Inclusion: criteria 1–3 must be present
1. Most prominent clinical feature is difficulty with language
2. Language deficits are the principal cause of impaired daily living activities
3. Aphasia should be the most prominent deficit at symptom onset and for the initial phases of the disease
Exclusion: criteria 1–4 must all be absent for a PPA diagnosis
1. Patterns of deficits are better accounted for by other nondegenerative nervous system or medical disorders
2. Cognitive disturbance is better accounted for by a psychiatric diagnosis
3. Prominent initial episodic memory, visual memory, and visuoperceptual impairments
4. Prominent initial behavioral disturbance
PPA has been mainly considered a form of presentation of frontotemporal lobar degeneration (FTLD), a group of clinical, pathological, and genetically heterogeneous disorders leading to degeneration of the frontal and temporal lobes. Recent international consensus has defined working research criteria for three main presentations of PPA [2]: nonfluent/agrammatic (nfvPPA), semantic (svPPA), and logopenic (lvPPA) subtypes (Table 3.2). Distinct pathological processes have been linked to different phenotypes. Nonfluent/agrammatic variant PPA has been associated with atrophy of the left posterior fronto-insular regions and FTLD tau-positive pathology inclusion disorders, notably Pick’s disease, corticobasal degeneration, and progressive supranuclear palsy [3]. Semantic variant PPA has been related to anterior bitemporal atrophy, including the temporal pole (although greater on the left) [4] and FTLD with ubiquitin and transactive response DNA-binding protein 43 immunoreactivity (FTLD TDP-43). The logopenic variant, while representing a pathologically more heterogenous group [5], is often associated with atrophy of the temporoparietal junction area, a pattern observed in Alzheimer’s disease (AD). In fact, more than half of the logopenic cases have AD pathology and the rest FTLD pathology [6–9]. Despite this classification consensus, uncertainties still persist regarding the actual existence of the three proposed variants. This classification caveats have been recently addressed by our group [10–12]. See Table 3.2.
Core features | Ancillary features | |
|---|---|---|
Nonfluent/agrammatic variant (nfvPPA) | Agrammatism in language production | Impaired comprehension of syntactically complex sentences |
Effortful, halting speech with inconsistent speech sound errors and distortions (apraxia of speech) | Absence of single-word comprehension or object knowledge impairments | |
Semantic variant (svPPA) | Impaired confrontation naming | Impaired object knowledge, particularly for low-frequency or low-familiarity items |
Impaired single-word comprehension | Surface dyslexia or dysgraphia | |
Absence of repetition or motor speech production deficits | ||
Logopenic variant (lvPPA) | Impaired single-word retrieval in spontaneous speech and naming | Speech (phonologic) errors in spontaneous speech and naming |
Impaired repetition of sentences and phrases | Absence of single-word comprehension and object knowledge or motor speech/agrammatism impairments |
Behavior Changes in PPA
Epidemiology and Frequency
Behavior changes are increasingly recognized at initial stages of progressive neurodegenerative disorders. A marked disturbance of behavior and personality, which is associated with degeneration of the orbitofrontal and medial prefrontal cortices, represents the clinical hallmark of the behavior variant of frontotemporal dementia (bvFTD). Diagnostic criteria for this syndrome include early socially inappropriate behavior, apathy or inertia, loss of sympathy or empathy, hyperorality/dietary changes, and perseverative, stereotyped, or compulsive/ritualistic behaviors [13].
The presence of prominent behavior symptoms excludes a diagnosis of PPA. However, and despite not being initially recognized [14], it is now accepted that some behavior changes may occur early in the disease course [1, 2]. Although scarce, studies have been consistent in indicated overall high frequency rates (>80 %) of behavioral changes in PPA. Banks and Weintraub have estimated a frequency of 91 % [15]. A more recent study has indicated a frequency of 88 % [16]. Another study has estimated frequencies between 81 and 100 %, in this case the variability being accounted for by the PPA subtype considered [17]. In a similar fashion, we were able to identify in our clinical series about 82 % of PPA patients who endorsed at least one behavior symptom. The emergence of behavior and personality changes may appear simultaneously [16, 18] or slightly after the language disturbances [19]. In addition, with disease progression, the number and severity of behavior symptoms increase in PPA, overlapping bvFTD and suggesting a continuum between both syndromes, at least in some patients [20].
Type of Symptoms
With respect to the symptoms most frequently endorsed by PPA patients in general, they include depression [15, 16, 18, 21, 22], apathy, anxiety, agitation, irritability [15, 16, 18, 22], abnormal appetite/eating disorders [16, 18, 22], lack of insight [23], and disinhibition [16, 22]. Nighttime and aberrant motor behaviors, euphoria, delusions, and hallucinations, although identified in some cases, have been less frequently reported [22].
Our group recently conducted an analysis of the presence of behavioral changes in a clinical series of 94 consecutive PPA patients. Information was obtained with the caregivers through a semi-structured interview based upon the behavior/personality part of the Blessed Dementia Rating Scale (BDRSBehavior) [24]. This part of the scale consists of eleven items that measure the presence or the absence of changes in specific personalities, interests, and drives: increased rigidity, increased egocentricity, coarsening of affect, impairment of regard of feelings for others, impairment of emotional control, diminished emotional responsiveness, hilarity in inappropriate situations, sexual misdemeanor, hobbies relinquished, growing apathy, and purposeless hyperactivity. The demographic and neuropsychological data characterization of the sample is provided in Table 3.3. Nonfluent PPA patients showed significantly higher scores on the aphasia severity scale when compared to the other two variants. Consistent with the diagnostic criteria, svPPA patients showed the lowest performance on the Snodgrass and Vanderwart Naming Test, object naming, and object identification when compared to the other two variants and also had a lower performance than the lvPPA group on vocabulary. As expected, the nfvPPA group was the one that showed a significantly higher frequency of speech production deficits (agrammatism, articulation deficits, and stuttering-like dysfluencies) when compared to the other groups. Patients with nfvPPA and lvPPA both had a high frequency of hesitations in speech production. Patients with lvPPA and with nfvPPA were significantly more impaired on sentence repetition, and nfvPPA patients were significantly more impaired in writing abilities (Table 3.3).
Table 3.3
Performance in selected language tests in patients with nonfluent, semantic, and logopenic primary progressive aphasia variants
Total n = 94 | nfvPPA n = 26 | svPPA n = 36 | lvPPA n = 32 | Statistics | p-value | Post hoc | |
|---|---|---|---|---|---|---|---|
Gender (F/M) | 45:49 | 10:16 | 16:20 | 19:13 | χ2 = 2.789 | 0.248 | n.s. |
Age at onset (mean±SD)(years) | 67.1(7.8) | 67.1(7.4) | 64.5(7.0) | 70.4(8.0) | F = 5.116 | 0.008 | lvPPA > svPPA* |
Age at assessment (mean±SD)(years) | 69.4(7.7) | 70.0(7.3) | 66.5(7.2) | 72.0(7.8) | F = 4.847 | 0.010 | lvPPA > svPPA* |
Education (mean±SD)(years) | 8.2(4.5) | 8.4(4.4) | 8.3(4.6) | 8.1(4.6) | F = 0.018 | 0.982 | n.s. |
Presence of agrammatism(Y/N) | 26:60 | 13:8 | 7:27 | 6:25 | χ 2 = 13.226 | 0.001 | nfvPPA < svPPA, lvPPA a |
Presence of articulation deficits(Y/N) | 12:78 | 9:14 | 0:36 | 3:28 | χ 2 = 19.143 | <0.001 | nfvPPA < svPPA, lvPPA a |
Presence of hesitations(Y/N) | 28:59 | 11:11 | 1:33 | 16:15 | χ 2 = 21.882 | <0.001 | lvPPA, nfvPPA < svPPA a |
Presence of stuttering-like dysfluencies(Y/N) | 18:70 | 11:11 | 1:34 | 6:25 | χ 2 = 18.488 | <0.001 | nfvPPA < svPPA, lvPPA a |
Aphasia severity rating scale (mean±SD)(/6) | 3.8(1.0) | 3.2(1.1) | 4.1(0.7) | 3.9(0.8) | F = 6.925 | 0.002 | nfvPPA < svPPA, lvPPA* |
Object naming (mean±SD)(% correct) | 72.6(28.3) | 78.0(24.7) | 62.8(32.1) | 78.9(24.0) | F = 3.472 | 0.035 | n.s. |
SVNT (mean±SD) (% correct) | 68.8(23.7) | 82.7(19.5) | 54.7(24.0) | 76.6(17.2) | F = 10.636 | <0.001 | svPPA < nfvPPA, lvPPA* |
Object identification (mean±SD)(% correct) | 97.9(6.1) | 99.5(1.8) | 95.3(9.2) | 99.5(2.5) | F = 4.846 | 0.010 | svPPA < nfvPPA, lvPPA* |
Word repetition words (mean±SD)(/30) | 29.5(1.7) | 29.0(2.8) | 29.9(0.3) | 29.6(1.1) | F = 2.331 | 0.103 | n.s. |
Sentence repetition (mean±SD)(/14) | 5.9(3.6) | 4.5(3.6) | 7.8(4.0) | 4.8(1.8) | F = 8.448 | <0.001 | nfvPPA, lvPPA < svPPA* |
Comprehension of oral commands (mean±SD)(/8) | 7.5(1.0) | 7.4(0.9) | 7.4(1.3) | 7.6(0.6) | F = 0.380 | 0.685 | n.s. |
Token test (mean±SD)(/22) | 12.5(5.1) | 10.7(4.6) | 13.9(5.8) | 12.4(4.2) | F = 2.598 | 0.081 | n.s. |
Presence of alexia (Y/N) | 39:44 | 15:11 | 14:17 | 10:16 | χ 2 = 4.369 | 0.358 | n.s. |
Presence of agraphia(Y/N) | 49:43 | 20:6 | 14:22 | 15:15 | χ 2 = 10.261 | 0.036 | nfvPPA < svPPA, lvPPA a |
Vocabulary (mean±SD)(% correct) | 62.7(24.2) | 68.1(18.7) | 53.8(23.5) | 77.2(23.4) | F = 3.512 | 0.042 | svPPA < lvPPA* |
The frequency of each behavioral symptom in isolation on the overall PPA sample, as assessed by the BDRSBehavior, is shown in Table 3.4. The most prevalent symptoms were growing apathy (54.3 %), followed by impairment of emotional control (37.2 %), hobbies relinquished (28.7 %), and increased rigidity (26.6 %) (Table 3.4). These findings confirm a case-control study, sought to examine if neuropsychiatric symptoms occur over and above expected in the normal population, which identified apathy as the most significant distinguishing feature between PPA patients and controls [18].
Table 3.4
Blessed Dementia Rating Scale scores and frequency (%) of each symptom for nonfluent, semantic, and logopenic primary progressive aphasia variants
Total n = 94 | nfvPPA n = 26 | svPPA n = 36 | lvPPA n = 32 | Statistics | p-value | Post hoc | |||
|---|---|---|---|---|---|---|---|---|---|
BDRS Behavior (mean±SD) (/11)a | 1.9(1.4) | 2.3(1.4) | 1.9(1.5) | 1.4(1.1) | F = 3.412 | 0.037 | nfvPPA > lvPPA* | ||
Item 1 | Increased rigidity (%) | 26.6 | 30.8 | 27.8 | 21.9 | χ 2 = 0.623 | 0.732 | n.s. | |
Item 2 | Increased egocentricity (%) | 8.5 | 7.7 | 11.1 | 6.3 | χ 2 = 0.545 | 0.761 | n.s. | |
Item 3 | Impairment of regard of feeling for others (%) | 7.4 | 7.7 | 8.3 | 6.3 | χ 2 = 0.110 | 0.947 | n.s. | |
Item 4 | Coarsening of affect (%) | 13.8 | 15.4 | 19.4 | 3.1 | χ 2 = 2.548 | 0.280 | n.s. | |
Item 5 | Impairment of emotional control (%) | 37.2 | 38.5 | 38.9 | 34.4 | χ 2 = 0.171 | 0.918 | n.s. | |
Item 6 | Hilarity in inappropriate situations (%) | 3.2 | 3.8 | 5.6 | 0 | χ 2 = 1.742 | 0.418 | n.s. | |
Item 7 | Diminished emotional responsiveness (%) | 5.3 | 7.7 | 2.8 | 3.1 | χ 2 = 0.807 | 0.668 | n.s. | |
Item 8 | Sexual misdemeanor (%) | 0 | 0 | 0 | 0 | – | – | – | |
Item 9 | Hobbies relinquished (%) | 29.8 | 46.2 | 30.6 | 12.5 | χ 2 = 6.409 | 0.041 | nfvPPA > lvPPA aa | |
Item 10 | Growing apathy (%) | 54.3 | 65.4 | 41.7 | 50.0 | χ 2 = 3.934 | 0.140 | n.s. | |
Item 11 | Purposeless hyperactivity (%) | 3.2 | 7.7 | 2.8 | 0 | χ 2 = 2.780 | 0.249 | n.s. | |
BDRS Total (mean±SD)(/28)b | 2.8(1.8) | 3.4(1.6) | 2.8(1.9) | 2.3(1.6) | F = 2.591 | 0.080 | n.s. | ||
BDRS ADL’s (mean±SD)(/8)c | 0.9(0.8) | 0.8(0.7) | 0.9(0.8) | 0.9(0.8) | F = 0.517 | 0.598 | n.s. | ||
BDRS Habits (mean±SD) (/9) da
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