A child with recurrent episodes of involuntary movements.
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Adolescent↗
Biomedical subjects
F Cardoso
Publications and source records attributed to F Cardoso.
Persistent Sydenham's chorea.
BACKGROUND: Sydenham's chorea (SC) occurs in 26% of patients with rheumatic fever (RF). Despite usually being described as a self-limited condition, few reports indicate that SC may persist in rare subjects. OBJECTIVE: To investigate the proportion of subjects with SC lasting more than 2 years and if clinical features differentiate patients with SC with a duration of less than 2 years (Group 1) from those with SC lasting more than 2 years (Group 2). METHODS: Prospective assessment of all patients with SC seen at our service from July 1993 through March 1998 analyzing the following: gender; age at onset; frequency of arthritis, carditis, family history of RF and SC; topographic distribution; and chorea severity on a 0-4 scale. RESULTS: Thirty-two patients (19 female, 13 male) were studied. In Group 1 (16 subjects, 50%) the follow-up period was 36.2 +/- 20.0 months; 50% were female; age at onset was 10.9 +/- 2.6 years; arthritis and carditis were present in 37.5% and 31.2%, respectively; family history of SC was reported by 18.7%; hemichorea was seen in 25.8% of subjects; and the mean intensity of chorea was 2.6 +/- 0.8. In Group 2, with a follow-up period of 34.1 +/- 18.9 months, 68.8% were female; age at onset was 9.3 +/- 3.9 years; arthritis and carditis were diagnosed in 18.7% and 50%, respectively; no patient reported a family history of SC; hemichorea was observed in 6.2% of subjects; and the mean intensity of chorea was 2.8 +/- 0.5. No difference was statistically significant. CONCLUSIONS: SC persists in half of our patients. Female gender, possibly related to endocrine factors, as well as the presence of carditis, indicating a more severe disease, may be risk factors for a longer duration of SC.
Acute Disease↗
Hemifacial spasm and arterial hypertension.
BACKGROUND: Hemifacial spasm (HFS) is a form of segmental myoclonus often associated with vascular compression of the seventh nerve at its exit in the brain stem. Although a few uncontrolled studies describe the association of arterial hypertension (AH) with HFS, their relationship remains to be elucidated. OBJECTIVES: To compare the clinical and radiologic features of patients with HFS and subjects with blepharospasm (BLS) with special emphasis on the presence of AH. MATERIAL AND METHODS: Chart review of all patients with HFS and BLS seen at a hospital-based movement disorders clinic from July 1993 through March 1998, analyzing the following parameters: gender, age at onset, frequency of AH, and abnormalities on computerized tomography (CT) or magnetic resonance imaging (MRI) studies. Neuroimaging studies (52 CT and 14 MRI) were performed in 45 subjects with HFS and in 21 with BFS. All patients were examined by one of the authors (FC). RESULTS: HFS and BLS were diagnosed in 48 (31 women, 17 men) and 34 (28 women, 6 men) patients, respectively. The age at onset was 50.1 +/- 12.6 years in HFS and 50.3 +/- 16.5 years in BLS. AH was diagnosed in 32 (66.7%) subjects with HFS and in 13 (38.2%) patients with BLS (chi-square p = 0.01). Neuroimaging evidence of vascular tortuosity in the cerebello-pontine angle was identified in 16 (25.4%) patients, all of whom had HFS (38.1%). Thirteen of 37 patients with AH who had imaging studies displayed vascular tortuosity in the cerebello-pontine angle (chi-square p = 0.01). CONCLUSIONS: AH is significantly more common in HFS than in BLS. AH is related to vascular tortuosity in the cerebello-pontine angle. Our findings suggest that AH may be an important risk factor for HFS.
Blepharospasm↗
Tourette syndrome: another cause of movement disorder of the ear.
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Adolescent↗
An akinetic-rigid syndrome, depression, and stereotypies in a young man.
A young man is presented who developed an akinetic-rigid syndrome shortly after a minor illness. Rituals and stereoptypies were prominent. At its most severe he was unable to feed himself. There was no response to L-dopa/cardopa treatment. A course of ECT was followed by a marked improvement in his condition. Attempts to stop ECT for more than a week have led to recurrence of his bradykinesia.
Adult↗
Association of circulating endothelium and noradrenaline with increased calcium-channel binding sites in the placental bed in pre-eclampsia.
OBJECTIVE: To evaluate factors contributing to both placental hypoperfusion and maternal vasoconstriction in pre-eclampsia. DESIGN: Single centre, comparative study of calcium-channel density and affinity in the placental bed of pregnant women with normotension and pre-eclampsia. SETTING: Teaching hospital. PARTICIPANTS: Twenty-two primigravidae in the third trimester of pregnancy: 10 with pre-eclampsia and 12 normotensive. METHODS: Plasma levels of endothelin-1 (by RIA) and noradrenaline (by HPLC-ED) were measured. Both pharmacological characterisation and anatomical localisation of dihydropyridine-sensitive binding sites (using radioligand-binding studies and autorradiographic techniques) were determined with 3H-isradipine in placental bed tissues to determine both the density (Bmax) and the affinity (Kd) of receptor sites. RESULTS: Higher plasma levels of endothelin-1 and noradrenalin were found in women with pre-eclampsia compared with normotensive women. Placental bed tissues bound 3H-isradipine in a saturable, reversible time and temperature-dependent manner with very low Kd values. Study of the 3H-isradipine specificity binding included the use of several dihydropyridine displacers. In the group with pre-eclampsia the Scatchard analysis of the results showed a significant increase (P < 0.001) both in the affinity [Kd = 0.23 nmol (0.04) vs 0.45 nmol (0.03), pre-eclampsia vs normotensive] and in the density of calcium-channel binding sites [Bmax = 77.70 fmol/mg (1.30) vs 64.30 fmol/mg (1 80) tissue, pre-eclampsia vs normotensive]. Autoradiography confirmed that in the placental bed tissue of those with pre-eclampsia there was a much higher silver grain density in the arteries walls, compared with normotensive women. CONCLUSIONS: In pre-eclampsia there is an increase in the maternal circulation of two strong vasoconstrictor factors (endothelin-1 and noradrenalin) and a sharp increase both in the density and the affinity of calcium-channel binding sites in placental bed central area. The latter may strongly contribute to the perpetuation of the uteroplacental hypoperfusion either by itself or by amplifying the local actions of circulating factors, such as endothelin-1 and noradrenalin.
Adult↗
Etiology of parkinsonism in a Brazilian movement disorders clinic.
OBJECTIVE: The aim of the present study is to investigate whether there are geographic differences in the etiology of parkinsonism (PA). BACKGROUND: 72% of patients with PA evaluated at movement disorders clinics in the Northern Hemisphere are diagnosed with Parkinson's disease (PD). Data regarding other regions are not available. METHODS: We reviewed the charts of all patients with PA seen at the Federal University of Minas Gerais Movement Disorders Clinic from July 1993 through October 1995. PA was diagnosed by the presence of at least two of the following: rest tremor, bradykinesia, rigidity, and postural instability. The different etiologies were diagnosed based on standard clinical criteria. RESULTS: During the period of the study, PA was recognized in 338 subjects. The following clinical diagnoses were made: PD (68.9%), drug-induced PA (DIP) (13.3%), vascular PA (4.7%), Progressive supranuclear palsy (PSP) (2%), multiple system atrophy (MSA) (1.8%), others (9.7%). Cinnarizine, haloperidol and flunarizine were the commonest drugs related to DIP. CONCLUSIONS: Similarly to other studies, PD accounts for about 70% of PA patients. However, there are differences between our results and previous series. DIP is much more common in the present series. This may be accounted for a more liberal use of antidopaminergic drugs in our environment, especially Calcium channel blockers. The lower frequency of MSA and PSP in our study may reflect a short follow-up, since many patients initially diagnosed with PD later are found to have Parkinson-plus syndromes.
Aged↗
Chorea in fifty consecutive patients with rheumatic fever.
Rheumatic fever (RF) remains a significant public health problem in developing countries. Although its incidence has been declining in developed areas, several outbreaks of RF have been identified in the United States since 1985. To study the prevalence and clinical features of Sydenham's chorea (SC), a well-recognized expression of RF, we examined 50 consecutive patients with RF seen at our Pediatric Cardiology Clinic. Chorea was rated on a scale of 0-4 (0 = no chorea; and 4 = severe chorea, patient unable to walk). Chorea, active or in remission, occurred in 13 patients (26%). The male-to-female ratio was 1.08 among the total population and 0.86 among the SC group. The mean age of the 50 patients at onset of RF was 8.4 years and the standard deviation (SD) 3.1 years. Carditis was diagnosed in 84% of patients with and without chorea, but arthritis was more common in the latter group (83.8%) than in the SC group (30.8%). The mean age at onset of SC was 9.2 years (SD 2.7). Involuntary movement, noticed by nine patients, was the most common initial symptom. Chorea went into remission in eight patients after 9.9 months (SD 13.4). Chorea was rated 3 in ten patients, 1 in two patients, and 4 in one patient. Among the five patients with active SC, three had hemichorea and two had generalized chorea. We recognized abnormal saccades in 80% of patients with active chorea, whereas just 13.5% of patients without chorea displayed this abnormality. SC remains an important cause of chorea in countries plagued by RF.
Age Distribution↗
Low-dose oral contraceptives and 24-hour ambulatory blood pressure.
OBJECTIVE: To evaluate in normotensive women the influence of low-dose oral contraceptives (OC, monophasic formulations containing 30 microg of estrogen) on 24-h blood pressure. METHODS: We evaluated prospectively in 15 normotensive healthy women (three smokers) the influence of OC on 24-h ambulatory blood pressure monitoring (ABPM). ABPM was performed (SpaceLabs 90207) before and after 6-9 months of use of OC. We also evaluated ABPM in eight women (two smokers) before and after 6-8 months on an intrauterine device (IUD) as contraceptive method--these were used as control subjects. RESULTS: OC produced a significant increase in 24-h ABPM values (from 120+/-3/75+/-2 to 128+/-4/81+/-2 mmHg, P < 0.04) which was particularly evident for night-time values (from 108+/-2/64+/-2 to 120+/-4/73+/-2 mmHg, P < 0.02). After OC, two normotensive women developed 'hypertensive values'. In OC users there was a slight but significant increase in body weight which did not correlate with the increase of blood pressure. In contrast, in the control group (IUD) neither ABPM values nor weight were modified by the contraceptive maneuver. CONCLUSIONS: In normotensive women, low-dose OC may increase blood pressure to an extent that, at least in some women, may affect blood pressure control towards 'hypertensive values'. This stresses the importance of monitoring blood pressure values during OC treatment.
Adult↗
Dystonia and dyskinesia.
Dystonia is defined as a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements, or abnormal postures. Tardive dyskinesia (TD), estimated to occur in 30% of patients treated with neuroleptics, encompasses a broad spectrum of hyperkinesias associated with exposure to these drugs. Dystonia is one of the most common expressions of tardive dyskinesia. This article aims to provide an overview of classification, phenomenology, epidemiology, genetics, pathophysiology, neuropsychologic aspects, and treatment of these two conditions.
Antipsychotic Agents↗
Frequency of the different mutations causing spinocerebellar ataxia (SCA1, SCA2, MJD/SCA3 and DRPLA) in a large group of Brazilian patients.
Spinocerebellar ataxia type 1 (SCA1), spinocerebellar ataxia type 2 (SCA2) and Machado-Joseph disease or spinocerebellar ataxia type 3 (MJD/SCA3) are three distinctive forms of autosomal dominant spinocerebellar ataxia (SCA) caused by expansions of an unstable CAG repeat localized in the coding region of the causative genes. Another related disease, dentatorubropallidoluysian atrophy (DRPLA) is also caused by an unstable triplet repeat and can present as SCA in late onset patients. We investigated the frequency of the SCA1, SCA2, MJD/SCA3 and DRPLA mutations in 328 Brazilian patients with SCA, belonging to 90 unrelated families with various patterns of inheritance and originating in different geographic regions of Brazil. We found mutations in 35 families (39%), 32 of them with a clear autosomal dominant inheritance. The frequency of the SCA1 mutation was 3% of all patients; and 6% in the dominantly inherited SCAs. We identified the SCA2 mutation in 6% of all families and in 9% of the families with autosomal dominant inheritance. The MJD/SCA3 mutation was detected in 30% of all patients; and in the 44% of the dominantly inherited cases. We found no DRPLA mutation. In addition, we observed variability in the frequency of the different mutations according to geographic origin of the patients, which is probably related to the distinct colonization of different parts of Brazil. These results suggest that SCA may be occasionally caused by the SCA1 and SCA2 mutations in the Brazilian population, and that the MJD/SCA3 mutation is the most common cause of dominantly inherited SCA in Brazil.
Adolescent↗
A Brazilian cohort of patients with Tourette's syndrome.
The clinical features of 32 patients (24 males) with Tourette's syndrome in Brazil were studied. The mean age at onset was 7.1 years, tics being the first symptom in 71% and hyperactivity in 29%. Blinking, grimacing, and shoulder elevation were the most common motor tics and sniffing, throat clearing, and grunting noises, the most frequent vocal tics. Coprolalia was present in 28%, echolalia in 16%, palilalia in 9%, and copropraxia in 25% of patients. Attention deficit and hyperactivity disorder was diagnosed in 63%, and obsessive compulsive behaviour in 44% of patients. In 84% of patients there was a family history of tics whereas attention deficit and hyperactivity disorder and obsessive compulsive behaviour were respectively present in relatives of 19% and 53% of the patients studied. These data suggest that Tourette's syndrome in Brazil is not clinically different from other countries, supporting the notion that genetic factors play the most important part in its aetiology.
Adolescent↗
Persistent dyskinesia and obsessive-compulsive behavior following alcohol withdrawal.
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Adult↗
Independent effect of maternal birth weight on infant birth weight.
The relationship between the birth weights of the mother and her infant was evaluated in a sample of 106 women consecutively delivered of a single live birth. Women were included in the study if their birth weight was available in hospital files or from another reliable source. Women that were themselves a twin or had diseases complicated the course of the pregnancy were excluded. A positive significant correlation was found between mother and infant birth weights (r = 0.29, p = 0.003). This correlation remained significant (partial r = 0.22, p = 0.039) after adjusting for other covariables such as the mother's age, height, education, whether the pregnancy was planned, number of cigarettes smoked daily during pregnancy, month of first antenatal visit and number of visits, weight at end-pregnancy, gestational age and sex of the newborn. In the multiple linear regression model, mother's birth weight explained 3% of the variation observed in infant birth-weight. A weaker crude correlation was also found between mothers' birth weight and gestational age (r = 0.19, p = 0.06). This study shows that maternal birth weight is an independent predictor of infant birth weight, and confirms previous findings suggesting that this maternal factor has a stronger effect on the birth weight than on the gestational age of the newborn.
Birth Weight↗
Peripherally induced tremor and parkinsonism.
OBJECTIVE: Trauma to the peripheral nervous system is a well-recognized cause of dystonia and tremor, but peripherally induced parkinsonism has not previously been documented. We seek to characterize peripherally induced tremor and parkinsonism and propose possible mechanisms for this phenomenon. DESIGN: Review of records of patients evaluated in the Movement Disorders Clinic between 1977 and 1993. In addition to demographic and clinical information, the records were screened for any potential predisposing factors. PATIENTS: Twenty-eight patients in whom the onset of tremor, parkinsonism, or both was anatomically and temporally related to local injury. INTERVENTION: The type and site of injury were verified by history and examination of records whenever possible. Severity of tremor and parkinsonism was assessed by clinical rating scales. Three patients with tremor and parkinsonism had their striatal [18F]-fluorodopa uptake and raclopride binding measured with positron emission tomography. MAIN OUTCOME MEASURE: Response to conventional antitremor and antiparkinsonian medication was assessed by a clinical rating scale. RESULTS: Severe local injury preceded the onset of movement disorder by 47.5 +/- 74.7 days (mean +/- SD). The mean age at onset of movement disorder was 46.5 +/- 14.1 years. Tremor was present in all 28 patients, 11 of whom exhibited additional parkinsonian features. In 20 patients, the movement disorder spread beyond the original site. Possible predisposing factors were identified in 13 patients; nine had essential tremor or a family history of essential tremor. In addition to tremor, dystonia and myoclonus were evident in 13 and three patients, respectively. Reflex sympathetic dystrophy was present in six patients. Tremor did not improve with medications, and only seven patients with parkinsonism responded to therapy with levodopa. CONCLUSION: Central reorganization in response to peripheral injury may give rise to a motor disturbance, including tremor and parkinsonism.
Adult↗
Clinical use of botulinum neurotoxins.
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Animals↗
Clozapine for acute and maintenance treatment of psychosis in Parkinson's disease.
In a prospective, open-label study, 12 patients manifesting psychosis associated with Parkinson's disease were treated with clozapine. Cognitive functioning and type of psychotic symptoms were measured prior to treatment, and changes in psychiatric and behavioral symptoms were studied by using the Behave-AD Scale. Significant resolution in psychotic symptoms was found and improvement in global behavioral status observed in all cases, with 10 patients maintaining improvement at follow-up. Careful initiation and titration of the drug resulted in few side effects, and dementia was not found to be a contraindication to such treatment.
Aged↗
Outcome after stereotactic thalamotomy for dystonia and hemiballismus.
The outcome after single or staged stereotactic thalamotomies in 17 patients with dystonia and 2 patients with hemiballismus is reviewed. All patients were severely disabled by their movement disorders despite optimal pharmacological therapies. Eight of the patients with dystonia (47%) showed moderate improvement immediately after the procedures. Six of these eight patients maintained their improvement, and two other patients with dystonia improved significantly, during the follow-up period (mean, 37.6 mo). The long-term outcome was better in patients with secondary dystonia (50% moderately or markedly improved at a mean of 41.0 mo) than in patients with primary dystonia (43% moderately or markedly improved at a mean of 32.9 mo). Excellent control was achieved in both of the patients who underwent thalamotomies for hemiballismus.
Adolescent↗