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Article

A stop-codon mutation in the BRI gene associated with familial British dementia.

Nature - 24 Jun 1999

Vidal R, Frangione B, Rostagno A, Mead S, Révész T, Plant G, Ghiso J

Abstract excerpt

Familial British dementia (FBD), previously designated familial cerebral amyloid angiopathy-British type, is an autosomal dominant disorder of undetermined origin characterized by progressive dementia, spasticity, and cerebellar ataxia, with onset at around the fifth decade of life. Cerebral amyloid angiopathy, non-neuritic and perivascular plaques and neurofibrillary tangles are the predominant pathological...

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Identifiers

PMID: 10391242
DOI: 10.1038/21637

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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