Frontotemporal Lobar Degeneration with FUS Immunoreactive Inclusions

Mild cognitive impairment and dementia.

“…FTLD refers to the underlying pathologic entities that cause degeneration in frontal and temporal regions. Previously, the terms Pick Complex or Pick's disease were used as synonyms for FTLD, but currently these are only used to designate a neuropathological diagnosis of a tauopathy with Pick bodies and cells (Neumann et al, 2016;Pang & Miller, 2016).…”

“…Importantly, not all FTLD-U cases have the TDP-43 proteinopathy. TDP-43-negative cases (also known as atypical FTLD-U), together with basophilic inclusion body disease and neuronal intermediate filament inclusion disease, have been shown to have abnormal fused in sarcoma (FUS) protein function and are now called FUS proteinopathies or FTLD-FUS, a rare cause of FTD (McMonagle & Kertesz, 2016;Neumann et al, 2016). It is important to note that logopenic PPA is not typically caused by FTLD (left temporoparietal atrophy is typical; Perry & Miller, 2018) and is usually associated with AD pathology (Norise et al, 2019).…”