Konno T, et al. - Aim of this study is to establish and validate diagnostic criteria for adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) due to colony stimulating factor 1 receptor (CSF1R) mutation. The authors reveal that for diagnosing ALSP with sufficient specificity for differentiating from cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and moderate specificity for other leukoencephalopathies, these diagnostic criteria are very sensitive. The outcomes propose that for the clinical diagnosis of ALSP, these criteria are useful.