Poster électronique

Un diagnostic, un seul ... le Lhermitte-Duclos !
T. Delchambre, O. Eker, F. Tahon, K. Boubagra, JA. Vasdev, A. Krainik, JF. Le Bas, S. Grand - Grenoble - France
Congrès 2011
Résumé
Objectifs
Connaître les caractéristiques morphologiques et fonctionnelles en IRM de la maladie de Lhermitte-Duclos (MLD).
Matériels et méthodes
Trois patients, atteints d'une maladie de MLD avec dans un cas un syndrome de Cowden, ont été explorés par IRM 3T (Philips), sur un plan morphologique avec des séquences T2/FLAIR/T1 sans et avec Gadolinium, et sur un plan fonctionnel pour deux cas par des séquences de perfusion/diffusion/spectroscopie mono voxel (TE court et TE long).
Résultats
Dans les 3 cas, nous retrouvons une masse de la fosse cérébrale postérieure tigrée, hypervasculaire sur la perfusion, présentant un ADC très discrètement diminué. Des modifications spectrales avec une diminution des résonances de la choline, du NAA, une concentration stable de créatine sont observées avec dans 1 cas d'un doublet de lactate.
Conclusion
La MLD (gangliocytome dysplasique cérébelleux) est rare, définie par une prolifération neuronale des couches granulaire et moléculaire du cortex cérébelleux associée à un amincissement de la substance blanche. La désorganisation
lamellaire corticale donne l'aspect tigré, avec une zone centrale en hyperT2 et une zone périphérique en hypoT2. Le lacis veineux autour des feuillets corticaux expliquent l'hypervascularisation. La restriction discète et hétérogène de la diffusion est vraisemblablement en rapport avec une relative hypercellularité. L'absence d'élévation de la choline n'est pas en faveur d'une tumeur proliférative et est concordante avec des phénomènes dysplasiques.
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2017-08-29 16.28 :
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2017-04-12 12.07 :
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