Progressive subcortical gliosis of Neumann: a clinicopathologic study of two cases with review

A clinico-pathological report is given of two cases of progressive dementia of fronto-temporal type associated with variable cortical neuronal loss and extensive subcortical and deep white matter gliosis. There was minimal demyelination of white matter, significant gliosis of thalamus and inferior olivary nuclei. The authors review the condition of progressive subcortical gliosis, compare such changes with white matter changes in other dementias, examine the relationship to Kraepelin's disease, contrast the disorder with atypical Pick's dementia, progressive dementia with motor neuron disease and Creutzfeldt-Jakob dementia.