Primary progressive aphasia: a clinical approach

Abstract

The primary progressive aphasias are a heterogeneous group of focal ‘language-led’ dementias that pose substantial challenges for diagnosis and management. Here we present a clinical approach to the progressive aphasias, based on our experience of these disorders and directed at non-specialists. We first outline a framework for assessing language, tailored to the common presentations of progressive aphasia. We then consider the defining features of the canonical progressive nonfluent, semantic and logopenic aphasic syndromes, including ‘clinical pearls’ that we have found diagnostically useful and neuroanatomical and other key associations of each syndrome. We review potential diagnostic pitfalls and problematic presentations not well captured by conventional classifications and propose a diagnostic ‘roadmap’. After outlining principles of management, we conclude with a prospect for future progress in these diseases, emphasising generic information processing deficits and novel pathophysiological biomarkers.Electronic supplementary materialThe online version of this article (10.1007/s00415-018-8762-6) contains supplementary material, which is available to authorized users.

Speech and language therapy for primary progressive aphasia across the UK: A survey of current practice

“…, Marshall et al . ). Semantic variant PPA (svPPA, most often associated with an underlying FTD pathology, sometimes called semantic dementia) results in difficulties in understanding word meanings.…”

“…The literature suggests that SLTs have a wide‐ranging role in supporting people with PPA (Marshall et al . , Volkmer ) but no one yet has a UK‐wide perspective on this.…”

Qualitative and Quantitative Analyses of Brain ¹⁸Fluoro-Deoxy-Glucose Positron Emission Tomography in Primary Progressive Aphasia

“…Globally, the combined use of QL and QN evidenced that the two procedures concordantly permitted to achieve a correct diagnosis in 8 of 18 (44.4%) cases, as lvPPA in 4 cases (5)(6)(7)(8) and as nfvPPA in 4 cases (12)(13)(14)(15). In the other 10 of 18 (55.6%) patients, only QN could be useful for the correct diagnosis, as lvPPA in 5 cases (1,2,3,11,16) and as nfvPPA in the remaining 5 cases (4,9,10,17,18).…”

“…These data suggest that early clinical characterization of PPA subtypes, increasing the ability in predicting underlying disease [5] and FTD/AD clinical evolution, could help to identify the most appropriate treatment and management. However, the early diagnosis of "in vivo" PPA variants, based on clinical signs and neuropsychological data, is difficult [6], even if most patients with language disorders do not have PPA, while some cases (about 40%) could present atypical variants [7].…”

“…Neuroimaging procedures are widely used. Standard magnetic resonance imaging (MRI) has high specificity [2,7], and left frontal inferior and insular atrophy is detected in nfvPPA, predominantly left-sided, bilateral anterior temporal atrophy in svPPA, and left temporoparietal atrophy in lvPPA [7,8]. However, MRI has low sensitivity in the early phases, in particular in nfvPPA and lvPPA [9,10], and only after disease progression, when atrophy affects larger areas, the diagnosis could be supported [11].…”