Primary Progressive Aphasia

Watchlist

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Retrieved

2021-01-23

Source

Orphanet

Trials

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Genes

GRN, TARDBP, C9orf72, APOE, LAMC2, MAPT, CSF2, NEFL, SMUG1, IGFALS, ECD, PRNP, ACHE, SOD1, TOMM40, TNFRSF10B, PSIP1, KHSRP, VCP, RIDA, ABAT, TNFRSF1B, SIGLEC7, BBC3, TREM2, IL33, FOXP2, LRRK2, BPIFA2, TP53, PTPN4, TCF3, TCF4, ALOX5, APOC1, BDNF, CAT, CHI3L1, CUX1, GBA, HCLS1, IL2RB, MSMB, PPA1, PSPN, PSEN1, PSPH, PTEN, REG1A, STXBP3, AOS

Drugs

Methylthioninium

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Primary progressive aphasia (PPA) is a neurodegenerative disorder, characterized by a primary dissolution of language, with relative sparing of other mental faculties for at least the first 2 years of illness. PPA is recognized as the language variant in the frontotemporal dementia (FTD; see this term) spectrum of disorders. PPA can be classified into 3 subtypes based on specific speech and language features: semantic dementia (SD), progressive non-fluent aphasia (PNFA) and logopenic progressive aphasia (lv-PPA) (see these terms).