Mutations within the CSF1R gene result in a subtype of dominantly inherited leukoencephalopathy named adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP), which is regarded as a single clinicopathologic entity of hereditary diffuse leukoencephalopathy with spheroids (HDLS) and pigmentary orthochromatic leukodystrophy (POLD) (Wider et al., 2009; Rademakers et al., 2011; Nicholson et al., 2013; Konno et al., 2014; Wider and Wszolek, 2014). Here, CSF1R is linked to Hereditary diffuse leukoencephalopathy with axonal spheroids and pigmented glia.