期刊
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
卷 67, 期 4, 页码 271-279出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/NEN.0b013e31816a12a6
关键词
dementia lacking distinctive histopathology; dystrophic neurites; frontoternporal lobar degeneration with motor neuron disease; frontotemporal lobar degeneration with ubiquitinated inclusions; immunohistochemistry; progranulin; TAR DNA-binding protein 43
资金
- NIA NIH HHS [U01 AG016976, P30 AG012300-159002, P30 AG012300, P50 AG005681, AG12300, P30 AG013854, P01-AG03991, P50-AG05681, P01 AG003991] Funding Source: Medline
TAR DNA-binding protein 43 (TDP-43) is a major component of the inclusions in frontotemporal lobar degeneration with ubiquitinated inclusions (FTLD-U). We studied TDP-43 pathology in the hippocampus and frontal cortex of autopsy brains from patients with FTLD-U (n = 68), dementia lacking distinctive histopathology (n = 4), other neurodegenerative diseases (n = 23), and controls (n = 12) using a sensitive immunohistochemistry protocol. Marked enhancement of staining of TDP-43-positive dystrophic neurites (DNs) was obtained, and we observed 2 previously unrecognized pathologic patterns (i.e. frequent long DNs in the CA1 region and frequent dot-like DNs in the neocortical layer 2) in 39% and 15% of the FTLD-U cases, respectively. Frequent long DNs, but not dot-like DNs, were significantly associated with progranulin mutations. Based on this evaluation, 4 FTLD-U cases showed no TDP-43 pathology and were reclassified as FTLD-U, non-TDP-43 proteinopathy, and 3 cases of dementia lacking distinctive histopathology were reclassified as FTLD-U. Of the cases with other neurodegenerative diseases, 43% showed TDP-43 pathology in the hippocampus, but only 4% showed TDP-43 pathology in the frontal cortex. No TDP-43 pathology was seen in controls. These results indicate that the sensitivity of the TDP-43 immunohistochemistry method affects both the extent and type of abnormalities detected. Moreover, assessment of abnormalities in both the hippocampus and frontal cortex may be diagnostically important in FTLD-U.
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