Abstract
Rheumatic chorea during pregnancy is important from obstetric, neurological as well as cardiac point of view. Management in a team of trio has resulted in a favourable outcome in the form of healthy baby and healthy mother after the delivery.

Introduction
Chorea is rapid jerky irregular movements that tend to occur in distal limbs or face which cannot be suppressed voluntarily.
The onset of new movement disorder during pregnancy chorea gravidarum is synonymous with rheumatic chorea (a variant of Sydenham’s chorea associated with rheumatic fever and streptococcal infection).

Sydenham’s chorea occurs in less than 10% of patients with rheumatic fever.1 The latent period of the presentation of Sydenham’s chorea after the streptococcal infection can be as long as few days to several months. Many patients who appear to have only chorea may reveal several decades later with evidence of typical rheumatic valvular heart disease.1 Hence even if they did not have diagnosed rheumatic heart disease the patients with Sydenham’s chorea, have been given secondary prophylaxis for prevention of recurrent attacks.

Case Report
A twenty year old lady, G2P1L0 with no living issue, married since 3 years with 37 weeks of gestation was admitted in the antenatal ward. For her previous pregnancy she was registered in antenatal outpatient department under our care. The foetus had breech presentation on antenatal checkup. However as per tradition she went to her parents’ house for delivery and ended up delivering the baby at home. The baby died immediately after birth presumed to be due to birth asphyxia.

Fifteen days later she started developing symptoms of involuntary movements of right half of body. She was diagnosed to have rheumatic chorea.
She conceived again three months later. The symptoms of chorea increased progressively during pregnancy. She had to be admitted under neurology care.
All basic investigations were in normal limits. Sonography revealed single live intrauterine gestation of 14.5 weeks. Subsequently she was investigated for other possible aetiologies, including Wilson's disease, space occupying lesion in brain and anti-phospholipid antibodies (APLA) syndrome.

ANF, Anti DsDNA, IgG and IgM cardiolipin antibody, lupus anticoagulant were detected negative.

Diagnosis and Management
In conjunction with neurologist and cardiologist, after ruling out Wilson’s disease, structural lesion in brain and APLA syndrome, our patient was diagnosed to have chorea gravidarum (rheumatic chorea associated with pregnancy).

Secondary prophylaxis was started with injection penidura 1.2 million units every 21 days. She was put on Sodium Valproate 500 mg once a day since 18 weeks of gestation with folic acid prophylaxis throughout the pregnancy.

At 36 weeks of gestation the anticonvulsant dose was increased to 750 mgs a day.

Fig. 1 : MRI of brain of the mother. No abnormalities detected.	Fig. 2 : Radiograph of the foetus showing normal findings without any gross radiologically detected anomalies.

She was taken for elective LSCS at 38 weeks of gestation for breech presentation (obstetric indication). She delivered a male child of 2.4 kg birth weight with Apgar score of 8,9,9.The baby did not have any congenital anomaly.

Her dose of Sodium Valproate was tapered to 500 mg once a day following delivery. Post operative period was uneventful. She was advised to continue prophylaxis with penicillin every three weeks. She was discharged without any symptoms of chorea with a healthy baby.

Discussion
There is no particular diagnostic test to detect rheumatic chorea. Diagnosis of rheumatic chorea is usually a diagnosis of exclusion after excluding other causes of chorea.

The usual age of presentation of the rheumatic chorea is in second decade of life, while Huntington’s chorea presents in the fourth decade of life. Patients who are asymptomatic may become symptomatic during pregnancy and patients who are previously symptomatic may have exacerbation of symptoms. Thus patients who have chorea at pregnancy maybe presumed to have rheumatic chorea and started on prophylactic treatment for prevention of recurrent infections though not diagnosed to have rheumatic heart disease.

Patients suffering from chorea have been documented to have presence of increased levels of dopamine in brain. Antidopaminergic drugs have been used to treat patient suffering from chorea. Haloperidol, chlorhexiphenedine, tetrabenzin have been the drugs of choice for treatment of chorea. However they have potential teratogenic effects on the baby and may cause parkinsonian features in mothers when used in pregnant women. Hence, sodium valproate though not the drug of choice is given as it has been proven to be effective and least teratogenic. This drug may cause neural tube defects. To reduce the risk of this side effect the drug was started after 18th week of gestation along with supplements of folic acid.

The pregnancy was totally uneventful and baby was normal at birth.

References

1. Harrison’s book of Medicine; 16th edition. 2005; Vol II : 1977-1979, 302.
   
2. Harrison’s book of Medicine; 2005; Vol I 16th edition 21, 139 .
3. K.D. Tripathi Book of pharmacology: 2003; 6th edition 375.