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In addition to atrophy of the cerebral cortex,cerebellus,and pantomesencephalic tegmentum,the cerebral white matter and a part of the white matter tracts within the brainstem show prominent high signal intensities on FLAIR images.These MR findings suggest that,in addition to the degeneration of the denatorubral and pallidoluysian systems,the pathological process extends to the white matter in DRPLA.This could be important for differentiating DRPLA from other clinically similar diseases such as Machado-Joseph disease or Huntington's disease. |
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