Abstract

Conclusions: FUS protein accumulation is characteristic in a group of FTLDs with ubiquitin-positive pathology. The clinical features are heterogeneous although the most common association is with bvFTD. Clinical and pathological evidence of MND may be found in cases previously diagnosed as NIFID. FUS-positive inclusions in both cerebral cortex and motor neurons in NIFID and aFTLD-U extend the spectrum of diseases with frontotemporal degeneration and involvement of the motor system.

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