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Atypical Parkinsonism (AP) syndromes are characterized by a wide spectrum of non-motor symptoms including prominent attentional and executive deficits. However, the cognitive profile of AP and its differences and similarities with that of Parkinson's Disease (PD) are still a matter of debate. The present meta-analysis aimed at identifying patterns of cognitive impairment in AP by comparing global cognitive functioning, memory, executive functions, visuospatial abilities, language, non-verbal reasoning, and processing speed test performances of patients with AP relative to healthy controls and patients with PD. All investigated cognitive domains showed a substantial impairment in patients with AP compared to healthy controls. When AP syndromes were considered separately, their cognitive functioning was distributed along a continuum from Multiple Systemic Atrophy at one extreme, with the least impaired cognitive profile (similar to that observed in PD) to Progressive Supranuclear Palsy, with the greatest decline in global cognitive and executive functioning (similar to Corticobasal Syndrome). These findings indicate that widespread cognitive impairment could represent an important clinical indicator to distinguish AP from other movement disorders.

We describe a patient who developed bilateral temporal lesions due to a haemorrhagic stroke. One year after onset, he showed marked apperceptive prosopagnosia and could not name people from their voices, although he had spared semantic knowledge about people he could not name and could access this knowledge from people's voice. Importantly, the patient was able to retrieve proper name of persons after their description and was able to name sounds and songs; he could also retrieve proper names of monuments, towns and cartoon characters upon visual presentation. No further relevant cognitive impairment was present. This clinical picture represents the auditory counterpart of prosopanomia and could be defined 'phonoanomia'. This case report suggests that naming people from their voice implies specific retrieval processes, and pose specific constraints to theoretical models of person recognition.

ObjectiveThe quarantine/self-isolation measures implemented to retard the spread of the 2019 coronavirus disease (COVID-19) may negatively affect the mental health of the population. The present study aimed to explore the impact of the psychological symptoms on the occurrence of cognitive failures in a large sample of home-dwelling Italian individuals during quarantine/self-isolation for COVID-19.MethodsWe employed an online questionnaire using a virtual platform of Google Moduli. The questionnaire included an assessment of cognitive failures evaluated by the Perceived Memory and Attentional Failures Questionnaire (PerMAFaQ) and of resilience, coping style, depression, anger, and anxiety.ResultsThe online questionnaire was completed by 4175 participants revealing that about 30% of participants complained of cognitive failures at least sometimes during quarantine/self-isolation, whereas some respondents reported very frequent cognitive failures. Moreover, resilience was found to mediate the relationships between depressive and anger symptoms and cognitive failures. Although no difference was found on PerMAFaQ among smart-workers, non-smart-workers, and those currently not at work, people not working at the moment complained of more frequent cognitive failures.ConclusionsThese findings indicate the need to implement psychological support intervention, particularly for vulnerable groups, to reduce anxiety, depression, and anger, and of psychoeducational interventions to enhance resilience reducing possible long-term cognitive consequences of the quarantine.

A relationship between sleep disorders and cognitive dysfunctions was reported in Parkinson’s Disease (PD), however, some studies did not confirm the link. A meta-analytic study was performed to investigate the relationship between sleep disorders and cognitive dysfunctions, and to clarify the evolution of cognitive status in PD patients with sleep disorders. The systematic literature search was performed up to November 2020 using PubMed, Scopus, and PsycINFO databases. We included studies published in peer-reviewed journals in English providing results about neuropsychological comparison between patients with or without sleep disorders. Meta-analysis on cross-sectional data included 54 studies for REM Sleep Behavior Disorder (RBD), 22 for Excessive Daytime Sleepiness (EDS), 7 for Obstructive Sleep Apnea (OSA), 13 for Restless Legs Syndrome (RLS), and 5 for insomnia, the meta-analysis on longitudinal data included 7 studies. RBD was related to deficits of global cognitive functioning, memory, executive functions, attention/working memory, language, and visuospatial abilities. EDS was associated with deficits of global cognitive functioning and attention and working memory abilities, whereas RLS and OSA were related to global cognitive dysfunction. Moreover, we revealed that PD patients with RBD and those with EDS performed worse than PD patients without sleep disorders at follow-up rather than baseline evaluation. Our results suggest that sleep disorders are associated with cognitive deficits supporting indirectly that these, especially the REM Sleep Behavior Disorder, reflect abnormalities of frontal networks and posterior cortical areas. Sleep disorders in patients with PD seem to also increase the risk for long-term cognitive decline.

The Corsi Block-Tapping test (CBT) is a measure of spatial working memory (WM) in clinical practice, requiring an examinee to reproduce sequences of cubes tapped by an examiner. CBT implies complementary behaviors in the examiners and the examinees, as they have to attend a precise turn taking. Previous studies demonstrated that the Prefrontal Cortex (PFC) is activated during CBT, but scarce evidence is available on the neural correlates of CBT in the real setting. We assessed PFC activity in dyads of examiner–examinee participants while completing the real version of CBT, during conditions of increasing and exceeding workload. This procedure allowed to investigate whether brain activity in the dyads is coordinated. Results in the examinees showed that PFC activity was higher when the workload approached or reached participants’ spatial WM span, and lower during workload conditions that were largely below or above their span. Interestingly, findings in the examiners paralleled the ones in the examinees, as examiners’ brain activity increased and decreased in a similar way as the examinees’ one. In the examiners, higher left-hemisphere activity was observed suggesting the likely activation of non-spatial WM processes. Data support a bell-shaped relationship between cognitive load and brain activity, and provide original insights on the cognitive processes activated in the examiner during CBT. Supplementary Information The online version contains supplementary material available at 10.1007/s00221-021-06073-0.

Background and purpose Although disabling fatigue is common in Parkinson disease (PD), available consensus-based diagnostic criteria have not yet been empirically validated. The aim of this study was to evaluate the clinimetric properties of the criteria. Methods A sample of outpatients with PD was evaluated for demographic, clinical, behavioral, and cognitive features. Fatigue was diagnosed according to the new diagnostic criteria and was rated by means of the Parkinson Fatigue Scale (PFS) and Fatigue Severity Scale (FSS). Acceptability, concurrent and discriminant validity, and interrater reliability were evaluated with binary logistic regression analyses and Cohen kappa (kappa). Results Of 241 included patients, 17 (7.1%) met the diagnostic criteria for PD-related fatigue. Eight of nine symptoms described in Section A of the diagnostic criteria occurred in >50% of patients with fatigue. Acceptability (missing data = 0.8%) of the criteria was good, as was their concurrent validity with the PFS (odds ratio = 3.65) and FSS (odds ratio = 3.63). The discriminant validity of fatigue criteria with other PD-related behavioral and cognitive features was good (odds ratio < 1.68). The interrater reliability was excellent (kappa = 0.92). Conclusions This is the first study to test the clinimetric properties of case definition diagnostic criteria for PD-related fatigue. Our results suggest that current diagnostic criteria may be useful in both clinical practice and research. Future longitudinal studies should examine their long-term stability.

Cognitive dysfunctions are highly prevalent in multiple sclerosis (MS) and negatively impact occupational and social functioning.In the present longitudinal study, we aimed at modeling cognitive changes and at assessing whether apathy could be a predictor of cognitive decline in MS.We assessed 67 people with MS at two-time points (baseline, T0; 4-year follow-up, T1), by means of several clinical, behavioural, and cognitive measures. We used a delta approach to measure cognitive decline during the follow-up period. We applied a mixed factorial design and a linear regression model to explore factors associated with cognitive changes over time.A higher level of apathy at baseline predicted the progressive cognitive decline at follow-up, whereas a higher level of depression did not. Among demographic and clinical characteristics, only low education level was significantly associated with cognitive decline over time. Interestingly, participants with persistent apathy (diagnosis of apathy at T0 and T1, A+A+) and those who developed apathy (A-A+) showed poorer inhibitory control and a larger decline in executive functioning during the 4-year follow-up than participants who had never received the diagnosis of apathy (A-A-).Apathy represents an early marker of cognitive decline in MS. These findings have important clinical and prognostic implications.

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).

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.

The concept of Cognitive Reserve (CR) refers to the individual differences allowing some people to cope better with brain pathology. The aim of the present review was to explore the relationship between CR and cognition in adulthood. This association has been addressed in several neuro(psycho)logical disorders and in healthy elderly people. However, few studies explored this issue in adulthood (age range 18–65), when individuals might take advantage the most from psychoeducational approaches aimed at increasing CR. For our systematic review, we selected studies assessing CR and adopting neuropsychological and experimental tasks for evaluation of cognitive functioning in healthy individuals. In the selected articles, we examined the relationship of singular proxies, composite indexes or standardized scales of CR with measures of general cognition, attention, inhibitory control, verbal fluency, constructional abilities, and verbal and spatial memory. The results suggested a positive relationship between singular CR proxies, composite indexes or standardized scales, and almost all the explored cognitive domains. No clear conclusion could be made on constructional abilities due to the scarcity of available studies. CR seems associated with better cognitive performance in adulthood, but definite methodological improvements and the use of standardized scales for CR are necessary to reduce inconsistencies among studies.

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.

Prism Adaptation (PA) is a non-invasive method to investigate visuomotor control. Recent neurostimulation studies have proposed an interpretation of the mechanisms underlying PA based on functioning of brain networks, instead of focusing on single brain areas. To test the functioning of the network during a classical PA procedure, here we used for the first time High-Definition transcranial Direct Current Stimulation (HD-tDCS) to simultaneously inhibit or facilitate brain activity in two main nodes of the network, namely the parietal cortex and the cerebellum, in healthy individuals. The main results showed that simultaneous anodal HD-tDCS over the two regions reduced terminal errors during exposure to prism glasses as compared to cathodal and sham stimulation. Conversely, cathodal HD-tDCS reduced after-effect as compared to anodal and sham stimulation following prism removal. Overall, these results provide new insights on the network related to the deployment of PA mechanisms and demonstrate the feasibility of using non-invasive HD-tDCS to modulate the adaptive mechanisms of PA.

Despite the clinical importance of psychological factors in migraine, the relationship between personality traits, depression, and migraine has been poorly investigated. To delineate the personality profile distinctive for migraineurs and to explore the relationship between personality traits and depression in migraineurs compared to non-migraineurs. A systematic literature search was performed up to March 2019 using PsycInfo (PROQUEST), PubMed and Scopus. Sixteen primary studies met inclusion and exclusion criteria and were included in the meta-analysis. The meta-analysis on the personality defined according to Psychobiological model revealed high Harm Avoidance (Hedges’g = 0.31; 95% confidence interval [CI] = 0.01–0.60), Persistence (Hedges’g = 0.37; 95% CI = 0.01to − 0.72) and low self-directedness (Hedges’g = − 0.33; 95% CI = −0.57 to − 0.09) in migraineurs. The meta-analysis on the personality defined according to Three Factor Model revealed high neuroticism (Hedges’g = 0.47; 95% CI = 0.32–0.63) and low extraversion (Hedges’g = − 0.08; 95% CI = − 0.14 to − 0.03) in migraineurs. Meta-regression analysis revealed that neuroticism moderated the relationship between depression and migraine. The findings evidenced that migraine is characterized by specific personality traits. Among them, neuroticism influenced the severity of depression in migraineurs, and, therefore, an early evaluation of the personality traits could allow identifying patients susceptible to develop migraine-associated psychopathological symptoms.

Background and purpose. Patients with prolonged disorders of consciousness (pDoC) have a high mortality rate due to medical complications. Since an accurate prognosis is essential for decision-making on patients’ management, we analysed data from an international multicentre prospective cohort study to evaluate two-year mortality rate and bedside predictors of mortality. Methods. We enrolled adult patients in prolonged vegetative state/unresponsive wakefulness syndrome (VS/UWS) or minimally conscious state (MCS) after traumatic and non-traumatic brain injury within 3 months post-injury. At enrolment we collected demographic (age; sex), anamnestic (etiology; time post-injury), clinical (Coma Recovery Scale Revised, CRS-R; Disability Rating Scale; Nociception Coma Scale-Revised) and neurophysiologic (EEG; somatosensory evoked and event-related potentials) data. Patients were followed-up to gather data on mortality up to 24 months post- injury. Results. Among 143 traumatic (n=55) and non-traumatic (n=88) patients (VS/UWS=68, 19 females; MCS=75, 22 females), 41 (28.7%) died within 24 months post-injury. Mortality rate was higher in VS/UWS (42.6%) than in MCS (16%; p Conclusions. This study demonstrated that a feasible multimodal assessment in the post-acute phase can help clinicians to identify patients with pDoC at higher risk of mortality within 24 months after brain injury. This evidence can help clinicians and patients’ families to navigate the complex clinical decision-making process and promote an international standardization of prognostic procedures for patients with pDoC.

People can decide whether the image of a hand represents a left or a right one. The laterality judgment mainly implies mentally imaging own hand movement (motor simulation) if the stimulus represents a palm, or analysing visual cues, as hand asymmetry, if the stimulus reproduces a dorsum. Here, by capitalizing on evidence underscoring the key role of thumb-palm complex in motor dexterity of human hand, we hypothesise that activation of motor or visual processes when judging hand laterality is due to the different relevance of palm-thumb and dorsum-thumb combinations to hand action. To test this thumb-anchored strategy, in a laterality judgment experiment, we concurrently manipulated the thumb position (flexed or extended) with respect to palm and dorsum, and the human likeness of the hand shape (influencing the salience of the thumb with respect to the hand shape). The main results demonstrated that viewing the flexed thumb from palm or dorsum elicited motor simulation, while viewing the extended thumb activated motor simulation when combined with palm but not dorsum. The present data highlight the pivotal role of the thumb in hand laterality judgments, consistent with its key role in human in-hand manipulation.

The COVID-19 pandemic and the measures to avert contagion heavily impacted individuals' mental health. In the present cross-sectional study, we investigate the relationship between cognitive reserve, coping modalities and the perceived stress during a chronic stage of COVID-19 pandemic by online administration of three standardized questionnaires in a sample of healthy volunteers covering a large lifespan (18-85 years). We found that positive orientation to problems and higher levels of cognitive reserve were associated with lower levels of stress. Conversely, coping strategies involving negation, substance consumption, and appeal to other people and religion to face everyday life, together with higher education, were associated with higher levels of stress. These results shade light on the long-term psychological consequences of COVID-19 and call for the development of psychological interventions improving coping and cognitive reserve, to preserve and restore mental health following the pandemic.

Multiple sclerosis (MS) is associated with a high prevalence of emotional and mood disorders. Emotional disorders may worsen during illness progression and affect the quality of life of patients and their families. MS is often associated with depression, with an increased risk of suicide, poor adherence to treatment, decreased functional status, and quality of life. The diagnosis and treatment of emotional and mood disorders in these patients is often challenging since several symptoms of these disorders overlap with those of MS. Other prevalent emotional disorders in MS include bipolar disorder, anxiety disorders, emotional blunting (apathy), and pseudobulbar affect. Early recognition and treatment of these comorbidities could contribute to the reduction of disability and even to decreased mortality. The aim of this chapter is to provide an up-to-date review of mood and emotional disorders that are often associated with MS, focusing on their epidemiology, clinical features, pathogenesis, assessment, and treatment. The interplay between the psychosocial impact of the chronic disability and the demyelinating structural lesions of the brain in precipitating emotional and mood disorders is discussed, as well as its implications for diagnosis and treatment.

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: Prospective memory (PM) is defined as memory for future intentions and it is typically divided into time-based and event-based PM. Deficit of PM has been reported in patients with Parkinson’s disease (PD) but no study has yet explored the association between motor subtypes (tremor dominant and rigidity/bradykinesia dominant) and performance on PM tasks. The aim of the study was to explore the role of motor subtypes in the defect of PM. Methods: Consecutive outpatients with tremor dominant (TD-PD) or rigidity/bradykinesia dominant (PIGD-PD) PD and healthy subjects (HCs) were enrolled and underwent a neuropsychological battery assessing PM, verbal memory and executive functions and questionnaires assessing apathy, functional autonomy, and perceived memory disturbances. Results: We enrolled 28 patients with TD-PD, 28 patients with PIGD-PD and 50 HCs. The three groups did not differ on demographic and cognitive variables. Patients with TD-PD performed worse on time-based PM tasks than patients with PIGD-PD and HCs; no significant difference was found among the three groups on event-based PM tasks. Executive dysfunctions contributed to reduced time-based PM scores in TD-PD. Moreover, severe deficit of time-based and more frequency of perceived failures of PM contributed to reduced functional autonomy in TD-PD. Conclusion: The finding of a poorer performance of patients with TD-PD than ones with PIGD-PD and HCs suggests a selective deficit of time-based PM abilities in TD-PD group; therefore, deficit of time-based PM might be considered as a distinctive non-motor symptom of TD-PD and it might affect the functional autonomy in this subtype of PD.