Neuropathologic analysis of brain tissue from FTLD-TDP patients. Ubiquitin immunohistochemistry in cases of familial FTLD-TDP demonstrates staining of (a) neurites and neuronal cytoplasmic inclusions in the superficial cerebral neocortex, (b) neuronal cytoplasmic inclusions in hippocampal dentate granule cells, and (c) neuronal intranuclear inclusions in the cerebral neocortex (arrows). Scale bar; (a) and (b) 40 μm, (c) 25 μm, insert 6 μm.
Specialty
Neurology, Psychiatry
Complications
Brain death
Frontotemporal lobar degeneration (FTLD) is a pathological process that occurs in frontotemporal dementia. It is characterized by atrophy in the frontal lobe and temporal lobe of the brain, with sparing of the parietal and occipital lobes.[1][2]
Common proteinopathies that are found in FTLD include the accumulation of tau proteins and TAR DNA-binding protein 43 (TDP-43). Mutations in the C9orf72 gene have been established as a major genetic contribution of FTLD, although defects in the granulin (GRN) and microtubule-associated proteins (MAPs) are also associated with it.[3]
^Whitwell, Jennifer L.; Anderson, Valerie M.; Scahill, Rachael I.; Rossor, Martin N.; Fox, Nick C. (2004). "Longitudinal Patterns of Regional Change on Volumetric MRI in Frontotemporal Lobar Degeneration". Dementia and Geriatric Cognitive Disorders. 17 (4): 307–310. doi:10.1159/000077160. ISSN 1420-8008.
^Lu, Po H.; Mendez, Mario F.; Lee, Grace J.; Leow, Alex D.; Lee, Hyun-Woo; Shapira, Jill; Jimenez, Elvira; Boeve, Bradley B.; Caselli, Richard J.; Graff-Radford, Neill R.; Jack, Clifford R.; Kramer, Joel H.; Miller, Bruce L.; Bartzokis, George; Thompson, Paul M. (2013-01-09). "Patterns of Brain Atrophy in Clinical Variants of Frontotemporal Lobar Degeneration". Dementia and Geriatric Cognitive Disorders. 35 (1–2): 34–50. doi:10.1159/000345523. ISSN 1420-8008. PMC 3609420. PMID 23306166.
^van der Zee, Julie; Van Broeckhoven, Christine (7 January 2014). "Dementia in 2013: Frontotemporal lobar degeneration—building on breakthroughs". Nature Reviews Neurology. 10 (2): 70–72. doi:10.1038/nrneurol.2013.270. PMID 24394289.
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