Lhermitte-Duclos Disease Associated with Syringomyelia

Overview

Journal Neuroradiology

Specialties Neurology
Radiology

Date 1996 Aug 1

PMID 8880711

Citations 8

Authors

C D Marcus

M Galeon

P Peruzzi

A Bazin

M H Bernard

M Pluot

B Menanteau

Affiliations

Soon will be listed here.

Abstract

A 23-year-old man presented with a 2-week history of intracranial hypertension. CT showed a large, nonenhancing cerebellar mass with surrounding calcification and displacement of the fourth ventricle. MRI revealed a septate lesion, with low signal on T1-weighted and high signal on T2-weighted images. The cerebellar tonsils were displaced below the foramen magnum and there was associated syringomyelia. The MRI features were characteristic of Lhermitte-Duclos (LD) disease (dysplastic gangliocytoma) and the diagnosis was confirmed following surgery. In this case, we emphasise the usefulness of MRI in the diagnosis of LD disease and consider the possible pathogenesis of the associated syringomyelia.

Citing Articles

Neuroimaging abnormalities in patients with Cowden syndrome: Retrospective single-center study.

Dhamija R, Weindling S, Porter A, Hu L, Wood C, Hoxworth J Neurol Clin Pract. 2018; 8(3):207-213.

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Action Semantics at the Bottom of the Brain: Insights From Dysplastic Cerebellar Gangliocytoma.

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Syringobulbia associated with posterior fossa meningioma: a review of the literature.

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Advanced MR imaging in Lhermitte-Duclos disease: moving closer to pathology and pathophysiology.

Thomas B, Krishnamoorthy T, Radhakrishnan V, Kesavadas C Neuroradiology. 2007; 49(9):733-8.

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Tonsillar herniation and cervical syringomyelia in association with posterior fossa tumors in children: a case-based update.

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