Pharmacotherapy for Sydenham's chorea: where are we and where do we need to be?

Introduction: Sydenham's chorea (SC) is the most common cause of acquired chorea in children. The existing literature describes it as a benign, self-remitting condition. However, recent evidence discloses the persistence of long-course neuropsychiatric and cognitive complications in adulthood, which imposes to redefine the concept of 'benignity' of such condition.

A Case of New Onset Cervical Dystonia in Pregnancy.

Background: Though uncommon, primary movement disorders can occur in pregnancy, the most common being restless legs syndrome and chorea gravidarum [1]. New onset dystonia in pregnancy has been reported four times previously with a resolution of symptoms within six months of delivery [2345]. Exacerbation of pre-existing movement disorders and the onset of de novo movement disorders during pregnancy support the hypothesis that female sex hormones play an important role in the regulation of basal ganglia circuitry.

Chorea gravidarum associated with systemic lupus erythematosus and antiphospholipid syndrome: case report.

Las manifestaciones neuropsiquiátricas en los pacientes con lupus eritematoso sistémico (LES) y síndrome antifosfolípido (SAF) secundario son muy frecuentes. En ambos casos, la fisiopatología se correlaciona con vasculopatía asociada a anticuerpos antifosfolípido y neurotoxicidad por anticuerpos y citocinas. La corea es el único trastorno del movimiento incluido en los 19 síndromes neuropsiquiátricos del LES según el American College of Rheumatology, con presentación inusual (prevalencia del 2%), y puede ocurrir como primera manifestación de la enfermedad.

Treatment of Secondary Chorea: A Review of the Current Literature.

Background: Chorea consists of involuntary movements affecting the limbs, trunk, neck or face, that can move from one body part to another. Chorea is conceptualized as being "primary" when it is attributed to Huntington's disease (HD) or other genetic etiologies, or "secondary" when it is related to infectious, pharmacologic, metabolic, autoimmune disorders, or paraneoplastic syndromes. The mainstay of the secondary chorea management is treating the underlying causative disorder; here we review the literature regarding secondary chorea.

Movement disorders in pregnancy.

Movement disorders in women during pregnancy are uncommon. Therefore, high quality studies are limited, and guidelines are lacking for the treatment of movement disorders in pregnancy, thus posing a significant therapeutic challenge for the treating physicians. In this chapter, we discuss movement disorders that arise during pregnancy and the preexisting movement disorders during pregnancy.

Chorea gravidarum: Case report and review of the literature.

Objective: To present a case of chorea gravidarum and conduct a review of the published literature on the treatment for this condition, and on maternal and fetal prognosis.

Methods: Case presentation of a 16-year-old primiparous patient admitted to a Level III public hospital at 8 weeks of gestation complaining of involuntary head and limb movements and right lower limb hyperreflexia lasting three days. The patient had a history of Sydenham chorea.

Identifying the aetiology of sudden acute abnormal involuntary movements in a primigravid.

A 20-year-old primigravid experienced sudden stiffening of the neck, upper and lower extremities and trunk associated with joint pains. She was generally well before hospital admission with no history of attacks, except for her inflammatory bowel disease that was treated more than a year ago. During physical examination, the patient manifested neck flexion deviated to the right, deviation of the eyes downward and to the right, spooning of the upper extremities, exhibition of milkmaid's grip, extension of both lower extremities and jerky speech.

Movement Disorders in Women.

Movement disorders such as Parkinson's disease (PD), restless legs syndrome (RLS), chorea, essential tremor, and Tourette syndrome, occur in men and women of all ages. Yet, considerable sex differences in epidemiology, clinical features, and treatment exist in these disorders. In this review, we highlight key differences in the evaluation and management of women with movement disorders, addressing sex-specific complications of treatment and unique challenges surrounding the management of movement disorders during pregnancy.

Chorea.

Chorea is an involuntary movement disorder characterised by flowing and rhythmic in nature. Hyperkinetic movement disorders such as myoclonus may be mistaken for chorea. Pathogenes of chorea is complex and results from dysfunction of network between motor nucleus of thalamus and subcortical nuclei including globus pallidus interna.

Sex differences in Parkinson's disease and other movement disorders.

Movement disorders including Parkinson's disease (PD), Huntington's disease (HD), chorea, tics, and Tourette's syndrome (TS) display sex differences in disease susceptibility, disease pathogenesis, and clinical presentation. PD is more common in males than in females. Epidemiologic studies suggest that exposure to endogenous and exogenous estrogen contributes to these sex differences.

Movement disorders in pregnancy.

Purpose Of Review: This review discusses movement disorders that occur during pregnancy, the treatment of preexisting movement disorders, and the influence the pregnant state has on movement disorders symptoms, in order to guide clinicians in providing better counseling for female patients who are pregnant or considering pregnancy.

Recent Findings: Unique considerations for movement disorders during pregnancy include investigations and their safety during pregnancy and the impact of treatment on both the pregnant patient and her fetus.

Summary: The most common movement disorders arising in pregnancy are restless leg syndrome and chorea gravidarum.

[Antiphospolipid syndrome related chorea gravidarum case with psychotic symptoms misdiagnosed as conversion disorder: case report].

Chorea gravidarum (CG) is a rare movement disorder characterized by rapid, irregular randomly distributed involuntary movements during pregnancy. Similar to Sydenham chorea, psychiatric symptoms may be observed in cases of CG. CG may be idiopathic or secondary to an underlying cause.

Chorea gravidarum: a rarity in West still haunts pregnant women in the East.

A pregnant woman, in her early 20s, presents with chorea following an emotional outburst. While the family members feel it to be a spirit haunting her, we try to establish the medical diagnosis of the present condition. There is a history of rheumatic fever in the past and examination reveals choreioathetoid jerky movements of her upper limbs with jerky speech, spooning of her limbs and demonstration of milkmaid's grip.

[Anaestheia for valve replacement in the second trimester of pregnancy].

Cardiac surgery in the pregnant woman gives rise to several anesthetic challenges, as the mother, but mainly the fetus, have a risk of high morbidity and mortality. In this context, the cardiopulmonary bypass is the most complex period, owing to the risks of fetal hypoxia it entails. Due to the absence, for ethical reasons, of prospective trials that provide generally accepted guidelines in intraoperative management, it means that physicians have to work based on case reports in the literature.

Gowers' Queen Square case notes on chorea: a 21st century re-appraisal.

Objective: To analyze the case notes of 127 patients with chorea admitted to the National Hospital at Queen Square, London, under the care of William Richard Gowers and review his contribution to the study of choreas.

Methods: We consulted the case books available at the Queen Square Library, from 1878 to 1911, comprising 42 volumes.

Results: 97 patients (76.

Pregnancy in patients with Sydenham's Chorea.

Background: Sydenham's 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 Sydenham's Chorea.

Methods: A chart review was performed of all patients with the diagnosis of Sydenham's Chorea followed up at our institution from 07/1993 through 08/2010 and who became pregnant.

Movement disorders and pregnancy: a review of the literature.

Pregnant patients are rarely encountered in the movement disorders clinic, but they present significant dilemmas regarding treatment and counseling for neurologists. While movement disorders in pregnancy once described those disorders arising de novo during pregnancy, such as chorea gravidarum or restless leg syndrome, advancing maternal age in Western countries will likely increase the number of women in whom pregnancy complicates a pre-existing movement disorder. Physicians treating these women must be aware of the impact of the movement disorder and its treatment on fertility, pregnancy, fetal development, lactation, and infant care.

[Chorea gravidarum. A case report].

Chorea gravidarum is an uncommon condition characterized by involuntary movements, speaking alterations and in the affective status during first trimester pregnancy, the incidence is 1 by each 2275 pregnancies, it is self limiting and resolves when the pregnancy ends, most of the cases are idiopathic and the rest is associated to the antiphospholipid syndrome, rheumatic fever, thyrotoxicosis, systemic lupus erythematosus, syphilis, Huntington disease or induced by drugs. The recurrences can occur in the subsequent pregnancies. The dopamine antagonists as the haloperidol and the chlorpromazine are useful to control the symptoms and are safe at lower doses.

Consecutive pregnancy with chorea gravidarum associated with moyamoya disease.

Chorea gravidarum is uncommon movement disorder of pregnancy, characterized by involuntary, abrupt, non-rhythmic movements. It can be idiopathic or secondary to the underlying pathology. A 28-year-old, primigravida woman who was 8 weeks and 6 days of gestation presented with a history of involuntary choreiform movements in the left side limbs and facial twitch for 2 weeks.

The differential diagnosis of chorea.

Chorea is a hyperkinetic movement disorder characterised by excessive spontaneous movements that are irregularly timed, randomly distributed and abrupt. In this article, the authors discuss the causes of chorea, particularly Huntington's disease and the genetic syndromes that may resemble it, including HDL1-3, inherited prion disease, spinocerebellar ataxias 1, 3 and 17, neuroacanthocytosis, dentatorubro-pallidoluysian atrophy (DRPLA), brain iron accumulation disorders, Wilson's disease, benign hereditary chorea, Friedreich's ataxia and mitochondrial disease. Acquired causes of chorea include vascular disease, post-infective autoimmune central nervous system disorders (PANDAS), drugs, systemic lupus erythematosus, antiphospholipid syndrome, thyrotoxicosis, AIDS, chorea gravidarum, and polycythaemia rubra vera.

Movement disorders in pregnancy.

Movement disorders are not commonly seen during pregnancy. As a result, there are few studies on whether disease manifestations are affected by the hormonal changes that occur during pregnancy or on the teratogenicity of commonly used medications for movement disorders on the developing fetus. This article discusses movement disorders that are seen only during pregnancy (chorea gravidarum) or that may present during pregnancy (restless legs syndrome), the effect that pregnancy has on symptoms and treatment (in Parkinson's disease, essential tremor, dystonia, tic disorders, and Wilson's disease), and the role of genetic testing for movement disorders in genetic counseling for pregnant women.