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.

[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.

[A case of chorea gravidarum with moyamoya disease].

A 16-year-old girl developed acute left choreic movements during her fourth week of pregnancy. She had sometimes had transient ischemic attacks since she was 10 years old. During the eighth week of pregnancy, a brain MRI showed old ischemic lesions deep in the right frontal white matter. Her angiograph revealed a complete obstruction of the terminal portion of the right internal carotid artery with a developed moyamoya net work. After her abortion, all involuntary movements completely subsided. The choreic movements might have been caused not only by ischemia, but also by enhanced dopaminergic sensitivity mediated by elevations in female sex hormones due to pregnancy.