Revisiting an immunoneuropsychiatry classic: Sydenham's chorea

Area of Science:

• Neurology
• Immunology
• Rheumatic Diseases

Background:

• Sydenham chorea (SC) is the primary neurological symptom of rheumatic fever.
• It involves involuntary movements and neuropsychiatric issues post-Group A streptococcal (GAS) infection.

Purpose of the Study:

• To review recent studies on SC pathogenesis, clinical features, and treatments.
• To synthesize current understanding of SC's autoimmune basis and therapeutic strategies.

Main Methods:

• Literature search of the PubMed database.
• Inclusion of original studies and systematic reviews on SC.

Main Results:

• Autoantibodies targeting neuronal antigens, particularly dopamine D2 receptors in the basal ganglia, are central to SC pathogenesis.
• This leads to dopaminergic dysfunction, causing motor and neuropsychiatric symptoms.
• A significant knowledge gap exists regarding how peripheral autoantibodies access the basal ganglia.

Conclusions:

• SC pathogenesis is strongly linked to autoantibodies affecting basal ganglia function.
• Symptomatic treatments include D2 antagonists and valproic acid.
• Corticosteroids, plasmapheresis, and immunoglobulin are considered for severe or refractory cases.