Phonemic fluency quantity and quality: Comparing patients with PSP, Parkinson's disease and focal frontal and subcortical lesions.

Progressive supranuclear palsy (PSP) can be difficult to distinguish from Parkinson's disease (PD), but has a much graver prognosis. PSP is characterised severely reduced output on measures of phonemic fluency, suggesting that it may be a specific marker of PSP. However, reduced phonemic fluency has also been noted in PD, and very few studies have actually compared phonemic fluency in PSP and PD. Although anecdotal reports suggest that phonemic fluency output in PSP may have specific characteristics, with more low-frequency words and perseverative errors, no study to date has formally explored this. Further investigation into phonemic fluency output and its cognitive and neuroanatomical correlates is now critical for improving our understanding of the verbal fluency in PSP. In this study, we compared phonemic fluency characteristics (including quantity, frequency and error rates) in patients with PSP, PD and focal frontal or subcortical lesions, and age- and education-matched healthy controls. We then compared these characteristics with performance on extensive neuropsychological testing. We found that PSP patients generated significantly fewer words than patients with PD and patients with right frontal focal lesions, and healthy controls. Phonemic fluency was also significantly reduced in patients with left frontal and subcortical focal lesions. However, there were no significant group differences in word frequency or error rates. Phonemic fluency was best predicted by performance on the Vocabulary and Hayling neuropsychological tests. We argue that these findings provide important evidence that reduced phonemic fluency is a hallmark of PSP and argue that the specificity of this impairment betrays an underlying impairment in energization, reflecting dysfunction of left frontal and subcortical networks.

Sensitivity and Specificity of the ECAS in Parkinson's Disease and Progressive Supranuclear Palsy.

Disentangling Parkinson's disease (PD) and progressive supranuclear palsy (PSP) may be a diagnostic challenge. Cognitive signs may be useful, but existing screens are often insufficiently sensitive or unsuitable for assessing people with motor disorders. We investigated whether the newly developed ECAS, designed to be used with people with even severe motor disability, was sensitive to the cognitive impairment seen in PD and PSP and able to distinguish between these two disorders. Thirty patients with PD, 11 patients with PSP, and 40 healthy controls were assessed using the ECAS, as well as an extensive neuropsychological assessment. The ECAS detected cognitive impairment in 30% of the PD patients, all of whom fulfilled the diagnostic criteria for mild cognitive impairment. The ECAS was also able to detect cognitive impairment in PSP patients, with 81.8% of patients performing in the impaired range. The ECAS total score distinguished between the patients with PSP and healthy controls with high sensitivity (91.0) and specificity (86.8). Importantly, the ECAS was also able to distinguish between the two syndromes, with the measures of verbal fluency offering high sensitivity (82.0) and specificity (80.0). In sum, the ECAS is a quick, simple, and inexpensive test that can be used to support the differential diagnosis of PSP.

ECAS A-B-C: alternate forms of the Edinburgh Cognitive and Behavioural ALS Screen.

BACKGROUND: The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) is a short assessment by which neuropsychological symptoms can be detected and quantified in people with ALS. To avoid potential practice effects with repeated administration, here we present alternative versions of the ECAS suitable for measuring change over time. OBJECTIVE: To develop two alternate versions of the ECAS: ECAS-B and ECAS-C. METHOD: One hundred and forty-nine healthy adult participants were recruited. Thirty participants completed a pilot study in developing the alternate versions. Two groups of 40 participants were administered the ECAS-B or ECAS-C and compared to published data of the original ECAS (ECAS-A) to determine equivalence. An additional 39 participants were administered the ECAS consecutively, either repeating the original version (ECAS-A-A-A) serially or the different versions (ECAS-A-B-C) to determine potential practice effects. Recordings of assessments were scored by a second researcher to determine inter-rater reliability. RESULTS: No significant differences were found between versions (A, B, C) of the composite performance measures of ALS Specific, ALS Non-Specific, and ECAS Total scores. Repeated serial administration of ECAS-A (A-A-A) produced some practice effects for composite scores, whereas no such effects were found when alternate versions were administered serially (A-B-C). Exceptionally high intra-class correlations were found for all three versions of the ECAS suggesting a high degree of rater agreement. CONCLUSION: The newly developed alternate forms of the ECAS are both highly equitable to the original ECAS-A and enable avoidance of practice effects, thus supporting their use in measuring cognition and behaviour over time.

Visual feature binding in younger and older adults: encoding and suffix interference effects.

Three experiments investigated younger (18-25 yrs) and older (70-88 yrs) adults' temporary memory for colour-shape combinations (binding). We focused upon estimating the magnitude of the binding cost for each age group across encoding time (Experiment 1; 900/1500 ms), presentation format (Experiment 2; simultaneous/sequential), and interference (Experiment 3; control/suffix) conditions. In Experiment 1, encoding time did not differentially influence binding in the two age groups. In Experiment 2, younger adults exhibited poorer binding performance with sequential relative to simultaneous presentation, and serial position analyses highlighted a particular age-related difficulty remembering the middle item of a series (for all memory conditions). Experiments 1-3 demonstrated small to medium binding effect sizes in older adults across all encoding conditions, with binding less accurate than shape memory. However, younger adults also displayed negative effects of binding (small to large) in two of the experiments. Even when older adults exhibited a greater suffix interference effect in Experiment 3, this was for all memory types, not just binding. We therefore conclude that there is no consistent evidence for a visual binding deficit in healthy older adults. This relative preservation contrasts with the specific and substantial deficits in visual feature binding found in several recent studies of Alzheimer's disease.