Rheumatic chorea in pregnancy: A rare case report

Syeda Bushra Fatima^1*, Suresh Rawte², Shubhangi Mande³, Swati Shiradkar⁴

¹Jr. Resident 3, ²Assistant Professor, ³Associate Professor and Unit Head, ⁴Professor and HOD, Department of OBGYN, Mahatma Gandhi Mission’s Medical College and Hospital, Aurangabad, Maharashtra, INDIA.

Email: s.bushra225@gmail.com

Abstract            Rheumatic heart disease often associated with chorea. Here we present a case of rheumatic heart disease with pregnancy, had chorea appeared first time in pregnancy. Those chorea movements disappeared two months postpartum.

Keywords: Rheumatic chorea, pregnancy.

INTRODUCTION

The word “Chorea” is derived from Greek word meaning dance. It is characterised by involuntary rhythmic movements which are random and brief. Jones Major Criteria for diagnosis of Rheumatic Fever include Carditis, Polyarthritis, Chorea, Erythema marginatum, and subcutaneous nodules. While Minor include fever, arthralgia, previous rheumatic fever or rheumatic heart disease, acute phase reactions: ESR/ CRP/ leucocytosis etc, and prolonged PR interval. Chorea gravidarum is a generic term used for chorea of any etiology occurring during pregnancy. The incidence is markedly reduced recently due to decline in incidence of rheumatic fever. It is one of the major component in Jones criteria for diagnosis of Rheumatic Fever. We report a rare case of rheumatic chorea during pregnancy.

CASE REPORT

A 19 year old primigravida presented to the outpatient department for antenatal check up. She was a diagnosed case of rheumatic heart disease with mitral stenosis and undergone Ballon Mitral Valvotomy 2 years back receiving Penicillin prophylaxis every 3 weeks. She had diastolic murmur and rest all Antenatal check up was normal. She had abnormal facial, hand and leg movements on right side ofn the body. Those movements were brief and non-rhythmic and used to get aggrevated with stress and reduce on its own within minutes. Those movements were absent during sleep and didn’t interfere her routine activites. Those involuntary movements started from beginning of 2nd trimester and gradually increased in intensity and frequency. Cardiology and Neurophysician consultation was done and those movements were diagnosed to have rhematic chorea. She reported history of fever, sore throat and fleeting joint pains during her childhood, suggestive of rheumatic fever. But denied such abnormal movements before the pregnancy. She had been investigated and found to be negative for Antiphosholipid antibody syndrome (APLA), ASO titre. Thyroid functions were normal.2 D-Echo revealed moderate Mitral stenosis with good biventricular function. As those chorea movements were non-troublesome, no medication required for those movements. Patient was receiving Inj. Penidura, digoxin and ramipril tablets besides iron and calcium tablets. At 38th week of gestation, she had an emergency LSCS for PROM with fetal distress. She gave birth to a male baby weighing 2.8 kg cried after bag and mask ventilation. Baby had no congenital anomaly. Intraoperative and postoperative stay was uneventful. Those involuntary movements reduced in frequency and intensity after the delivery. Completely disappeared after 2 months. Neurophysician consultation was done and those movements were diagnosed to have rhematic chorea.

DISCUSSION

Chorea is a greek word for dance. Chorea is an involuntary abnormal movement characterized by abrupt, brief, non-rhythmic, nonrepetitive movement of any limb, often associated with non-patterned facial grimaces.¹ Chorea gravidarum is the term given to chorea occurring during pregnancy. The condition is rare now due to decline in rheumatic fever which was a major cause of chorea gravidarum before. Wilson and Preece found that the overall incidence of chorea gravidarum was approximately 1 case per 300 deliveries. In recent times, most cases of chorea appearing during pregnancy are caused by SLE, Huntington disease, APL syndrome, Wilson’s disease and Idiopathic.² 80 %occur during first pregnancy and 50 % start during first trimester. 1/3rd begin in 2nd trimester of affected women, 2/3rd of patients it lasts till puerperium. Recurrence in 21% may occur in subsequent pregnancy particularly if APLA is the cause. Carditis was found in 87% of fatal cases.³ The oestrogen and progestational hormones may sensitize the dopamine receptors previously at stria nigra level and induce chorea in individuals who are vulnerable to this complication by virtue of pre-existing pathology. Oestrogen can influence neural activity in the hypothalamus, and limbic system directly through modulation of neural excitability and they have complex multiphasic efferents on nigrostriatal sensitivity.⁴ There is no particular diagnostic test to detect the rheumatic chorea. Diagnosis is usually by excluding other causes of chorea. Some 35% of patients with chorea gravidarum have a definite history of rheumatism and as expected, this patient had a positive history suggestive of rheumatic fever during her childhood.¹ 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.³ Patients who are asymptomatic may become symptomatic during pregnancy and those who are symptomatic previously may have exacerbation of symptoms.² Chorea may be unilateral hemichorea. The patients may attempt to disguise chorea by incorporating it into mannerisms or gestures. In mild chorea, patients are generally unaware of involuntary movements. Chorea movements are more distressing to observers than to the individuals. Treatment consists of rest and seclusion, careful feeding, and emotional support. Antidopaminergic drugs have been the drugs of choice for treatment of chorea. However, they have potential teratogenic effects on the fetus and may cause parkinsonian fetures in mothers when used in pregnant women. Drug treatment is indicated for those with severe disabling chorea or disturbed sleep.⁵

CONCLUSION

Pregnancy is the time of changing hormonal milieu, it can lower the threshold for chorea and emotional stress may precipitate it. Mild choreic movements can be relieved by isolation, hydration and rest. Rheumatic chorea still haunts the pregnant women in the developing countries, though it is rare in the developed world.

REFERENCES

1. Pathania M, et al. Demonstration of choreic movements in a case of chorea gravidarum ; BMJ Case Rep 2013. doi:10.1136/bcr-2012-008234
2. Sheela et. al / A case report: Chorea gravidarum ; Int J Biol Med Res. 2011; 2(3): 814-815
3. Sunita Ghike, et al, A rare case of chorea gravidarum ; South Asian Federation of Obstetrics and Gynecology, May – Aug 2010, 2 (2) : 145-146
4. Kaplan and Sadock's synopsis of Psychiatry.(10th ed), 123.
5. Gravidorum C. Article by Tarakad.S. Ramachandran. Chorea gravidarum : A case report and review. Md Med J. 1997:46(8):436-439.