An 8-year-old girl is brought to the physician for abnormal behavior. She has been acting inappropriately, crying, and laughing without apparent reason for the past 2 weeks. At school, she was restless and easily distracted. Last week, her parents noted strange movements of the girl's hands that improved when she slept. On the day before presentation, her parents noticed rapid jerking movements of her face and feet. The girl has had minor, self-resolving upper respiratory infections but has otherwise been healthy. She takes no medications and her vaccinations are up-to-date. Examination shows an alert and cooperative girl with normal vital signs. Facial grimacing and tongue fasciculations are seen. The lungs are clear to auscultation and a II/VI holosystolic murmur is heard at the apex. When the girl speaks, distinct changes in the volume and pitch of her voice are noted. Muscular tone is mildly decreased globally. Pronator sign is positive, and the relaxation phase of the patellar reflex is delayed.
Which of the following is the most likely diagnosis?

Question

An 8-year-old girl is brought to the physician for abnormal behavior.  She has been acting inappropriately, crying, and laughing without apparent reason for the past 2 weeks.  At school, she was restless and easily distracted.  Last week, her parents noted strange movements of the girl's hands that improved when she slept.  On the day before presentation, her parents noticed rapid jerking movements of her face and feet.  The girl has had minor, self-resolving upper respiratory infections but has otherwise been healthy.  She takes no medications and her vaccinations are up-to-date.  Examination shows an alert and cooperative girl with normal vital signs.  Facial grimacing and tongue fasciculations are seen.  The lungs are clear to auscultation and a II/VI holosystolic murmur is heard at the apex.  When the girl speaks, distinct changes in the volume and pitch of her voice are noted.  Muscular tone is mildly decreased globally.  Pronator sign is positive, and the relaxation phase of the patellar reflex is delayed. 
Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

Explanation: 11ec2b69_bfbc_babe_8f63_25b242917880_MD0005_00 Acute rheumatic fever (ARF) is a complication of untreated streptococcal pharyngitis. Patients and families may not remember the initial self-resolving infection. The diagnosis is based on the J♥NEScriteria (Table). Two major or 1 major and 2 minor criteria are required, although the diagnosis can be made on the presence of Sydenham chorea (SC) or carditis alone. This patient's examination findings are classic for SC (also known as Saint Vitus dance), the most common acquired chorea in children. Emotional lability and decline in school performance are typically the earliest neurologic manifestations, followed by distal hand movements that progress to facial grimacing and feet jerking. Movements are typically irregular and rapid. Patients also have decreased strength throughout, and the relaxation phase of the patellar reflex is usually delayed. In addition, pronator drift (involuntary hyperpronation of extended arms) is present. The diagnosis is based on clinical presentation. SC usually develops 1-8 months after the initialstreptococcal infection; carditis and arthritis develop within 3 weeks. This patient's murmur is concerning for carditis and should be evaluated by echocardiography. (Choice A) Attention deficit hyperactivity disorder is characterized by inattention, impulsivity, and hyperactivity that persist for >6 months. The physical examination is otherwise normal. (Choice B) Huntington disease is an autosomal dominant disorder caused by a trinucleotide repeat expansion. The psychiatric symptoms, chorea, and dementia typically manifest at age 30-50; onset is uncommon during childhood. (Choice D) Tardive dyskinesia is a movement disorder that occurs in patients on dopamine receptor-blocking agents (eg, antipsychotic agents, metoclopramide). Chorea is a side effect of these medications; this child takes no medications. (Choice E) Tourette syndrome is characterized by involuntary motor and vocal tics that begin at age 2-15. The physical examination is otherwise normal and chorea is not seen. Educational objective: Sydenham chorea is one of the major manifestations of acute rheumatic fever and is the most commonly acquired chorea in children. It is characterized by emotional lability and irregular, rapid jerking movements of the face, hands, and feet. References: Sydenham's chorea in western Pennsylvania. (http://www.ncbi.nlm.nih.gov/pubmed/16533893) Prevention of rheumatic fever and diagnosis and treatment of acute Streptococcal pharyngitis: a scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research: endorsed by the American Academy of Pediatrics. (http://www.ncbi.nlm.nih.gov/pubmed/19246689) Seminar on choreas. (http://www.ncbi.nlm.nih.gov/pubmed/16781989)

An 8-Year-Old Girl Is Brought to the Physician for Abnormal