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. Se describe el caso de una paciente de 17 años con corea desencadenada durante el embarazo como manifestación inicial de LES y SAF.Neuropsychiatric symptoms of systemic lupus erythematosus (SLE) and secondary antiphospholipid syndrome (APS) are very common. In both cases, pathophysiology is correlated to vasculopathy associated to antiphospholipid antibodies as well as neurotoxicity mediated by antibodies and cytokines. Chorea, instead, is the only movement disorder included in the 19 SLE's neuropsychiatric syndromes described by the American College of Rheumatology. Nevertheless, its presence is unusual with a prevalence of about 2%, and could appear as an early manifestation of the disease. The case of a 17-year-old pregnant woman in whom chorea was an early manifestation of SLE and APS.

Chorea gravidarum: Case report and review of the literature / Corea Gravídica: reporte de caso y revisión de la literatura

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. Treatment with antipsychotics and benzodiazepines was given to manage symptoms, and with benzathine penicillin to address the etiology, achieving control at 14 weeks. Treatment was discontinued at 35 weeks and the patient went on to have normal delivery at 39 weeks. A search was conducted in the Medline via PubMed, UptoDate, Medscape and Google Scholar databases using the terms "Pregnancy and Chorea Gravidarum". The search was limited to case reports and case series or review articles published between 2000 al 2019. RESULTS: Seven case reports and one review of the topic were found. In 4 of the 7 cases, treatment was based on haloperidol, benzodiazepines and chlorpromazine. Penicillin was used in one of two cases with a history of Sydenham chorea. Maternal and fetal prognosis was good in 6 of 7 cases, there was 1 case of intrauterine growth restriction. CONCLUSIONS: Treatment of gestation chorea is primarily expectant and the goal is to reduce symptoms. Maternal and fetal prognosis is good.

TITULO: COREA GRAVÍDICA: REPORTE DE CASO Y REVISIÓN DE LA LITERATURA. OBJETIVO: presentar un caso de corea gravídica y hacer una revisión sistemática de la literatura publicada sobre el tratamiento y pronóstico materno-fetal de la entidad. METODOS: se presenta el caso de una paciente de 16 años, primigesta, que ingresa a un hospital público de tercer nivel de complejidad, con 8 semanas de gestación, con clínica de 3 días de evolución caracterizada por movimientos involuntarios de la cabeza y las extremidades, con hiperreflexia en miembro inferior derecho. Antecedente de Corea de Sydenham. Se da tratamiento con antisicóticos y benzodiacepinas para la sintomatología y etiológico con penicilina benzatínica y control del cuadro a las 14 semanas. Suspendió el tratamiento a las 35 semanas. Parto normal a las 39 semanas. Se realizó una búsqueda en la base de datos de Medline vía PubMed, UptoDate, Medscape y Google Académico empleando los términos: "Pregnancy" and "Chorea Gravidarum". La búsqueda se limitó a reportes y series de casos o artículos de revisión, desde el año 2000 al 2019. RESULTADOS: se encontraron siete reportes de casos y una revisión de tema. El tratamiento se realizó con haloperidol benzodiacepinas y clorpromacina en cuatro de los siete casos. En uno de los dos casos con antecedentes de corea de Sydenham se utilizó penicilina. El pronóstico materno-fetal fue bueno en seis de siete casos, en un caso hubo restricción de crecimiento intrauterino. CONCLUSIONES: el tratamiento de la corea gestacional principalmente es expectante y su finalidad es disminuir la sintomatología, el pronóstico materno-fetal es bueno.

Chorea gravidarum: Case report and review of the literature / Corea gravídica: reporte de caso y revisión de la literatura

RESUMEN Objetivos: presentar un caso de corea gravídica y hacer una revisión sistemática de la literatura pu- blicada sobre el tratamiento y pronóstico materno- fetal de la entidad. Materiales y métodos: se presenta el caso de una paciente de 16 años, primigesta, que ingresa a un hospital público de tercer nivel de complejidad, con 8 semanas de gestación, con clínica de 3 días de evo- lución caracterizada por movimientos involuntarios de la cabeza y las extremidades, con hiperreflexia en miembro inferior derecho. Antecedente de Corea de Sydenham. Se da tratamiento con antisicóticos y benzodiacepinas para la sintomatología y etiológico con penicilina benzatínica y control del cuadro a las 14 semanas. Suspendió el tratamiento a las 35 semanas. Parto normal a las 39 semanas. Se realizó una búsqueda en la base de datos de Medline vía PubMed, UptoDate, Medscape y Google Académico empleando los términos: "Pregnancy" and "Chorea Gravidarum". La búsqueda se limitó a reportes y series de casos o artículos de revisión, desde el año 2000 al 2019. Resultados: se encontraron siete reportes de casos y una revisión de tema. El tratamiento se realizó con haloperidol benzodiacepinas y clorpromacina en cuatro de los siete casos. En uno de los dos casos con antecedentes de corea de Sydenham se utilizó penicilina. El pronóstico materno-fetal fue bueno en seis de siete casos, en un caso hubo restricción de crecimiento intrauterino. Conclusión: el tratamiento de la corea gestacional principalmente es expectante y su finalidad es disminuir la sintomatología, el pronóstico materno- fetal es bueno.

ABSTRACT Objectives: 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. Materials and 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. Treatment with antipsychotics and benzodiazepines was given to manage symptoms, and with benzathine penicillin to address the etiology, achieving control at 14 weeks. Treatment was discontinued at 35 weeks and the patient went on to have normal delivery at 39 weeks. A search was conducted in the Medline via PubMed, UptoDate, Medscape and Google Scholar databases using the terms "Pregnancy and Chorea Gravidarum". The search was limited to case reports and case series or review articles published between 2000 al 2019. Results: Seven case reports and one review of the topic were found. In 4 of the 7 cases, treatment was based on haloperidol, benzodiazepines and chlorpromazine. Penicillin was used in one of two cases with a history of Sydenham chorea. Maternal and fetal prognosis was good in 6 of 7 cases, there was 1 case of intrauterine growth restriction. Conclusion: Treatment of gestation chorea is primarily expectant and the goal is to reduce symptoms. Maternal and fetal prognosis is good.

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. She also showed uncontrollable tremors of the neck and occasional flailing of upper extremities. Her preliminary laboratory tests were within normal range. It was worth noting here that her family's medical history was unremarkable. In this article, the process of arriving at the final diagnosis and treatment would be discussed.

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. We review the complex relationship between estrogen and movement disorders, including the putative neuroprotective effects of estrogen in PD and the modulatory effects on RLS and chorea associated with autoimmune disease. Further understanding of sex-specific and hormonal effects on clinical features will be important to optimize the management of women with movement disorders in the future.

Movimientos anormales y embarazo / Abnormal movements and pregnancy

Los movimientos anormales corresponden a una de las patologías neurológicas que con menor frecuencia se presentan durante el embarazo. A excepción del temblor esencial y el síndrome de piernas inquietas, los demás son raros. Los estudios en este grupo de pacientes son escasos y antiguos dada la baja prevalencia. Se busca con este artículo dar a conocer al personal médico en general las características clínicas de patologías como la corea gravidarum, la distonía y la enfermedad de Huntington entre otros, y su relación con el embrazo, la frecuencia y las opciones adecuadas de tratamiento, dada la susceptibilidad de las pacientes y el alto riesgo de efectos adversos en el feto con sus respectivas complicaciones a corto y largo plazo. Para la revisión de la literatura se estructuró una estrategia de búsqueda utilizando los términos movimientos anormales, embarazo, corea gravidarum, síndrome de piernas inquietas, distonía, enfermedad de Parkison (MeSH y no Mesh), los cuales se articularon con operadores booleanos en las siguientes bases de datos: pubmed, ebscohost y embase, filtrando los resultados por artículos de revisión.

Abnormal movements are one of the rare neurological conditions that present during pregnancy. With the exception of essential tremor and restless legs syndrome, the rest are rare. Studies on this patient group are limited and old, given the low prevalence. With this article, it is sought to provide information to general medical professionals on the clinical characteristics of diseases including, among others, chorea gravidarum, dystonia, and Huntington's disease, as well as their relationship with pregnancy, their frequency, and appropriate treatment options, given the susceptibility of the patients and the high risk of adverse effects in the foetus with their respective short and long-term complications. A structured strategy was employed for the review of the literature, using the terms, abnormal movements, pregnancy, chorea gravidarum, restless legs syndrome, dystonia, Parkinson's disease (MeSH and non-MeSH), which were formulated with Boolean operators in the following data bases: PubMed, EBSCOhost and Embase, filtering the results for review articles.

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. There is extensive evidence that estrogen prevents dopaminergic neuron depletion induced by neurotoxins in PD animal models and therefore is neuroprotective. Estrogen may also decrease the efficacy of other neuroprotective substances such as caffeine in females but not males. Sex chromosomes can exert effects independent of sex steroid hormones on the development and maintenance of the dopamine system. As a result of hormone, chromosome and other unknown effects, there are sexual dimorphisms in the basal ganglia, and at the molecular levels in dopaminergic neurons that may lead to distinct mechanisms of pathogenesis in males and females. In this review, we summarize the evidence that estrogen and selective estrogen receptor modulators are neuroprotective in PD and discuss potential mechanisms of action. We also briefly review how sex differences in basal ganglia function and dopaminergic pathways may impact HD, chorea, and tics/Tourette's syndrome. Further understanding of these sex differences may lead to novel therapeutic strategies for prevention and treatment of these diseases.

[Anaestheia for valve replacement in the second trimester of pregnancy]. / Anestesia para sustitución valvular en el segundo trimestre del embarazo.

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. These procedures also require team coordination to be successful. The case is presented of a 19 weeks pregnant woman, who required a mitral valve replacement, which was achieved with success, and enabled her to complete her pregnancy without complications. Details are provided on the published references on which our management was based.

[Antiphospolipid syndrome related chorea gravidarum case with psychotic symptoms misdiagnosed as conversion disorder: case report]. / Konversiyon bozuklugu yanlis tanisi konan psikotik belirtilerin oldugu antifosfolipid sendromu iliskili kore gravidarum: olgu sunumu.

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. One of the most common causes of CG is antiphospholipid syndrome. Herein we present a case of recurrent CG that was considered to be due to antiphospholipid syndrome. The patient had a history of 3 pregnancy losses and her fourth pregnancy was treated appropriately, resulting in the birth of healthy full-term baby. During the patient's first pregnancy CG was accompanied by psychotic symptoms and was misdiagnosed as conversion disorder.

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. Laboratory investigations did not reveal anything interesting. We discuss the diagnosis and management of this patient.

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. There are genetic and non genetic causes of chorea. Huntington's disease is most common genetic cause of chorea. Clinical manifestations of Huntington's disease are mainly neurological and psychiatric. Recently non neurological clinical manifestations of this disease have been described. Genetic test for Huntington's disease is available which may be done for diagnosis and detection of family members at risk of developing disease. Other genetic causes of chorea are neuroacanthocytosis and Wilson's disease. Treatment of genetic causes of chore is usually symptomatic with exception of Wilson's disease. Sydenham's chorea is a neurological manifestation of acute rheumatic fever and most important cause of chorea seen in paediatric population. Treatment includes penicillin prophylaxis and drugs such as sodium valproate and carbamazepine. Diagnosis of chorea is mainly clinical. Family history is very important in diagnosis of genetic causes of chorea. In other patients a detailed work up is required before a final diagnosis is made. Hematological and blood chemistry investigations are helpful in diagnosis of some of the patients. Neuro imaging may also be useful mainly in Huntington's disease patients. Metabolic disorders and drugs are very important causes of non genetic chorea. Early diagnosis is important because majority of the patients respond to the treatment.

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. 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 Sydenham's 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.

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. This review summarizes retrospective series and case reports to both guide clinicians and to stimulate and direct the design of prospective studies.

[Chorea gravidarum. A case report]. / Corea gravídica. Reporte de un caso.

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. A 22 years-old patient, second pregnancy, without prenatal care, who was admitted to the labor at 36.1 weeks of gestation and psychosis of pregnancy presenting involuntary movements, dysnea and behavior disorder of three days evolution. The initial treatment was with diazepam, two days after the patient presented amniotic rupture without uterine activity, the pregnancy was interrupted by C- section and a male newborn was obtained. She had a favorable evolution during puerperium. She was discharged of the hospital four days later, having only abnormal movement in the right hand. Antistreptolysins antibodies of 333, PCR 1:80 and negative rheumatoid factor. She had four normal pregnancies more after, being the last the 10th of February of 2007.

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 symptoms started just after onset of severe emesis gravidarum. There was no meaningful medical history or family history, and she was taking no regular medication. Magnetic resonance imaging of the brain revealed moyamoya disease. The symptoms, as well as the hyperemesis gravidarum, improved with gestational age; however, they were sustained up to 30 weeks of gestation. She delivered 2.61 kg, healthy male neonate at 40 weeks' pregnancy by vaginal delivery under epidural analgesia. After 3 years later, she got pregnant again, and delivered at 41 weeks of pregnancy without complication. She experienced similar but milder symptoms to that of the first pregnancy until the late second trimester. After the delivery, full evaluation was lost due to her refusal. In this report, we present the case of a patient with chorea gravidarum in which moyamoya disease acts as an etiologic factor and a review of literature with management proposal.