Emergence of artistic talent in logopenic variant of primary progressive aphasia: a case report.

The case report covers the investigation of a 59-year-old woman, with the diagnosis of logopenic variant of primary progressive aphasia (lvPPA), who presented de novo visual artistic talent after the onset of language deficits. Similar cases have been described in patients with semantic and non-fluent/agrammatic variants of primary progressive aphasia. However, this is the first related case with lvPPA reported in the literature. Upon her initial visit at the Neurology department in 2020, a neurological and neuropsychological assessment were conducted to establish the diagnosis. A neuropsychological reassessment was performed in 2022. Neuroimaging data indicated decreased blood flow of the left hemisphere, while neuropsychological deficits were diffuse with predominant language production disorder. A description of the patient's portraits and suggestions regarding possible neural mechanisms associated with the manifestation of this ability are provided.

The Parkinson's Disease-Cognitive Rating Scale: Greek Normative Data, Clinical Utility and Cultural Considerations.

BACKGROUND: The Parkinson's Disease-Cognitive Rating Scale (PD-CRS) is a comprehensive screening procedure for the evaluation of cognitive impairment in patients with Parkinson's disease (PD). OBJECTIVES: In the present study we adjusted the PD-CRS for the Greek population, developed normative data and examined its clinical utility for the assessment of cognitive functioning in Greek PD patients. In addition, the correlation of clinical characteristics with cognitive performance in PD patients was examined. METHODS: Three hundred four community-dwelling healthy adults and 59 patients with PD, completed the adapted PD-CRS. RESULTS: Healthy adults outperformed the PD patients on the total, the cortical and subcortical scores of the PD-CRS. Normative data indicated effects of both education and age on the PD-CRS. The optimal total PD-CRS cutoff score for the identification of cognitive impairment in a heterogeneous sample of PD patients, with regard to the severity of cognitive difficulties, was 79, yielding a modest sensitivity and specificity. Clinical characteristics of the patients (i.e., disease duration and functional disease burden) were related to poor performance on the PD-CRS. CONCLUSIONS: The Greek version of the PD-CRS is a useful instrument for the assessment of cognition in PD. Future prospective studies should examine its clinical utility to identify PD-cognitive subtypes (i.e., PD patients with mild cognitive impairment), to monitor cognitive changes, as well as its predictive accuracy for subsequent dementia.

Hereditary diffuse leukoencephalopathy with spheroids mimicking primary progressive aphasia: report of a Greek case.

INTRODUCTION: Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is an adult onset leukodystrophy, causally related to mutations in the colony-stimulating factor 1 receptor (CSF1R) gene. We report the unique case of a Greek HDLS patient, demonstrating an unusual phenotype, reminiscent of primary progressive aphasia (PPA). METHODS: A 63-year-old woman was referred with a 2-year history of deteriorating language and memory deficits, apathy, and two generalized tonic-clonic seizures. Neurological and neuropsychological examination revealed prominent aphasia with a pattern consistent with nonfluent variant of PPA. However, brain MRI disclosed confluent T2 and FLAIR white matter hyperintensities with frontal emphasis, whereas genetic testing corroborated the diagnosis of HDLS. DISCUSSION: PPA-like patterns may rarely develop in the context of HDLS. Prompt diagnosis of this leukoencephalopathy is essential, since preliminary data suggest that it could represent a potentially treatable disorder.

Selective executive impairments as neuroimmunological manifestations of the human immunodeficiency virus.

Executive processes that predominantly effect people living with human immunodeficiency virus remain to be understood. In the present case-control study, components summarizing executive functions were empirically determined to clarify the nature of executive difficulties observed in individuals with human immunodeficiency virus. One hundred and five seropositive and 62 seronegative healthy adults without comorbidities underwent a comprehensive executive function assessment. Test data were reduced via principal components analysis and component scores were used to investigate whether seropositive adults exhibit selective difficulties in specific executive processes. A three-component solution was found, consisting of updating, inhibition and set-shifting. Group differences between seropositive and seronegative participants were observed only in the updating component. In the present exploratory analyses, significant findings emerged that suggest a selective executive impairment associated with the updating/working memory process in young to middle adulthood seropositive individuals without comorbidities.

Computerized cognitive rehabilitation for treatment of cognitive impairment in multiple sclerosis: an explorative study.

In this explorative study, forty-seven patients with relapsing-remitting multiple sclerosis were randomized to a custom 6-week cognitive rehabilitation intervention (n = 23) using the BrainHQTM web-based platform and to a control group condition (n = 24). Cognitive rehabilitation intervention consisted of two 40-minute sessions per week. All patients were tested with the Brief International Cognitive Assessment for Multiple Sclerosis battery, the Stroop Color-Word Test, and the trail making test, while the Beck Depression Inventory - Fast Screen questionnaire was used as a measure of mood and the cognitive reserve index as a measure of cognitive reserve. We used the reliable change index, to calculate clinically meaningful changes of performance, and to discriminate between responders and non-responders of this intervention. Statistically significant improvement of the group receiving treatment was observed mainly on measures of verbal and non-verbal episodic memory and, to a lesser extent, on reading speed, selective attention/response inhibition, and visual attention. Verbal memory and visual attention improvements remained significant after considering the corrected for multiple comparisons level of significance. According to reliable change index scores, 12/23 (52.2%) of patients in the intervention group presented meaningful improvement in at least one measure (Greek Verbal Learning Test: 26%, Brief Visuospatial Memory Test-Revised: 17.4%, Stroop-Words test: 13%). This explorative study provides evidence that, at least in the short term, cognitive rehabilitation may improve the cognitive performance of multiple sclerosis patients.

Time reference, morphology and prototypicality: tense production in stroke aphasia and semantic dementia in Greek.

The present study aims at investigating verb inflection in aphasia and semantic dementia. In particular, it addresses the contribution of time reference and morphological complexity. Moreover, it investigates whether the lexical properties of the verb, such as argument structure and lexical aspect interact with the production of tense. Ten individuals with (different types of) stroke aphasia and five individuals with semantic dementia and their respective control groups conducted a sentence completion task. Three tenses were tested: past perfective, past imperfective and present. All tenses had to be produced with three different verb classes, which differed with respect to syntactic and semantic properties: unergative, unaccusative and transitive verbs. The findings imply problems with marking aspect and an interaction between inflection and lexical aspect but no effect of morphological complexity or across the board difficulties with reference to the past in aphasia. Moreover, the results suggest problems with inflection in semantic dementia, an interaction between inflection and lexical aspect and a selective difficulty with imperfective tenses. The study contributes to a better understanding of inflection problems in aphasia and it provides evidence for inflection problems in semantic dementia.

Screening for Executive Impairment in Patients with Frontotemporal Dementia: Evidence from the Greek Version of the Frontier Executive Screen.

OBJECTIVES: The aim of the present study was to adjust the frontier executive screen (FES) for the Greek population, to develop normative data, and to investigate its ability to discriminate patients diagnosed with frontotemporal dementia from healthy individuals. METHODS: The FES was administered to 142 community-dwelling healthy adults (age: M = 65.9, SD = 8.5; education: M = 10.8, SD = 4.3; sex: 59% female) and 32 patients diagnosed with frontotemporal dementia (age: M = 69.3, SD = 8.6; education: M = 11.7, SD = 4.8; sex: 31% female). Correlation and regression analyses were performed to determine the association between the FES scores, demographic, and clinical characteristics. Cronbach's α coefficient was used to determine internal consistency. Group differences on the FES were examined with independent samples t-test and Mann-Whitney test. Discriminant and ROC analyses were used to determine diagnostic accuracy and to identify the optimal cutoff score for the discrimination between groups. RESULTS: Regression analyses indicated associations between demographic characteristics and FES scores (age: R2 = .08; education: R2 = .33). Internal consistency was marginally acceptable (α = .69). Patients scored lower than healthy participants on the total FES score (d = 1.91) and its three subscores (verbal fluency: η2 = .60; inhibition: η2 = .52; working memory: d = 0.90). The results indicated high diagnostic accuracy (94%) and the optimal cutoff score was 7 (91% sensitivity, 78% specificity). CONCLUSIONS: The Greek version of the FES is a useful tool for the brief evaluation of executive functions in patients diagnosed with frontotemporal dementia.

Behavioral disturbances differentiate frontotemporal lobar degeneration subtypes and Alzheimer's disease: evidence from the Frontal Behavioral Inventory.

BACKGROUND: Behavioral assessment is useful for the diagnosis of frontotemporal lobar degeneration (FTLD). We explored the ability of the Frontal Behavioral Inventory (FBI) to discriminate between patients with distinct subtypes of FTLD and patients with Alzheimer's disease (AD), as well as the influence of demographic variables on FBI scores. METHODS: The FBI was administered to the caregivers of 87 patients diagnosed with FTLD [64 behavioral variant FTLD, 19 aphasic variant FTLD (primary progressive aphasia), and 4 motor/extrapyramidal variant (corticobasal syndrome)] and 30 patients with AD. Patients with AD were older than patients with FTLD. The two groups did not differ with respect to duration of illness, level of education, or sex ratio. RESULTS: Age significantly predicted disinhibited positive behaviors, such as perseverations and irritability, whereas education did not contribute to FBI ratings. Classification accuracy for the discrimination of AD and mixed FTLD groups was 81%. Moreover, 88.3% and 83.7% accuracy was achieved for the discrimination of AD and behavioral variant FTLD, and AD and primary progressive aphasia groups, respectively. The Total Negative subscale of the FBI, which summarizes the presence of deficit (negative) behaviors, was the best discriminator. A cut-off score of 17 provided 83% sensitivity and 98% specificity in distinguishing between FTLD and AD patients. CONCLUSIONS: The FBI is a sensitive and specific tool for the differential diagnosis of FTLD from AD. The optimal cut-off point for the detection of FTLD patients was lower than that initially proposed.

The cognitive and behavioral correlates of functional status in patients with frontotemporal dementia: A pilot study.

Objective: Frontotemporal dementia (FTD) impinges significantly on cognition, behavior, and everyday functioning. Goal of the present study is the detailed description of behavioral disturbances and functional limitations, as well as the investigation of associations between cognition, behavior, and functional impairment among FTD patients. Given the importance of maintaining a satisfying functional status as long as possible, this study also aims to identify the cognitive correlates of compensatory strategy use in this clinical group. Methods: A total of 13 patients diagnosed with FTD (behavioral variant FTD = 9, non-fluent variant primary progressive aphasia = 3, semantic dementia = 1) were administrated a broad range of neuropsychological tests for the assessment of different cognitive abilities. Behavioral symptomatology and performance on everyday activities were rated with informant-based measures. Descriptive statistics were used for the delineation of behavioral and functional patterns, whereas stepwise multiple regression analyses were performed to identify associations between cognition, behavior, and functional status. Results: Negative symptoms, especially apathy, were found to predominate in the behavior of FTD patients. Instrumental tasks, such as housework and leisure activities, appeared to be the most impaired functional domains. Working memory was the strongest cognitive correlate of performance across various domains of everyday functioning, whereas working memory along with short-term verbal memory accounted for a great proportion of variance in compensatory strategy use. Behavioral disturbances and especially negative symptoms were also found to contribute significantly to functional impairment in FTD. Conclusions: Executive dysfunction, as well as behavioral disturbances contribute significantly to functional disability in FTD. Early interventions tailored at these domains may have the potential to improve functional outcomes and delay the rate of functional decline among FTD patients.

Inhibitory control is associated with medication adherence in young HIV patients without comorbidities.

OBJECTIVE: In the present study we evaluated the incremental contribution of executive cognition (EC) subprocesses to antiretroviral medication adherence. METHOD: A comprehensive EC test battery assessing updating/working memory, mental flexibility, and inhibitory control, along with measures assessing non-executive cognitive functions were completed by 100 individuals with HIV. Medication adherence was determined via a visual analogue self-report scale and the Medication Adherence Questionnaire. Potential predictors, including demographic and clinical characteristics and neuropsychological performances on EC and other cognitive tasks were regressed to medication adherence. Predictive variables related to executive processes were added in the final block of the hierarchical regression model in order to assess their incremental predictive ability on medication adherence. RESULTS: 23% of the variance in the visual analogue scale was explained by treatment complexity, memory and EC performance. A measure of inhibitory control, in particular, predicted self-reported medication adherence above and beyond demographic, clinical and other cognitive factors. CONCLUSIONS: The contribution of EC to self-reported medication adherence in young seropositive adults was limited, but inhibitory control was associated with proper medication management above and beyond demographic, clinical and other cognitive functions.

Preliminary validation of the apraxia battery for adults-second edition (ABA-2) in Greek patients with dementia.

BACKGROUND: Apraxia is considered a supportive feature in Alzheimer's disease (AD) patients. It has been reported that patients with frontotemporal dementia (FTD) may present apraxia, especially in the buccofacial area. The Apraxia Battery for Adults (ABA-2) is a brief and practical battery for praxis impairment and has been validated in Greek post-stroke patients. AIM: To validate and evaluate ABA-2 test, translated and culturally adapted, in a sample of Greek demented patients. PATIENTS AND METHODS: Patients diagnosed with FTD (n = 20) and AD (n = 20) were included in the study. Age-, gender-, and education- matched healthy controls (n = 20) were also tested. All participants completed Adenbrooke's Cognitive Examination-Revised (ACE-R), Frontal Rating Scale (FRS), Frontal Behavioral Inventory (FBI), and ABA-2 battery. Sensitivity and specificity of ABA-2 were calculated, as well as its consistency and statistical significance for diagnosing apraxia. RESULTS: The ABA-2 was able to differentiate demented patients from healthy controls with a sensitivity of 77.5% and specificity of 95%. Its validity was confirmed with Cronbach's alpha coefficient > 0.7, indicating satisfactory internal reliability. Statistically significant differences were found when comparing total ABA-2 score (p < 0.0001), as well as 3 out of 6 subtests of ABA-2, between the two study groups. Age, gender and education were not correlated with ABA-2 score. CONCLUSION: ABA-2 is a valid, reliable and sensitive battery to differentiate demented patients from healthy individuals in the Greek population. We propose the modification of ABA-2 to a 5-subtest tool, to be administered as a bed-side test.

Lexical and Grammatical Factors in Sentence Production in Semantic Dementia: Insights From Greek.

Purpose: Language production in semantic dementia (SD) is characterized by a lexical-semantic deficit and largely preserved argument structure and inflection production. This study investigates (a) the effect of argument structure on verb retrieval and (b) the interrelation between inflection marking and verb retrieval in SD. Method: Seven individuals with SD and 7 healthy controls performed 2 sentence elicitation tasks. In Experiment 1, participants described the action taking place in a video. In Experiment 2, they watched the same videos preceded by a phrase prompting the production of past tense. Three verb classes were tested: (a) unergative (e.g., to walk), (b) unaccusative (e.g., to fall), and (c) transitive with 1 object (e.g., to read a book). Results: There was not any quantitative difference among the verb classes in Experiment 1, but error analysis hinted at difficulties related with argument structure complexity. The findings of Experiment 2 suggest no general effect of inflection on verb retrieval; nevertheless, inflection marking impeded the retrieval of verbs with complex argument structure. Large individual variation was established. Conclusions: Argument structure complexity may challenge speakers with SD. Verb retrieval and inflection marking seem to interrelate at the expense of the former. Inflection production may be affected at severe stages of the disease. Supplemental Material: https://doi.org/10.23641/asha.6030779.

Estimating Everyday Neuropsychological Functioning in Multiple Sclerosis: Reliability and Validity of the Greek Multiple Sclerosis Neuropsychological Questionnaire.

The Multiple Sclerosis Neuropsychological Questionnaire is a brief screening questionnaire for the assessment of everyday neuropsychological competence of patients with Multiple Sclerosis. The aim of the present study was to examine psychometric properties of the Greek version of the instrument. One hundred and three MS patients and 60 informants participated in the present study and completed the questionnaire. From the initial patient sample, 51 participants completed broadly used neuropsychological tests and measures estimating cognitive failures and depression. Moreover, after a six-month interval the MSNQ was administered to 58 patients from the initial sample in order to explore test-retest reliability. Cronbach's α was 0.92 and 0.93 for patient and informant forms, respectively. The patient form was correlated significantly with measures of cognitive failures and depression. Low correlations were found between the informant form and performance on cognitive tests. In regard to the patient form, significant correlation was observed between repeated administrations and, psychometrically, the three-factor structure was preferable than the one-factor structure. The present study confirms the already established pattern of correlations among the two MSNQ forms, neuropsychological test performance and depression measurements. Additional research is needed in order to define a cut-off score for the MSNQ-I providing further information about the diagnostic interpretability of the instrument.