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Article

Neuropathologic heterogeneity in HDDD1: a familial frontotemporal lobar degeneration with ubiquitin-positive inclusions and progranulin mutation.

Alzheimer disease and associated disorders - 1 Jan 2000

Behrens Maria I, Mukherjee Odity, Tu Pang-hsien, Liscic Rajka M, Grinberg Lea Tenenholz, Carter Deborah, Paulsmeyer Katherine, Taylor-Reinwald Lisa, Gitcho Michael, Norton Joanne B, Chakraverty Sumi, Goate Alison M, Morris John C, Cairns Nigel J

Abstract excerpt

Hereditary dysphasic disinhibition dementia (HDDD) describes a familial disorder characterized by personality changes, and language and memory deficits. The neuropathology includes frontotemporal lobar atrophy, neuronal loss and gliosis and, in most cases, abundant Abeta plaques and neurofibrillary tangles (NFTs). A Pick/Alzheimer's spectrum was proposed for the original family (HDDD1). Here we report the...

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PMID: 17334266
DOI: 10.1097/WAD.0b013e31803083f2

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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