Classification and pathology of primary progressive aphasia.

Neurology 2013 Nov 18;81(21):1832-9. Epub 2013 Oct 18.

From the Manchester Academic Health Sciences Centre (J.M.H., C.G., J.C.T., A.M.T.R., D.N., D.d.P., P.P., D.M.A.M., J.S.S., M.J.), Cerebral Function Unit, Greater Manchester Neuroscience Centre, Salford Royal NHS Foundation Trust, Salford; and Institute of Brain, Behaviour and Mental Health (J.M.H., D.N., D.M.A.M., J.S.S., M.J.), University of Manchester, UK.

Objective: We aimed to determine the extent to which patients with progressive language impairment conform to 2011 primary progressive aphasia (PPA) classification and to examine clinicopathologic correlations within PPA variants.

Methods: Sixty-two consecutive patients with pathologically confirmed dementia who presented clinically with aphasia were identified. Patients with insufficient clinical information were excluded. PPA classifications were applied to anonymized clinical data taken from patients' initial assessment by raters who were blinded to clinical and pathologic diagnosis.

Results: The final cohort comprised 52 patients, 30 of whom met basic PPA criteria. Twenty-five patients met one of the 3 PPA classifications (13 logopenic, 8 nonfluent/agrammatic, and 4 semantic). Five patients did not meet the criteria for any of the PPA variants. All patients who met semantic variant PPA and 75% of patients who met nonfluent/agrammatic variant PPA classifications had frontotemporal lobar degeneration spectrum pathology. Pathologies were heterogeneous in patients who met logopenic variant PPA criteria (46% Alzheimer disease [AD], 8% AD mixed with dementia with Lewy bodies, 23% frontotemporal lobar degeneration, and 23% other).

Conclusion: The 2011 PPA recommendations classify a large proportion of patients who meet basic PPA criteria. However, some patients had aphasic syndromes that could not be classified, suggesting that the 2011 recommendations do not cover the full range of PPA variants. Classification of semantic variant PPA provides a good prediction of underlying pathology. Classification of logopenic variant does not successfully differentiate PPA due to AD from PPA due to other pathologies.

Download full-text PDF

Source
http://dx.doi.org/10.1212/01.wnl.0000436070.28137.7bDOI Listing
November 2013
46 Reads

Publication Analysis

Top Keywords

patients met
20
variant ppa
16
ppa
15
ppa criteria
12
ppa classifications
12
patients
11
patients meet
8
frontotemporal lobar
8
basic ppa
8
lobar degeneration
8
ppa variants
8
logopenic variant
8
semantic variant
8
primary progressive
8
progressive aphasia
8
variant
5
met
5
ppa 75%
4
75% patients
4
degeneration spectrum
4

Similar Publications