I DEFICIT COGNITIVI NELLA SCLEROSI LATERALE AMIOTROFICA: DALLA VALUTAZIONE AI CORRELATI NEURALI

Il presente lavoro si propone di validare, mediante l’impiego di metodiche psicometriche e di neuroimmagine, l’ Edinburgh Cognitive and Behavioural ALS Screen (ECAS) come test adatto ad indicizzare il profilo cognitivo dei pazienti affetti da Sclerosi Laterale Amiotrofica (SLA). I risultati supportano l’ipotesi che, nella SLA, il deterioramento cognitivo, collegato alla degenerazione lobare frontotemporale, aumenta con l’incremento del livello di disabilità motoria. In tale contesto, l’ Edinburgh Cognitive and Behavioural ALS Screen (ECAS) si attesta come un test di screening utile per esaminare le funzioni cognitive e comportamentali solitamente compromesse nella SLA, dal momento che i risultati da esso forniti sono fortemente associati ai cambiamenti nell’architettura microstrutturale cerebrale dei pazienti SLA

Speech Rhythm Variation in Early-Stage Parkinson's Disease: A Study on Different Speaking Tasks

Patients with Parkinson's disease (PD) usually reveal speech disorders and, among other symptoms, the alteration of speech rhythm. The purpose of this study is twofold: (1) to test the validity of two acoustic parameters—%V, vowel percentage and VtoV, the mean interval between two consecutive vowel onset points—for the identification of rhythm variation in early-stage PD speech and (2) to analyze the effect of PD on speech rhythm in two different speaking tasks: reading passage and monolog. A group of 20 patients with early-stage PD was involved in this study and compared with 20 age- and sex-matched healthy controls (HCs). The results of the acoustic analysis confirmed that %V is a useful cue for early-stage PD speech characterization, having significantly higher values in the production of patients with PD than the values in HC speech. A simple speaking task, such as the reading task, was found to be more effective than spontaneous speech in the detection of rhythmic variations

Non-motor impairments affect walking kinematics in Parkinson disease patients: A cross-sectional study

Background: In patients with Parkinson disease (PD), severe postural and gait impairments are rarely observed in early stage of disease and non-motor symptoms (NMS) are often overlooked. Objective: This observational study aimed to characterize the impact of non-motor impairments on walking kinematics in early stages PD patients, and to assess the differences of gait parameters and NMS between PD patients with and without mild cognitive impairment (MCI). Methods: Twenty-six patients with Modified Hoehn and Yahr Scale score≤2 were evaluated for NMS using Kings Parkinson's Pain Scale, Parkinson Fatigue Severity scale, Parkinson Anxiety Scale, Beck Depression Inventory and Epworth Sleepiness Scale, kinematic parameters through an inertial sensor and cognitive performance by a comprehensive neuropsychological battery. Results: Fatigue had a moderate negative correlation with step cadence, and a moderate to strong positive correlation with gait duration, Timed Up and Go (TUG) and TUG Dual Task (p < 0.01). Pain showed positive moderate correlation with gait duration (p < 0.01). Twelve patients resulted affected by MCI and reported significantly worse scores in gait duration, pain and fatigue (p < 0.05). According to cognitive z scores, PD-MCI group showed a moderate negative correlation between visuospatial abilities and fatigue (p < 0.05). Conclusions: NMS significantly affect walking kinematics whereas a limited role of cognitive status on motor performance occur in the early PD stages

Cognitive dysfunctions and psychological symptoms in migraine without aura: a cross-sectional study

BACKGROUND: The occurrence of cognitive dysfunctions and psychological symptoms, as well as their mutual relationships, in migraine patients are still debated. The aim of the study was to characterize the cognitive profile and psychological symptoms (i.e. depression, anxiety and apathy) in drug-naïve migraine without aura (MwoA) patients. METHODS: Seventy-two consecutive MwoA patients, referred to the Italian University Headache Clinic and 72 healthy subjects (HCs) were enrolled. Patients, during an attack-free period, and HCs completed Montreal Cognitive Assessment (MoCA), Beck Depression Inventory-II (BDI-II), Self-version of Apathy Evaluation Scale (AES-S) and State and Trait Anxiety Inventory (STAI-Y-1 and 2). Clinical parameters of disease severity (i.e. disease duration, migraine attacks per month, mean pain intensity during migraine attacks, migraine disability and impact on daily life) were recorded. RESULTS: Although performance of MwoA patients on MoCA was above Italian cut-off threshold (<15.5) suggesting presence of cognitive impairment, MwoA patients achieved significantly lower scores than HCs on total MoCA scale (22.3 ± 2.7 versus 25.4 ± 2.3) and on its attention (4.9 ± 1.1 versus 5.6 ± 0.7), memory (1.8 ± 1.4 versus 3.1 ± 1.3), visuospatial (3.2 ± 0.9 versus 3.6 ± 0.6) and executive subscales (2.6 ± 1.1 versus 3.1 ± 0.8). In addition, we observed significant correlations between MoCA executive domain subscore and the attack-related disability score (MIDAS). As for behavioral profile, the percentage of depressive symptoms (4.2 %), high state and trait anxiety (13.9 and 9.7 %, respectively), and apathy (11.1 %) in MwoA patients were similar to that of HCs. No significant associations of behavioural symptoms with cognitive performance and clinical parameters were found. CONCLUSIONS: Drug-naïve MwoA patients are characterized by subtle cognitive dysfunctions and low percentage of behavioural symptoms. The results support the importance of searching for subclinical cognitive disturbances in patients with MwoA, who deserve to be followed-up to verify whether they develop clinically relevant disorders over time

Correlates of the discrepancy between objective and subjective cognitive functioning in non-demented patients with Parkinson’s disease

Abstract: Background: Subjective complaints of cognitive deficits are not necessarily consistent with objective evidence of cognitive impairment in Parkinson’s disease (PD). Here we examined the factors associated with the objective-subjective cognitive discrepancy. Methods: We consecutively enrolled 90 non-demented patients with PD who completed the Parkinson’s Disease Cognitive Functional Rating Scale (subjective cognitive measure) and the Montreal Cognitive Assessment (MoCA; objective cognitive measure). The patients were classified as “Overestimators”, “Accurate estimators”, and “Underestimators” on the basis of the discrepancy between the objective vs. subjective cognitive measures. To identify the factors distinguishing these groups from each other, we used chi-square tests or one-way analyses of variance, completed by logistic and linear regression analyses. Results: Forty-nine patients (54.45%) were classified as “Accurate estimators”, 29 (32.22%) as “Underestimators”, and 12 (13.33%) as “Overestimators”. Relative to the other groups, the “Underestimators” scored higher on the Fatigue Severity Scale (FSS), Beck Depression Inventory (BDI), and Parkinson Anxiety Scale (p < 0.01). Logistic regression confirmed that FSS and BDI scores distinguished the “Underestimators” group from the others (p < 0.05). Linear regression analyses also indicated that FSS and BDI scores positively related to objective-subjective cognitive discrepancy (p < 0.01). “Overestimators” scored lower than other groups on the MoCA’s total score and attention and working memory subscores (p < 0.01). Conclusion: In more than 45% of consecutive non-demented patients with PD, we found a ‘mismatch’ between objective and subjective measures of cognitive functioning. Such discrepancy, which was related to the presence of fatigue and depressive symptoms and frontal executive impairments, should be carefully evaluated in clinical setting

COVID-19 Severity in Multiple Sclerosis: Putting Data Into Context

Background and objectives: It is unclear how multiple sclerosis (MS) affects the severity of COVID-19. The aim of this study is to compare COVID-19-related outcomes collected in an Italian cohort of patients with MS with the outcomes expected in the age- and sex-matched Italian population. Methods: Hospitalization, intensive care unit (ICU) admission, and death after COVID-19 diagnosis of 1,362 patients with MS were compared with the age- and sex-matched Italian population in a retrospective observational case-cohort study with population-based control. The observed vs the expected events were compared in the whole MS cohort and in different subgroups (higher risk: Expanded Disability Status Scale [EDSS] score &gt; 3 or at least 1 comorbidity, lower risk: EDSS score ≤ 3 and no comorbidities) by the χ2 test, and the risk excess was quantified by risk ratios (RRs). Results: The risk of severe events was about twice the risk in the age- and sex-matched Italian population: RR = 2.12 for hospitalization (p &lt; 0.001), RR = 2.19 for ICU admission (p &lt; 0.001), and RR = 2.43 for death (p &lt; 0.001). The excess of risk was confined to the higher-risk group (n = 553). In lower-risk patients (n = 809), the rate of events was close to that of the Italian age- and sex-matched population (RR = 1.12 for hospitalization, RR = 1.52 for ICU admission, and RR = 1.19 for death). In the lower-risk group, an increased hospitalization risk was detected in patients on anti-CD20 (RR = 3.03, p = 0.005), whereas a decrease was detected in patients on interferon (0 observed vs 4 expected events, p = 0.04). Discussion: Overall, the MS cohort had a risk of severe events that is twice the risk than the age- and sex-matched Italian population. This excess of risk is mainly explained by the EDSS score and comorbidities, whereas a residual increase of hospitalization risk was observed in patients on anti-CD20 therapies and a decrease in people on interferon

DMTs and Covid-19 severity in MS: a pooled analysis from Italy and France

We evaluated the effect of DMTs on Covid-19 severity in patients with MS, with a pooled-analysis of two large cohorts from Italy and France. The association of baseline characteristics and DMTs with Covid-19 severity was assessed by multivariate ordinal-logistic models and pooled by a fixed-effect meta-analysis. 1066 patients with MS from Italy and 721 from France were included. In the multivariate model, anti-CD20 therapies were significantly associated (OR&nbsp;=&nbsp;2.05, 95%CI&nbsp;=&nbsp;1.39–3.02, p&nbsp;&lt;&nbsp;0.001) with Covid-19 severity, whereas interferon indicated a decreased risk (OR&nbsp;=&nbsp;0.42, 95%CI&nbsp;=&nbsp;0.18–0.99, p&nbsp;=&nbsp;0.047). This pooled-analysis confirms an increased risk of severe Covid-19 in patients on anti-CD20 therapies and supports the protective role of interferon

Exploring visuospatial abilities and their contribution to constructional abilities and nonverbal intelligence

The present study aimed at exploring relationships among the visuospatial tasks included in the Battery for Visuospatial Abilities (BVA), and at assessing the relative contribution of different facets of visuospatial processing on tests tapping constructional abilities and nonverbal abstract reasoning. One hundred forty-four healthy subjects with a normal score on Mini Mental State Examination completed the BVA plus Raven’s Coloured Progressive Matrices and Constructional Apraxia test. We used Principal Axis Factoring and Parallel Analysis to investigate relationships among the BVA visuospatial tasks, and performed regression analyses to assess the visuospatial contribution to constructional abilities and nonverbal abstract reasoning. Principal Axis Factoring and Parallel Analysis revealed two eigenvalues exceeding 1, accounting for about 60% of the variance. A 2-factor model provided the best fit. Factor 1 included sub-tests exploring “complex” visuospatial skills, whereas Factor 2 included two subtests tapping “simple” visuospatial skills. Regression analyses revealed that both Factor 1 and Factor 2 significantly affected performance on Raven’s Coloured Progressive Matrices, whereas only the Factor 1 affected performance on Constructional Apraxia test. Our results supported functional segregation proposed by De Renzi, suggesting clinical caution to utilize a single test to assess visuospatial domain, and qualified the visuospatial contribution in drawing and non-verbal intelligence test

Theory of Mind and Its Neuropsychological and Quality of Life Correlates in the Early Stages of Amyotrophic Lateral Sclerosis

This study aims to explore the potential impairment of Theory of Mind (ToM; i.e., the ability to represent cognitive and affective mental states to both self and others) and the clinical, neuropsychological and Quality of Life (QoL) correlates of these cognitive abnormalities in the early stages of amyotrophic lateral sclerosis (ALS), a multisystem neurodegenerative disease recently recognized as a part of the same clinical and pathological spectrum of frontotemporal lobar degeneration. Twenty-two consecutive, cognitively intact ALS patients, and 15 healthy controls, underwent assessment of executive, verbal comprehension, visuospatial, behavioral, and QoL measures, as well as of the ToM abilities by Emotion Attribution Task (EAT), Advanced Test of ToM (ATT), and Eyes Task (ET). ALS patients obtained significantly lower scores than controls on EAT and ET. No significant difference was found between the two groups on ATT. As regard to type of ALS onset, patients with bulbar onset performed worse than those with spinal onset on ET. Correlation analysis revealed that EAT and ET were positively correlated with education, memory prose, visuo-spatial performances, and "Mental Health" scores among QoL items. Our results suggest that not only "cognitive" but also "affective" subcomponents of ToM may be impaired in the early stages of ALS, with significant linkage to disease onset and dysfunctions of less executively demanding conditions, causing potential impact on patients' "Mental Health.