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1.
Artigo em Inglês | MEDLINE | ID: mdl-32484726

RESUMO

Background: A cognitive impairment, ranging from frontotemporal dementia (FTD) to milder forms of dysexecutive or behavioral dysfunction, is detected in 30-50% of patients affected by amyotrophic lateral sclerosis (ALS) at diagnosis. Such condition considerably influences the prognosis, and possibly impacts on the decision-making process with regards to end-of-life choices. The aim of our study is to examine the changes of cognitive and behavioral impairment in a large population of ALS from the time of diagnosis to a 6-month follow-up (IQR 5.5-9.0 months), and to examine to what extent the progression of cognitive impairment affects survival time and rate of disease progression.Methods: We recruited 146 ALS patients classified according to revised criteria of ALS and FTD spectrum disorder. In a multidisciplinary setting, during two subsequent visits we examined clinical features with ALSFRS-r score, FVC% and BMI, and cognitive status with an extensive neuropsychological evaluation.Results: At second examination, one-third of patients showed a worsening of cognitive impairment, namely 88% of ALSbi, 27% of ALSci, 40% of ALScbi, and, interestingly, also 24% of cognitive normal ALS developed a significant cognitive dysfunction. We find that those who changed their cognitive status presented a lower ALSFRS-r score at t1 and a shorter survival time compared to those who did not change, regardless of the type of cognitive impairment.Conclusion: We show how cognitive disorders in ALS patients can not only be present at diagnosis, but also manifest during disease and influence the progression of motor deficit and the prognosis.


Assuntos
Esclerose Amiotrófica Lateral , Demência Frontotemporal , Esclerose Amiotrófica Lateral/complicações , Cognição , Demência Frontotemporal/complicações , Humanos , Estudos Longitudinais , Testes Neuropsicológicos
2.
Neurology ; 94(8): e802-e810, 2020 02 25.
Artigo em Inglês | MEDLINE | ID: mdl-31907290

RESUMO

OBJECTIVE: To assess the determinants of amyotrophic lateral sclerosis (ALS) phenotypes in a population-based cohort. METHODS: The study population included 2,839 patients with ALS diagnosed in Piemonte, Italy (1995-2015). Patients were classified according to motor (classic, bulbar, flail arm, flail leg, predominantly upper motor neuron [PUMN], respiratory) and cognitive phenotypes (normal, ALS with cognitive impairment [ALSci], ALS with behavioral impairment [ALSbi], ALSci and ALSbi combined [ALScbi], ALS-frontotemporal dementia [FTD]). Binary logistic regression analysis was adjusted for sex, age, and genetics. RESULTS: Bulbar phenotype correlated with older age (p < 0.0001), women were more affected than men at increasing age (p < 0.0001), classic with younger age (p = 0.029), men were more affected than women at increasing age (p < 0.0001), PUMN with younger age (p < 0.0001), flail arm with male sex (p < 0.0001) and younger age (p = 0.04), flail leg with male sex with increasing age (p = 0.008), and respiratory with male sex (p < 0.0001). C9orf72 expansions correlated with bulbar phenotype (p < 0.0001), and were less frequent in PUMN (p = 0.041); SOD1 mutations correlated with flail leg phenotype (p < 0.0001), and were less frequent in bulbar (p < 0.0001). ALS-FTD correlated with C9orf72 (p < 0.0001) and bulbar phenotype (p = 0.008), ALScbi with PUMN (p = 0.014), and ALSci with older age (p = 0.008). CONCLUSIONS: Our data suggest that the spatial-temporal combination of motor and cognitive events leading to the onset and progression of ALS is characterized by a differential susceptibility to the pathologic process of motor and prefrontal cortices and lower motor neurons, and is influenced by age, sex, and gene variants. The identification of those factors that regulate ALS phenotype will allow us to reclassify patients into pathologically homogenous subgroups, responsive to targeted personalized therapies.


Assuntos
Esclerose Amiotrófica Lateral/classificação , Esclerose Amiotrófica Lateral/epidemiologia , Proteína C9orf72/genética , Disfunção Cognitiva/epidemiologia , Demência Frontotemporal/epidemiologia , Transtornos Motores/epidemiologia , Superóxido Dismutase-1/genética , Fatores Etários , Idoso , Esclerose Amiotrófica Lateral/genética , Disfunção Cognitiva/genética , Comorbidade , Feminino , Demência Frontotemporal/classificação , Demência Frontotemporal/genética , Humanos , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Transtornos Motores/classificação , Transtornos Motores/genética , Mutação , Fenótipo , Fatores Sexuais
3.
Neurology ; 93(10): e984-e994, 2019 09 03.
Artigo em Inglês | MEDLINE | ID: mdl-31409738

RESUMO

OBJECTIVE: To assess the association of the degree of severity of motor impairment to that of cognitive impairment in a large cohort of patients with amyotrophic lateral sclerosis (ALS). METHODS: This is a population-based cross-sectional study on patients with ALS incident in Piemonte, Italy, between 2007 and 2015. Cognitive status was classified according to the revised ALS-FTD Consensus Criteria. The King system and the Milano Torino Staging system (MiToS) were used for defining the severity of motor impairment. RESULTS: Of the 797 patients included in the study, 163 (20.5%) had ALS-frontotemporal dementia (FTD), 38 (4.8%) cognitive and behavioral impairment (ALScbi), 132 (16.6%) cognitive impairment (ALSci), 63 (7.9%) behavioral impairment (ALSbi), 16 (2.0%) nonexecutive impairment, and 385 (48.2%) were cognitively normal. According to King staging, the frequency of cases with ALS-FTD progressively increased from 16.5% in stage 1-44.4% in stage 4; conversely, the frequency of ALSci, ALSbi, and ALScbi increased from King stage 1 to King stage 3 and decreased thereafter. A similar pattern was observed with the MiToS staging. ALS-FTD was more frequent in patients with bulbar involvement at time of cognitive testing. Patients with C9ORF72 expansion (n = 61) showed more severe cognitive impairment with increasing King and MiToS stages. CONCLUSION: Our findings suggest that ALS motor and cognitive components may worsen in parallel, and that cognitive impairment becomes more pronounced when bulbar function is involved. Our data support the hypothesis that ALS pathology disseminates in a regional ordered sequence, through a cortico-efferent spreading model.


Assuntos
Esclerose Amiotrófica Lateral/diagnóstico , Esclerose Amiotrófica Lateral/psicologia , Disfunção Cognitiva/diagnóstico , Disfunção Cognitiva/psicologia , Progressão da Doença , Vigilância da População , Idoso , Idoso de 80 Anos ou mais , Esclerose Amiotrófica Lateral/genética , Proteína C9orf72/genética , Disfunção Cognitiva/genética , Estudos de Coortes , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos , Vigilância da População/métodos , Estudos Prospectivos , Sistema de Registros
4.
Acta Neurol Scand ; 139(5): 438-445, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30712314

RESUMO

OBJECTIVES: Amyotrophic lateral sclerosis (ALS) is not only a motor disorder: More than 50% of patients have cognitive dysfunctions over the course of the disease. At the same time, mood disorders may also occur in ALS patients following diagnosis due to the fatal prognosis; however, little data are available on any depression beforehand. Starting from these considerations, the aim of our study was to investigate the occurrence of depression in Italian ALS patients prior to diagnosis, evaluating its prevalence in the subjects who have developed cognitive dysfunctions and in those who did not. MATERIALS AND METHODS: We included 318 patients, establishing the presence of depression in the 5 years before ALS diagnosis. Patients underwent a complete battery of neuropsychological tests with the aim to evaluate the executive functions, behavior, language, and memory. RESULTS: Before diagnosis, 40 patients with ALS had been diagnosed with depression: Among them, 29 patients had cognitive impairment over the course of the disease and only 11 did not develop any cognitive alteration (OR 1.46; 95% CI: 1.26-1.66, adjusted for sex, age, and disease phenotype, P: 0.038). Moreover, there is a significant difference in survival time between ALS patients with depression before ALS, compared to ALS patients without previous depression (P: 0.006). CONCLUSIONS: We reported a high prevalence of depression in the past in patients with ALS and cognitive impairment, as compared to patients without cognitive deficits, showing also that patients with both had a shorter survival time. These aspects require multidisciplinary approach at disease onset.


Assuntos
Esclerose Amiotrófica Lateral/complicações , Esclerose Amiotrófica Lateral/psicologia , Disfunção Cognitiva/etiologia , Depressão/epidemiologia , Adulto , Idoso , Disfunção Cognitiva/epidemiologia , Depressão/complicações , Feminino , Humanos , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos , Prevalência
5.
Artigo em Inglês | MEDLINE | ID: mdl-29451027

RESUMO

We describe a 64-year-old woman, suffering from late-onset obsessive-compulsive disorder (OCD) from the age of 57, who developed dysarthria and dysphagia, spastic diplegic, and proximal muscles weakness. Needle electromyography showed no active denervation. Neuropsychological evaluation showed intact cognitive functioning. We diagnosed upper motor neuron disease (MND), with no known genetic correlates. Brain magnetic resonance (MRI) detected bilateral hippocampal atrophy with sclerosis of right hippocampus. 18F-FDG positron emission tomography (PET) showed moderate right temporal cortex thinning. Six months later, motor and behavioral symptoms worsened. Neuropsychological examination revealed long-term memory deficit and executive dysfunction. MRI and PET evidenced severe worsening of atrophy in temporal and frontal lobes. Four years later a definitive diagnosis of primary lateral sclerosis (PLS) and FTD was made. To our knowledge, this is the first report of PLS and FTD with OCD at onset.


Assuntos
Encéfalo/diagnóstico por imagem , Demência Frontotemporal/complicações , Doença dos Neurônios Motores/complicações , Transtorno Obsessivo-Compulsivo/complicações , Progressão da Doença , Feminino , Fluordesoxiglucose F18 , Demência Frontotemporal/diagnóstico por imagem , Demência Frontotemporal/fisiopatologia , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Doença dos Neurônios Motores/diagnóstico por imagem , Transtorno Obsessivo-Compulsivo/diagnóstico por imagem , Tomografia por Emissão de Pósitrons
6.
Neuropsychiatr Dis Treat ; 13: 909-916, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28356745

RESUMO

PURPOSE: Interest in assessing denial is still present, despite the criticisms concerning its definition and measurement. We tried to develop a questionnaire (Illness Denial Questionnaire, IDQ) assessing patients' and caregivers' denial in relation to their illness/disturbance. PATIENTS AND METHODS: After a preliminary study, a final version of 24 dichotomous items (true/false) was selected. We hypothesized a theoretical model with three dimensions: denial of negative emotions, resistance to change, and conscious avoidance, the first two composing the actual Denial and the last representing an independent component of the illness denial behavior. The IDQ was administered to 400 subjects (219 patients and 181 caregivers) together with the Anxiety-Depression Questionnaire - Reduced form (AD-R), in order to assess concurrent validity. Confirmatory factor analysis (CFA), internal consistency indices (Cronbach's α and McDonald's ω), and test-retest analysis were performed. RESULTS: CFA and internal consistency indices (Cronbach's α: 0.87-0.96) indicated a clear and meaningful three-factor structure of IDQ, for both patients and caregivers. Further analyses showed good concurrent validity, with Denial and its subscale negatively associated with anxiety and depression and avoidance positively associated with anxiety and depression. The IDQ also showed a good stability (r from 0.71 to 0.87). CONCLUSION: The IDQ demonstrated good psychometric properties. Denial of negative emotions and resistance to change seem to contribute to a real expression of denial, and conscious avoidance seems to constitute a further step in the process of cognitive-affective elaboration of the illness.

7.
G Ital Med Lav Ergon ; 38(2): 69-78, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27459838

RESUMO

The approach to patients affected by motor neuron disease (MND) and their caregivers requires specific training for the care-team. In fact, the progression of the disease, with the decline of physical--and sometimes cognitive--function, the increasing difficulties in speaking, breathing, and swallowing and the need of invasive choices, as the artificial nutrition and tracheostomy, constitute a challenge for the health professionals, often generating distress. For this reason, their cohesion and sharing abilities are fundamental. Psychologist assumes a strategic role in supporting and facilitating the analysis of clinical cases and of the team's intra/interpersonal dynamics. For this aim, he/she needs specific training and instruments. We here present a semi-structured interview--the Motor Neuron Disease-Psychological Interview (MoNeDi-PI)--which may guide in the psychological assessment of patients affected by MNDs and their caregivers. It can also be a handy reference tool for other members of the healthcare team providing necessary information about the patient and caregiver in order to optimize clinical decision making about which health interventions to apply.


Assuntos
Cuidadores/psicologia , Doença dos Neurônios Motores/psicologia , Doença dos Neurônios Motores/reabilitação , Enfermeiras e Enfermeiros/psicologia , Equipe de Assistência ao Paciente , Estresse Psicológico/etiologia , Adaptação Psicológica , Adulto , Idoso , Idoso de 80 Anos ou mais , Progressão da Doença , Feminino , Pessoal de Saúde/psicologia , Necessidades e Demandas de Serviços de Saúde , Humanos , Masculino , Pessoa de Meia-Idade , Doença dos Neurônios Motores/diagnóstico , Equipe de Assistência ao Paciente/normas , Equipe de Assistência ao Paciente/tendências , Índice de Gravidade de Doença , Inquéritos e Questionários
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