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1.
Neurol Sci ; 2024 Sep 19.
Artigo em Inglês | MEDLINE | ID: mdl-39298029

RESUMO

BACKGROUND: Migraine is a debilitating neurological condition linked to various psychological comorbidities. The aims of our study are: 1) to evaluate potential psychopathological differences between patients in treatment with anti-CGRP monoclonal antibodies (SPECIFIC group) and those with onabotulinumtoxin-A/oral pharmacotherapy (Group NON-SPECIFIC), 2) to compare treatment efficacy over time between groups, and examined whether psychopathological comorbidities can influence it. METHODS: This is a post-hoc ambispective study: a retrospective analysis of patient-level real-life data prospectively collected for clinical evaluation. We enrolled 102 patients with chronic migraine (CM), 64 in treatment with erenumab or galcanezumab, and 38 with botulinum toxin or oral pharmacotherapies. Psychopathological variables are assessed at baseline, whereas clinical factors over time. RESULTS: The NON-SPECIFIC group showed more pronounced emotion regulation difficulties (DERS, p = 0.001), alexithymia (TAS-20, p = 0.012), and impulsiveness (BIS-11, p = 0.002) with respect to the SPECIFIC group. Moreover, treatment efficacy overtime was more pronounced in the group with anti-CGRP treatment with a reduction of migraine frequency overtime, pain intensity, and improved quality of life up to six months post-treatment. Psychopathological comorbidity did not influence treatment efficacy. CONCLUSIONS: Our study highlighted more pronounced psychopathological comorbidities in patients in treatment with NON-SPECIFIC therapies in terms of impulsivity, alexithymia, and emotion regulation. Moreover, treatment efficacy overtime was more pronounced and stable over time in the SPECIFIC group, and psychopathological comorbidity did not influence it.

2.
Neurol Sci ; 2024 Aug 06.
Artigo em Inglês | MEDLINE | ID: mdl-39103734

RESUMO

INTRODUCTION: Several studies demonstrated the utility of immersive virtual reality (VR) as a complementary approach to conventional therapy for improving motor, psychological and cognitive impairment in some pathological conditions. Our pilot study aims to evaluate for the first time: 1) sense of presence, tolerability and usability of VR immersive experience in patients with early stages of Huntington disease (eHDp) compared to healthy controls (HC); 2) correlation between the use of technology/cybersickness and the variables of presence/usability; 3) correlation between clinical characteristics (genetic, motor, functional and cognitive) and VR's variables. METHOD: We recruited 10 eHDp and 10 age, gender and education matched HC. Participants completed questionnaires about sense of presence, usability, tolerability and technology use profile. Subjects were exposed to different VR scenarios from a first-person perspective through a standalone VR headset. RESULTS: Our results showed no significant statistical difference between eHDp and HC for the sense of presence (p=0.910), usability (p=0.744) and tolerability (p=0.730) during the VR experience. Familiarity with the use of technology was also comparable between groups (p=0.676). Regarding correlations in eHDp group, our results showed no correlations between use of technology/tolerability and the sense of presence/usability. Moreover, clinical characteristics of eHDp (genetic, motor, functional and cognitive scores) did not influence the sense of presence, tolerability and usability. CONCLUSION: Our research presents preliminary evidence for the applicability of VR in eHDp. These results open up the possibility to explore future applications of this methodology in rehabilitation (i.e., cognitive training, physiotherapy), diagnosis and psychological support in Huntington disease patients.

3.
Neurol Sci ; 45(8): 3785-3790, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38483677

RESUMO

INTRODUCTION: Parkinson's disease (PD) is a neurodegenerative disorder characterized by motor and non-motor symptoms, including alteration in emotional processing and recognition of emotions. We explored the effects of PD on the emotional behavioral ratings using a battery of affective visual stimuli selected from the International Affective Picture System (IAPS). METHODS: Twenty-two patients diagnosed with idiopathic PD and 22 healthy controls (HC), matched by age, gender, and education, were enrolled in the study. Following a clinical assessment, each participant was asked to evaluate the arousal and valence of affective visual stimuli, and response time was recorded. Disease-specific measures including the MDS Unified Parkinson's Disease Rating Scale (MDS UPDRS) and the Non-Motor Symptom Scale (NMSS) were also collected. RESULTS: PD patients exhibited higher arousal responses compared to HC for negative/unpleasant pictures (scoring 7.32 ± 0.88 vs 5.43 ± 2.06, p < 0.001). The arousal response to negative/unpleasant pictures was correlated with measures of non-motor burden in PD (MDS UPDRS I and NMSS, rho = 0.480 and p = 0.023, rho = 0.533 and p = 0.010, respectively). CONCLUSION: Impaired emotional processing characterizes PD patients with mild disease and is related to the non-motor symptom burden. Given the importance of emotional processing for the development and maintenance of close interpersonal relationship and for coping with specific medical situations, it is crucial to direct PD patients towards therapeutic interventions focused on the recognition and processing of emotions.


Assuntos
Nível de Alerta , Emoções , Doença de Parkinson , Humanos , Doença de Parkinson/psicologia , Doença de Parkinson/fisiopatologia , Feminino , Masculino , Nível de Alerta/fisiologia , Idoso , Pessoa de Meia-Idade , Emoções/fisiologia , Índice de Gravidade de Doença , Estimulação Luminosa/métodos
4.
Mov Disord Clin Pract ; 11(4): 363-372, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38264920

RESUMO

BACKGROUND: Cognitive changes in Huntington's disease (HD) precede motor manifestations. ENROLL-HD platform includes four cognitive measures of information processing speed (IPS). Our group is eager to seek clinical markers in the life stage that is as close as possible to the age of onset (ie, the so called prodromal HD phase) because this is the best time for therapeutic interventions. OBJECTIVES: Our study aimed to test whether cognitive scores in prodromal ENROLL-HD mutation carriers show the potential to predict the severity of motor and behavioral changes once HD became fully manifested. METHODS: From the global ENROLL-HD cohort of 21,343 participants, we first selected a premanifest Cohort#1 (ie, subjects with Total Motor Score (TMS) <10 and Diagnostic Confidence Level (DCL) <4, N = 1.222). From this cohort, we then focused on a prodromal Cohort#2 of subjects who were ascertained to phenoconvert into manifest HD at follow-up visits (ie, subjects from 6 ≤ TMS≤9 and DCL <4 to TMS≥10 and DCL = 4, n = 206). RESULTS: The main results of our study showed that low IPS before phenoconversion in Cohort#2 predicted the severity of motor and behavioral manifestations. By combining the four IPS cognitive measures (eg, the Categorical Verbal Fluency Test; Stroop Color Naming Test; Stroop Word Reading; Symbol Digit Modalities Test), we generated a Composite Cognition Score (CCS). The lower the CCS score the higher the TMS and the apathy scores in the same longitudinally followed-up patients after phenoconversion. CONCLUSIONS: CCS might represent a clinical instrument to predict the prognosis of mutation carriers who are close to manifesting HD.


Assuntos
Doença de Huntington , Humanos , Estudos Longitudinais , Estudos Retrospectivos , Prognóstico , Doença de Huntington/diagnóstico , Progressão da Doença , Cognição
5.
Neurol Sci ; 45(3): 1079-1086, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37770762

RESUMO

BACKGROUND: This study is aimed at assessing the clinimetric properties and feasibility of the Italian version of the Montreal Cognitive Assessment (MoCA) in patients with Huntington's disease (HD). METHODS: N = 39 motor-manifest HD patients, N = 74 Parkinson's disease (PD) patients and N = 92 matched HCs were administered the MoCA. HD patients further underwent the Unified Huntington's Disease Rating Scale (UHDRS), self-report questionnaires for anxiety and depression and a battery of first- and second-level cognitive tests. Construct validity was tested against cognitive and behavioural/psychiatric measures, whereas ecological validity against motor-functional subscales of the UHDRS. Sensitivity to disease severity was tested, via a logistic regression, by exploring whether the MoCA discriminated between patients in Shoulson-Fahn stage ≤ 2 vs. > 2. The same analysis was employed to test its ability to discriminate HD patients from HCs and PD patients. RESULTS: The MoCA converged towards cognitive and behavioural measures but diverged from psychiatric ones, being also associated with motor/functional measures from the UHDRS. In identifying patients with cognitive impairment, adjusted MoCA scores were highly accurate (AUC = .92), yielding optimal diagnostics at the cut-off of < 19.945 (J = .78). The MoCA was able to discriminate patients in the middle-to-advanced from those in the early-to-middle stages of the disease (p = .037), as well as to differentiate HD patients from both HCs (p < .001) and PD patients (p < .001). CONCLUSIONS: The MoCA is a valid, diagnostically sound and feasible cognitive screener in motor-manifest HD patients, whose adoption is thus encouraged in clinical practice and research.


Assuntos
Disfunção Cognitiva , Doença de Huntington , Humanos , Doença de Huntington/complicações , Doença de Huntington/diagnóstico , Estudos de Viabilidade , Disfunção Cognitiva/etiologia , Disfunção Cognitiva/complicações , Testes de Estado Mental e Demência , Testes Neuropsicológicos , Itália
6.
Neurol Sci ; 44(12): 4343-4348, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37432564

RESUMO

INTRODUCTION: Multiple sclerosis neuropsychological questionnaire (MSNQ) is a brief questionnaire useful for screening patient's and informant's self-perception of cognitive dysfunctions in daily life activities. Our study aims to evaluate the MSNQ validity in Huntington's disease (HD) mutation carriers and to correlate MSNQ scores with neurological, cognitive, and behavioral variables. METHODS: The study was conducted on a sample of 107 subjects from presymptomatic to the middle stage of HD recruited at LIRH Foundation and C.S.S. Mendel Institute in Rome. Unified Huntington's Disease Rating Scale (UHDRS), an internationally standardized and validated scale, was used to evaluate motor, functional cognitive, and behavioral domains. RESULTS: Our results showed that in HD subjects, MSNQ has a unidimensional factor structure. Correlational analyses indicated a good correlation between the MSNQ-patient version (MSNQ-p) and clinical variables, specifically with cognitive dysfunction and behavioral alterations. Moreover, higher scores in MSNQ-p were associated with higher motor disease and functional impairment showing that patients in advanced stage of HD perceive a greater cognitive impairment. These results confirm the questionnaire's reliability. CONCLUSIONS: The present study demonstrates the validity and adaptability of MSNQ in the HD population proposing it as a cognitive tool during routine clinical follow-ups, although further research is needed to determine an optimal cut-off score for this measure.


Assuntos
Doença de Huntington , Esclerose Múltipla , Humanos , Doença de Huntington/complicações , Doença de Huntington/diagnóstico , Esclerose Múltipla/complicações , Esclerose Múltipla/diagnóstico , Esclerose Múltipla/psicologia , Reprodutibilidade dos Testes , Testes Neuropsicológicos , Inquéritos e Questionários
7.
J Neurol ; 270(11): 5408-5417, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37462754

RESUMO

BACKGROUND: Progressive cognitive decline is an inevitable feature of Huntington's disease (HD) but specific criteria and instruments are still insufficiently developed to reliably classify patients into categories of cognitive severity and to monitor the progression of cognitive impairment. METHODS: We collected data from a cohort of 180 positive gene-carriers: 33 with premanifest HD and 147 with manifest HD. Using a specifically developed gold-standard for cognitive status we classified participants into those with normal cognition, those with mild cognitive impairment, and those with dementia. We administered the Parkinson's Disease-Cognitive Rating Scale (PD-CRS), the MMSE and the UHDRS cogscore at baseline, and at 6-month and 12-month follow-up visits. Cutoff scores discriminating between the three cognitive categories were calculated for each instrument. For each cognitive group and instrument we addressed cognitive progression, sensitivity to change, and the minimally clinical important difference corresponding to conversion from one category to another. RESULTS: The PD-CRS cutoff scores for MCI and dementia showed excellent sensitivity and specificity ratios that were not achieved with the other instruments. Throughout follow-up, in all cognitive groups, PD-CRS captured the rate of conversion from one cognitive category to another and also the different patterns in terms of cognitive trajectories. CONCLUSION: The PD-CRS is a valid and reliable instrument to capture MCI and dementia syndromes in HD. It captures the different trajectories of cognitive progression as a function of cognitive status and shows sensitivity to change in MCI and dementia.


Assuntos
Disfunção Cognitiva , Doença de Huntington , Doença de Parkinson , Humanos , Doença de Huntington/complicações , Doença de Huntington/diagnóstico , Doença de Huntington/genética , Testes Neuropsicológicos , Disfunção Cognitiva/diagnóstico , Disfunção Cognitiva/etiologia , Disfunção Cognitiva/psicologia , Cognição , Doença de Parkinson/complicações , Doença de Parkinson/diagnóstico
8.
Front Psychol ; 13: 1031871, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36533005

RESUMO

Background: This study aimed at assessing the diagnostic properties of the Frontal Assessment Battery (FAB) as to its capability to (1) discriminate healthy controls (HCs) from patients with Huntington's disease (HD) and (2) identify cognitive impairment in this population. Materials: Thirty-eight consecutive HD patients were compared to 73 HCs on the FAB. Patients further underwent the Montreal Cognitive Assessment (MoCA) and the Unified Huntington's Disease Rating Scale (UHDRS). Receiver-operating characteristics (ROC) analyses were run to assess both intrinsic-i.e., sensitivity (Se) and specificity (Sp), and post-test diagnostics, positive and negative predictive values (PPV; NPV) and likelihood ratios (LR+; LR-), of the FAB both in a case-control setting and to identify, within the patient cohort, cognitive impairment (operationalized as a below-cut-off MoCA score). In patients, its diagnostic accuracy was also compared to that of the cognitive section of the UHDRS (UHDRS-II). Results: The FAB and UHDRS-II were completed by 100 and 89.5% of patients, respectively. The FAB showed optimal case-control discrimination accuracy (AUC = 0.86-0.88) and diagnostic properties (Se = 0.68-0.74; Sp = 0.88-0.9; PPV = 0.74-0.8; NPV = 0.84-0.87; LR+ = 5.6-7.68; LR- = 0.36-0.29), performing even better (AUC = 0.9-0.91) at identifying cognitive impairment among patients (Se = 0.73-1; Sp = 0.86-0.71; PPV = 0.79-0.71; NPV = 0.82-1; LR+ =5.13-3.5; LR- = 0.31-0) and comparably to the UHDRS-II (89% vs. 85% of accuracy, respectively; p = 0.46). Discussion: In HD patients, the FAB is highly feasible for cognitive screening aims, being also featured by optimal intrinsic/post-test diagnostics within both case-control and case-finding settings.

9.
Front Aging Neurosci ; 14: 1031908, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36408105

RESUMO

Background: The semiology and determinants of apathy are largely unknown across amyotrophic lateral sclerosis (ALS), Parkinson's disease (PD), and Huntington's disease (HD), due to both motor and non-motor confounders. This study thus aimed at (1) profiling apathy in ALS, PD, and HD and (2) exploring its clinical determinants. Materials: Consecutive ALS (N = 99), PD (N = 73), and HD (N = 25) patients underwent a motor-free assessment of apathy (Dimensional Apathy Scale, DAS), global cognition, anxiety and depression. Function was assessed through disease-specific scales. The DAS was also completed by N = 101 healthy controls (HCs). Between-group comparisons on DAS scores were implemented by covarying for all applicable confounders. Predictive models on DAS scores were built through multiple, stepwise regressions. Results: Parkinson's disease and HD, but not ALS, patients were more apathetic than HCs-with HD patients also selectively showing lower initiation and poorer goal-directed planning than HCs. Higher apathetic features were detected in PD and HD as compared to ALS. Education was a protective factor against apathy in ALS. Anxiety was a risk factor for global apathy in ALS, HD, and to a lesser extent, in PD, whereas, protective only toward affective disintegration in PD and ALS. Cognitive inefficiency was a risk factor toward apathy in both PD and ALS. Depression was a risk factor for executive-related apathy in PD. Discussion: This study provides unprecedented insights into the heterogeneous semiology and determinants of apathy across ALS, PD, and HD via the DAS, in turn informing clinical practice and research.

10.
J Pers Med ; 12(6)2022 May 25.
Artigo em Inglês | MEDLINE | ID: mdl-35743649

RESUMO

(1) Background: Sleep patterns are frequently disrupted in neurodegenerative disorders such as Huntington disease (HD); however, they are still poorly understood, especially their association with clinic features. Our study aimed to explore potential correlations between sleep features and motor, cognitive, behavioural and functional changes in manifest HD subjects. (2) Methods: We enrolled 42 patients who were assessed by the Pittsburgh Sleep Quality Index (PSQI) and Insomnia Severity Index (ISI) questionnaires; clinical features were evaluated by the validated ENROLL-HD platform assay, including the Unified Huntington's Disease Rating Scale (UHDRS) and the Problem Behaviours Assessment Short Form (PBA-s). (3) Results: We found a significant association between the patients' perception of sleep abnormalities and scores of impaired independence, cognitive and motor performances. Specifically, sleep efficiency (PSQI-C4 subscores) and the use of sleep medications (PSQI-C6 subscores) seem to be more frequently associated with the severity of the disease progression. (4) Conclusion: sleep abnormalities represent an important part of the HD clinical profile and can impair patients' quality of life by affecting their level of independence, cognition performance and mental well-being.

12.
Neurol Sci ; 43(6): 3803-3810, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35064344

RESUMO

Migraineurs show impaired cognitive functions interictally, mainly involving information processing speed, basic attention, and executive functions. We aimed to assess executive impairment in migraine patients with different attack frequencies through a task-switching protocol designed to assess different sub-processes of executive functioning. We enrolled 42 migraine patients and divided them into three groups based on the attack frequency: 13 subjects had episodic migraine with a low frequency (LFEM, 4-7 migraine days per month), 14 subjects had high-frequency episodic migraine (HFEM, 8-14 days) and, finally, 15 subjects presented chronic migraine (≥ 15 headache days/month, CM); we compared them to 20 healthy control (HC), matched to both gender and education. Patients with high headache frequencies (CM and HFEM) showed worse performance than LFEM and HC controls, as indicated by poor accuracy, increased switch cost, and reaction times. Our study demonstrated a difference in task-switching abilities in patients with high frequency or chronic migraine compared with low-frequency episodic migraine and healthy controls. These difficulties in executive control processes could be related to altered functioning of the frontal cortex and its cortical and subcortical connections.


Assuntos
Transtornos de Enxaqueca , Cefaleia , Humanos
13.
J Pers Med ; 12(1)2022 Jan 03.
Artigo em Inglês | MEDLINE | ID: mdl-35055351

RESUMO

We focused on Cognitive Reserve (CR) in patients with early Huntington Disease (HD) and investigated whether clinical outcomes might be influenced by lifetime intellectual enrichment over time. CR was evaluated by means of the Cognitive Reserve Index questionnaire (CRIq), an internationally validated scale which includes three sections: education, working activity, and leisure time. The clinical HD variables were quantified at three different time points (baseline-t0, 1 year follow up-t1 and 2 years follow up-t2) as per the Unified Huntington's Disease Rating Scale (UHDRS), an internationally standardized and validated scale including motor, cognitive, functional and behavioral assays. Our sample consisted of 75 early manifest patients, withclinical stage scored according to the Total Functional Capacity (TFC) scale. Our correlational analysis highlighted a significant inverse association between CRIq leisure time (CRIq_LA) and longitudinal functional impairment (namely, the differential TFC score between t2 and t0 or ΔTFC) (p < 0.05), and the multidimensional progression of HD as measured by the composite UHDRS (cUHDRS, p < 0.01). CRIq_LA was significantly and positively associated with better cognitive performances at all time points (p < 0.05). Our results suggest that higher is the CRIq_LA, milder is the progression of HD in terms of functional, multidimensional and cognitive outcome.

14.
J Pers Med ; 12(1)2022 Jan 17.
Artigo em Inglês | MEDLINE | ID: mdl-35055435

RESUMO

The "Spazio Huntington-A Place for Children" program was launched in 2019. The aim was to contact at risk kids within Huntington disease (HD) families, to provide counseling to their parents and to start a prospective follow-up of kids suspicious to manifest pediatric HD (PHD). We met 25 at risk kids in two years, four of whom with PHD and highly expanded (HE) mutations beyond 80 CAG repeats. We rated motor, neuropsychological and behavioral changes in all PHD kids by the Unified HD Rating Scale (UHDRS)-total motor score (TMS) and additional measures of (1) cognitive level (Leiter International Performance Scale), (2) adaptive functioning (Adaptive Behavior Assessment Systems), (3) receptive language (Peabody Picture Vocabulary Test) and (4) behavioral abnormalities (Child Behavior Check List and Children's Yale-Brown Obsessive Compulsive Scale). All PHD kids showed a severe progression of neurological and psychiatric manifestations including motor, cognitive and behavioral changes. The magnetic resonance imaging contributed to confirm the suspicious clinical observation by highlighting very initial striatum abnormalities in PHD. Spazio Huntington is a program to prospectively study PHD, the most atypical face of HD, and may represent the basis to recruit PHD patients in future clinical trials.

15.
J Huntingtons Dis ; 10(4): 459-468, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34602494

RESUMO

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 < 0.05) patients and HC (p < 0.01). Early-mild HD patients had lower scores than preHD patients (p < 0.001) and HC (p < 0.001). Compared to HC, preHD and early-mild HD participants made more errors as trial complexity increased. Moreover, arithmetic word-problem solving scores were significantly associated with measures of global cognition (p < 0.001), frontal-executive functions (p < 0.001), attention (p < 0.001) visual working memory (p < 0.001), mental rotation (p < 0.001), and confrontation naming (p < 0.05). CONCLUSION: Arithmetic word-problem solving is affected early in the course of HD and is related to deficient processes in frontal-executive and mentalizing-related processes.


Assuntos
Doença de Huntington , Biomarcadores , Cognição , Progressão da Doença , Função Executiva , Humanos , Doença de Huntington/genética , Testes Neuropsicológicos , Resolução de Problemas
16.
Brain Behav ; 11(7): e02151, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-34110097

RESUMO

INTRODUCTION: Behavioral and cognitive changes can be observed across all Huntington disease (HD) stages. Our multicenter and retrospective study investigated the association between cognitive and behavioral scale scores in manifest HD, at three different yearly timepoints. METHODS: We analyzed cognitive and behavioral domains by the Unified Huntington's Disease Rating Scale (UHDRS) and by the Problem Behaviors Assessment Short Form (PBA-s), at three different yearly times of life (t0 or baseline, t1 after one year, t2 after two years), in 97 patients with manifest HD (mean age 48.62 ± 13.1), from three ENROLL-HD Centers. In order to test the disease progression, we also examined patients' motor and functional changes by the UHDRS, overtime. RESULTS: The severity of apathy and of perseveration/obsession was associated with the severity of the cognitive decline (p < .0001), regardless of the yearly timepoint. The score of irritability significantly and positively correlated with perseveration errors in the verbal fluency test at t0 (r = .34; p = .001), while the psychosis significantly and negatively correlated with the information processing speed at t0 (r = -.21; p = .038) and significantly and positively correlated with perseveration errors in the verbal fluency test at t1 (r = .35; p < .0001). The disease progression was confirmed by the significant worsening of the UHDRS-Total Motor Score (TMS) and of the UHDRS-Total Functional Capacity (TFC) scale score after two-year follow-up (p < .0001). CONCLUSION: Although the progression of abnormal behavioral manifestations cannot be predicted in HD, the severity of apathy and perseveration/obsessions are significantly associated with the severity of the cognitive function impairment, thus contributing, together, to the disease development and to patients' loss of independence, in addition to the neurological manifestations. This cognitive-behavior pattern determines a common underlying deficit depending on a dysexecutive syndrome.


Assuntos
Doença de Huntington , Comportamento Problema , Adulto , Cognição , Estudos Transversais , Humanos , Pessoa de Meia-Idade , Estudos Retrospectivos , Índice de Gravidade de Doença
18.
Neurol Sci ; 42(12): 4997-5006, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33728549

RESUMO

INTRODUCTION: The study aims at investigating psychometric properties of the Edinburgh cognitive and behavioural ALS screen (ECAS) in Parkinson's (PD) and Huntington's (HD) diseases. The sensitivity and specificity of the ECAS in highlighting HD and PD cognitive-behavioural features and in differentiating between these two populations and from healthy controls (HC) were evaluated. Moreover, correlations between the ECAS and traditional cognitive measures, together with core clinical features, were analysed. METHODS: Seventy-three PD patients, 38 HD patients, and 49 education-matched healthy participants were enrolled. Participants were administered the ECAS, together with other cognitive screening tools and psychological questionnaires. Patients' behavioural assessment was also carried out with carers. RESULTS: The ECAS distinguished between HD patients and HC and between the two clinical syndromes with high sensitivity and specificity. Even if the diagnostic accuracy of the ECAS in distinguishing between PD and HC was low, the PD cognitive phenotype was very well described by the ECAS performances. Convergent validity of the ECAS against other traditional cognitive screening was observed, as well as correlations with psychological aspects and typical clinical features, especially for the HD group. CONCLUSIONS: The ECAS represents a rapid and feasible tool, useful also in other neurodegenerative disorders affecting verbal-motor abilities than the amyotrophic lateral sclerosis such as PD and HD. Clinical applications in these neurodegenerative conditions require further investigations and, probably, some adaptations of the original test.


Assuntos
Esclerose Lateral Amiotrófica , Transtornos Cognitivos , Doença de Huntington , Doença de Parkinson , Esclerose Lateral Amiotrófica/complicações , Esclerose Lateral Amiotrófica/diagnóstico , Humanos , Doença de Huntington/complicações , Doença de Huntington/diagnóstico , Testes Neuropsicológicos , Doença de Parkinson/complicações , Doença de Parkinson/diagnóstico , Sensibilidade e Especificidade
19.
Neurol Sci ; 42(11): 4583-4589, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33651198

RESUMO

The Multiple Sclerosis Neuropsychological Questionnaire (MSNQ) is a brief questionnaire useful for screening patients with multiple sclerosis (MS) at risk for cognitive impairment. It includes a patient self-assessment (MSNQ-p) and a section for the caregiver (informant) (MSNQ-i). This study's aim was to validate the Italian version of MSNQ and to compare MSNQ scores with Symbol Digit Modality Test (SDMT), Beck Depression Inventory (BDI), and Expanded Disability Status Scale (EDSS) score, measuring cognitive skills, mood status, and physical disability respectively. We enrolled 122 MS patients (and related caregivers) at MS center of Tor Vergata University Hospital of Rome. The final study sample consisted of 122 patients with MS (90 relapsing-remitting, 24 secondary progressive, and 8 primary progressive). Our results highlighted that MSNQ has a unidimensional factor structure. Correlational analyses found a good correlation between both versions (MSNQ-p and MSNQ-i) of the questionnaire. Both MSNQ-p and MSNQ-i were correlated with clinical variables, specifically with cognitive impairment, mood disorder, and with disability. The Italian version of MSNQ is reliable and useful as screening tool to identify MS patients at high risk of cognitive impairment.


Assuntos
Transtornos Cognitivos , Esclerose Múltipla , Transtornos Cognitivos/diagnóstico , Transtornos Cognitivos/etiologia , Humanos , Itália/epidemiologia , Esclerose Múltipla/complicações , Esclerose Múltipla/diagnóstico , Recidiva Local de Neoplasia , Testes Neuropsicológicos , Inquéritos e Questionários
20.
Eur J Cancer Care (Engl) ; 30(4): e13430, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-33728750

RESUMO

OBJECTIVE: This multicentre study aimed to develop a measure of the perception of care dependence in patients diagnosed with cancer and to test its psychometric properties. METHODS: The questionnaire was developed based on findings emerged from a meta-synthesis and from qualitative studies conducted in three hospitals in Italy. The draft questionnaire was tested for face and content validity and pilot-tested with patients. The questionnaire was completed by care-dependent patients with cancer. Test-retest was conducted to verify stability. Exploratory factor analysis (EFA) was performed using a Maximum Likelihood robust estimator. RESULTS AND CONCLUSION: The Scale-Content Validity Index was 0.92. The final 15-item questionnaire was completed by 208 patients admitted to two hospitals. The EFA yielded a two-factor model including a positive and a negative perception of care dependence. Factor score determinacy coefficients, Cronbach's alpha coefficients, composite reliability coefficients and Intraclass Correlations Coefficients yielded satisfactory results confirming internal consistency and stability. The hedonic balance score is also available as a single indicator of subjective well-being. The study provides initial validation of the Care DEeP Questionnaire that can be used by cancer nurses to assess positive and negative patient experiences with care dependence and to personalise and improve their care.


Assuntos
Percepção , Análise Fatorial , Humanos , Psicometria , Reprodutibilidade dos Testes , Inquéritos e Questionários
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