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  Megalencephalic leukoencephalopathy with subcortical cysts in an adult: quantitative proton MR spectroscopy and diffusion tensor MRI

Brockmann, K., Finsterbusch, J., Terwey, B., Frahm, J., & Hanefeld, F. (2003). Megalencephalic leukoencephalopathy with subcortical cysts in an adult: quantitative proton MR spectroscopy and diffusion tensor MRI. Neuroradiology, 45(3), 137-142. Retrieved from http://www.springerlink.com/content/bqa8473tkucvuvnp/fulltext.pdf.

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Item Permalink: http://hdl.handle.net/11858/00-001M-0000-0012-F153-6 Version Permalink: http://hdl.handle.net/11858/00-001M-0000-0027-E3E4-E
Genre: Journal Article

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20740.pdf (Publisher version), 0B
 
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 Creators:
Brockmann, K., Author
Finsterbusch, J.1, Author              
Terwey, B., Author
Frahm, J.1, Author              
Hanefeld, F., Author
Affiliations:
1Biomedical NMR Research GmbH, MPI for biophysical chemistry, Max Planck Society, ou_578634              

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Free keywords: leukoencephalopathy; subcortical cysts; magnetic resonance spectroscopy; diffusion tensor imaging
 Abstract: A 37-year-old macrocephalic woman was investigated for increasing gait disturbance due to longstanding spasticity and ataxia. MRI showed widespread bilateral increase in signal from cerebral white matter on T2-weighted images. Numerous subcortical cysts were visible in anterior-temporal and parietal regions. These clinical and neuro radiological features are those of megalencephalic leukoencephalopathy with subcortical cysts (MLC), a recently delineated whitematter disease with onset in childhood. Quantitative localised proton MR spectroscopy of white matter revealed marked reduction of N- acetylaspartate, creatine, and choline with normal values for myoinositol, consistent with axonal loss and astrocytic proliferation. Diffusion tensor imaging showed an increased apparent diffusion coefficient and reduced anisotropy in affected white matter pointing to reduced cell density with an. increased extracellular space. These findings are in line with histological changes alterations known to occur in MLC.

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Language(s): eng - English
 Dates: 2003-03
 Publication Status: Published in print
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 Rev. Method: Peer
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Title: Neuroradiology
Source Genre: Journal
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Pages: - Volume / Issue: 45 (3) Sequence Number: - Start / End Page: 137 - 142 Identifier: -