<unk>  (HDLS) is a rare adult onset autosomal dominant disorder characterized by cerebral white matter degeneration with demyelination and axonal<unk>  leading to progressive cognitive and motor dysfunction.<unk>  are axonal swellings with discontinuous or absence of myelin sheaths. It is believed that the disease arises from primary microglial dysfunction that leads to secondary disruption of axonal integrity, neuroaxonal damage, and focal axonal<unk>  leading to demyelination.<unk>  in HDLS resemble to some extent those produced by shear stress in a closed head injury with damage to axons, causing them to swell due to blockage of axoplasmic transport. In addition to trauma, axonal<unk>  can be found in aged brain, stroke, and in other degenerative diseases. In HDLS, it is uncertain whether demyelination occurs prior to the axonal<unk>  or what triggers neurodegeneration after apparently normal brain and white matter development, although genetic deficits suggest that demyelination and axonal pathology may be secondary to microglial dysfunction. The clinical syndrome in patients with HDLS is not specific and it can be mistaken for Alzheimer's disease, frontotemporal dementia, atypical Parkinsonism, multiple sclerosis, or corticobasal degeneration.
Hereditary diffuse leukoencephalopathy with spheroids
Powassan encephalitis
Toxic leukoencephalopathy
Autoimmune encephalitis
Transmissible spongiform encephalopathy
Progressive multifocal leukoencephalopathy
Malignant multiple sclerosis
Variant Creutzfeldt–Jakob disease