Table 1.

Some ‘fingerprints’ of primary progressive aphasia at diagnosis

Initial suspiciona	Is it primary progressive aphasia?
Speech/language symptoms	Spelling errors (recent onset), work/social activities impacted, progressive decline
Other symptoms	Difficulty using/learning devices, increased difficulty hearing in busy environments, changes in personality and inter-personal interactions (often subtle)b

Syndromic diagnosis	nfvPPA	svPPA	lvPPA
Speech/language: history	Hesitant speech with errors of pronunciation and/or grammar, binary (especially ‘yes/no’) confusions	Asking the meaning of familiar words, keeping personal ‘dictionaries’	Losing thread of sentences, frequent tip-of-tongue hesitations in conversation, occasional jargon (neologisms)
Speech/language: examination	Speech apraxia: obviously effortful, distorted speech sounds with ‘groping’ after target sound, reduced articulatory agility (difficulty repeating syllable strings, e.g. ‘pa-ke-ta’) Grammatical errorsc	Impaired single word comprehensiond: unable to define words/identify items nominated by examiner Regularisation errors on reading aloud, e.g. sounding yacht as ‘yached’ (‘surface dyslexia’)	Impaired verbal working memory: errors repeating phrases/sentences, more prominent with increasing target length, despite intact repetition of single words; reduced digit span
Other clinical featurese	Orofacial apraxia Neurological (especially extrapyramidal) signsf	Visual agnosiag New interests (e.g. puzzles, numbers, music), clockwatching	Difficulty with route-finding Limb apraxia Nonverbal episodic memory impairment Visuospatial difficulties
Brain MRI atrophy signatures	Left anterior peri-Sylvian	Predominantly left anterior, mesial and inferior temporalh	Left posterior temporal/parietal

In this table, we summarise symptoms, signs and other features we have found useful in corroborating an initial clinical suspicion of primary progressive aphasia (PPA) and in differentiating between the major variant syndromes: nonfluent/agrammatic (nfvPPA), semantic (svPPA) and logopenic (lvPPA) (see also Figs. 1, 2, 3). The table is informed in part by surveys of caregivers for people living with primary progressive aphasia [5, 112]; however, not all patients with a given diagnosis will exhibit every feature, the chronology of deficits varies between individuals and nonverbal cognitive, behavioural and neurological features tend to become more prominent later in the illness (see text). a These features in our experience tend to develop early across syndromes and are useful in distinguishing PPA from other presentations with ‘word finding difficulty’, including health anxiety or functional cognitive disorder; b may include changes in humour, sociability, initiative, etc. as well as mood symptoms; c tends to be less prominent than speech apraxia but may sometimes lead the presentation, more reliably detected in written sentences if speech is very effortful; d this must be distinguished from anomia due to impaired word retrieval, which is very prominent in both svPPA and lvPPA and commonly seen across the PPA spectrum, and in other patients presenting to memory clinics; e by definition, initially subordinate to the language problem but of diagnostic and clinical relevance if present; f mild features of atypical parkinsonism (or less commonly, motor neuron disease) may be evident at presentation; g unable to recognise and/or demonstrate use of familiar items by sight); h we question the diagnosis of svPPA if the characteristic atrophy profile is absent, MRI features in other syndromes are substantially more variable