Abstract

Rheumatic chorea (RC), also known as Sydenham’s chorea, is a late manifestation of acute rheumatic fever (RF) and represents one of the main diagnostic criteria of the disease. RC is a well-recognized autoimmune movement disorder elicited by group A beta-hemolytic streptococcal (GABHS) pharyngitis. Clinically, RC is characterized by involuntary arrhythmic movements and cognitive and behavioral symptoms. RC supposedly results from basal ganglia dysfunction triggered by cross-reactive antibodies produced during GABHS infection. Recent studies have unveiled specific antibody targets in the basal ganglia possibly implicated in neural circuits dysfunction and, therefore, in the emergence of neurological symptoms. In this chapter, we summarize current evidence on clinical and pathophysiological aspects of RC.

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