Learn how and when to remove this template message Chorea is characterized by brief, semi-directed, irregular movements that are not repetitive or rhythmic, but appear to flow from one muscle to the next. Walking may become difficult, and include odd postures and leg movements. Unlike ataxia , which affects the quality of voluntary movements, or Parkinsonism , which is a hindrance of voluntary movements, the movements of chorea and ballism occur on their own, without conscious effort. Thus, chorea is said to be a hyperkinetic movement disorder. When chorea is serious, slight movements will become thrashing motions; this form of severe chorea is referred to as ballism , or ballismus. Psychological symptoms may precede or accompany this acquired chorea and may be relapsing and remitting.
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Prognosis Background Chorea gravidarum CG is the term given to chorea occurring during pregnancy. This is not an etiologically or pathologically distinct morbid entity but a generic term for chorea of any cause starting during pregnancy. Chorea is an involuntary abnormal movement, characterized by abrupt, brief, nonrhythmic, nonrepetitive movement of any limb, often associated with nonpatterned facial grimaces.
Incidence Most of the more common and serious movement disorders rarely occur during reproductive years. Hence clinicians are not very familiar with chorea gravidarum. Willson and Preece found that the overall incidence of chorea gravidarum was approximately 1 case per deliveries.
The condition is much more rare now. Zegart and Schwartz found that one patient had been encountered in the course of , deliveries in 3 major Philadelphia hospitals. In recent times, most cases of chorea appearing during pregnancy are caused by other diseases eg, systemic lupus erythematosus [SLE], Huntington disease. In general, about half the cases are idiopathic, with rheumatic fever and antiphospholipid syndrome APLS underlying most of the remainder.
Recurrences may occur in subsequent pregnancies, particularly if antiphospholipid syndrome is the cause. A family history of transient chorea is not unusual. References Pathophysiology Several pathogenetic mechanisms for chorea gravidarum have been offered, but none have been proven.
The suggestion is that estrogens and progestational hormones may sensitize dopamine receptors presumably at a striatal level and induce chorea in individuals who are vulnerable to this complication by virtue of preexisting pathology in the basal ganglion.
Pathologic changes found at autopsy in chorea gravidarum include perivascular degenerative changes in the caudate nucleus. Pathology of rheumatic brain disease is of a nonspecific arteritis with endothelial swelling, perivascular lymphocytic infiltration, and petechial hemorrhages.
Aschoff bodies are not present in the brain. Severe neuronal loss occurs in the caudate nucleus and putamen. The same pathologic changes have been reported for chorea gravidarum, but all those patients also had cardiac disease. Presumably, as the inflammation resolves, the chorea disappears and degenerative changes are left in small arterioles.
Several lines of evidence suggest that heightened dopamine activity occurs either by denervation hypersensitivity or by aberrant sprouting of dopamine terminals on the remaining striatal neurons. A possible relationship between chorea gravidarum and moyamoya disease has been reported in a year-old pregnant patient.
Koide et al reported that from , 8 patients were diagnosed with clinically definite opsoclonus-myoclonus syndrome a movement disorder at Tokyo Metropolitan Neurological Hospital. The relationship between opsoclonus-myoclonus syndrome and pregnancy, like chorea gravidarum, remains unclear.
During sleep, the movements disappear. The chorea may be unilateral hemichorea. The patient may attempt to disguise chorea by incorporating it into a mannerism or gesture. Choreic movements largely affect the extremities but vary greatly in complexity and temporal expression from one patient to another. The patient may be restless and fidgety and is often unaware of it and may not complain about it; hence, the clinician might be misled or totally miss the diagnosis.
Generally, the affected limb is hypotonic; joints are floppy, and knee jerks are pendular. Normally the arms dangle by the sides, but with chorea ie, hypotonia , they flail about. Wrist and fingers assume the shape of a dinner fork with abduction of the thumb. At times, continuous involuntary movements may be impossible to sustain. Protruded tongue darts in and out uncontrollably. Varying hand strength is referred to as "milkmaid" grip.
Choreic movements are rapid, purposeless, irregular, jerky movements that seem to randomly flow from one part of the body to another.
Obtain a thorough past history including a history of rheumatic fever and confidential inquiry about illicit drug use and any psychiatric treatment with neuroleptics or metoclopramide ie, dopamine antagonists. Relationship to rheumatic heart disease Chorea gravidarum is linked strongly to rheumatic fever.
Isolated recurrences of chorea among a group of 60 children with a history of Sydenham chorea followed an episode of streptococcal pharyngitis by a week, 3 months, or even 6 months. This does not imply that all cases of chorea gravidarum are related to an immediately preceding streptococcal infection; the fact that chorea recurs in the same woman with several pregnancies is statistically against this.
Moreover, Jonas et al were able to document that a woman with chorea and a history of acute rheumatic fever had been free of streptococcal infection for 15 months prior to the presentation of chorea in the sixth month of pregnancy.
Rheumatic encephalopathy Rheumatic encephalopathy is reflected in the EEG findings. Slow waves Hz can occur continually or in intermittent rhythmic paroxysms. They may be generalized or predominantly over the frontal and central regions. Changes may be unilateral in hemichorea. References Physical Physical examination includes a careful general, systemic, and neurologic examination.
Look especially for involuntary movements and mental status changes. References Causes The most probable cause of chorea gravidarum is the reactivation by some mechanism of subclinical damage to basal ganglia resulting from previous rheumatic encephalopathy. Oral contraceptives and possibly other mechanisms may activate the same mechanism.
In , Miranda et al reported of a case of chorea associated with the use of the oral contraceptives, in which antibasal ganglia antibodies have also been detected, suggesting an immunological basis to the pathogenesis of this disorder. The criteria are 1 the presence of autoantibodies, 2 the presence of antibodies in target tissue, 3 the induction of disease in an animal model by passive transfer of the antibody, 4 the induction of disease in an animal model by autoantigen immunization, and 5 improvement of clinical symptoms after removal of the antibodies with plasma exchange.
Prognosis Background Chorea gravidarum CG is the term given to chorea occurring during pregnancy. This is not an etiologically or pathologically distinct morbid entity but a generic term for chorea of any cause starting during pregnancy. Chorea is an involuntary abnormal movement, characterized by abrupt, brief, nonrhythmic, nonrepetitive movement of any limb, often associated with nonpatterned facial grimaces. Incidence Most of the more common and serious movement disorders rarely occur during reproductive years. Hence clinicians are not very familiar with chorea gravidarum.
What is Chorea Gravidarum & How is it Treated?
What Is Chorea?