Handicap as a Measure of Perceived-Health Status in Parkinson's Disease.

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.

Clinical and functional correlates of parkinsonism in a population-based sample of individuals aged 75 + : the Pietà study.

BACKGROUND: Parkinsonism is strongly associated with ageing, and many studies have suggested that parkinsonian signs may affect up to half of older adults and is associated with a wide range of adverse health outcomes. We compared clinical and functional characteristics of oldest-old community-dwelling individuals with parkinsonism (parkinsonian group [PG]) to individuals without parkinsonism (non-parkinsonian group [NPG]. METHODS: The Pietà study is a population-based study conducted in Caeté, southeast Brazil, involving 607 individuals aged 75 + years submitted to an extensive clinical evaluation. A subset of 65 PG individuals (61.5% women, median age of 82 years) was compared to 542 NPG individuals (64.8% women, median age of 80 years). RESULTS: PG individuals had significantly more functional impairment, clinical comorbidities (including number of falls, loss of bladder control and dysphagia) and major depression. Multivariate analysis revealed that older age, higher UPDRSm scores, lower category fluency test (animals/minute) and delayed recall memory scores were associated with PG. This group was also more cognitively impaired, with lower performance than NPG individuals in the Mini-Mental State Examination, category fluency test (animals/minute), clock drawing and in delayed recall (p < 0.001 for all tests). UPDRSm scores were the most contributing factor to cognition that independently explained variability in functionality of the entire sample. CONCLUSION: Individuals aged 75 + years with parkinsonism were significantly more clinically and functionally impaired in this population-based sample. Cognitive dysfunction explained most of the loss of functionality in these patients. UPDRS-m scores contributed independently to explain variability in functionality in the whole sample.

Guidelines for Parkinson's disease treatment: consensus from the Movement Disorders Scientific Department of the Brazilian Academy of Neurology - motor symptoms.

The treatment of Parkinson's disease (PD) is challenging, especially since it is considered highly individualized. The Brazilian Academy of Neurology has recognized the need to disseminate knowledge about the management of PD treatment, adapting the best evidence to the Brazilian reality. Thus, the main published treatment guidelines were reviewed based on the recommendations of group from the Movement Disorders Scientific Department of the Brazilian Academy of Neurology.

Guidelines for Parkinson's disease treatment: consensus from the Movement Disorders Scientific Department of the Brazilian Academy of Neurology - motor symptoms / Diretrizes para o tratamento da doença de Parkinson: consenso do Departamento Científico de Transtornos do Movimento da Academia Brasileira de Neurologia - sintomas motores

ABSTRACT The treatment of Parkinson's disease (PD) is challenging, especially since it is considered highly individualized. The Brazilian Academy of Neurology has recognized the need to disseminate knowledge about the management of PD treatment, adapting the best evidence to the Brazilian reality. Thus, the main published treatment guidelines were reviewed based on the recommendations of group from the Movement Disorders Scientific Department of the Brazilian Academy of Neurology.

Resumo O tratamento da doença de Parkinson (DP) constitui um desafio, especialmente por ser considerado muito individualizado. A Academia Brasileira de Neurologia (ABN) identificou a necessidade de disseminar o conhecimento sobre o manejo do tratamento da DP, adaptando as melhores evidências à realidade brasileira. Assim, foi realizada uma revisão sobre as principais orientações de tratamento publicadas, baseada nas recomendações elaboradas por um grupo de especialistas em transtornos do movimento do departamento científico da ABN.

Parkinsonism in a population-based study of individuals aged 75+ years: The Pietà study.

INTRODUCTION: Parkinsonism is one of the most prevalent neurological syndromes in the elderly. There are only a few epidemiological studies focusing on parkinsonism in oldest-old individuals, particularly in Latin America. This study aims to investigate the prevalence and characteristics of parkinsonism in subjects aged 75 + years living in the community. METHODS: The Pietà study is a population-based investigation on brain aging conducted in Caeté, southeast Brazil. A sample composed of 610 community-dwelling individuals aged 75 + years (48.7% of the total population within this age range) underwent clinical, neurological, cognitive and functional assessments. RESULTS: The sample comprised mostly women (61.5%), with mean age of 83.3 years and mean schooling of 2.5 years. Parkinsonism was identified in 65 subjects (crude prevalence = 10.7%). Parkinson's disease, Parkinsonism + dementia syndrome, drug-induced parkinsonism, vascular parkinsonism and Lewy-body dementia were identified in, respectively, 19 (29.2%), 19 (29.2%), 8 (12.3%), 4 (6.1%) and 1 (1.5%) subjects. In 14 individuals (21.5%), the etiology of parkinsonism could not be determined. The most important vascular risk factor was hypertension (64.6%). Cognitive evaluation disclosed dementia in 37 (56.9%) subjects. Mean Pfeffer's functional activities questionnaire score was 12.2 points and 22 (33.8%) subjects were impaired in basic activities of daily living. CONCLUSION: Parkinsonism was common in this oldest-old population, being associated with dementia and vascular risk factors, particularly hypertension. Different confounders, such as concomitant dementia and exposure to anti-dopaminergic drugs, were present in this population, challenging the definition of causes of parkinsonism.

Brief considerations on the dispensation profile of the botulinum toxin type A by the Brazilian Unified Health System for treatment of dystonias: Datasus data.

Botulinum toxin injections are the most effective approach for the treatment of focal dystonia. Despite growing demand and clinical indications over the years, there are few reports or publications of its use and benefit to patients seen at the Sistema Único de Saúde - SUS (Unified Health System). Analyzing the Datasus data (Unified Health System Information Department of Brazilian Ministry of Health), it was noticed that in Brazil the percentage of dystonic patient benefited from this procedure is still low. We therefore suggest some strategies to increase the dispensation of the toxin by the Brazilian Unified Health system for the dystonic patients.

Brain SPECT in Sydenham's chorea in remission.

BACKGROUND: Sydenham's chorea, a major manifestation of rheumatic fever, is characterized by chorea, behavioral changes, and cognitive dysfunction. Perfusion changes in the basal ganglia are the most frequent imaging findings observed in patients with Sydenham's chorea. METHODS: Twelve adult women with Sydenham's chorea in remission underwent brain single-photon emission computed tomography (SPECT). Their scans underwent a quantification process to evaluate the perfusion of Brodmann's areas of the frontal lobes and basal ganglia. The results were compared with the findings from a control group that was matched by age. RESULTS: A pattern of hyperperfusion in the left putamen was observed in the patient group (P = 0.02). No significant difference was observed in relation to other brain regions. CONCLUSIONS: The findings of brain SPECT suggest that perfusion abnormalities of the basal ganglia may persist even after the remission of abnormal movements in patients with Sydenham's chorea.

Síndrome de opsoclonia-mioclonia-ataxia em paciente com AIDS / Opsoclonus-myoclonus-ataxia syndrome in an AIDS patient

É relatado aqui o caso de uma mulher de 38 anos com AIDS que desenvolveu a síndrome de opsoclonia-mioclonia-ataxia em um período diferente dos outros casos já relatados na literatura. A síndrome de opsoclonia-mioclonia-ataxia já tinha sido relatada como manifestação inicial de AIDS, assim como no momento da soroconversão de HIV e na síndrome de reconstituição imune. Este caso é único, uma vez que a paciente tinha contagem elevada de CD4 e carga viral negativa no momento em que a síndrome de opsoclonia-mioclonia-ataxia ocorreu.

We report the case of a 38-year-old woman with AIDS who developed opsoclonus-myoclonus-ataxia syndrome during a period different from other cases reported in literature. Opsoclonus-myoclonus-ataxia syndrome had already been reported as the initial neurological presentation of AIDS, as well as at the time of HIV-seroconversion and immune reconstitution syndrome. Our case is unique since the patient had an elevated CD4 count and negative viral load in the period when the opsoclonus-myoclonus-ataxia syndrome occurred.

Prevalence and clinical classification of tremor in elderly--a community-based survey in Brazil.

Prevalence data on different types of tremor among the elderly population are very scarce. The objective of this study was to study the prevalence of tremor in a community-dwelling elderly population in the town of Bambuí, Brazil. The authors studied 1186 inhabitants aged≥64 years. This was a 2-phase study in which all participants who screened positive in a questionnaire for tremor and parkinsonism or who used drugs capable of causing/suppressing tremor were examined. In this population, the prevalence rate was 17.4% for tremor, 7.4% for essential tremor, 5.6% for parkinsonian tremor, 2.8% for enhanced physiological tremor, and 1.6% for other causes. There were no gender differences in prevalence rates for all types. Patients who had Parkinson's disease with tremor were older than those who had essential tremor, whereas patients who had enhanced physiological tremor were significantly younger. The age-specific prevalence of tremor increased with advancing age for both men and women. The prevalence of tremor in the studied population was high and increased with advancing age. Essential tremor, parkinsonian tremor, and enhanced physiological tremor were the most commonly identified causes. © 2013 Movement Disorder Society.

Opsoclonus-myoclonus-ataxia syndrome in an AIDS patient.

We report the case of a 38-year-old woman with AIDS who developed opsoclonus-myoclonus-ataxia syndrome during a period different from other cases reported in literature. Opsoclonus-myoclonus-ataxia syndrome had already been reported as the initial neurological presentation of AIDS, as well as at the time of HIV-seroconversion and immune reconstitution syndrome. Our case is unique since the patient had an elevated CD4 count and negative viral load in the period when the opsoclonus-myoclonus-ataxia syndrome occurred.

Psychosis following acute Sydenham's chorea.

Sydenham's chorea (SC) is characterized by motor, mainly choreic involuntary movements, and psychiatric symptoms, including anxiety, depression, obsessive-compulsive and attention-deficit/hyperactivity disorders. Symptoms of SC may be considered as the result of basal ganglia dysfunction determined by autoimmune mechanisms elicited by streptococcal infection. We report on a case of a 13-year-old boy with brief psychotic episode following acute SC. His hallucinations and delusions remitted after treatment with risperidone, paralleling motor improvement. The neurobiological basis of psychotic symptoms remains largely elusive. This case report provides further insight about the involvement of basal ganglia dysfunction on psychosis.

[The chorea of Zezé]. / A coréia do Zezé.

The authors analyse the correspondence between Eça de Queiroz, a well-known Portuguese writer, and his wife, in 1900, describing the neurological disease of their second son, José Maria d'Eça de Queiroz, know as Zezé. In those letters, the writer mentions chorea as his son diagnosis, explaining in detail its clinical picture. In addition, the writer reveals the medical thoughts of the time about the aetiology, treatment and natural history of Sydenham's Chorea.

Dopamine dysregulation syndrome in Parkinson's disease: case report / Síndrome de desregulação dopaminérgica na doença de Parkinson: relato de caso

We report a 67-year-old man with Parkinson's disease for 9 years who developed compulsive use of levodopa. This phenomenon is the main feature of the dopamine dysregulation syndrome. Other related symptoms presented by our patient were mood fluctuation and increased writing activity suggestive of punding.

Relatamos sobre um homem de 67 anos de idade com doença de Parkinson por 9 anos e que desenvolveu uso compulsivo de levodopa. Esse fenômeno é a principal característica da síndrome de desregulação dopaminérgica. Outros sintomas apresentados pelo paciente foram flutuações do humor e atividade de escrita aumentada, comportamento este sugestivo de punding.

Sydenham's chorea is associated with decreased verbal fluency.

BACKGROUND: Sydenham's chorea (SC) is a disorder associated with rheumatic fever and related to Streptococcus-induced immune reaction cross-reactive with basal ganglia antigens. Obsessive-compulsive disorder and other behavioral abnormalities have been described in SC. There are scarce data of cognitive function in this condition. OBJECTIVE: To assess verbal fluency in patients with SC. PATIENTS AND METHODS: We have compared the semantic (SVF) and phonemic verbal fluency (PVF) of 20 subjects with SC and 40 controls matched by age, gender and years of school. RESULTS: The scores of the control group on the SVF and PVF tests were 26.3 +/- 5.7 and 18.0 +/- 6.4 whereas the SC patients scored 25.1 +/- 6.9 and 12.5 +/- 8.2. PVF was significantly decreased in the SC group (p < 0.01). CONCLUSION: SC is associated with reduction of PVF. This finding may reflect disruption of the dorsolateral prefrontal-striatal circuit caused by the auto-immune process characteristic of SC.

Parkinsonism and Parkinson's disease in the elderly: a community-based survey in Brazil (the Bambuí study).

Several community-based surveys on the prevalence of Parkinsonism and Parkinson's disease have been conducted worldwide, with variations on their methodology and results. The objective of this study is to assess the prevalence of Parkinsonism and its causes in a cohort of individuals age 64 years or older in Bambuí, a Brazilian town. In phase I, 1,186 people older than 64 years responded to a 9-question screening questionnaire for Parkinsonism. In phase II, all subjects who scored > or = 2 points on the test were examined independently by at least 2 movement disorder-trained physicians. A movement disorder senior specialist excluded or confirmed the diagnosis in all suspected cases. The response rate was high for both phases (96% for phase I and 94% for phase II). The prevalence rate per 100 population over 64 years of age in this group was 7.2% for Parkinsonism of all types (n = 86). The most frequent causes were idiopathic Parkinson's disease and drug-induced Parkinsonism, with prevalence rates of 3.3% (n = 39) and 2.7% (n = 32), respectively. The prevalence of vascular Parkinsonism was 1.1% (n = 13). We found 1 case of posttraumatic Parkinsonism and another with multiple system atrophy. In this first population-based study of Parkinsonism conducted in Brazil, the prevalence in a cohort of elderly subjects was higher than the observed in other populations worldwide, especially because of the high rates of drug-induced and vascular Parkinsonism. The prevalence of Parkinson's disease was similar to that observed in elderly people in door-to-door surveys in other American, European, and Eastern countries.

Dopamine dysregulation syndrome in Parkinson's disease: case report.

We report a 67-year-old man with Parkinson's disease for 9 years who developed compulsive use of levodopa. This phenomenon is the main feature of the dopamine dysregulation syndrome. Other related symptoms presented by our patient were mood fluctuation and increased writing activity suggestive of punding.

[The initial testing and the discrimination property of the UFMG Sydenham's Chorea Rating Scale (USCRS)]. / Testagem inicial e capacidade discriminatória da escala "UFMG Sydenham's Chorea Rating Scale (USCRS)".

Recently we developed and validated the Universidade Federal de Minas Gerais (UFMG) Sydenham's chorea Rating Scale (USCRS) to systematically assess SC patients. In this study, we assessed 97 children and adults with SC (mean age +/- SD, 15.5 +/- 5.9; male/female, 31/66) seen at the Movement Disorders Clinic at UFMG employing the USCRS. The patients were divided into 4 groups according to their clinical status: acute (n=19), recurrent (n=17), persistent (n=19) and remission (n=42). The mean +/- SEM USCRS scores for each group were: 47.7 +/- 4.7 for acute group, 29.5 +/- 2.6 for recurrent group, 17.6 +/- 3.1 for persistent group and 1.1 +/- 0.2 for remission group. All pair comparisons were statistically significant (p<0.05). Our results indicate that the USRSC can reasonably discriminate groups of SC patients in different clinical stages of the disease.