Both diseases present an insidious course with an initial frontal lobe or cognitive syndrome of varying duration developing into a variable focal, multisystem encephalopathy, where reported Parkinsonian features in the CSF1R-related HDLS/ALSP cases may correspond to extrapyramidal and dystonic features in HDLS-S [4, 14, 33–35]. Here, CSF1R is linked to Hereditary diffuse leukoencephalopathy with axonal spheroids and pigmented glia.