Leukoencephalopathy could be a prominent finding in some patients with DRPLA, explaining, at least in part, their clinical evolution. In our case, the disproportion between the severity of white matter damage and vascular changes does not support a cardinal role for ischemic mechanisms in leukoencephalopathy.
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The corresponding record at NLM can be accessed at https://www.ncbi.nlm.nih.gov/pubmed/15210537