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Corresponding author. This article has been cited by other articles in PMC. Supplementary material 2 MP3 kb. Supplementary material 3 MP3 kb. Supplementary material 4 MP3 kb. Supplementary material 5 DOCX 14 kb. Electronic supplementary material The online version of this article Introduction The primary progressive aphasias PPA are a diverse group of disorders that collectively present with relatively focal degeneration of the brain systems that govern language.

Table 1 Summary of key language features and cognitive, neurological, neuroanatomical and neuropathological associations in syndromes of primary progressive aphasia. Open in a separate window. Table 2 A framework for assessment of language functions, directed particularly to progressive aphasias.

Communication task Cognitive process Key history Clinical test Neuropsychological test SYND Message production Idea Generating verbal idea Reduced initiation of conversation a Describe a recent vacation; generating words by initial letter e. Edmunds h Dabul Apraxia battery for adults. Second, Pro-Ed: Austin i Kay et al. Table 3 Examples of spoken and written language output in patients with canonical syndromes of primary progressive aphasia.

Syndrome Speech transcriptions Written sentences nfvPPA: prominent speech apraxia The lady is drying a the plate, which she has washed. The man a boy um a cookie is on there. Um a daughter a no no um sister… And when she gonna throwen on them. And they broken and book on there on there stool on round by them.

And lady and she was having a washing on there and they be taps on them and drain is come on the floor one there. And um…And this man are there bear maybe going in the went on there… working out the window.

With water running down. The lvPPA cases show marked word retrieval difficulty impaired message content reflected in prolonged pauses when speaking and trailing off of written sentences and in addition, incorrect syllable selection impaired message structure; underlined despite intact sentence construction; the speech sample of the lvPPA case here illustrates the challenge of reliably assessing spoken grammar in the setting of severe word-finding difficulty lvPPA logopenic variant primary progressive aphasia, nfvPPA nonfluent—agrammatic variant primary progressive aphasia, svPPA semantic variant primary progressive aphasia.

This Table presents some clinical observations that are not currently emphasised in standard diagnostic formulations but which we have found useful in the bedside diagnosis of the major syndromes of primary progressive aphasia lvPPA logopenic variant primary progressive aphasia, nfvPPA nonfluent—agrammatic variant primary progressive aphasia, svPPA semantic variant primary progressive aphasia. A clinical framework for assessing language functions in primary progressive aphasia When confronted by an aphasic patient, it is important firstly to establish the context of the language disturbance.

Canonical syndromes of primary progressive aphasia: nonfluent—agrammatic Clinical presentation Patients with nfvPPA present with slow, effortful, hesitant and distorted speech Table 3 ; Supplementary sound files 1 and 2.

Key associations General intellect is often remarkably well preserved, though a degree of executive dysfunction is usual and may be accompanied behaviourally by apathy or impulsivity [ 29 , 30 ].

Canonical syndromes of primary progressive aphasia: semantic Clinical presentation In striking contrast to nfvPPA, patients with svPPA exhibit well structured, well articulated language that is relentlessly bereft of substance Table 3 ; Supplementary sound file 3. Neuroanatomy On neuroimaging, svPPA has a hallmark pattern of asymmetric, focal cerebral atrophy chiefly involving the dominant anteroinferior and mesial temporal lobe, including amygdala and anterior hippocampus [ 9 , 48 ].

Key associations A behavioural syndrome similar to that defining the behavioural variant of frontotemporal dementia characteristically develops in svPPA and indeed, these syndromes can occasionally be difficult to distinguish, even after a careful history. Canonical syndromes of primary progressive aphasia: logopenic Clinical presentation The most recently described of the major PPA syndromes is in more than one sense the most problematic.

Neuroanatomy The key neuroimaging association is asymmetric atrophy mainly involving the temporo-parietal junction zone of the dominant hemisphere, appearing as widening of the posterior left Sylvian fissure on a T1-weighted coronal MRI scan [ 68 ] Fig.

Key associations A number of patients with lvPPA exhibit generalised anxiety, irritability and increased clinging emotionally and physically to their primary caregivers [ 29 , 70 , 71 ].

Toward the diagnosis: a path with pitfalls Accurate and early identification of PPA syndromes is essential for clinical counselling and planning appropriate management. We next consider some common potential pitfalls on the path to diagnosis. The patient with comorbidities Interpretation of apparent language deficits should be cautious in older patients with a history of developmental dyslexia or longstanding peripheral hearing loss, especially when the recent symptoms target phonology or articulation and if additional cognitive involvement is subtle.

An outline of management Patients with PPA generally live for a number of years following diagnosis, with evolving deficits and specific needs at each stage of the illness. Non-pharmacological strategies Management begins with diagnosis; this is often delayed due to the lack of experience with these conditions in the wider medical community and the value of a clear explanation for patients and families should never be underestimated.

Pharmacological interventions There are currently no disease modifying treatments for PPA and evidence for efficacy of symptomatic treatments is scant. Conclusions and future directions Recognition of the major PPA syndromes has transformed our understanding of the language system and given us a new picture of selective neural vulnerability in degenerative brain disease.

Electronic supplementary material Below is the link to the electronic supplementary material. Supplementary material 1 MP3 kb 3. Supplementary material 2 MP3 kb audio file. Supplementary material 3 MP3 kb 1.

Supplementary material 4 MP3 kb 1. Compliance with ethical standards Conflicts of interest The authors declare that they have no conflicts of interest. Footnotes Charles R. Contributor Information Charles R.

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