Atypical, slowly progressive behavioural variant frontotemporal dementia associated with C9ORF72 hexanucleotide expansion.

doi:10.1136/jnnp-2011-301883

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Article

Atypical, slowly progressive behavioural variant frontotemporal dementia associated with C9ORF72 hexanucleotide expansion.

Journal of neurology, neurosurgery, and psychiatry - 1 Apr 2012

Khan Baber K, Yokoyama Jennifer S, Takada Leonel T, Sha Sharon J, Rutherford Nicola J, Fong Jamie C, Karydas Anna M, Wu Teresa, Ketelle Robin S, Baker Matthew C, Hernandez Mariely-Dejesus, Coppola Giovanni, Geschwind Daniel H, Rademakers Rosa, Lee Suzee E, Rosen Howard J, Rabinovici Gil D, Seeley William W, Rankin Katherine P, Boxer Adam L, Miller Bruce L

Abstract excerpt

BACKGROUND: Some patients meeting behavioural variant frontotemporal dementia (bvFTD) diagnostic criteria progress slowly and plateau at mild symptom severity. Such patients have mild neuropsychological and functional impairments, lack characteristic bvFTD brain atrophy and have thus been referred to as bvFTD 'phenocopies' or slowly progressive (bvFTD-SP). The few patients with bvFTD-SP that have been studied at...

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PMID: 22399793
DOI: 10.1136/jnnp-2011-301883
PMCID: PMC3388906

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