Although white matter diseases with numerous axonal spheroids mimic the pathology of colony stimulating factor-1 receptor (CSF-1R)-mutant leukoencephalopathy, such as dentatorubral-pallidoluysian atrophy (DRPLA) [17]. However, in our cases, there were no progressive myoclonus, epilepsy, ataxia, or dementia, which are the typical symptoms of DRPLA. This evidence concerns the gene CSF1R and Dentatorubral pallidoluysian atrophy.