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Lhermitte-Duclos disease: MR diffusion and spectroscopy.

Alessandra Gaballo¹, Michele Palma, Franca Dicuonzo

¹U.O. Neuroradiologia, Cattedra di Neuroradiologia, Azienda Ospedaliera Policlinico Consorziale, Università degli Studi di Bari, Bari, Italy.

La Radiologia Medica

|November 18, 2005

Summary

Lhermitte-Duclos disease (LDD) is diagnosed using advanced MRI techniques. Diffusion MRI and MR Spectroscopy reveal characteristic findings of this rare cerebellar condition.

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Area of Science:

Neurology
Radiology
Neuro-oncology

Background:

Lhermitte-Duclos disease (LDD), also known as dysplastic cerebellar gangliocytoma, is a rare condition characterized by a slow-growing cerebellar mass.
It is associated with Cowden's syndrome in 40% of cases, an autosomal dominant disorder with malignant potential.

Observation:

Two patients with LDD underwent conventional MRI, Diffusion MRI, and MR Spectroscopy.
Conventional MRI demonstrated thickened cerebellar folia and a cerebellar mass with mild compression of the fourth ventricle, leading to hydrocephalus.
Diffusion imaging revealed hyperintensity and hypointensity on the ADC map, while Spectroscopy showed an elevated lactate peak and reduced choline peak.

Findings:

The observed MRI findings, including specific diffusion characteristics and spectroscopic abnormalities (increased lactate, decreased choline), are indicative of abnormal anaerobic glycolysis and demyelination.

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These neuroradiological findings correlate with the understanding of LDD as a malformative condition.

Implications:

MRI combined with Diffusion Imaging and MR Spectroscopy is the preferred imaging modality for diagnosing LDD.
These advanced imaging techniques facilitate a definitive diagnosis, aiding in the understanding and management of LDD and its potential association with Cowden's syndrome.