Neurologic complications of rheumatic fever.

Sydenham chorea, also known as St. Vitus dance, is a major clinical criterion for the diagnosis of acute rheumatic fever. Clinically, it results in a combination of movement disorders and complex neuropsychiatric symptoms. Cardiac damage due to rheumatic fever may also predispose to neurologic complications later in life. Rheumatic heart disease (RHD) is associated with heart remodeling, cardiac arrhythmias, and ischemic stroke. Furthermore, chronically damaged heart valves are predisposed to infection. Septic brain embolism, a known complication of infective endocarditis, may result in brain ischemia, hemorrhage, and spread of the infection to the brain.

The "sick dancers": The construction of medical knowledge about the "epidemic of dance" in Itapagipe, Salvador, Bahia (1882-1901).

The goal of this paper is to analyze a little-known set of documents referring to a "Dancing Epidemic" that took place in Itapagipe, a suburb of Salvador, capital of the province of Bahia, Brazil, in 1882. Through the studies of a group of physicians, especially Raimundo Nina Rodrigues (1862-1906), a psychiatrist and anthropologist from the Bahia School of Medicine, the medical knowledge built on this unique phenomenon in Brazilian history is examined. The case in particular involved a crowd that spread through the streets of Itapagipe, attracting the interest of the medical classes, who were intrigued by the symptoms of motor incoordination the patients manifested. Inspired by foreign literature, but developing their own theories, Rodrigues and colleagues created a unique body of knowledge about the infirmity.

Huntington disease and Huntington disease-like in a case series from Brazil.

The aim of this study was to identify the relative frequency of Huntington's disease (HD) and HD-like (HDL) disorders HDL1, HDL2, spinocerebellar ataxia type 2 (SCA2), SCA17, dentatorubral-pallidoluysian degeneration (DRPLA), benign hereditary chorea, neuroferritinopathy and chorea-acanthocytosis (CHAC), in a series of Brazilian families. Patients were recruited in seven centers if they or their relatives presented at least chorea, besides other findings. Molecular studies of HTT, ATXN2, TBP, ATN1, JPH3, FTL, NKX2-1/TITF1 and VPS13A genes were performed. A total of 104 families were ascertained from 2001 to 2012: 71 families from South, 25 from Southeast and 8 from Northeast Brazil. There were 93 HD, 4 HDL2 and 1 SCA2 families. Eleven of 104 index cases did not have a family history: 10 with HD. Clinical characteristics were similar between HD and non-HD cases. In HD, the median expanded (CAG)n (range) was 44 (40-81) units; R(2) between expanded HTT and age-at-onset (AO) was 0.55 (p=0.0001, Pearson). HDL2 was found in Rio de Janeiro (2 of 9 families) and Rio Grande do Sul states (2 of 68 families). We detected HD in 89.4%, HDL2 in 3.8% and SCA2 in 1% of 104 Brazilian families. There were no cases of HDL1, SCA17, DRPLA, neuroferritinopathy, benign hereditary chorea or CHAC. Only six families (5.8%) remained without diagnosis.

PRRT2 mutation causes paroxysmal kinesigenic dyskinesia and hemiplegic migraine in monozygotic twins.

PRRT2 gene mutations have recently been identified as a causative gene of Paroxysmal kinesigenic dyskinesia (PKD), a rare movement disorder characterised by the occurrence of chorea, dystonia or athetosis triggered by sudden action. Some patients have additional intermittent neurologic disorders like infantile convulsions. The association with migraine has been rarely reported in this condition. Here we report the coexistence of PKD and hemiplegic migraine in twins harbouring a heterozygous mutation in PRRT2. Two monozygotic twins manifesting PKD together with repeated episodes of migraine with some severe attacks of hemiplegic migraine have been followed and treated for more than 10 years. Molecular genetic analysis disclosed the c.649_650insC, p.R217Pfs*8 heterozygous mutation in both twins. This mutation was segregating from the mother who likewise harboured the same mutation c.649dupC although she had never manifested PKD but complained of rare common migraine attacks in her past history. The association of PKD and hemiplegic migraine has been previously reported in one large family, associated to febrile convulsions and afebrile seizures in some individuals, but our report relates this association of symptoms to a mutation in PRRT2. The co-occurrence of both hemiplegic migraine and PKD in monozygotic twins expands the phenotypic spectrum of intermittent manifestations related to PRRT2 and perhaps suggests an additional causing gene for hemiplegic migraine.

Chorea in primary antiphospholipid syndrome is associated with rheumatic fever.

The aim of the study is to evaluate the frequency of chorea in a cohort of primary antiphospholipid syndrome (PAPS) patients and their possible clinical and laboratory associations. The records of 88 PAPS patients, fulfilling Sapporo criteria, followed up at the rheumatology outpatient clinic, were analyzed in order to determine the frequency of chorea. Risk factors for chorea, clinical manifestations, associated comorbidities, serologic features and treatment strategies were analyzed. Eighty-eight PAPS patients were evaluated. Mean age was 40.6 ± 11.1 years, and 91% of them were Caucasian and 91% women. Four (4.5%) patients with chorea were identified: 2 of them (50%) had only one chorea episode and 2 (50%) had recurrent chorea. All patients had chorea onset before PAPS diagnosis. Mean age, gender and ethnical distribution were comparable in groups with or without seizures (P > 0.05). Interestingly, the comparison of the 4 PAPS patients with chorea with those without this abnormality (n = 84) demonstrated a lower BMI [21.1 (18-24.2) vs. 27.5 (17.5-40.9) kg/m(2), P = 0.049] and frequency of venous events (0 vs. 63.1%, P = 0.023) in the first group. A higher frequency of rheumatic fever (75% vs. 0, P < 0.001) and thrombocytopenia (75 vs. 21.4%, P = 0.041) was observed in PAPS individuals with chorea. Both groups were alike regarding the other clinical APS manifestations, disease duration, risk factors for cerebrovascular diseases, use of drugs and antiphospholipid antibodies (P > 0.05). This study demonstrated that 4.5% of PAPS patients had chorea, predominately before PAPS diagnosis, and this neurological abnormality was associated with rheumatic fever and thrombocytopenia. These data reinforce the need for RF diagnosis in those PAPS patients with chorea.

Sydenham's chorea.

Sydenham's chorea (SC) is the neurologic expression of acute rheumatic fever (ARF). Despite the declining incidence of ARF worldwide, it remains the most common cause of acute chorea in children. It is characterized by a combination of motor and nonmotor features. In addition to chorea, among the first are decreased muscle tone and tics. Nonmotor features include obsessions, compulsions, attention deficit, emotional lability, decreased verbal fluency, and executive dysfunction. Most patients present with nonneurologic features of ARF, such as carditis and arthritis. The pathogenesis is thought to involve streptococcus-induced antibodies which cross-react with antigens of the basal ganglia. The diagnosis is made on purely clinical grounds since there is no biological marker of the illness. The management is based on use of antichoreic agents, such as valproic acid and neuroleptics, and prophylaxis of new bouts of streptococcus infection with antibiotics. Although the motor features of SC come into spontaneous remission in the majority of patients, a significant proportion of individuals remain with persistent chorea.

Executive functioning in adult patients with Sydenham's chorea.

Sydenham's chorea (SC) is characterized by a combination of motor and behavioral findings. Cognitive function has been scarcely studied in this condition. The aim of this study is to investigate executive functions in adult patients with SC. We performed neuropsychological tests to evaluate executive functions in controls and adult patients with persistent and in remission SC. Patients with SC have impairment in Tower of London task, reduced verbal fluency, and lower scores in the Stroop test. A subset of adult patients with SC present with executive dysfunction, even when chorea is in remission.

Subtle cognitive deficits in adults with a previous history of Sydenham's chorea during childhood.

OBJECTIVE: To evaluate the neuropsychological profile and health-related quality of life (HRQOL) of adults who had rheumatic fever (RF) during childhood with and without Sydenham's chorea (SC). METHODS: Three groups of patients were assessed: adults who had RF with SC during childhood (SC group), adults who had RF without SC during childhood (RF group), and controls (CT group). A range of neuropsychological tests looked at several cognitive domains. HRQOL was measured through a Brazilian version of the Short Form 36 (SF-36) health survey. RESULTS: Twenty patients were included in the SC group, 23 patients in the RF group, and 19 patients in the CT group. The 3 groups were homogeneous regarding sex (P = 0.078), age (P = 0.799), schooling (P = 0.600), socioeconomic status (P = 0.138), intelligence quotient (P = 0.329), and scores for anxiety (P = 0.156) and depression (P = 0.076). The SC group demonstrated inferior performance in tests that assessed attention (Digit Span Forward [P = 0.005], Corsi Block Forward [P = 0.014]), speeded information processing (Trail Making A [P = 0.009], Symbol Search [P = 0.042]), and executive functions and working memory (Corsi Block Backward [P = 0.028]), and higher scores for attention deficit scale (P = 0.030) when compared with the RF and CT groups. They also showed a tendency toward lower scores in the physical aspects, vitality, emotional aspects, and mental health domains of the SF-36. The RF group had a lower score for the general health domain than the CT group (P = 0.030). CONCLUSION: Patients who had SC during childhood can exhibit inferior performance in tasks that evaluate attention, speeded information processing, executive functions, and working memory in adult life. Therefore, there is indirect evidence of the persistence of dysfunction in cerebral circuits involved with the basal ganglia. They also presented a worse self-evaluation in HRQOL that was not related to cognitive impairments.

Higher prevalence of obsessive-compulsive spectrum disorders in rheumatic fever.

OBJECTIVE: This study aims to compare the prevalence of obsessive-compulsive spectrum disorders (OCSD) in psychiatric outpatients with and without a history of rheumatic fever (RF). METHODS: An analytical cross-sectional study assessing a large sample of consecutive psychiatric outpatients at a Brazilian private practice was conducted during a 10-year period. Psychiatric diagnoses were made by a senior psychiatrist based on Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition. Best-estimate diagnosis procedure was also performed. RESULTS: The total sample comprised 678 subjects, 13 of whom (1.92%) presented with a previous history of RF. This group showed a higher prevalence of subclinical obsessive-compulsive disorder (P=.025) and OCSD (P=.007) when compared to individuals with no such history. CONCLUSIONS: A previous history of RF was associated with OCSD. These results suggest that clinicians should be encouraged to actively investigate obsessive-compulsive symptoms and related disorders in patients with a positive history of RF.

Sydenham's chorea: clinical observations from a Brazilian movement disorder clinic.

We retrospectively evaluated the clinical and epidemiological characteristics of 100 patients suffering from Sydenham's chorea (SC). Our analysis revealed a recent, progressive decline in the number of new cases. Onset of SC was frequently reported between 7 and 12 years of age, being more frequent in females. Patients with generalized or severe chorea showed a higher risk of presenting gait abnormalities and behavioral symptoms. Chorea was transitory and remitted within the first 6 months in about 50% of patients but was persistent in 40%. Almost all patients with persistent chorea remitted after a protracted course of the disease.

Migraine headache in patients with Sydenham's chorea.

We compared the frequency of migraine among Sydenham's 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 Tourette's syndrome.

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.

Obsessive-compulsive symptoms in adults with history of rheumatic fever, Sydenham's chorea and type I diabetes mellitus: preliminary results.

OBJECTIVE: Rheumatic fever (RF) associated with Sydenham's chorea (a neurological variant of RF), but not RF without chorea, has been acutely related to obsessive-compulsive symptomatology/disorder (OCS/OCD). This study investigated the presence of OCS in adults who had RF with or without chorea in childhood. METHOD: The Yale-Brown Obsessive-Compulsive Scale (Y-BOCS) was used to evaluate OCS in 38 adults with history of RF (13 with chorea; 25 without chorea) or diabetes (controls; n = 19). RESULTS: The OCS was similar in both groups, although the intensity of symptoms was not clinically relevant. Moreover, subjects with RF with or without chorea did not score differently in the Y-BOCS. CONCLUSION: The similar occurrence of OCS in patients with history of RF and diabetes suggests that the development of this symptomatology, triggered by group A beta-haemolytic streptococcus infections, is restricted to the RF acute phase, occurred during infancy, and did not seem to predispose the appearance of OCS in adulthood.

Obsessive-compulsive spectrum disorders in rheumatic fever with and without Sydenham's chorea.

BACKGROUND: Recent findings suggest that acute-phase rheumatic fever (RF) patients present with higher frequencies of obsessive-compulsive disorder (OCD) and tic disorders. Until now, there have been no such studies in RF in non-acute phases. OBJECTIVE: To verify whether patients with a history of RF with or without Sydenham's chorea (SC) present with higher rates of OCD, tic disorders, and other obsessive-compulsive (OC) spectrum disorders (such as body dysmorphic disorder [BDD]) than controls. METHOD: Between February 1999 and December 2002, 59 consecutive outpatients with non-acute RF (28 with and 31 without SC) from an RF clinic and 39 controls from an orthopedics clinic were blindly assessed for OC spectrum disorders using structured interviews to assign DSM-IV diagnosis. Data were analyzed with Fisher exact and chi(2) tests to compare frequencies of disorders, and Kaplan-Meier survival analyses were used to obtain age-corrected rates. RESULTS: The age-corrected rates of tic disorders were higher in patients with RF without SC (N = 3; 14.39%) (p =.003) when compared with controls. Age-corrected rates for OC spectrum disorders (OCD, tic disorders, and BDD) combined were higher both in RF without SC (N = 4; 20.65%) and RF with SC (N = 5; 19.55%) groups than in controls (N = 1; 2.56%) (p =.048). CONCLUSIONS: RF, even in the non-acute phase, may increase the risk for some OC spectrum disorders, such as OCD, tic disorders, and BDD. These data, although preliminary, reinforce the idea that OC spectrum disorders may share common underlying pathophysiologic mechanisms and vulnerability factors with RF or that RF could trigger central nervous system late manifestations such as OC spectrum disorders.

Sydenham's chorea--clinical and evolutive characteristics.

CONTEXT: During the last 12 years we have observed an increase in the frequency of Sydenham's chorea in our country. We have observed that some of our patients have presented recurrence of the chorea despite regular treatment with benzathine penicillin. OBJECTIVE: The aim of our study was to evaluate clinical and evolutive characteristics of Sydenham's chorea in a group of patients followed in our Pediatric Rheumatology Unit. TYPE OF STUDY: Retrospective study. SETTING: Section of Pediatric Rheumatology - Discipline of Allergy, Clinical Immunology and Rheumatology - Department of Pediatrics - UNIFESP - EPM. PARTICIPANTS: Two hundred and ninety patients with rheumatic fever followed between 1986 and 1999. METHODS: We reviewed the records of 290 patients with rheumatic fever followed between 1986 and 1999. All patients were diagnosed according to the revised Jones criteria (1992). We included 86 patients that presented Sydenham's chorea as one of the major criteria (one or more attacks) and evaluated their clinical and evolutive characteristics as well the treatment. RESULTS: Fifty-five patients were girls and 31 were boys. The mean age at onset was 9.7 years and mean follow-up period was 3.6 years. The 86 Sydenham's chorea patients presented 110 attacks of chorea. We observed isolated chorea in 35% of the patients, and 25 (29%) presented one or more recurrences. We included only 17 of the 25 patients for further analysis, with a total of 22 recurrences of which 14 were attacks of chorea, because it was not possible to precisely detect the interval between attacks in the other patients. The approximate interval between the attacks ranged from 4 to 96 months. In 71% of the patients there was no failure in the secondary prophylaxis with benzathine penicillin, which was performed every 3 weeks. CONCLUSION: Despite the regular use of secondary benzathine penicillin prophylaxis, children with rheumatic fever have a high risk of Sydenham's chorea recurrence.

Sydenham's chorea: clinical and evolutive characteristics

CONTEXT: During the last 12 years we have observed an increase in the frequency of Sydenham's chorea in our country. We have observed that some of our patients have presented recurrence of the chorea despite regular treatment with benzathine penicillin. OBJECTIVE: The aim of our study was to evaluate clinical and evolutive characteristics of Sydenham's chorea in a group of patients followed in our Pediatric Rheumatology Unit. TYPE OF STUDY: Retrospective study. SETTING: Section of Pediatric Rheumatology - Discipline of Allergy, Clinical Immunology and Rheumatology - Department of Pediatrics - UNIFESP - EPM. PARTICIPANTS: Two hundred and ninety patients with rheumatic fever followed between 1986 and 1999. METHODS: We reviewed the records of 290 patients with rheumatic fever followed between 1986 and 1999. All patients were diagnosed according to the revised Jones criteria (1992). We included 86 patients that presented Sydenham's chorea as one of the major criteria (one or more attacks) and evaluated their clinical and evolutive characteristics as well the treatment. RESULTS: Fifty-five patients were girls and 31 were boys. The mean age at onset was 9.7 years and mean follow-up period was 3.6 years. The 86 Sydenham's chorea patients presented 110 attacks of chorea. We observed isolated chorea in 35 percent of the patients, and 25 (29 percent) presented one or more recurrences. We included only 17 of the 25 patients for further analysis, with a total of 22 recurrences of which 14 were attacks of chorea, because it was not possible to precisely detect the interval between attacks in the other patients. The approximate interval between the attacks ranged from 4 to 96 months. In 71 percent of the patients there was no failure in the secondary prophylaxis with benzathine penicillin, which was performed every 3 weeks. CONCLUSION: Despite the regular use of secondary benzathine penicillin prophylaxis, children with rheumatic fever have a high risk of Sydenham's chorea recurrence

The psychiatric symptoms of rheumatic fever.

OBJECTIVE: This study examined the frequency and age at onset of psychiatric disorders among children with rheumatic fever, Sydenham's chorea, or both and a comparison group. METHOD: Twenty children with rheumatic fever, 22 with Sydenham's chorea, and 20 comparison children were assessed by means of a semistructured interview and rating scales for tic disorders and obsessive-compulsive disorder. RESULTS: Obsessive-compulsive symptoms were more frequent in both the Sydenham's chorea and rheumatic fever groups than in the comparison group. The Sydenham's chorea group had a higher frequency of major depressive disorder, tic disorders, and attention deficit hyperactivity disorder (ADHD) than both the comparison and rheumatic fever groups. ADHD symptoms were associated with a higher risk of developing Sydenham's chorea. CONCLUSIONS: Both the rheumatic fever and Sydenham's chorea groups were associated with a higher risk of developing neuropsychiatric disorders than the comparison group. ADHD appears to be a risk factor for Sydenham's chorea in children with rheumatic fever.

Obsessive-compulsive and related symptoms in children and adolescents with rheumatic fever with and without chorea: a prospective 6-month study.

OBJECTIVE: The incidence and course of neuropsychiatric symptoms were determined in pediatric patients with rheumatic fever. METHOD: The Leyton Obsessional Inventory and National Institute of Mental Health Global Obsessive-Compulsive Scale were used to evaluate children and adolescents who had rheumatic fever with Sydenham's chorea (N=30) or without chorea (N=20). They were assessed three times over 6 months from the onset of rheumatic fever. Psychiatric diagnoses were also determined. RESULTS: Obsessive-compulsive symptoms abruptly appeared and peaked during the 2 months after the onset of rheumatic fever in 21 patients with chorea (70.0%) and were absent in all patients without chorea. Obsessive-compulsive disorder (OCD) was diagnosed in five patients with chorea (16.7%). CONCLUSIONS: The association between Sydenham's chorea and OCD supports suggestions that similar mechanisms involving the basal ganglia underlie both disorders. Obsessive-compulsive symptoms occurred at the beginning of rheumatic fever, so early psychopathological assessments are essential.