TY - JOUR
T1 - Hippocampal sclerosis in tau-negative frontotemporal lobar degeneration
AU - Josephs, Keith A.
AU - Dickson, Dennis W.
N1 - Funding Information:
KAJ is supported by a NIH Roadmap Multidisciplinary Clinical Research Career Development Award Grant (K12/NICHD)-HD49078 and the H. Robert and Clarice Smith and Abigail Van Buren Alzheimer's Disease Research Program of the Mayo Foundation. DWD is supported by NIH grants: P50-AG16574, P01-AG17216 and P01-AG14449. The Jacksonville brain bank has contributions from the State of Florida Alzheimer Disease Initiative, the Society for Progressive Supranuclear Palsy, the Einstein Aging Study (P01-AG03949) and the Udall Center for Excellence in Parkinson's Disease Research (P50-NS40256). We extend our thanks to our colleagues with whom we work in aging and neurodegenerative disease research, namely David S. Knopman, MD, Joseph E. Parisi, MD, Ronald C. Petersen, PhD, MD, Bradley F. Boeve and Neill Graff-Radford, MD We particularly extend our gratitude to the patients and their families for participating in research on aging and neurodegenerative disease.
PY - 2007/11
Y1 - 2007/11
N2 - 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.
AB - 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.
KW - Frontotemporal lobar degeneration
KW - Hippocampal sclerosis
KW - Motor neuron disease
KW - Ubiquitin
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U2 - 10.1016/j.neurobiolaging.2006.07.010
DO - 10.1016/j.neurobiolaging.2006.07.010
M3 - Article
C2 - 16930776
AN - SCOPUS:34548575051
SN - 0197-4580
VL - 28
SP - 1718
EP - 1722
JO - Neurobiology of aging
JF - Neurobiology of aging
IS - 11
ER -