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Purpose The current cross-sectional study had three objectives: (1) to assess the prevalence of depression and demoralization in a sample of prostate cancer (PCa) patients; (2) to examine whether masculine self-esteem and depression were associated with demoralization; and (3) to evaluate the role of resilience as a factor buffering the effects of masculine self-esteem and depression on demoralization. Methods 197 PCa patients aged 48 to 79 years (M = 67.19; SD = 6.83) answered questions about masculine self-esteem, depression, resilience, and demoralization. An ANOVA was conducted to examine whether the association between demoralization and depressive symptoms was linear. A chi-square test was calculated to determine differences between depression and demoralization. Finally, a hierarchical multiple linear regression analysis with interaction terms was conducted to examine the associations between masculine self-esteem, depression, resilience, and demoralization. Results Depression scores increased linearly with demoralization severity, but demoralization scores were higher than depression scores (21.3% vs. 15.2%). Lower scores on masculine self-esteem and higher scores on depressive symptoms were associated with greater demoralization. Resilience significantly moderated the association between masculine self-esteem and demoralization, but not between depression and demoralization. Conclusion Assessment of depression, masculine self-esteem, resilience, and demoralization in the clinical setting is critical for improving the mental health status of PCa patients.

Cognitive impairment is an essential feature of Huntington’s disease (HD) and dementia is a predictable outcome in all patients. However, validated instruments to assess global cognitive performance in the field of HD are lacking. We aimed to explore the utility of the Parkinson’s disease-Cognitive Rating Scale (PD-CRS) for the screening of global cognition in HD. A multicenter cohort of 132 HD patients at different disease stages and 33 matched healthy controls were classified as having preserved cognition, mild cognitive impairment (HD-MCI) or dementia (HD-Dem) according to the Clinical Dementia Rating and Functional Independence Score. The PD-CRS and the Mini-Mental State Examination were administered. Receiver operating characteristic curve analysis was used to determine optimal cutoffs to differentiate patients according to their cognitive status. A PD-CRS cutoff score ≤ 81/82 was optimal to detect HD-MCI (sensitivity = 93%; specificity = 80%; area under the curve (AUC) = 0.940), and ≤ 63/64 was optimal to detect HD-Dem (sensitivity = 90%; specificity = 87%; AUC = 0.933). MMSE scores failed to show robust psychometric properties in this context. The PD-CRS is a valid and reliable instrument to assess global cognition in HD in routine clinical care and clinical trials.

Objective Closing-in (CI) consists in copying drawings near to (near-CI) or superimposed on (adherent-CI) the model and often occurs in dementia and mild cognitive impairment (MCI). We assessed whether a visuo-constructional task not requiring a grapho-motor response is sensitive to identify CI in Alzheimer's disease (AD), fronto-temporal dementia (FTD), vascular dementia (VD), and MCI. Method We enrolled a sample of 162 patients with dementia (AD, FTD, or VD), 66 individuals with MCI, and 20 healthy adults (HAs) who completed a neuropsychological assessment and the Stick Design test that requires arranging matches for reproducing geometrical figures. Results CI in the Stick test was slightly (but not significantly) more frequent than that observed in the copying drawing task. CI frequency on both tests was high and similar in AD and FTD and lower in VD and MCI. On both tests, near-CI was the most common error in VD and MCI, whereas adherent-CI prevailed in patients with AD; a similar percentage of near-CI and adherent-CI was observed in FTD. Last, in the participants with dementia and MCI, the number of CIs was significantly correlated with an index of control and executive functions but not with spatial short-term memory. Conclusions CI occurs with the same frequency in AD, VD, and FTD, but the prevalent type of CI varies across syndromes. The Stick Design test is a useful task to assess CI and its specific forms in dementia and MCI. (PsycINFO Database Record (c) 2020 APA, all rights reserved).

Objective Weight loss is common in patients with Parkinson’s disease (PD). It has been reported that low Body Mass Index (BMI) is associated with disease progression in these patients, but only a few data are available on the relationship between BMI and cognitive dysfunctions in PD patients. In the present study we systematically assessed the possible relationship between BMI index and specific cognitive defects. Method We enrolled a prospective sample of 37 PD individuals and 30 healthy controls (HC) of similar age, sex, and education. The BMI was calculated in each participant, who underwent a neuropsychological assessment exploring the general cognitive skills, frontal/executive, visuo-spatial, visuo-constructional and memory abilities. Results We showed that PD group had significant lower BMI value compared to HC group. In PD patients, the BMI was negatively correlated to disease duration and number of errors at the Stroop-Color Word Test, and positively to score on Frontal Assessment Battery (FAB). Moreover, a regression analysis revealed that, the BMI in PD patients was associated with disease duration and score on FAB. Conclusions Our findings contribute to reveal that the relationship between height and weight is strongly related to frontal cognitive dysfunctions in PD patients.

Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a monogenic small-vessel disease that is characterized by a wide range of neurologic and neuropsychological impairments. Constructional impairments have been reported in some cases but have never been assessed systematically.To evaluate constructional abilities and their cognitive and neural correlates in nondemented individuals with CADASIL.Thirty individuals with CADASIL who were not affected by clinically relevant cognitive deterioration and 30 healthy controls (HC) underwent an extensive cognitive assessment and paper-and-pencil visuoconstructional tasks in order to detect constructional impairments. Performance on the visuoconstructional tasks was correlated with the cognitive assessment scores and with quantitative indices of regional gray matter atrophy (obtained via FreeSurfer image analysis) and white matter involvement.The individuals with CADASIL achieved significantly lower scores on the cognitive assessment compared with the HC. Poor visuoconstructional abilities were observed in seven (23.3%) of the individuals with CADASIL when performing the copy drawing task and in nine (30%) when performing the Rey Complex Figure Test. Logistic regression revealed that visuoconstructional impairments were significantly associated with scores on the Frontal Assessment Battery and the Attentional Matrices Test. Morphometric results revealed that scores on the visuoconstructional tasks were related to gray matter atrophy of the left frontal lobe and right parietal lobe.Impairments on visuoconstructional tasks are quite common in individuals with CADASIL, even in the lack of clinically relevant cognitive deterioration, and are critically related to frontal and parietal atrophy.

Background Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder with progressive loss of upper and lower motor neurons. Non-motor-symptoms, such as cognitive, emotional, autonomic, and somatosensory alterations, have been also described. Interoception represents the link between the body and brain, since it refers to the ability to consciously perceive the physical condition of the inner body, including one’s heartbeat (i.e., interoceptive sensitivity, IS). Objectives To evaluate IS in ALS patients by means of a well-established task: the heartbeat perception task. Moreover, we evaluated possible correlations between IS and neuropsychological, affective, and disease-related characteristics. Methods Fifty-five ALS patients (mean-age = 60.3 ± 12.5 years; mean disease-duration = 20.9 ± 18.8 months) and 41 caregivers (CG) underwent the heartbeat perception task and an extensive evaluation of motor, cognitive, body awareness, affective, and emotion domains. Results ALS patients showed lower IS than CG (0.68 ± 0.24 vs 0.82 ± 0.16; p = 0.003). Significant correlations were found between IS and self-reported measures of alexithymia (subscale of Toronto Alexithymia scale-20 “difficulties in describing feelings”; rho = − .391, p = .003) and interoceptive awareness (subscale of Multidimensional assessment of interoceptive awareness “not worrying about pain”; rho = .405, p = .002). No significant differences were found on questionnaires for depression and anxiety between patients with ALS and their caregivers (p > .05). Conclusions ALS patients show reduced interoceptive sensitivity that is associated with poorer ability to describe feelings and with lower focalization on pain, regardless of cognitive and motor impairment. Alteration of interoception may represent a specific behavioural sign within the spectrum of emotion processing deficits described in ALS patients.

Personality traits are pat terns of thoughts, feelings and actions that are usually assessed by means of psychometric questionnaires. In the present study we described the Phenomenological Personality Factor (PPF), a short questionnaire assessing the personality traits, taking into account the different interpretative models of personality. Methods. A sample of 554 healthy subjects (357 female; 197 males) aged 18-60 years were enrolled. Each participant was required to complete PPF, by indicating the presence/absence of the individual personality trait, and the Italian version of the Affective Neuroscience Personality Scale (ANPS). Results. The principal component analysis showed that seven factors explained the 35.07% of the total variance. Moreover, the cor relation analysis revealed that the PPF components were significantly and positively associated with the ANPS scales. Conclusions. Our findings suggest that the PPF is a useful questionnaire to assess the person ality traits, and the adaptive functioning, in healthy individuals Riassunto. Introduzione. I tratti di personalità rappresentano schemi di pensieri, sentimenti e azioni spesso valutati mediante questionari psicometrici. Nel presente studio noi descriviamo il questionario Phenomenological Personality Factor (PPF), un breve strumento sviluppato per valutare i tratti di personalità in soggetti sani, tenendo in considerazione i diversi modelli interpretativi della personalità. Metodi. Sono stati reclutati 554 soggetti sani (357 femmine; 197 maschi), di età compresa tra i 18 e i 60 anni. A ogni partecipante è stato chiesto di completare il PPF, indicando la presenza/assenza dello specifico tratto di personalità, e la versione Italiana dell’Affective Neuroscience Personality Scale (ANPS). Risultati. L’analisi delle com ponenti principali ha estratto sette fattori che spiegavano il 35,07% della varianza totale. Inoltre, l’analisi di correlazione ha mostrato che le componenti del PPF erano associate significativamente e positivamente con le scale dell’ANPS. Conclusioni. I nostri risultati suggeriscono che il PPF è un utile strumento per valutare l’occorrenza dei tratti di personalità, e il funzionamento adattivo, nei soggetti sani.

Introduction: Subjects can improve their performance on memory for action phrases if, during the encoding condition, they self-perform actions associated with verbs (subject-performed condition), or if they perceive the actions carried out by experimenter (experimenter-performed condition), with respect to a verbal task condition in which they only read or listen to the stimuli. This facilitation is labeled "Enactment effect" (EE), and is thought to be associated with episodic integration processes binding actions and nouns together in a coherent representation. Only recently, studies addressed EE in AD individuals reporting significant improvements on memory tasks in the subject-performed encoding condition. However, no studies tried to explore the cognitive mechanisms supporting EE in AD individuals. Method: Performance on recognition and cued recall tasks for action phrases were assessed in a sample of 32 mild-to-moderate AD individuals and 30 healthy adults, in verbal, subject-performed and experimenter-performed encoding conditions. Moreover, a cognitive assessment was completed to explore the possible correlates of EE in our participants. Results: Results showed that both subject-performed and experimenter-performed encoding conditions produced similar advantages over the verbal condition, in both memory tasks in both groups. Moreover, these memory advantages were strongly associated to executive processes, in both AD and healthy adults. Conclusions: The present study confirmed that EE is spared in mild to moderate AD. Our findings supported the role of episodic integration processes and suggested a contribution of executive processes in EE.

Background: Arithmetic word-problem solving depends on the interaction of several cognitive processes that may be affected early in the disease in gene-mutation carriers for Huntington’s disease (HD). Objective: Our goal was to examine the pattern of performance of arithmetic tasks in premanifest and manifest HD, and to examine correlations between arithmetic task performance and other neuropsychological tasks. Methods: We collected data from a multicenter cohort of 165 HD gene-mutation carriers. The sample consisted of 31 premanifest participants: 16 far-from (>12 years estimated time to diagnosis; preHD-A) and 15 close-to (≤12 years estimated time to diagnosis; preHD-B), 134 symptomatic patients (early-mild HD), and 37 healthy controls (HC). We compared performance between groups and explored the associations between arithmetic word-problem solving and neuropsychological and clinical variables. Results: Total arithmetic word-problem solving scores were lower in preHD-B patients than in preHD-A (p

Timed neuropsychological tests do not take into account physical impairment during scoring procedures. Dysarthria and upper limb impairment can be easily measured with the PATA rate test (PRT) and the nine-hole pegboard test (9HPT). We recently validated a normalization method for timed neuropsychological tests using the PRT and 9HPT (p9NORM). We now validate the p9NORM in Parkinson’s disease (Yarnall et al. Neurology 82(4):308–316; 2014) and multiple system atrophy (MSA). We enrolled twenty-six patients with PD, eighteen patients with MSA, and fifteen healthy controls (HC). p9NORM was applied to patients with abnormal PRT and/or 9HPT. All subjects were tested with a comprehensive neuropsychological battery. No differences emerged in demographics across groups: (PD: mean age ± SD 66 ± 8; education 9 ± 4 years; MSA: age 60 ± 8; education 10 ± 4 years; HC: age 61 ± 12; education 9 ± 4 years). In MSA patients, the scores on the trail making test (TMT-A p = 0.003; TMT-B p = 0.018), attentional matrices (AM; p = 0.042), and symbol digit modalities test (SDMT p = 0.027) significantly differed following application of p9NORM. In PD patients, the TMT-A (p < 0.001), TMT-B (p = 0.001), and AM (p = 0.001) differed after correction. PD and MSA showed cognitive impairment relative to HC performance. When comparing MSA with PD, the SDMT, AM, and fluencies were similar. TMT-A and -B raw scores were different between groups (p = 0.006; p = 0.034), but these differences lost significance after p9NORM corrections (p = 0.100; p = 0.186). We confirm that the p9NORM can be successfully used in both PD and MSA patients, as it mitigates the impact of disability on timed tests, resulting in a more accurate analysis of cognitive domains.

Objective Patients with Huntington’s disease (HD) may show impairments of frontal cortical–subcortical circuits with difficulties on cognitive flexibility tasks. One marker of poor flexibility is “perseveration” behavior, which refers to inappropriate and involuntary production of iterative responses not adequate to the current task demands. This study explored frequency, type, and possible cognitive mechanisms of verbal perseverations in a large sample of HD patients. Method A sample of 128 patients with HD underwent phonological and category verbal fluency tests to assess perseverative errors, within a wide neuropsychological, psychopathological, motor, and functional assessment. Results Perseverative errors in verbal fluency task occurred in 89 (69.5%) patients. Patients showing perseverations scored significantly lower than patients without perseverations on tasks tapping executive and motor functions and on functional independence scales. Logistic regression analysis revealed a significant independent association of verbal perseverations with scores on Trail Making Test only. Conclusions: Verbal perseverations are frequent in HD patients and are likely related with impairments of attentional switching and working memory, hampering tracking of verbal responses already produced. Perseverative behavior may serve as a useful clinical marker of cognitive and functional impairment in patients with HD.

Objective In visuo-constructional tasks, patients may reproduce drawings near-to or superimposed on a model, showing the so-called "Closing-in" (CI), often ascribed to a defect in inhibitory control. CI has been described in neurological conditions, but no studies have explored CI in Huntington's disease (HD), a neurodegenerative disorder often involving the frontal cortical-subcortical circuits. We searched for the occurrence of CI in HD patients and systematically investigated its correlates to find a clinical marker of the frontal/executive dysfunctions in the early examination of HD patients. Method We assessed 130 HD participants, who performed a graphic coping task and a neuropsychological, psychiatric, motor, and functional assessment. Results CI occurred in 52/130 (40%) HD patients, with 43/52 (82.7%) superimposing their copy directly on the model. MANOVA showed that HD patients with CI scored significantly poorer on Symbol digit modality test, Stroop-color word - reading test, Stroop-color word - interference test, Trail making test - part B, and Phonological verbal fluency test. However, a logistic regression analysis revealed that the significant predictor of the occurrence of CI was the score on Stroop-color word - interference test. Conclusions HD patients may show CI in graphic tasks, and it could be related to a defect in inhibitory control impeding the switch of attention from the model to the copying space, and releasing a default tendency which causes an attraction of hand movement towards the focus of visual attention. CI might be a useful clinical marker for the early detection of frontal/executive defects in HD patients.

Motor simulation implies that the same motor representations involved in action execution are re-enacted during observation or imagery of actions. Neurofunctional data suggested that observation of letters or abstract paintings can elicit simulation of writing or drawing gestures. We performed four behavioural experiments on right-handed healthy participants to test whether observation of a static and complex geometrical figure implies re-enactment of drawing actions. In Experiment 1, participants had to observe the stimulus without explicit instruction (observation-only condition), while performing irrelevant finger tapping (motor dual task), or while articulating irrelevant verbal material (verbal dual task). Delayed drawing of the stimulus was less accurate in the motor dual-task (interfering with simulation of hand actions) than in verbal dual-task and observation-only conditions. In Experiment 2, delayed drawing in the observation only was as accurate as when participants encoded the stimulus by copying it; in both conditions, accuracy was higher than when participants were instructed to observe the stimulus to recall it later verbally (observe to recall), thus being discouraged from engaging motor simulation. In Experiment 3, delayed drawing was as accurate in the observation-only condition as when participants imagined copying the stimulus; accuracy in both conditions was higher than in the observe-to-recall condition. In Experiment 4, in the observe-only condition participants who observed the stimulus with their right arm hidden behind their back were significantly less accurate than participants who had their left arm hidden. These findings converge in suggesting that mere observation of a geometrical stimulus can activate motor simulation and re-enactment of drawing actions.

In visuo-constructional assessment, brain-damaged individuals may copy figures near to or superimposed on the model, showing the Closing-in (CI). CI has been largely investigated in dementia, and often ascribed to impairments of the attention/executive abilities ("Attraction hypothesis"). Only a few dated studies investigated frequency of CI in brain-damaged individuals, without clarifying the genesis of the phenomenon. We aimed at testing the "Attraction hypothesis" in 27 individuals with focal frontal cortical or sub-cortical brain lesions by a dual-task experimental paradigm. The participants underwent a neuropsychological battery and a copying task to be performed alone (single task condition), or concurrently with a simple or a complex verbal secondary task (dual-task conditions). CI was found in 66% of frontal-damaged individuals, who scored significantly lower than healthy adults on all neuropsychological measures; brain-damaged individuals showing CI performed worse than frontal-damaged individuals without CI on frontal and visuo-constructional measures. In the dual-task condition with the complex secondary task CI was significantly enhanced, with a weaker tendency to self-correction, in individuals with CI compared to individuals without CI. These findings would confirm that the CI in brain-damaged individuals is related to reduction of attentional resources, consistently with the "Attraction hypothesis".

Background Attentional difficulties are very frequent among patients affected by Huntington’s disease (HD), a rare autosomal dominant neurodegenerative disorder. Aims In the present study we aimed to assess whether and how different types of attention are associated to CAG size, disease duration, motor disorders, functional dysfunction and psychopathological signs in symptomatic HD patients. Methods We recruited a prospective sample of 22 symptomatic HD patients and 22 healthy controls who underwent a clinical, neuropsychological and psychopathological assessment, and an evaluation of the sustained, selective and divided attention. Results Correlation analyses showed that performance on the motor section of the Unified Huntington’s Disease Rating Scale was significantly associated to scores on the Stroop-color word test and Cancellation task. Moreover, performance on the Total Functional Capacity and functional section of the Unified Huntington’s Disease Rating Scale were significantly related to scores on the Trail making test. Last, score on the Frontal Behavioural Assessment was correlated with performance on the Stroop-color word test. Conclusions Specific clinical features are associated to different types of attention in our HD patients. The severity of motor dysfunctions is strongly related to the ability to selectively pay attention on a target (selective attention) and to sustain it for extended time (sustained attention), whereas the decreasing of the functional abilities is associated to difficulties in paying attention on multiple tasks simultaneously (divided attention). Psychopathological signs are associated to the ability to direct the attention towards relevant stimuli by inhibiting the irrelevant ones (selective attention).

Introduction Huntington’s disease (HD) is an autosomal dominant neurodegenerative disease, caused by the expansion of a CAG repeat within exon 1 of IT15 gene. HD exhibits the typical phenomenon of genetic anticipation and the symptoms of the disease appear earlier and more severe in subsequent generations due to meiotic instability. The CAG repeat accounts only for approximately 56%–70% of the variation in age at onset in HD. It is likely that modifying genetic variations, which segregate independently from the primary mutation, could influence the age at onset. Aims of the study Genetic, pathological, and clinical similarities exist between HD and spino-cerebellar ataxias (SCAs). In this study, seven SCAs genes have been studied as modifiers of age at onset in a cohort of HD patients. Materials and Methods We enrolled 50 HD patients. For every HD subject, CAG repeats have been measured on the larger allele of ATXN1, ATXN2, ATXN3, CACNA1A, ATXN7, PPP2R2B, and TBP genes. Regression analysis was used to evaluate the effects of CAG repeats in SCAs genes on age at motor, cognitive and psychiatric onset in HD patients. Results We did not find extensive correlations between CAG repeats in SCA genes and age at onset of HD. The only exceptions were represented by ATXN2 and CACNA1 genes for age at motor onset, and ATXN2 for age at psychiatric onset. When a multiple regression model was tested, a small additional effect on age at motor onset was identified only for CACNA1A. CAG repeats in expanded HD gene and larger CACNA1A alleles account for 64% of age at motor onset in HD patients. Conclusions CACNA1A gene could represent a mild genetic modifier of age at onset in HD patients. Further studies, conducted on larger HD patients‘ cohorts, are needed to confirm our data.

Animal-assisted therapy (AAT) includes a set of nonpharmacological interventions aimed at improving human health through the use of trained or untrained animals. In recent decades, AAT has been trialed for different neurological and psychiatric disorders. In patients with dementia, interaction with animals seems to have a positive influence on aggressiveness and anxiety and to ameliorate quality of life and relationship skills. In psychiatric patients, AAT seems to increase motivation and self-esteem, improve prosocial conduct, and decrease behavioral problems. The aim of this study is to review the literature on AAT for elderly people with dementia and psychiatric disorders. Other fields of possible application for AAT are suggested.

Objectives: Alzheimer’s disease (AD) patients may show the Closing-in (CI), a tendency to reproduce figures close to or superimposed on the model. AD patients with CI might manifest reduced functional independence compared to AD patients without CI, but no study directly assessed if CI can hamper common daily living activities. To address this issue here we investigated whether AD patients with CI veer their walking trajectory toward irrelevant objects more often than AD patients without CI. Methods: Fifty AD individuals, and 20 age- and education-matched healthy adults, underwent a graphic copying task to detect CI and a newly developed walking task to assess the tendency to veer toward irrelevant objects and to bump into them. All participants also completed a comprehensive neuropsychological battery to assess dementia severity; impairments in frontal/executive, visuo-spatial, visuo-constructional, and memory domains; and functional independence in daily living activities. Results: Graphic CI occurred in 34/50 (68%) AD patients (AD-CI group) who achieved significantly lower scores on frontal/executive abilities, and daily living functioning than AD individuals not showing CI. Most AD-CI patients (20/34; 58.8%) also showed at least one veering error in the walking task. Participants with CI and veering errors showed significantly poorer performance on Stroop test, and lower level of functional independence than AD individuals with CI in isolation. Conclusions: CI on graphic tasks can identify difficulties in walking and in complying with everyday activities in AD patients. These observations demonstrate the value of assessing CI in copying tasks. (JINS, 2018, 24, 437–444)

Introduction LRRK2 gene mutations underlie the most common mendelian form of Parkinson's Disease (PD), designated PARK8, that shows clinical features similar to those in idiopathic PD (IPD). We assessed the cognitive functions and the cerebral metabolism measured with 18F-fluorodeoxyglucose (FDG)-PET in PARK8 patients compared with IPD. Patients and methods We enrolled eight PARK8 patients and eight IPD patients, comparable for onset age, stage, severity and duration of disease. Mean age ± SD was 61.8 ± 10.9 years and disease duration 8.4 ± 5.8 in PARK8, and 61.4 ± 14.7 and 7.0 ± 4.8 in IPD. All subjects underwent a wide neuropsychological assessment and a FDG-PET. Cerebral regional relative metabolic maps were evaluated using voxel-based analysis with statistical parametric mapping. Results Motor and non-motor phenotype and neuropsychological evaluation did not differ between PARK8 and IPD. We detected one case of dementia and four of Mild Cognitive Impairment in each group. At FDG-PET, compared to controls, IPD patients revealed a significant relative posterior cortical hypometabolism in the left temporal and inferior parietal and in the right inferior and superior parietal regions, whereas patients with LRRK2 mutation, at a less conservative threshold, showed only a relative left inferior parietal hypometabolism. No differences were found between patients with PARK8 and IPD. Conclusions The results confirm a comparable cognitive profile between LRRK2 and IPD patients. FDG-PET study showed a pattern of posterior cortical hypometabolism in both groups, although less severe in LRRK2-related PD. The milder decrease of cortical metabolism in PARK8 might be due to a less marked pathological involvement compared to IPD.

Perseveration is the involuntary production of iterative responses. This study explored graphic perseverative errors in Alzheimer's disease and vascular dementia, to comprehend the neuropsychological correlates of this behavior.We performed a retrospective analysis of graphic productions in 114 individuals with clinical diagnosis of Alzheimer's disease and in 63 individuals with clinical diagnosis of vascular dementia, who completed frontal-executive, visuo-spatial, visuo-constructional, and verbal memory tests. For assessing perseverative behavior, we considered recurrent perseverations (inappropriate re-drawing of a figure drawn in a previous trial) and continuous perseverations (inappropriate replication of the same figure or of its element within one trial) produced in a standard copying task.Two or more graphic perseverative errors occurred in 66/114 individuals with Alzheimer's disease (57.9%) and in 31/63 individuals with vascular dementia (49.2%). Participants with moderate-to-severe dementia produced a significantly higher number of perseverations than individuals with mild dementia. Logistic regression revealed a significant relationship of graphic perseverations with frontal and visuo-constructive impairments, in both Alzheimer's disease and vascular dementia.Graphic perseverations are frequent in both Alzheimer's disease and vascular dementia. Frontal-executive defects can hamper inhibition of iterative graphic productions. Identification of graphic perseverations can be useful for detection and monitoring of frontal-executive impairments throughout the disease course in individuals with dementia.

INTRODUCTION Closing-in (CI) is a peculiar phenomenon consisting in the tendency to copy drawings close to or superimposed on a model. Recent findings showed that CI can be associated with frontal/executive dysfunctions, likely determining a failure in high-level monitoring of attention-action circuits. CI has been often observed in demented patients, but scarce data are available about CI in patients with Parkinson's disease (PD). In the present study, we detected occurrence of CI and investigated the cognitive processes associated to this phenomenon in a large sample of non-demented PD patients. METHODS: We retrospectively analysed graphic reproductions in a copying task of 100 non-demented PD patients who had also completed cognitive, behavioural, and motor screening assessment. RESULTS: CI phenomenon occurred in 50/100 non-demented PD patients (50%; 118/700 drawings). Among these patients, 46/50 patients copied drawings close to the model (near-CI), 2/50 patients superimposed their copy directly on the model (adherent-CI), whereas 2/50 patients showed both near-and adherent-CI. MANOVA showed that non-demented PD patients with CI showed more severe impairment on frontal/executive functions and visuo-constructional abilities than non-demented PD patients without CI. However, the logistic regression model revealed that occurrence of CI was significantly associated to lower scores on frontal/executive tasks only. CONCLUSION: CI can be found in a large proportion of non-demented PD patients, and it is related to frontal monitoring defects that could hamper inhibition of action and attention toward a model.

Mirror writing is a striking behaviour that is common in children and can reemerge in adults following brain damage. Skilled deliberate mirror writing has also been reported, but only anecdotally. We provide the first quantitative study of skilled deliberate mirror writing. K.B. can write forward or backward, vertically upright or inverted, with the hands acting alone or simultaneously. K.B. is predominantly left handed, but writes habitually with his right hand. Of his writing formats, his left hand mirror writing is by far the most similar in style to his normal handwriting. When writing bimanually, he performs better when his two hands make mirror-symmetrical movements to write opposite scripts than if they move in the same direction to write similar scripts. He has no special facility for reading mirrored text. These features are consistent with prior anecdotal cases and support a motor basis for K.B.'s ability, according to which his skilled mirror writing results from the left hand execution of a low-level motor program for a right hand abductive writing action. Our methods offer a novel framework for investigating the sharing of motor representations across effectors.

OBJECTIVE Closing-in (CI) in visuo-constructional tasks occurs when a drawing is reproduced close to or superimposed on the original model. CI has been often observed in Alzheimer disease (AD) patients and only rarely investigated in patients with vascular dementia (VD). Recent studies suggested that CI in AD patients represents a default behavior released by frontal-executive impairments, but the cognitive mechanisms behind this phenomenon in VD patients have not been clarified. We aimed to ascertain whether the same mechanisms could determine CI in VD and in AD patients. For this purpose we explored whether CI is related to frontal-executive or visuospatial impairments in a prospective sample of AD and VD patients, and investigated whether CI can be induced by a secondary task in patients with either disease. METHOD Sixty-three VD patients and 70 AD patients underwent a neuropsychological assessment of frontal-executive and visuospatial skills, and a copying drawings task for CI. Moreover, VD and AD patients without CI also performed a drawing copying task combined with a simple or a demanding verbal task. RESULTS In both VD and AD patients CI was related to frontal-executive impairments, with more severe forms of CI related to greater frontal-executive defects. In AD patients the tendency to deviate toward a salient model was significantly enhanced by reduction of cognitive resources, whereas VD could compensate for this tendency. CONCLUSIONS CI is related to the tendency to deviate toward a salient model and can be released by frontal-executive impairments in demented patients, independently from clinical diagnosis.

Background: In visuo-constructional copying tasks, individuals with dementia can reproduce distorted or simplified figures resulting in the so-called ‘Constructional apraxia’ (CA) impairment. CA has been often described in Alzheimer’s disease (AD), Vascular dementia (VAD), or Fronto-temporal dementia (FTD), and less often in patients with Parkinson’s disease and associated dementia (PDD). There have been suggestions that different cognitive mechanisms could account for CA in different forms of dementia, but this hypothesis has not been directly verified. In the present study we explored visuo-constructional abilities in a sample of AD, VAD, FTD and PDD patients. We also investigated the cognitive factors associated to CA in these patients’ groups. Methods: We enrolled 72 AD patients, 61 VAD patients, 33 FTD patients and 32 PDD patients. All the patients underwent a copying drawings task to assess CA, and an extensive neuropsychological assessment of frontal/ executive, visuo-spatial, and memory skills. Results: FTD patients showed significant higher scores on drawing copying task compared to all other groups, whereas similar scores were observed between AD, VAD and PDD patients. Moreover, CA was strongly related to visuo-spatial impairments in AD and PDD patients and to frontal/executive impairments in VAD and FTD patients. Conclusion: Our findings suggest that CA could be ascribed to distinctive cognitive defects in the different forms of dementia.

Mirror writing (MW) is a rare disorder in which a script runs in direction opposite to normal and individual letters are reversed. The disorder generally occurs after left-hemisphere lesions, is transient and is observed on the left hand, whereas usually motor impairments prevent assessment of direction of right handwriting. We describe a left-handed patient with complete left hand mirror writing, still evident 2 years after a hemorrhagic stroke in left nucleo-capsular region. Since the patient could write with his right hand he underwent several writing tasks with either hand, and a thorough assessment to clarify the nature of MW. MW was evident in writing to dictation with left hand only, both in right and left hemispace, but the patient could modify his behavior when a verbal instruction was provided. No mirror errors were found in reading words, in copying geometric figures and in spatial orientation tasks. MW in our patient could be accounted for by a failure in automatic transformation of grapho-motor programs to write with the left hand. A lack of concern (a sort of anosodiaphoria) and a poor cognitive flexibility could contribute to long-term persistence of MW.

INTRODUCTION The tendency to reproduce figures close to or superimposed on the model in copying tasks, the so-called Closing-in (CI) phenomenon, has been rarely reported in individuals affected by Parkinson's disease and associated dementia (PD-D). We aimed to comprehend the genesis of CI in PD-D individuals by assessing whether reduction of attention/executive resources can increase the tendency to deviate toward the model. METHOD: Thirty PD-D individuals underwent a neuropsychological evaluation exploring visuo-spatial, visuo-constructional, executive and memory abilities. An experimental copying task was employed in single and in two dual task conditions to assess whether a (verbal) cognitive load can increase CI in these individuals, compared with a matched sample of healthy adults. RESULTS: CI occurred in 19/30 (63.3%) PD-D individuals: 4 of them produced copies close to the model (near-CI), whereas 15 individuals also superimposed their copies on the model (adherent-CI) besides showing near-CI. Presence of CI in PD-D individuals was significantly correlated with executive dysfunction but not with general cognitive impairment. In the experimental copying task the tendency to deviate toward the model was strongly correlated with executive dysfunction, and significantly increased when PD-D individuals were engaged in dual-task compared with single task conditions. Some individuals attempted to counteract CI, but this trend significantly decreased in dual-task compared with the single task conditions. In healthy adults the drift toward the model was significantly smaller than in PD-D individuals; in dual-task conditions healthy adults only showed a significant decrease in their ability to compensate for this drift. CONCLUSIONS: In PD-D individuals the tendency to deviate toward a salient model is strongly enhanced by a concurrent cognitive load. Such preliminary findings would suggest a relationship between reduction of executive attentional resources and the genesis of CI in PD-D individuals.

Cognitive models of drawing are mainly based on assessment of copying performance of adults, whereas only a few studies have verified these models in young children. Moreover, developmental investigations have only rarely performed a systematic examination of the contribution of perceptual and representational visuo-spatial processes to copying and drawing from memory. In this study we investigated the role of visual perception and mental representation in both copying and drawing from memory skills in a sample of 227 typically developing children (53% females) aged 7-10 years. Participants underwent a neuropsychological assessment and the Rey-Osterrieth Complex Figure (ROCF). The fit and invariance of the predictive model considering visuo-spatial abilities, working memory, and executive functions were tested by means of hierarchical regressions and path analysis. Results showed that, in a gender invariant way, visual perception abilities and spatial mental representation had a direct effect on copying performance, whereas copying performance was the only specific predictor for drawing from memory. These effects were independent from age and socioeconomic status, and showed that cognitive models of drawing built up for adults could be considered for predicting copying and drawing from memory in children.

Background Apathy and depression are behavioral manifestations that may occur often in Alzheimer's disease (AD) patients. AD patients may also show Closing-in (CI) phenomenon, in graphic copying tasks. Recent evidence would suggest that apathetic symptoms are related to frontal dysfunctions in AD patients, whereas the cognitive bases of depressive symptoms in AD are still unclear. Recent studies demonstrated that frontal dysfunctions are also involved in the genesis of CI in AD patients. Objective Since frontal dysfunctions are thought to be more strongly related to apathetic than depressive symptoms, here we tested the hypothesis that CI is significantly associated with apathy in AD patients. Methods Forty-four AD patients were enrolled for this study. All patients completed a neuropsychological evaluation of visuo-spatial, frontal/executive, visuo-constructional, and memory skills. Moreover, graphic copying tasks were employed to detect CI, and behavioral scales to assess apathetic and depressive symptoms. Results CI and apathetic and depressed symptoms occurred in more than half of the present AD sample, but regression models revealed that the number of CI was significantly related to apathy only. The number of CI was also significantly correlated with severity of apathetic but not of depressive symptoms. Conclusion The present study demonstrated that CI and apathy are correlated with each other in mild to moderate AD, likely because they share common pathogenic mechanisms related to frontal/executive dysfunctions.

Environmental dependency (ED) phenomena, including utilization behavior and imitation behavior, are clinical manifestations typically observed in patients with the behavioral variant of fronto-temporal dementia (bvFTD), who may also show the closing-in (CI) phenomenon. Here, we explored the neuropsychological correlates of ED and CI in bvFTD, and the association of ED with CI to clarify the mechanisms underlying these clinical manifestations. Thirty-one bvFTD patients underwent a wide cognitive assessment in addition to special tasks to detect occurrence of CI and ED phenomena. Both ED and CI phenomena were present in more than half of the sample. Logistic regression analyses revealed that both ED and CI phenomena were significantly associated with poor scores on frontal neuropsychological tests. Although ED and CI often co-occurred, 3/12 patients with CI did not show ED, and 5/18 patients with ED did not show CI. A logistic regression model showed that the presence of ED was not significantly associated to CI. CI and ED are associated to progressive derangement of frontal functions in bvFTD. However, specific frontal dysfunctions might explain the occurrence of either phenomenon in isolation. (JINS, 2015, 21, 1–7)

Closing-in (CI) refers to copying drawings near to or superimposed on the original model, and is often observed in Alzheimer's disease (AD) patients. Contrasting hypotheses have been suggested to explain CI, but no prospective study has directly verified these interpretations. We evaluated the role of frontal/executive versus visuo-spatial impairments in a prospective sample of AD patients, and also explored whether different types of CI are related to specific neuropsychological tasks. We enrolled 64 AD patients who underwent copying tasks and an extensive neuropsychological assessment of visuo-spatial and visuo-constructional skills, frontal/executive abilities and anterograde memory. AD patients with CI showed more severe impairment on frontal/executive functions than AD patients without CI. Moreover, the tendency to produce copies superimposed on the model was selectively associated with poor inhibitory control for irrelevant responses. On this basis, we suggest that different CI phenomena could be ascribed to distinctive frontal/executive defects. (JINS, 2013, 19, 1–7)

Background Akinetic Mutism (AM) is a clinical condition of lacking of almost all motor functions including speech, gestures, and facial expression.1 Two subtypes of AM are distinguished. Apathetic AM involves mesencephalic and diencephalic regions, whereas hyperpathic AM is associated to anterior cingulate gyrus and adjacent frontal lobes.2 AM has been observed in different pathological conditions, but it has never been described in Huntington’s disease (HD), a neurodegenerative disease caused by cytosine-adenine-guanine (CAG)-repeat expansion in the huntingtin gene. Case History We described AM in T.A., a 27-year-old Ukrainian male with genetically confirmed HD. T.A. developed HD symptoms in his late teenage years with likely juvenile onset of the disorder. PET scan showed reduced FDG uptake in bilateral striatum and in bilateral frontal and parietal regions. We assessed AM in T.A. with dedicated tasks, in addition to a neuropsychological examination to explore cognitive abilities. Neuropsychological examination showed poor performances on inhibitory control skills, selective and sustained attention abilities, and on anterograde verbal memory tasks. Moreover, T.A. showed diminished motivation with preserved consciousness level and inability to respond to auditory or visual commands. Conclusions Our findings would be consistent with the hypothesis that hyperpathic AM in T.A. is related to frontal/executive dysfunctions, and would contribute to refine the hyperpathic AM in HD. References Cairns H, Oldfield RC, Pennybacker JB, Whitteridge D. Akinetic mutism with an epidermoid cyst of the 3rd ventricle. Brain 1941;64(4):273–290 Nagaratnam N, Nagaratnam K, Ng K, Diu P. Akinetic mutism following stroke. J Clin Neurosci 2004;11(1):25–30. doi:10.1016/j.jocn.2003.04.002