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Article

Fatal familial insomnia: clinical and pathologic heterogeneity in genetic half brothers.

Neurology - 1 Dec 1998

Johnson M D, Vnencak-Jones C L, McLean M J

Abstract excerpt

We describe clinical and pathologic features of a patient with fatal familial insomnia (FFI) whose prion (PrP) genotype is D178N coupled with methionine at codon 129 on his mutant allele and valine at codon 129 on his normal allele. A cousin (genetic half brother) with identical PrP genotypes exhibited strikingly different clinical and pathologic changes. Comparison of these cousins shows the phenotypic...

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Identifiers

PMID: 9855529
DOI: 10.1212/wnl.51.6.1715

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

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