A 10-year-old boy is brought to the physician by his parents due to restlessness and involuntary jerking. He takes no medications and his vaccinations are up-to-date. His parents do not recall any recent injuries or illnesses other than a sore throat 3 months ago. On examination, the patient has rapid, irregular jerking movements involving his face, arms, and legs. This patient is at greatest risk for developing which of the following conditions?

Question

A 10-year-old boy is brought to the physician by his parents due to restlessness and involuntary jerking.  He takes no medications and his vaccinations are up-to-date.  His parents do not recall any recent injuries or illnesses other than a sore throat 3 months ago.  On examination, the patient has rapid, irregular jerking movements involving his face, arms, and legs.  This patient is at greatest risk for developing which of the following conditions?

Quizplus · Accepted Answer

11ec160b_38e9_2eef_9249_934626e68c99_MD0001_00 This patient's restlessness and purposeless jerking movements 3 months after having a sore throat are consistent with Sydenham chorea, a hyperkinetic extrapyramidal movement disorder that is the most common acquired chorea of childhood. Sydenham chorea is a neurologic manifestation of acute rheumatic fever that occurs 1-8 months after group A β-hemolytic streptococcal infection. It is caused by a delayed onset autoimmune reaction involving anti-streptococcal antibodies that cross-react with the basal ganglia. Because Sydenham chorea is a manifestation of acute rheumatic fever, patients with this disorder are at risk for chronic rheumatic heart disease. (Choice A) Although migratory polyarthritis is an early manifestation of acute rheumatic fever, it is generally transient and rarely, if ever, deforming. (Choice B) Early-onset Alzheimer dementia is associated with Down syndrome (trisomy 21). Increased expression of the amyloid precursor protein gene located on chromosome 21 is thought to be the cause. (Choice C) Parkinson disease is an extrapyramidal hypokinetic movement disorder consisting of tremor, rigidity, akinesia, and postural instability. Jerking extremity movements are generally not seen in Parkinson disease, except in cases of levodopa overdose. (Choice D) Poststreptococcal glomerulonephritis occurs following infection with specific nephritogenic strains of group A Streptococcus. However, the nephritis manifests within 1-6 weeks and is typically self-limited. Renal failure is not part of the clinical syndrome associated with acute rheumatic fever. Educational objective: Sydenham chorea presents with involuntary, rapid, irregular jerking movements involving the face, arms, and legs. It occurs months after group A streptococcal infection and is one of the major clinical manifestations of acute rheumatic fever. Patients with this condition carry a high risk of chronic valvular disease. __________ References: Diagnostic criteria of acute rheumatic fever. (http://www.ncbi.nlm.nih.gov/pubmed/24424191)

A 10-Year-Old Boy Is Brought to the Physician by His