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Published on July 14, 2016

Author: neelammalik33

Source: authorstream.com

Slide1: LEUKODYSTROPHIES DR.MARYAM AISHA Slide3: Leukodystrophies are genetically determined metabolic diseases, in which the underlying biochemical abnormality interferes with the normal build-up and/or maintenance of myelin, which leads to hypo- (or arrested) myelination, or dysmyelination with resultant demyelination. Slide8: Axial T2WI in a child with Canavan disease shows periventricular , deep, and subcortical WM involvement, plus thalami (white arrow) and globi pallidi (white curved) involvement; very characteristic. Slide10: Coronal FLAIR MR shows bilateral and symmetric periventricular and deep white matter signal abnormality but sparing of sub-cortical U-fibers (white arrow) in child with metachromatic leukodystrophy . Coronal T2WI MR in another case of metachromatic leukodystrophy shows characteristic diffuse deep and periventricular white matter involvement, with sparing of the cerebellar white matter (white arrow). Slide11: a Axial T2-weighted fast spin-echo b diffusion-weighted c ADC map Slide12: X-linked adrenoleukodystrophy with preferential involvement of descending pyramidal tract A-C. T2-weighted image shows demyelination of internal capsule, descending pyramidal tract (arrows, A) and cerebellar deep white matter (arrows, B). The peritrigonal white matter is relatively spared(C). D. On gadolinium-enhanced T1-weighted image, enhancing bilateral descending pyramidal tracts (arrows) are shown. Slide18: MR spectroscopy (MRS)  allows tissue to be interrogated for the presence and concentration of various metabolites .   It is fair to say that MRS often does not add a great deal to an overall MR study but does increase specificity , and may help in improving our ability to predict histological grade. Slide19: The value of conventional diffusion-weighted imaging in the diagnostic work-up of leukodystrophies Allows confident differentiation of certain edema types, in particular myelin edema A llows better understanding of the underlying histopathological phenomena in leukodystrophies as well as the dynamics of the disease processes A useful adjunct to the characterization of the different entities in the process of pattern recognition As a very crude classification scheme, leukodystrophies characterized by absent, low, intermediate and highgrade myelin edema may be distinguished, depending on the diffusion-weighted imaging presentation

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