Chorea as the presenting feature of acute rheumatic fever in childhood; case reports from a low-prevalence European setting

Abstract

Background Despite a notable decrease in acute rheumatic fever (ARF) incidence in the past few decades, there are still cases in our setting. Sydenham chorea (SC) may be the initial manifestation for this condition in childhood in a significant proportion of children. We report two cases of choreoathetosis in children as the first manifestation of ARF. Case presentation A previously healthy 8-year-old boy presented with right hemichorea with a predominance in the brachial region, orofacial dyskinesias and speech difficulties for the past 2 weeks. The only medical history of interest was a common catarrhal illness 3 weeks before and nonspecific bilateral tenosynovitis in both feet since a year prior. A brain computerized tomography was normal and the echocardiogram showed mild mitral and aortic regurgitation, meeting ARF criteria. He demonstrated clinical improvement with treatment based on prednisone and carbamazepine. The second patient was a 10-year-old girl with choreic movements of the right half of the body and repetitive right eye closure of 1 week duration. She had symptoms of fever and rash the previous week and pharyngitis that resolved without antibiotic 2 months before. Blood tests revealed elevated C reactive protein (12 mg/dl) and erythrocyte sedimentation rate (96 mm/h). Brain magnetic resonance was normal and echocardiogram showed left ventricle dilation and mild mitral regurgitation, leading to the diagnosis of ARF. Due to neurological involvement, she received corticosteroids and intravenous immunoglobulin treatment, with worsening of neurological symptoms that required valproic acid with remission of the hemichorea. In addition skin lessions compatible with erythema marginatum appeared on the upper limbs. Conclusions SC should be the main diagnostic consideration in cases of hemichorea with normal neuroimaging in children. The cases reported highlight the need to maintain a high index of suspicion even in settings where incidende of ARF is low and the need to perform cardiological investigations in all patients with suspected SC, due to the possibility of subclinical valve lesions. Good adherence to secondary prophylaxis is crucial to avoid chorea relapses and worsening valve disease.

One Side of the Story; Clues to Etiology in Patients with Asymmetric Chorea

“…Sydenham’s disease is the most common cause of acquired chorea in children, and often presents asymmetrically or unilaterally [ 8 71 ]. Although there are few cases currently seen in resource-rich countries, it remains quite prevalent in many resource-limited countries [ 71 ]. Although known for its asymmetry, in one follow-up study of 65 children, 78% were reported as generalized versus 22% hemichorea.…”

“…The etiology of Sydenham’s disease is proposed to be an autoimmune response generated by antibodies to streptococcus A, often in the setting of rheumatic fever. Onset of symptoms is generally 1–8 months after the acute infection, although cases are reported of abnormal movements as the presenting symptom [ 71 72 ].…”

Treatments and Outcomes Among Patients with Sydenham Chorea

“…We identified 1479 patients with IPD (median [IQR] age at onset, 10 [8-13] years in 1354 patients; 985 of 1426 [69.1%] female and 441 of 1426 [30.9%] male) were identified from 307 articles…”

Sydenham’s chorea in a female child with rheumatic fever in Bhutan: a case report

“…Sydenham chorea represents the most common cause of acquired chorea in childhood [1]. It is one of the major criteria for the diagnosis of acute rheumatic fever [2]. In Sydenham chorea there is both neurological abnormalities and psychiatric disorder.…”

“…The neurological abnormalities comprise of involuntary choreatic movements, incoordination of voluntary movement, muscular weakness and hypotonia [3]. Psychiatric disorders include emotional lability, hyperactivity, distractibility, obsessions and compulsions [2,3]. Choreatic movements are involuntary, irregular, purposeless, non-rhythmic, abrupt, rapid and unsustained.…”