Different stages of white matter changes in the original HDLS family revealed by advanced MRI techniques.

摘要: BACKGROUND The temporal evolution of white matter (WM) changes on MR examinations in hereditary diffuse leukoencephalopathy with spheroids (HDLS) is largely unknown. Our purpose was to investigate the these WM diffusion weighted/tensor imaging (DWI/DTI) and Spectroscopy (MRS). METHODS A newly diagnosed patient HDLS from original Swedish family followed prospectively 5 MRI as well DWI/DTI MRS during 16 months. RESULTS The DTI eigenvalues demonstrated that suggested early myelin axonal disturbances normal appearing (NAWM). showed a rim decreased progressively expanding through initial frontal periventricular zones, indicated complete destruction axons area behind front. findings were suggestive NAWM. CONCLUSION We describe three stages development corresponding lesions outside, vicinity of, characteristic centrifugally progressing ventricular horns. The by NAWM support primary degeneration, proposed report, rather than degeneration secondary demyelination. These could help differential diagnosis HDLS.