Lack of Frank Agrammatism in the Nonfluent Agrammatic Variant of Primary Progressive Aphasia

Dement Geriatr Cogn Dis Extra. 2016 Sep 23;6(3):407-423. doi: 10.1159/000448944. eCollection 2016 Sep-Dec.

Abstract

Background/aims: Frank agrammatism, defined as the omission and/or substitution of grammatical morphemes with associated grammatical errors, is variably reported in patients with nonfluent variant primary progressive aphasia (nfPPA). This study addressed whether frank agrammatism is typical in agrammatic nfPPA patients when this feature is not required for diagnosis.

Method: We assessed grammatical production in 9 patients who satisfied current diagnostic criteria. Although the focus was agrammatism, motor speech skills were also evaluated to determine whether dysfluency arose primarily from apraxia of speech (AOS), instead of, or in addition to, agrammatism. Volumetric MRI analyses provided impartial imaging-supported diagnosis.

Results: The majority of cases exhibited neither frank agrammatism nor AOS.

Conclusion: There are nfPPA patients with imaging-supported diagnosis and preserved motor speech skills who do not exhibit frank agrammatism, and this may persist beyond the earliest stages of the illness. Because absence of frank agrammatism is a subsidiary diagnostic feature in the logopenic variant of PPA, this result has implications for differentiation of the nonfluent and logopenic variants, and indicates that PPA patients with nonfluent speech in the absence of frank agrammatism or AOS do not necessarily have the logopenic variant.

Keywords: Apraxia of speech; Diagnostic criteria; Differential diagnosis; Frontotemporal dementia.