Neuropathology of chromosome-17-linked dementia

We describe the characteristic neuropathologic changes in chromosome-17-lin ked dementia. Identified cases to date demonstrate a uniform pathology cons isting of neuronal loss, gliosis and vacuolization of frontal, anterior cin gulate and temporal cortex and occipital association areas, Similar changes are present in substantia nigra, ventral striatum and amygdala, Hippocampu s itself is spared, whereas the afferent perforant tract is consistently af fected. Characteristic neuronal inclusions consisting of lattice-like neuro filaments are present in subcortical nuclei and glial cytoplasmic tau-posit ive inclusions are present throughout the white matter. These pathologic ch anges appear to be unique for chromosome-17-linked dementia.