Management, treatment, and clinical approach of Sydenham's chorea in children: Italian survey on expert-based...

Area of Science:

• Neurology
• Immunology
• Pediatrics

Background:

• Sydenham's chorea (SC) is an autoimmune neurological disorder and a key diagnostic criterion for acute rheumatic fever.
• It primarily affects children (ages 5-15), particularly in developing nations, presenting with involuntary movements and neuropsychiatric symptoms.
• Pathophysiology involves an autoimmune response targeting the basal ganglia, especially against dopamine.

Purpose of the Study:

• To investigate current management practices and identify discrepancies in Sydenham's chorea treatment among Italian specialists.
• To establish a consensus on diagnostic and therapeutic approaches for SC.

Main Methods:

• A survey was distributed across Italy to neurologists, pediatricians, child neuropsychiatrists, and rheumatologists.
• Fifty-one physicians responded, providing data on their diagnostic and treatment preferences for SC.

Main Results:

• A consensus favored hospitalization for suspected SC, alongside laboratory tests and brain MRI.
• Oral prednisone and IVIG were commonly agreed upon treatments, but duration and plasmapheresis showed varied opinions.
• Haloperidol was the preferred symptomatic treatment, with strong support for post-SC penicillin prophylaxis and steroid therapy, though durations differed.

Conclusions:

• Significant agreement exists on initial management and some therapies for SC, but treatment protocols require further standardization.
• Neuropsychological and cardiological follow-up assessments are recommended.
• Further research is needed to guide evidence-based treatment decisions for this challenging rheumatic fever complication.