presentation

Chronic headache for five years followed by recently blurred vision.

Patient Data

Age: 35 years
Gender: Male

MRI Brain

mri

There is an intra-axial, ill-defined, altered signal intensity mass lesion in the left medial cerebellum returning signals are hypo to isointense on T1 WI, isointense to hyperintense on T2 with a striated/tigroid appearance and isointense on T2 FLAIR. Subtle linear enhancement on the post-contrast images is likely due to internal vessels.

Mass effect is manifested as compression of the cerebellar vermis and 4th ventricle outlet/ upper part of the central canal with moderate retrograde dilatation of the ventricular system along with trans-ependymal seepage.

Case Discussion

MRI Brain features, age of the patient and course of the disease with presentation are more in favor of Lhermitte-Duclos disease  (dysplastic gangliocytoma of the cerebellum).

Unfortunately, headaches are very common, and therefore the history of chronic headaches does not absolutely confirm the chronicity of this condition, and more aggressive lesions should be considered, particularly medulloblastoma SHH activated. A review of diffusion-weighted imaging would be useful (currently unavailable). 

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