History
Chorea gravidarum (CG) is a rare clinical entity often without an identifiable cause, but its early recognition is important to decrease the maternal and fetal morbidity and mortality. The diagnosis requires a detailed patient history and physical examination along with thorough laboratory evaluation.
The clinical presentation is variable as chorea can present in various forms including generalized, focal, multifocal or hemichorea. It can also be unilateral or bilateral and include upper extremities and/or lower extremities. There are many different forms of chorea including Huntington's disease, paralytic, persistent, recurrent, tetanoid, functional, maniacal, hemichorea, and chorea gravidarum. [21]
Patients may attempt to disguise chorea by incorporating movements into a mannerisms or gestures. Some patients may appear simply restless or fidgety. Some may be unaware of the abnormal movements and, thus, may not complain about chorea or abnormal movements. Additionally, stress may aggravate the movements of CG and the movements disappear during sleep. These factors may lead to misdiagnosis of the condition.
As the exact etiology of CG is not clear, getting a comprehensive history from the patient is important. A thorough past medical history including rheumatic fever, history of recent infection with group A beta-hemolytic streptococcus (GABHS), and family history of chorea also needs to be identified. A pre-pregnancy history of Sydenham’s chorea has an increased risk of development if CG. [4] Of note, there are published case reports from the early 1900s in which women with normal pregnancies before rheumatic fever developed chorea in subsequent pregnancies after having rheumatic fever. [22, 23]
Immune-mediated conditions, including antiphospholipid antibody syndrome (APS) and systemic lupus erythematosus (SLE) may also predispose patients to a higher risk of CG. Therefore, a history of dermatological and joint complaints, clotting abnormalities, and spontaneous abortions may point to these etiologies being the cause of CG.
History should also focus on reviewing prescribed medications, particularly dopamine agonists, as well as inquiring about substance abuse and illicit drug use. Prior use of oral contraceptives (OC) also helps in supporting the diagnosis of recurrence of CG. Fernando et al reported the first case linking estrogen containing oral contraceptives to chorea. Chorea may also reappear in CG patients who later take OCs or use topical estrogen. [24]
A detailed history should be obtained to rule out other diagnoses that may manifest as chorea during pregnancy such as thyrotoxicosis, Wilson’s disease, and Huntington’s disease. Though patients with these diseases may have chorea during pregnancy, they are etiologically distinct pathological process and not typically considered to be CG.
Physical
Clinical manifestations of chorea gravidarum (CG) may include the following:
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Involuntary muscle movements: Typically the dance-like movements of chorea, which are rapid, purposeless, irregular, jerky movements that seem to randomly flow from one part of the body to another. These movements worsen with stress or anxiety and completely disappear during sleep. Generally, the affected limb may be hypotonic; joints are floppy, and knee jerks are pendular. Wrist and fingers assume the shape of a dinner fork with abduction of the thumb.
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Facial grimacing: Involuntary movements are often associated with non-patterned grimacing of the face due to incoordination of muscles.
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Milkmaid’s grip: Due to varying hand strength, when the patient tries to shake someone’s hand they may grip and release the fingers over and over again. This action appears similar to milking a cow, thus is referred to as milkmaid’s grip.
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Darting tongue sign: When the patient tries to protrude his tongue, it may slide in and out uncontrollably. [25]
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Dysarthria: Chorea movements may involve facial muscle leading to dysarthria. However, it should be noted that dysarthria can be present in some cases even without facial chorea. [26]
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Mental status changes: Some cases of CG may present with confusion and agitation. This is more likely when the etiology of CG is related to autoimmune disease.
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Neuropsychiatric symptoms: Chorea movements can be preceded by neuropsychiatric symptoms like emotional lability, mild cognitive changes, and psychosis. [27] Other clinical manifestations include personality changes, depression, chronic cognitive deficits, hypnic hallucinations, delirium, and Tourette-like symptom. [28]
The cerebral manifestation of rheumatic fever has sometimes historically been referred to as rheumatic brain disease. This may present as Sydenham’s chorea associated with mental status changes, emotional lability to hysterical traits, psychotic delusions, hallucinations, seizures, and papilledema depending on the severity of illness. [29, 30, 31] Encephalopathy associated with rheumatic fever, historically referred to as rheumatic encephalopathy, may be reflected in the EEG findings of 3–6 Hz slow waves, particularly over the frontal and central regions. [32]
The diagnosis of CG relies on a complete physical examination in which the involuntary, non-rhythmic, abrupt movements of chorea are identified during pregnancy, particularly in the first trimester. Case reports have documented dystonia as a sole presentation in the first trimester with resolution after delivery as a form of CG. Authors hypothesized that transient dystonia in these patients has a similar pathophysiology of CG due to the hyperkinetic nature of dystonia. [33]
Though CG is not a life-threatening condition, hyperthermia, rhabdomyolysis, myoglobinuria, and death have been reported in severe cases. [7]
Causes
There is evidence that chorea gravidarum (CG) is a sequela of rheumatic fever and autoimmune diseases. Although CG is a rare entity today, the etiology is most probably autoimmune in nature in industrialized nations, whereas it is rheumatic in nature in developing nations.
Causes contributing to CG include:
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Lupus anticoagulant
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Anticardiolipin antibody
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Systemic lupus erythematosius
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Vascular malformation in the basal ganglia region
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Cerebrovascular disease involving the basal ganglia region
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Oral contraceptive pills
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Illicit substance abuse
Causes of chorea in during pregnancy include:
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Thyrotoxicosis
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Wilson’s disease
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Huntington’s disease
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Neuroacanthocytosis
Tables
| Primary differential diagnosis |
|---|
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| Secondary differential diagnosis | |
|---|---|
| Drugs/toxicity |
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| Infectious |
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| Genetic |
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| Inherited disorders of metabolism |
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| Metabolic/endocrine disorders |
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| Vascular/trauma |
|
| Other systemic disorders |
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| Miscellaneous |
|
