De- or Hypomyelination in Myelinopathia Centralis Diffusa/Leukoencephalopathy with Vanishing White Matter? Insights from Localized Proton MRS
Dreha-Kulaczewski S, Dechent P, Wilken B, Brockmann K, Finsterbusch J, Hanefeld F, Frahm J, Grtner J
De- and hypomyelination are discussed in the pathogenesis of white matter (WM) disorders. Both produce distinct metabolite patterns detectable by proton MRS as demyelination is characterized by elevated Cho, whereas hypomyelination causes decreased concentrations of Cho. The newly discovered Myelinopathia Centralis Diffusa/Leukoencephalopathy with Vanishing White Matter (MCD/VWM) is neuropathologically characterized by loss of WM tissue. However the pathophysiology leading to the profound MRI-detectable WM changes already at onset of symptoms is still not fully understood. Ten patients with MCD/VWM were studied longitudinally using localized proton MRS. At early stages, a relatively high Cho was detected, indicating an initial demyelinating process.