A 24 year old right handed Pakistani woman who was eight weeks’ pregnant presented with a two day history of involuntary movements of her left arm, hand. According to Willson and Preece, the first description of chorea with onset during pregnancy (chorea gravidarum) was made by Horstius in The English. A pregnant woman, in her early 20s, presents with chorea following an emotional outburst. While the family members feel it to be a spirit.
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A pregnant woman, in her early 20s, presents with chorea following an emotional outburst. While the family members feel it to be a spirit haunting her, we try to establish the medical diagnosis of the present condition.
There is a history of rheumatic fever in the past and examination reveals choreioathetoid jerky movements of her upper limbs with jerky speech, spooning of her limbs and demonstration of milkmaid’s grip. Laboratory investigations did not reveal anything interesting.
We discuss the diagnosis and management of this patient.
Chorea gravidarum is a rare movement disorder of pregnancy. With the declining incidence of rheumatic fever, chorea underlying rheumatic heart disease has become a rarity in the developed world. However, isolated cases still occur in the developing countries and obstetricians need to be aware of this rare manifestation. Though the exact aetiology remains unknown, prompt diagnosis and treatment reduces maternal morbidity and prevents any complication.
This is a case of chorea gravidarum in third trimester of pregnancy with a normal fetal outcome. The history was elicited from her mother-in law who told that patient started having abnormal movements of her tongue which progressed to involve her both arms in the time span of a day when she heard the news of her father’s death.
The involuntary grabidarum in her tongue and arms were neglected as the attendants took her to a local priest thinking it as an evil spirit in the hills. The patient herself complained of excessive salivation and she denied any movement disturbance. She had no antenatal health check-ups in this pregnancy. The first and second trimesters of her pregnancy had been uneventful.
Three weeks prior to admission in our hospital, she had fever, sore throat and migrating polyarthralgia involving her left wrist followed by left knee joint. Her symptoms had subsided after taking some antibiotics and analgesics from a local health centre. This gragidarum was suggestive of an episode of rheumatic fever.
However, she denied any history of taking long-term injectable penicillin for the same. There was no history of psychiatric illnesses in the past. There was also no history of any neuroleptic drug or metocloperamide intake. On examination, patient was right-handed, afebrile, pale, conscious, oriented, emotionally labile but depressed.
The tongue was darting even at rest without patient being able to control it. She had choreioathetoid, purposeless, irregular and jerky movements bilaterally in her upper limbs. The patient was constantly trying to cover her head with her cloth each time she was having choric movements.
The patient was fidgety. Tone was slightly reduced in upper limbs than lower limbs. Power and reflexes were normal in all four limbs. Sensory system and all cranial nerve examination were gravidarhm with flexor plantar response. There were no cerebellar signs and signs of meningeal irritation. There was no family history of any neurological disease. Laboratory studies were as shown in table 1. Slit-lamp examination of the eyes was normal.
The differentials which were considered on history and examination were chorea gravidarum, systemic lupus erythematosus, primary anti-phospholipid antibodies, viral encephalitis, Huntington disease and Wilson disease. The negative reports of laboratory investigations of the antinuclear antibodies and C reactive protein helped in excluding systemic lupus erythematosus and primary antiphospholipid antibody. The absence of associated symptoms of fever, coryza helped in excluding viral encephalitis.
There was no family history of Huntington disease and disease progression was graviddarum not suggestive of Huntington disease. The slit-lamp examination and copper estimation helped exclude Wilson disease. The positive history of rheumatic fever in the past and precipitation of these symptoms during pregnancy indicated towards the likelihood of chorea gravidarum which was rheumatic in origin.
The patient was admitted in a quiet isolated ward and given one dose of benzathine penicillin, iron and calcium supplements were given along with multivitamins. Attendants were advised to keep her away from her house where her father had died recently.
She was also on a chotea follow-up with a psychiatrist for counselling. However, she is still seen on follow-up and is on penicillin prophylaxis to prevent rheumatic heart disease.
[Chorea gravidarum. A case report].
Chorea is an involuntary abnormal movement characterised by abrupt, brief, non-rhythmic and non-repetitive movement of any limb, often associated with non-patterned facial grimaces. The choreic movements may be caused by ischaemia or enhanced dopaminergic sensitivity mediated by increased female hormones during pregnancy. Chorea gravidarum is considered a syndrome rather than a specific disease entity. In recent times, most cases of chorea appearing during pregnancy in developed countries were caused by disease entities like systemic lupus erythematosus and Huntington disease.
The decline in new cases of chorea gravidarum is probably the result chlrea a decline in rheumatic fever which was a major cause prior to the era when antibiotics came into use for Streptococcal pharyngitis.
However, the scenario is different in developing countries like India where rheumatic fever still occurs frequently. Group A streptococcal infection induces antibodies against the host neural tissues by a phenomenon called molecular mimicry. The chogea laboratory investigations did not show any evidence of recent streptococcal infection. As reported by Dilenge et al5 most cases of isolated Sydenham’s chorea will have negative serology. Rheumatic chorea is one of the three circumstances in which the diagnosis of acute rheumatic fever can be made without strict adherence to the Jones criteria.
The patient had mild form of chorea which was aggravated by stress of her kin’s death and it subsided with one dose of long-acting pencillin, proper seclusion, rest, multivitamins and multimineral therapy.
Patients with Sydenham’s chorea are at risk for the development of rheumatic carditis particularly mitral stenosis and to prevent this, a regimen of daily penicillin prophylaxis should be instituted and maintained. The present patient was a case of rheumatic chorea without carditis with previous history of rheumatism but she was at risk of developing rheumatic heart disease in future.
Therefore, patients with chorea, even in the absence of other manifestations of rheumatic fever, require long-term antibiotic prophylaxis. Chorea in this case was helpful to the patient as she was started on penicillin prophylaxis which would prevent development of rheumatic heart disease in future. MP was the treating physician for the patient and performed detailed clinical examination. SU was the psychiatrist who was regularly advising the patient. AS is the obstetrician who carefully assisted in the patient delivering a healthy fetus.
BSL compiled the case report and submitted it online. Provenance and peer review: Not commissioned; externally peer reviewed. National Center for Biotechnology InformationU. Published online Jun Author information Copyright and License information Disclaimer. Correspondence to Bhagat Singh Lali, moc. Abstract A pregnant woman, in her early 20s, presents with chorea following an emotional outburst.
Chorea Gravidarum. | History of Medicine | JAMA Neurology | JAMA Network
Background Chorea gravidarum is a rare movement disorder of pregnancy. Open in a separate window. Differential diagnosis The differentials which were considered on history and examination were chorea gravidarum, systemic lupus erythematosus, primary anti-phospholipid antibodies, viral encephalitis, Huntington disease and Wilson disease.
Treatment The patient was admitted in a quiet isolated ward and given one dose of benzathine penicillin, iron and calcium supplements were given along with multivitamins. Discussion Chorea is an involuntary abnormal movement characterised by abrupt, brief, non-rhythmic and non-repetitive movement of any limb, often associated with non-patterned facial grimaces.
Pregnancy is the time of changing hormonal milieu, it can lower the threshold for chorea and emotional outburst can precipitate it.
Post-streptococcal autoimmune disorders of the central nervous system. Curr Opin Neurol ; Willson P, Preece AA. Arch Intern Med ; Restricted unilateral Sydenham’s chorea: J Child Neurol ; Support Center Support Center.