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Multiple Choice

Which childhood movement disorder is a complication of rheumatic fever and presents with choreiform movements, more common in girls, and is associated with hyperactivity and OCD?

Sydenham chorea is an autoimmune movement disorder that can follow rheumatic fever in children. It presents with involuntary, irregular choreiform movements and is more common in girls. Behavioral changes are typical, with hyperactivity and obsessive–compulsive features often appearing, reflecting involvement of the basal ganglia circuits. The underlying mechanism involves antibodies generated after a group A streptococcal infection that cross-react with neurons in the basal ganglia, leading to the characteristic motor and behavioral symptoms. Onset usually occurs weeks to months after the streptococcal illness and may be accompanied by motor incoordination and emotional lability; it often resolves gradually over months to a year, though recurrences can happen and long-term penicillin prophylaxis is used to prevent future rheumatic fever. Hemiballismus arises from a lesion in the subthalamic nucleus and is typically unilateral with wild flinging movements, not tied to rheumatic fever. Huntington disease–related chorea is a genetic neurodegenerative condition usually seen in adults (though juvenile forms exist) with a progressive course and cognitive/psychiatric symptoms, not specifically linked to rheumatic fever. Blepharospasm is a focal dystonia of the eyelids, not a generalized chorea linked to rheumatic fever.

Sydenham chorea is an autoimmune movement disorder that can follow rheumatic fever in children. It presents with involuntary, irregular choreiform movements and is more common in girls. Behavioral changes are typical, with hyperactivity and obsessive–compulsive features often appearing, reflecting involvement of the basal ganglia circuits. The underlying mechanism involves antibodies generated after a group A streptococcal infection that cross-react with neurons in the basal ganglia, leading to the characteristic motor and behavioral symptoms. Onset usually occurs weeks to months after the streptococcal illness and may be accompanied by motor incoordination and emotional lability; it often resolves gradually over months to a year, though recurrences can happen and long-term penicillin prophylaxis is used to prevent future rheumatic fever.

Hemiballismus arises from a lesion in the subthalamic nucleus and is typically unilateral with wild flinging movements, not tied to rheumatic fever. Huntington disease–related chorea is a genetic neurodegenerative condition usually seen in adults (though juvenile forms exist) with a progressive course and cognitive/psychiatric symptoms, not specifically linked to rheumatic fever. Blepharospasm is a focal dystonia of the eyelids, not a generalized chorea linked to rheumatic fever.