Trattamento corticosteroideo versus terapia convenzionale nella corea reumatica

The present retrospective observational study on thirty children with Sydenham chorea shows that steroid treatment seems to be more effective than symptomatic treatment in both clinical remission and clinical improvement of symptoms.

Atypical presentation of rheumatic fever

Sydenham Chorea (St. Vitus dance) occurs in about 10-15% of children with acute rheumatic fever. Herein, we present the case of a 5-year-old male child with hemichorea and arthralgia. The child also presented with mild mitral regurgitation and mild aortic regurgitation. Appropriate management is essential to prevent mortality, morbidity, and psychosocial disability in such cases. We would also like to shed light on the challenges faced in the management of chorea in young children with key emphasis on the anticipation of adverse reactions to commonly used medications.

Sydenham’s chorea in Sudan

Background: Sydenham’s chorea (SC), is the most common form of acquired chorea in childhood, it is considered as neurological complication of streptococcal pharyngitis. Our aim was to determine the clinical pattern, association of Sydenham’s chorea with other manifestation of ARF and the laboratory findings of Sydenham’s chorea among Sudanese patients.Methods: This study is descriptive , retrospective cross- sectional study. Fifty patients with different age groups, were diagnosed as having Sydenham’s chorea & followed up at The National center for Neurological sciences, in the period (Jan2017 to Nov2019). Data were obtained after patients consent of by personal interview or personal review of patients records through a designed questionnaire including demographic data, symptoms, co morbid illness, risk factors, physical examination and related investigations.Results: The patients’ median age was 13.7 years: 88% of the cases occurred between 7-17 years with female predominance(35) . Generalized chorea was seen in 33 (66%) and hemichorea 17 (34%) patients. weakness and hypotonia were common, behavior change (44%), dysarthria (71%), gait change(17.20%)and deterioration of handwriting (13%) Arthritis occurred in (36%), carditis 30 (60%), arthritis and carditis in 18(36%), and pure chorea 14 (28%). Erythema marginatum and subcutaneous nodules were not observed in our patients. Only13 patients (26%) gave a history of pharyngitis.Conclusion : There were clear evidence of familial predisposition. Sydenham’ chorea commonly presents acutely in majority of patients. Chorea firstly appeared with oro-fascial movement followed by limb involvement. Minority of patients gave a history of pharyngitis. Brain imaging was normal.

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

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.

Acute chorea: case series from the emergency room of a Brazilian tertiary-level center

ABSTRACT Background: Chorea is a movement disorder characterized by random, brief and migratory involuntary muscle contractions. It is defined as acute when present within hours to days. Three main causes for this scenario have emerged as most likely: vascular, toxic-metabolic and inflammatory. Objectives: To identify the prevalence of the main etiologies and major clinical findings of acute chorea in the emergency room of a tertiary-level referral center; and to suggest an approach for guiding the diagnostic workup and clinical management. Methods: We retrospectively reviewed the clinical aspects and neuroimaging data of 10 patients presenting with acute chorea at the neurological emergency room of our hospital from 2015 to 2019. Results: Stroke was the most common etiology (50% of the cases). All of them were ischemic. It was noteworthy that only one case demonstrated the classical ischemic topographic lesion at the contralateral subthalamic nuclei. Regarding nonvascular etiologies, nonketotic hyperglycemia was the major cause, followed by drug-related chorea. One patient showed inflammatory etiology, which was probably Sydenham chorea reactivation. Conclusion: Acute chorea is an uncommon and challenging problem at the emergency room, often associated with potentially treatable causes. We suggest that use of the acronym DANCE (Diagnosis of chorea, Acute stroke protocol, Normal glucose levels, Check neuroimaging, Exposure to drugs) could form a potential initial approach in the evaluation, in order to emphasize causes that require prompt proper management (e.g. thrombolysis).