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          Institute: MPI für biophysikalische Chemie     Collection: Biomedizinische NMR ForschungsGmbH (Prof. Jens Frahm)     Display Documents

ID: 20740.0, MPI für biophysikalische Chemie / Biomedizinische NMR ForschungsGmbH (Prof. Jens Frahm)
Megalencephalic leukoencephalopathy with subcortical cysts in an adult: quantitative proton MR spectroscopy and diffusion tensor MRI
Authors:Brockmann, K.; Finsterbusch, J.; Terwey, B.; Frahm, J.; Hanefeld, F.
Date of Publication (YYYY-MM-DD):2003-03
Title of Journal:Neuroradiology
Issue / Number:3
Start Page:137
End Page:142
Review Status:Peer-review
Audience:Not Specified
Abstract / Description: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.
Free Keywords:leukoencephalopathy; subcortical cysts; magnetic resonance spectroscopy; diffusion tensor imaging
Comment of the Author/Creator:Date: 2003, MAR
External Publication Status:published
Document Type:Article
Communicated by:N. N.
Affiliations:MPI für biophysikalische Chemie/AG Jens Frahm
External Affiliations:Univ Gottingen, Childrens Hosp, Dept Paediat & Neuropaediat,; Robert Koch Str 40, D-37075 Gottingen, Germany; Univ Gottingen, Childrens Hosp, Dept Paediat & Neuropaediat, D-37075 Gottingen, Germany; Zentralkrankenhaus, Inst Magnet Resonanz Diagnost, D-28205 Bremen, Germany
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