Binary reversals: a diagnostic sign in primary progressive aphasia.

BACKGROUND: Binary reversals (exemplified by 'yes'/'no' confusions) have been described in patients with primary progressive aphasia (PPA) but their diagnostic value and phenotypic correlates have not been defined. METHODS: We conducted a retrospective cohort study analysing demographic, clinical, neuropsychological, linguistic and behavioural data from patients representing all major PPA syndromes (non-fluent/agrammatic variant, nfvPPA; logopenic variant, lvPPA; semantic variant, svPPA) and behavioural variant frontotemporal dementia (bvFTD). The prevalence of binary reversals and behavioural abnormalities, illness duration, parkinsonian features and neuropsychological test scores were compared between neurodegenerative syndromes, and the diagnostic predictive value of binary reversals was assessed using logistic regression. RESULTS: Data were obtained for 83 patients (21 nfvPPA, 13 lvPPA, 22 svPPA, 27 bvFTD). Binary reversals occurred in all patients with nfvPPA, but significantly less frequently and later in lvPPA (54%), svPPA (9%) and bvFTD (44%). Patients with bvFTD with binary reversals had significantly more severe language (but not general executive or behavioural) deficits than those without reversals. Controlling for potentially confounding variables, binary reversals strongly predicted a diagnosis of nfvPPA over other syndromes. CONCLUSIONS: Binary reversals are a sensitive (though not specific) neurolinguistic feature of nfvPPA, and should suggest this diagnosis if present as a prominent early symptom.

Preserved musical working memory and absolute pitch in posterior cortical atrophy.

Working memory for nonverbal auditory information is essential for everyday functioning but its cognitive organisation is not well understood. Here we addressed this issue in a musician, YA, with absolute pitch (AP, the uncommon ability to categorise and label individual musical pitches without an external reference) who developed posterior cortical atrophy. We assessed YA's AP ability and her working memory for pitch and rhythmic patterns using procedures modelled on a standard test of auditory verbal working memory (digit span), referenced to age-matched, cognitively-normal AP and non-AP possessing musicians. YA had retained AP and performed comparably to healthy older AP and non-AP musicians on all musical working memory tasks, despite impaired auditory verbal working memory. These findings suggest that the cognitive mechanisms for auditory verbal working memory, nonverbal (pitch and rhythm) working memory and AP are at least partly dissociable, and both musical working memory and AP can be spared despite posterior parietal degeneration.

EXPRESS: Pure-tone audiometry and dichotic listening in primary progressive aphasia and Alzheimer's disease.

Hearing is multifaceted and the relative contributions of peripheral and central hearing loss are rarely considered together in the context of dementia. Here, we assessed peripheral (as measured with pure-tone audiometry) and central (as measured with dichotic listening) hearing in 19 patients with typical amnestic Alzheimer's disease (tAD), 10 patients with logopenic variant primary progressive aphasia (lvPPA), 11 patients with nonfluent/agrammatic variant PPA (nfvPPA), 15 patients with semantic variant PPA (svPPA), and 28 healthy age-matched individuals. Participants also underwent neuropsychological assessment and magnetic resonance image scanning, allowing us to use voxel-based morphometry to assess associations between hearing scores and grey matter volume. Dichotic listening was impaired in all patient groups relative to healthy controls. In the combined patient (but not healthy control) cohort, dichotic listening scores were significantly correlated with measures of global cognitive functioning and speech-based neuropsychological tasks. Pure-tone audiometry scores were not significantly elevated in any patient group relative to the healthy control group, and no significant correlations were observed between peripheral hearing and neuropsychological task performance in either the combined patient or healthy control cohorts. Neuroanatomically, dichotic listening performance was associated with grey matter volume in a bilateral fronto-temporo-parietal network over the combined patient cohort, but no correlates were identified for pure-tone audiometry. Our findings highlight the importance of speech parsing mechanisms beyond elementary sound detection in driving cognitive test performance, underline the importance of assessing central hearing alongside peripheral hearing in people with dementia, and further delineate the complex auditory profiles of neurodegenerative dementias.

Musical experience influences socio-emotional functioning in behavioural variant frontotemporal dementia.

Objectives: On phenotypic and neuroanatomical grounds, music exposure might potentially affect the clinical expression of behavioural variant frontotemporal dementia (bvFTD). However, this has not been clarified. Methods: 14 consecutive patients with bvFTD fulfilling consensus diagnostic criteria were recruited via a specialist cognitive clinic. Earlier life musical experience, current musical listening habits and general socio-emotional behaviours were scored using a bespoke semi-quantitative musical survey and standardised functional scales, completed with the assistance of patients' primary caregivers. Associations of musical scores with behavioural scales were assessed using a linear regression model adjusted for age, sex, educational attainment and level of executive and general cognitive impairment. Results: Greater earlier life musical experience was associated with significantly lower Cambridge Behavioural Inventory (Revised) scores (ß ± SE = -17.2 ± 5.2; p = 0.01) and higher Modified Interpersonal Reactivity Index (MIRI) perspective-taking scores (ß ± SE = 2.8 ± 1.1; p = 0.03), after adjusting for general cognitive ability. Number of hours each week currently spent listening to music was associated with higher MIRI empathic concern (ß ± SE = 0.7 ± 0.21; p = 0.015) and MIRI total scores (ß ± SE = 1.1 ± 0.34; p = 0.014). Discussion: Musical experience in earlier life and potentially ongoing regular music listening may ameliorate socio-emotional functioning in bvFTD. Future work in larger cohorts is required to substantiate the robustness of this association, establish its mechanism and evaluate its clinical potential.