An axial CT scan of a patient presenting a<unk> , the base disorder to the family of disorders that HDLS falls under. In HDLS, there is enlargement of the lateral ventricles and marked thinning or weakening of cerebral white matter. The loss of white matter is caused by myelin loss. These changes are associated with<unk>  gliosis, moderate loss of axons and many axonal<unk> . Activated or ameboid microglia and macrophages that contain myelin debris, lipid droplets and brown autofluorescent pigment granules are found in the areas with demyelination and axonal<unk> . In severely degenerated areas there are many large, reactive astrocytes filled with glial fibrils. In autopsy cases, it has been shown that white matter abnormalities are relatively confined to the cerebrum while avoiding the cerebellum and many of the major fiber tracts of the nervous system. The exception is the corticospinal tracts(pyramidal tracts) in the brainstem and sometimes spinal cord. The brain pathology of HDLS resembles that of Nasu-Hakola disease (polycystic lipomembranous osteodysplasia with sclerosing<unk> .
Hereditary diffuse leukoencephalopathy with spheroids
Powassan encephalitis
Toxic leukoencephalopathy
Autoimmune encephalitis
Transmissible spongiform encephalopathy
Progressive multifocal leukoencephalopathy
Malignant multiple sclerosis
Variant Creutzfeldt–Jakob disease