Débora Palma Maia

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 Parkinsons 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 ≥ 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 Parkinsons 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 Parkinsons disease was similar to that observed in elderly people in door‐to‐door surveys in other American, European, and Eastern countries.

Obsessive compulsive behavior, hyperactivity, and attention deficit disorder in Sydenham chorea.

The authors investigated obsessive-compulsive behavior, obsessive-compulsive disorder (OCD), and attention deficit and hyperactivity disorder (ADHD) in 50 healthy subjects, 50 patients with rheumatic fever without chorea, and 56 patients with Sydenham chorea. Obsessive-compulsive behavior, OCD, and ADHD were more frequent in the Sydenham chorea group (19%, 23.2%, 30.4%) than in the healthy subjects (11%, 4%, 8%) and in the rheumatic fever without chorea group (14%, 6%, 8%). ADHD was more common in persistent Sydenham chorea.

Treatment of Sydenham chorea with corticosteroids

Despite treatment with valproic acid and neuroleptics, a significant proportion of patients with Sydenham chorea (SC) remain with chorea. We evaluated the effect of intravenous methyl‐prednisolone followed by oral prednisone in patients with SC refractory to conventional treatment. Patients were enrolled in the study if they failed to improve with conventional treatment, despite the development of side effects. Chorea was rated on a 0 to 4 score. Five patients, 3 of them women, were included in the study. The median pretreatment rating score of the chorea was 3 (range, 3–4) and dropped to 1 (range, 0–2) after a median follow‐up of 7 months (range, 3–7 months). Two patients developed Cushing syndrome. Our data suggest that intravenous methyl‐prednisolone followed by oral prednisone is an effective and well‐tolerated treatment of refractory SC.

UFMG Sydenham's chorea rating scale (USCRS): Reliability and consistency

Despite the renewed interest in Sydenhams chorea (SC) in recent years, there were no valid and reliable scales to rate the several signs and symptoms of patients with SC and related disorders. The Universidade Federal de Minas Gerais (UFMG) Sydenhams Chorea Rating Scale (USCRS) was designed to provide a detailed quantitative description of the performance of activities of daily living, behavioral abnormalities, and motor function of subjects with SC. The scale comprises 27 items and each one is scored from 0 (no symptom or sign) to 4 (severe disability or finding). Data from 84 subjects, aged 4.9 to 33.6 years, support the interrater reliability and internal consistency of the scale. The USCRS is a promising instrument for rating the clinical features of SC as well as their functional impact in children and adults.

Sydenham’s chorea may be a risk factor for drug induced parkinsonism

Sydenham’s chorea, the most common cause of acquired chorea in childhood, is a delayed complication of group A β-haemolytic streptococcal infection.1 It is thought to be caused by antibodies induced by streptococci which cross react with basal ganglia antigens.2 Despite the decrease in Sydenham’s chorea in developed countries, there is a renewed interest in this condition because of the hypothesis that a similar mechanism may play a role in the pathogenesis of a subset of patients with tics and other neuropsychiatric disorders.3 The treatment of Sydenham’s chorea is based on the combination of penicillin and antichoreic drugs (valproic acid and/or dopamine antagonists). At the movement disorders clinic of the Federal University of Minas Gerais (MDC-UFMG), located in an area where Sydenham’s chorea remains endemic, we have been struck by the occurrence of drug induced parkinsonism among patients with Sydenham’s chorea. We therefore decided to investigate the incidence of this complication in a cohort of patients with the condition, as well as in a group of subjects with Tourette’s syndrome. The latter is an interesting choice for a control group as Tourette’s syndrome and Sydenham’s chorea share several clinical features—for example, childhood onset, the constellation of motor and behavioural disturbances, and the response to neuroleptic agents.4 In the first part of the study we undertook a retrospective …

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.

Migraine headache in patients with Sydenham's chorea

We compared the frequency of migraine among Sydenhams chorea (SC) patients, rheumatic fever (RF) patients without neurological symptoms and matched controls. Migraine was more frequent in SC patients (12/55, 21.8%) than in controls (9/110, 8.1%) and as common as in the RF group (10/55, 18.2%). Our data are in agreement with previous studies reporting higher frequency of migraine in other basal ganglia disorders, such as essential tremor and Tourettes syndrome.

Dopamine dysregulation syndrome in Parkinson's disease: case report

We report a 67-year-old man with Parkinsons 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.

Pregnancy in patients with Sydenham’s Chorea

BACKGROUND Sydenhams Chorea is a frequent cause of chorea during pregnancy, chorea gravidarum. The aim of this article is to describe the effect of pregnancy in a consecutive series of patients with diagnosis of Sydenhams Chorea. METHODS A chart review was performed of all patients with the diagnosis of Sydenhams Chorea followed up at our institution from 07/1993 through 08/2010 and who became pregnant. RESULTS From 66 patients, 20 became pregnant. Of these 20 patients, 15 (75%) developed chorea gravidarum. Generalized chorea was found in 67% of these 15 patients, focal or multifocal chorea was identified in 20% and 13.4% developed hemichorea. In 80% of cases chorea began in the first 6 months of gestation. Three women with previous persistent chorea experienced worsening of the movement disorder during pregnancy. Remission occurred after delivery in 11 patients whereas the other four remained with non-disabling chorea during the first 12 months after delivery. Abortion occurred in two patients (13%). All patients with chorea gravidarum subsequently treated with oral contraceptives developed recurrence of chorea. CONCLUSIONS Chorea gravidarum is a frequent complication of pregnancy in patients with previous history of Sydenhams Chorea and an increased risk of miscarriage should be considered. Our findings confirm the notion that chorea gravidarum results from hormonal changes acting on previously dysfunctional basal ganglia.

Sensory tricks in focal dystonia and hemifacial spasm

Sensory tricks are sensory, often but not exclusively tactile, stimuli usually in the body part affected by the movement disorder that produce a meaningful alleviation of dystonia. The frequency and clinical features of sensory tricks in different types of dystonia are poorly studied in the literature. There is no information regarding the presence of a similar phenomenon in HFS.