| Prognosis | Slowest disease progression among FTLD-TDP subtypes (mean duration ~9.7 years). |
| Chronic traumatic encephalopathy (CTE) | Associated with repetitive traumatic-brain-injury; features unique perivascular tau deposits at the depths of cortical sulci. |
| Morphology | Granulofilamentous NCIs with abundant fine granular neuritic pathology across all cortical layers. |
| Distribution | Widespread cortical and subcortical involvement. |
| Genetics | Most FTLD-FUS cases are sporadic; familial FUS mutations more commonly cause als than pure FTD |
| Clinical | Rapidly progressive frontotemporal degeneration with remarkably short disease duration (~2.1 years). |
| FUS protein | FUS is an RNA-binding protein involved in transcription, splicing, and DNA repair; cytoplasmic aggregation impairs these nuclear functions |
| Subtypes | Three histological patterns — NIFID (neuronal intermediate filament inclusion disease), aFTLD-U (atypical FTLD with ubiquitin inclusions), and BIBD (basophilic inclusion body disease) |
| Clinical presentation | Typically young-onset behavioral variant FTD (often before age 40), frequently with prominent psychiatric features |
| Key distinction from FTLD-TDP | FUS inclusions are immunoreactive for all FET family proteins (FUS, EWSR1, TAF15), unlike tdp-43 inclusions (Neumann et al., 2011) |
| Databases | OMIMOrphanetClinicalTrialsPubMed |