An average clinical profile from published studies shows that the median onset age for HDLS patients is 44.3 years with a mean disease duration of 5.8 years and mean age of death at 53.2 years. As of 2012, there have been around 15 cases identified with at least 11 sporadic cases of HDLS. HDLS cases have been located in Germany, Norway, Sweden, and the United States, showing an international distribution focusing between Northern Europe and the United States. Through the study of numerous kindred, it was found that the disease did not occur among just males or females, but rather was evenly distributed indicative of an autosomal rather than a sex-linked genetic disorder. It was also observed that the HDLS cases did not skip generations as it would occur with a recessive inheritance, and as such has been labeled autosomal dominant.
Hereditary diffuse leukoencephalopathy with spheroids
Powassan encephalitis
Toxic leukoencephalopathy
Autoimmune encephalitis
Transmissible spongiform encephalopathy
Progressive multifocal leukoencephalopathy
Malignant multiple sclerosis
Variant Creutzfeldt–Jakob disease