Several motor predominant cases of ALSP caused by heterozygous CSF1R mutations has been reported to mimic primary progressive multiple sclerosis (PPMS) (Inui et al., 2013; Saitoh et al., 2013; Sundal et al., 2015; Prieto-Morin et al., 2016). Here, CSF1R is linked to Hereditary diffuse leukoencephalopathy with axonal spheroids and pigmented glia.