| H2 Haplotype | The H2 haplotype is protective and is in linkage disequilibrium with the inversion polymorphism that reduces 4R-tau expression. |
| MAPT | Most cases are sporadic with H1 haplotype association |
| Chromosome 17q21.31 | Region linked to FTDP-17 shows overlap |
| Familial cases | Rare but reported with MAPT mutations |
| Databases | OMIMOrphanetClinicalTrialsPubMed |
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