SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
A woman with chorea gravidarum in the absence of SLE is described. She later developed severe chorea while using the contraceptive pill, and high titered antiphospholipid antibodies were detected in serum. A rapid recovery after high dose i.v. methylprednisolone was observed. Deterioration occurred in a later pregnancy.
Explore the source record for details and available documents.
Sydenham's chorea (chorea minor, St. Vitus dance, rheumatic encephalitis), described by Thomas Sydenham in 1686, is considered one of the major manifestations of rheumatic fever (1, 2, 3, 4). Clinically it is characterized by involuntary movements, hypotonia, dysarthria, emotional disorders, and less frequently, by other neurological manifestations such as weakness, headache, seizures and sensory abnormalities (1,4). The motor disorders may be generalized or unilateral, in this case constituting a hemichorea (3). Chorea may present associated to other rheumatic fever manifestations during an acute episode, or in isolated form, characterizing the so-called "pure" chorea (5, 6, 7). Its etiology and pathophysiological mechanisms are still unclear, although its relation with a previous pathophysiological group A Beta-hemolytic streptococcus infection is well established (8). There is also evidence of the participation of immunological mechanisms in its pathogenesis, such as the finding of serum anti-nucleus caudatus and anti-subthalamic antibodies (9) and increase in IgG levels in cerebrospinal fluid of patients with chorea (10). In developed countries due to the reduction in rheumatic fever incidence and decrease in frequency of chorea as its manifestation (3, 11), the latter has become rare. However, in developing countries rheumatic fever remains a public health problem. In Brazil, in the last years an increase in the incidence of chorea has been observed as part of the clinical picture of rheumatic fever (12). The present study reports the clinical and laboratory findings of 187 cases of Sydenham's chorea followed-up during the period of January 1980 to December 1990 in two university centers in the city of Sao Paulo, Brazil.
Chorea associated with systemic lupus erythematosus (SLE) has been reported in only 28 patients. The clinical and laboratory features of these cases are reviewed here, along with those of a 7-year-old boy who, we believe, represents the youngest child reported to date. In approximately half of these 29 individuals, most of whom were children, chorea preceded other manifestations of SLE. The age range and clinical characteristics of lupus-associated chorea were not appreciably different from those of Sydenham's chorea and most of the patients in whom chorea developed before other manifestations of SLE were initially assumed to have Sydenham's chorea. Systemic lupus erythematosus should especially be considered if chorea begins in the older child or is associated with a persistently elevated erythrocyte sedimentation rate.
A rare complication of oral contraceptive therapy is the induction of chorea. We here describe five cases of chorea in patients receiving low- or high-dose estrogen-containing contraceptives. All patients were nulliparous, young (average age 19 years), and became symptomatic shortly (average of 5 weeks) after initiation of contraceptive therapy. Two patients previously suffered an episode of Syndenham chorea; one experienced chorea in the course of Henoch-Schönlein purpura; and two had a history of congenital cyanotic heart disease without chorea. Dyskinesia resolved in all patients upon discontinuing the medication. Patients with preexisting striatal abnormalities appear more susceptible to oral contraceptive-induced chorea which is reversible on drug discontinuation. The mechanism of oral contraceptive-induced chorea is unknown, but clinical and experimental data suggest that it involves altered central dopaminergic activity.
Neurologic complications of systemic lupus erythematosus (SLE) are common, but chorea is rare. Three cases of chorea associated with SLE are presented as well as a review of 28 cases from the world literature. Chorea may be the first and at times the only sign prior to the establishment of diagnosis of SLE. Under such conditions the establishment of the correct diagnosis is difficult, and the chorea may be attributed to a more common etiology, Also the SLE-related chorea may recur in the same subject during the course of the disease. Finally, the chorea associated with SLE can be brought under control by use of haloperidol (HALDAL).
In the last few years both neuropathological and neurochemical data have contributed towards a classification of the pathogenesis of degenerative chorea of which Huntington's chorea shows clear evidence of heredity. The role of GABA, DA and Ach in determining chorea seems clear. In 7 patients with degenerative hypotonic-hyperkinetic chorea (the acid metabolites into the cerebrospinal fluid are not available) a neuropharmacological study was conducted. The symptomatology was evaluated both through clinical rating and through phasic and tonic performance tests. The neuropharmacological tests were both acute and chronic. In the acute tests the effect of modification of various neurotransmitters (Ach, DA, NA, GABA) was evaluated. In chronic treatment the effectiveness of haloperidol, PCPA and bromocryptine was evaluated. The results of the acute study were positive both as regards increment of DA (receptors stimulation) and GABA. The chronic study shows any effect of PCPA, positive results both with bromocryptine and with haloperidol. Our results confirm a DA/GABA interaction in the pathogenesis of degenerative chorea.
Sydenham's chorea can be successfully treated with haloperidol, an agent that is known to interfere with the binding of dopamine to its receptors. This suggests that dopamine and its metabolic end product, homovanillic acid (HVA), might be elevated in Sydenham's chorea. To test this hypothesis, the urine of three patients with the clinical diagnosis of Sydenham's chorea was analyzed for HVA and vanillylmandelic acid (VMA). Urinary HVA and the HVA:VMA ratio were significantly higher in these three patients compared with seven control children. Urinary VMA was not different in these two groups. It is suggested that increased dopamine metabolism is involved in the pathogenesis of Sydenham's chorea and that the determination of urinary HVA and the HVA:VMA ratio may be helpful in establishing this diagnosis. We report a case that demonstrates the use of urinary HVA determination in the diagnosis of Sydenham's chorea.
The hospital records of 28 children (mean age, 9.4 years) with typical Sydenham's chorea were reviewed. Nineteen of 28 patients had antistreptolysin O titers of greater than or equal to 200 Todd units. Other causes of chorea were excluded by appropriate laboratory and clinical follow-up studies. At the onset of the movement disorder, 17 of 28 patients had abnormal EEGs consisting of irregular posterior slowing in 15, sharp epileptic spikes in 5, and high-voltage sharp waves in 2. Two patients with spikes predominantly in the temporal lobe regions developed complex partial seizures. On follow-up evaluation, the EEGs returned to normal within one to four weeks. Seizures did not recur after therapy with anticonvulsants. Seizures have been reported only rarely in association with Sydenham's chorea. Our observation suggests that seizures may occur during chorea but may often be masked by frequent choreic movements and thus not recognized. The EEG changes and seizures were transient in our patients studied so far.
Recurrent chorea is described in a 61-year-old woman who had had chorea gravidarum when she was younger. The recurrent chorea appeared to be induced by a topical vaginal cream that contained conjugated estrogen. This case is consistent with the existence of a recurrent syndrome of hormone-induced chorea. The effect of estrogen on the basal ganglia is complex and not fully understood.
Hyperthyroidism is invariably accompanied by nervous system dysfunctions. Irritability, emotional lability and hyperkinesia are the signs and symptoms most frequently observed. Chorea or choreoathetosis are only rarely associated with hyperthyroidism. It is the purpose of this work to describe the case of a young girl in whom chorea was the main manifestation of thyrotoxicosis. The chorea receded and disappeared as the patient became euthyroid. Hyperthyroidism, therefore, is to be considered an unusual cause of chorea and every patient with choreiform movements should be examined also for thyroid function.
A case of hemi-chorea is described in a young woman, initially following administration of an oral contraceptive pill and recurring in a subsequent early pregnancy. The chorea ceased with the removal of the aetiological factor on each occasion. Subsequent challenge with a combined oestrogen/progestogen pill resulted in recurrence of the chorea, but the patient has been successfully maintained on a progestogen without any further symptoms. Thus it would appear that oestrogen component was the precipitating factor for the chorea.
Two cases of polycythaemic chorea are described, both of which were complicated by severe heart disease. The first was a child with patent ductus arteriosus and coarctation of the aorta causing severe cyanosis and secondary polycythaemia. Chorea began intermittently at an early age, becoming continuous by his fifth birthday. The second was a middle-aged male with tight mitral stenosis and a story of paralytic chorea in his teens. Polycythaemia rubra vera was eventually diagnosed two years after mitral valvotomy, some seven years after the onset of chorea.
Subdural hematoma has rarely been implicated as a cause of chorea. We describe a case of chorea occurring several months after evacuation of a traumatic subdural hematoma. No other causes of chorea were found. In this case and one previously reported case, signs of tentorial herniation were present prior to surgery. Herniation may damage the basal ganglia, with resultant chorea.
We report a 21-year-old woman in whom chorea was associated with antiphospholipid antibodies. In August 1986, she developed involuntary movement which started in the right hand but subsided spontaneously. In September 1988, she again developed right-sided involuntary movements which started in the right hand but rapidly progressed to involve the whole of the right side. In September 1990, she was admitted to our hospital for investigation of choreiform movements, because her involuntary movements had progressed to involve all four extremities. She had no family or past history of chorea, psychiatric, rheumatological or vascular disease. On admission, she had difficulty in speaking and swallowing due to choreiform movements of her mouth and tongue. Her gait was unsteady. On walking she had wild gyrations of the arms. Choreiform movements of all four extremities, neck, face, mouth and tongue were present at rest, more marked on the right side. There was no other neurological deficits. She had none of the classical features of SLE. She had none of the complications commonly associated with antiphospholipid antibody syndrome (APS) (i.e., recurrent spontaneous abortion, thrombosis and thrombocytopenia). Laboratory tests revealed that antinuclear antibody was present. Cardiolipin antibody (VDRL) was positive but specific tests for syphilis were negative. Anticardiolipin antibodies were present. All coagulation studies have failed to reveal lupus anticoagulant. Brain CT, MRI, 123IMP-SPECT and cerebral angiography were normal. Associated with her chorea, she had the serological but not the clinical features of APS. We suggest that antiphospholipid antibodies should be looked for in all unexplained cases of chorea, even when the associated clinical signs of APS are absent.
Sydenham's chorea was observed in 65 patients in Tunisia during the period 1971-1976. The average age of onset was 10.8 years, and girls were affected twice as often as boys. Sydenham's chorea is a seasonal disorder; it usually develops between the months of november and march, and its frequency is closely related to that of Bouillaud's disease. A study of the past history of infections disease or rheumatic disorder (ARF), and biological tests for inflammation (sedimentation rate, blood fibrin levels, antistreptolysins O, and culture of throat swabs), showed that it is possible to distinguish cases of true chorea occurring alone from those in which it is associated with a rheumatic affection. These facts are discussed in the light of the data published in the literature. The authors conclude that sydenham's chorea and acute rheumatic fever are but two unrelated expressions of a streptococcal infection. Anti-inlammatory treatment with corticoids, therefore, is only indicated in the presence of signs of rheumatic affection.
Three weeks after commencing oral contraception for the first time, a 26-year-old woman with a history of rheumatic carditis and chorea minor presented with sudden recurrence of hyperkinesis. Withdrawal of the contraceptive agent was followed by rapid remission of the hyperkinesis, which suggests a casual relationship between the contraceptive and this condition and an analogy between this case and so-called chorea gravidarum. A young non-pregnant woman presenting with chorea minor should be specifically questioned by ingestion of oral contraceptives. If there is a past history of rheumatic fever with or without chorea, oral contraceptives are contraindicated.
During the last two years two patients with hypokinetic appearance of Huntington's chorea--beginning in childhood--were investigated in the Neurological Univ.-Clinic of Vienna. Both of the patients descend from families with primary hyperkinetic Huntington's chorea in adult life. The problem of the early manifest hypokinetic form--when the family case history is not attended--are of special interest for the first symptoms can lead into false diagnosis. Therefore oftenly systematic treatment will be done at first just during the adult life, when already the complete picture of Huntington's chorea has appeared. From the point of eugenic deliberations it is very interesting for relatives of families with Huntington's chorea to be informed about the heredity, the most lately appearance of the disease and by the possiblity of anteposition.