title: Hippocampal sclerosis in tau-negative frontotemporal lobar degeneration.
abstract: Tau-negative frontotemporal lobar degeneration (FTLD) can be divided into those with motor neuron disease (FTLD-MND), and those without MND, but with ubiquitin-immunoreactive neuronal inclusions (FTLD-U). Some authors group FTLD-U and FTLD-MND together as tau-negative FTLD, but others separate them on the basis of clinical, pathologic and imaging differences. In 103 cases of pathologically confirmed, tau-negative FTLD (FTLD-MND and FTLD-U), we assessed the frequency of hippocampal sclerosis defined as neuronal loss in the subicular or CA1 regions of the hippocampus. The subjects in the FTLD-U group were older at death and had longer disease duration. After adjusting for age at death and disease duration, we found a significant difference in the frequency of hippocampal sclerosis in the FTLD-U group (79%) compared to FTLD-MND group (26%) (p=0.02). The difference in frequency of HpScl in FTLD-U compared to FTLD-MND is further evidence that they are separate clinicopathologic entities.
authors.affiliation: Department of Neurology, Behavioral Neurology & Movement Disorders, Mayo Clinic, Rochester, MN 5590, United States. josephs.keith@mayo.edu
authors.forename: Keith A
authors.initials: KA
authors.lastname: Josephs
authors.forename: Dennis W
authors.initials: DW
authors.lastname: Dickson
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003704:Dementia / Q000235:genetics* / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006624:Hippocampus / Q000737:chemistry* / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012598:Sclerosis; D016875:tau Proteins / Q000032:analysis* / Q000172:deficiency / Q000235:genetics
chemical_list: D016875:tau Proteins