Survival, development, proliferation, and activation of mononuclear phagocytic cells and central nervous system (CNS) microglia are regulated by CSF1R. CSF1R gene mutations are considered an underlying cause of primary brain microgliopathies and are linked to CNS damage of ALSP (20, 34–36). Here, CSF1R is linked to Hereditary diffuse leukoencephalopathy with axonal spheroids and pigmented glia.