RESUMO
Background: Handicap is a patient-centered measure of health status that encompasses the impact of social and physical environment on daily living, having been assessed in advanced and late-stage Parkinson's Disease (PD). Objective: To characterize the handicap of a broader sample of patients. Methods: A cross-sectional study of 405 PD patients during the MDS-UPDRS Portuguese validation study, using the MDS-UPDRS, Unified Dyskinesias Rating Scale, Nonmotor symptoms questionnaire, PDQ-8 and EQ-5D-3L. Handicap was measured using the London Handicap Scale (LHS). Results: Mean age was 64.42 (±10.3) years, mean disease duration 11.30 (±6.5) years and median HY 2 (IQR, 2-3). Mean LHS was 0.652 (±0.204); "Mobility," "Occupation" and "Physical Independence" were the most affected domains. LHS was significantly worse in patients with longer disease duration, older age and increased disability. In contrast, PDQ-8 did not differentiate age groups. Handicap was significantly correlated with disease duration (r = -0.35), nonmotor experiences of daily living (EDL) (MDS-UPDRS-I) (r = -0.51), motor EDL (MDS-UPDRS-II) (r = -0.69), motor disability (MDS-UPDRS-III) (r = -0.49), axial signs of MDS-UPDRS-III (r = -0.55), HY (r = -0.44), presence of nonmotor symptoms (r = -0.51) and PDQ-8 index (r = -0.64) (all P < 0.05). Motor EDL, MDS-UPDRS-III and PDQ-8 independently predicted Handicap (adjusted R 2 = 0.582; P = 0.007). Conclusions: The LHS was easily completed by patients and caregivers. Patients were mild-moderately handicapped, which was strongly determined by motor disability and its impact on EDL, and poor QoL. Despite correlated, handicap and QoL seem to differ in what they measure, and handicap may have an added value to QoL. Handicap seems to be a good measure of perceived-health status in a broad sample of PD.
RESUMO
INTRODUCTION: Chorea is well described in a group of patients with Systemic Lupus Erythematosus (SLE). There is less information, however, on other movement disorders as well as non-motor neuropsychiatric features such as obsessive-compulsive symptoms (OCS), executive dysfunction and attention deficit and hyperactivity disorder (ADHD) in subjects with SLE. METHODS: Fifty-four subjects with SLE underwent a battery of neuropsychiatric tests that included the Mini Mental State Examination, the Montreal Cognitive Assessment, the Frontal Assessment Battery (FAB), the FAS verbal and the categorical (animals) semantic fluency tests, the Obsessive and Compulsive Inventory - Revised, the Yale-Brown Obsessive and Compulsive Scale and Beck's Anxiety and Depression Scales. ADHD was diagnosed according to DSM-IV criteria. SLE disease activity and cumulative damage were evaluated according to the modified SLE Disease Activity Index 2000 (mSLEDAI-2K) and the SLICC/ACR, respectively. RESULTS: Six (11.1%) and 33 (61.1%) patients had cognitive impairment according to the MMSE and MoCA, respectively. Eleven (20.4%) had abnormal FAB scores, and 5 (9.3%) had lower semantic fluency scores than expected. The overall frequency of cognitive dysfunction was 72.2% (39 patients) and of neuropsychiatric SLE was 77.8% (42 patients). Two patients (3.7%) had movement disorders. Fifteen (27.8%) had OCS and 17 (31.5%) met diagnostic criteria for ADHD. ADHD and OCS correlated with higher disease activity, p=0.003 and 0.006, respectively. Higher cumulative damage correlated with lower FAB scores (p 0.026). CONCLUSIONS: Executive dysfunction, ADHD, OCS, and movement disorders are common in SLE. Our finding suggests that there is frequent basal ganglia dysfunction in SLE.
Assuntos
Transtorno do Deficit de Atenção com Hiperatividade/complicações , Transtornos Cognitivos/complicações , Comportamento Compulsivo/complicações , Função Executiva/fisiologia , Lúpus Eritematoso Sistêmico/complicações , Comportamento Obsessivo/complicações , Adulto , Ansiedade/complicações , Ansiedade/fisiopatologia , Ansiedade/psicologia , Transtorno do Deficit de Atenção com Hiperatividade/fisiopatologia , Transtorno do Deficit de Atenção com Hiperatividade/psicologia , Gânglios da Base/fisiopatologia , Transtornos Cognitivos/fisiopatologia , Transtornos Cognitivos/psicologia , Comportamento Compulsivo/fisiopatologia , Comportamento Compulsivo/psicologia , Depressão/complicações , Depressão/fisiopatologia , Depressão/psicologia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/fisiopatologia , Lúpus Eritematoso Sistêmico/psicologia , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos , Comportamento Obsessivo/fisiopatologia , Comportamento Obsessivo/psicologia , Escalas de Graduação PsiquiátricaRESUMO
Huntington's disease (HD) is a rare neurodegenerative disease with a multitude of symptoms, which requires access to specialized multidisciplinary care for adequate management. The aim of this study was to survey the characteristics of care in various HD centers in South America (SA). Methods A questionnaire was sent to 24 centers involved in the care for HD patients in SA. Results Of the total 24 centers, 19 (79.2%) are academic units. The majority of centers (62.5%) are general movement disorders clinics. Multidisciplinary care is available in 19 (79.2%) centers and in 20 (83.3%) care is provided free of charge. Genetic testing and counseling are available in 25 and 66.6% of centers, respectively. The majority of centers (83.3%) have no institutional support for end-stage care. Conclusions Although HD centers in SA are committed to providing multidisciplinary care, access to genetic counseling and end-stage care are lacking in most centers.
Assuntos
Assistência Integral à Saúde/estatística & dados numéricos , Atenção à Saúde/estatística & dados numéricos , Doença de Huntington/terapia , Aconselhamento Genético , Testes Genéticos , Humanos , Assistência Centrada no Paciente/estatística & dados numéricos , América do Sul , Inquéritos e QuestionáriosRESUMO
BACKGROUND: Takayasu's arteritis (TA) has been associated with many conditions. Herein described is a case of TA in a patient with rheumatic fever complicated with Sydenham's chorea. CASE REPORT: A 17-year-old female presented at age 6 with rheumatic fever followed by chorea a month later. At the age of 16, she developed a blood pressure discrepancy between the arms and faint pulses. Computed tomography angiography revealed diffuse aortic involvement and narrowing of the arteries. DISCUSSION: The presence of rheumatic fever and Sydenham's chorea in TA raises the possibility of an immunological basis for the pathogenesis of the disease.
RESUMO
BACKGROUND: Sydenham's chorea is associated with dysfunction of fronto-striatal circuits induced by cross-reactive antibodies to group A ß-hemolytic streptococcus. High susceptibility of extrapyramidal effects of neuroleptics in patients with Sydenham's chorea suggests underlying nigro-striatal dysfunction. OBJECTIVE: To study the presence of parkinsonism in patients with a history of Sydenham's Chorea. METHODS: We used the UFMG Sydenham's Chorea Rating Scale (USCRS) and the Unified Parkinson's Disease Rating Scale (UPDRS) part III, respectively, to determine the presence of chorea and parkinsonian symptoms and signs in 25 adults with a history of previous Sydenham's Chorea currently without chorea or use of anti-choreic drugs. RESULTS: Bradykinesia was found in 64% of subjects. There was a statistically significant correlation between bradykinesia and hemichorea (-0.412; p = 0.036) and bradykinesia and generalized chorea (0.412; p = 0.036). There was no correlation between bradykinesia and use of anti-choreic drugs. CONCLUSIONS: Bradykinesia is common in patients with Sydenham's Chorea in remission. This finding suggests an immune-mediated dysfunction of the nigro-striatal system.
Assuntos
Coreia/complicações , Hipocinesia/fisiopatologia , Transtornos Parkinsonianos/etiologia , Adolescente , Adulto , Coreia/diagnóstico , Coreia/tratamento farmacológico , Feminino , Humanos , Masculino , Estudos Retrospectivos , Índice de Gravidade de Doença , Estatísticas não Paramétricas , Adulto JovemAssuntos
Coreia/etiologia , Discinesias/etiologia , Hiperglicemia/complicações , Antipsicóticos/uso terapêutico , Encéfalo/patologia , Coreia/patologia , Diabetes Mellitus Tipo 2/complicações , Discinesias/patologia , Feminino , Humanos , Hipoglicemiantes/uso terapêutico , Insulina/uso terapêutico , Corpos Cetônicos/urina , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Debilidade Muscular/etiologia , Exame Neurológico , Risperidona/uso terapêuticoRESUMO
Huntington's disease (HD) is a rare neurodegenerative disease with a multitude of symptoms, which requires access to specialized multidisciplinary care for adequate management. The aim of this study was to survey the characteristics of care in various HD centers in South America (SA).
A questionnaire was sent to 24 centers involved in the care for HD patients in SA.
Of the total 24 centers, 19 (79.2%) are academic units. The majority of centers (62.5%) are general movement disorders clinics. Multidisciplinary care is available in 19 (79.2%) centers and in 20 (83.3%) care is provided free of charge. Genetic testing and counseling are available in 25 and 66.6% of centers, respectively. The majority of centers (83.3%) have no institutional support for end-stage care.
Although HD centers in SA are committed to providing multidisciplinary care, access to genetic counseling and end-stage care are lacking in most centers.
A doença de Huntington (DH) é uma doença neurodegenerativa rara que requer tratamento multidisciplinar especializado para manejo adequado. O objetivo do presente trabalho foi pesquisar as características da assistência à saúde em centros de DH na América do Sul (AS).
Um questionário foi enviado para 24 centros envolvidos no cuidado de pacientes com DH na AS.
Dos 24 centros, 19 (79,2%) são unidades acadêmicas. A maioria (62,5%) são clínicas de distúrbios dos movimentos. Cuidado multidisciplinar é disponível em 19 (79,2%) dos centros e em 20 (83,3%), o tratamento é gratuito. O teste e o aconselhamento genético estão disponíveis em 25 e 66,6% dos centros, respectivamente. Não há suporte institucional para cuidado terminal em 83,3% dos centros.
Apesar dos centros de DH na AS terem compromisso com o provimento de cuidados multidisciplinares, o acesso a aconselhamento genético e a tratamento na fase terminal são falhos na maioria dos centros.