Abnormal movements x1 week
7yF w/ no PMH brought in by her parents with progressively worsening involuntary movements x 1 week, slurred speech x 3 days. The movements improve but persist during sleep. No trouble eating/swallowing or handling her secretions. She has remained alert, coherent, interactive over this time course.
PMH / PSH
PMH / PSH: none
Afebrile, HR 91, 112/77, RR 22, 98% RA
Gen: AO x 3, NAD, nontoxic
HEENT: Atraumatic, no signs of injury, EOMI, conj wnl, PERRL
Cardiovascular: s1s2, regular rhythm, 2/6 systolic murmur loudest over apex
Pulmonary: CTAB, no wheeze, no rhonchi, no crackles, chest wall WNL
Abdominal: Scaphoid, soft, no distention, no mass, no tenderness, no hernia
Skin: warm, dry
Neuro: + dysarthria. Persistent, involuntary, flailing movements of entire body, seemingly at random, limbs > trunk (see video)
SILT throughout, 5/5 strength throughout. Normal tone. Normal coordination, no pronator drift, no ataxia
What tests would confirm your diagnosis?
TTE, ASO titers, Throat Culture, ESR/CRP levels
The diagnosis of Rheumatic Fever is predominantly clinical and made using the Jones Criteria. There must be evidence of preceding GAS infection AND the patient must exhibit 2 major manifestations OR 1 major + 2 minor manifestations.
What is your therapy of choice?
1. Eradicate GAS with either IM Penicillin G x 1 or Amoxicillin x 10 days
2. Prophylaxis against future GAS with IM Penicillin G every 28 days or Oral Azithromycin every day
3. Symptomatic relief: most cases of Sydenham Chorea self resolve within a few weeks but for distressing symptoms consider dopamine receptor blockade, carbamazepine, VPA, steroids. For severe chorea consider steroids, IVIG, plasmapharesis. Arthritis can be managed with aspirin + NSAIDs
What is the expected prognosis?
Most patients make a full recovery within a few weeks. However, our patient’s chorea continued to worsen despite a prescribed course of prednisone upon discharge home. She followed up with her neurologist at an outside hospital and was subsequently admitted for IVIG and plasmapheresis. She responded well to therapy and has recovered nearly back to baseline.
Our patient is suffering from Sydenham chorea, a manifestation of Acute Rheumatic Fever.
ARF is a nonsuppurative sequela of group A Streptococcus (GAS) pharyngitis. The mechanism is likely cross reacting antibodies against a person’s own tissues. Genetics and underlying serotypes (strongly influenced by hygiene – thus the lower rates of disease in developed countries… but that’s a discussion onto itself) play a strong role in who develops the disease.
The diagnosis of the disease is predominantly clinical and made using the Jones Criteria. There must be evidence of preceding GAS infection AND the patient must exhibit 2 major manifestations OR 1 major + 2 minor manifestations.
- Sydenham’s chorea
- Subcutaneous nodules
- Erythema marginatum
- Elevated ESR/CRP
- Prolonged PR on ECG
So, how do you establish a preceding GAS infection? By using laboratory markers as surrogates: positive strep culture, elevated and/or rising ASO titers and elevated ESR/CRP levels.
However, there are 3 important exceptions:
- Chorea as the only manifestation
- Indolent carditis as the only manifestation
- Individuals with a history of rheumatic heart disease
Why do these matter? Because chorea (and carditis) often develop > 4 weeks after initial infection at which point ASO titers and inflammatory markers begin to fall.
In our patient, the chorea would have been diagnostic alone. However, she did have a murmur + mitral insufficiency on echo (major criteria), and elevated ASOt + ESR/CRP levels (minor criteria).
Three major goals of treatment:
- 1. Eradicate GAS – 2 options:
- Intramuscullar Penicillin G x 1
- Amoxicillin x 10 days
- Prophylaxis against future GAS – 2 options:
- IM Penicillin G every 28 days
- Oral Azithromycin every day
- Symptomatic relief
- Sydenham Chorea Management:
- Most cases self resolve within a few weeks
- For distressing symptoms: dopamine receptor blockade, carbamazepine, VPA, steroids
- For severe chorea: steroids, IVIG, plasmapharesis
- Arthritis Management: Aspirin + NSAIDs
- Sydenham Chorea Management:
Most patients make a full recovery within a few weeks.
- Dajani AS, Ayoub E, Bierman FZ, Bisno AL, Denny FW, Durack DT, Ferrieri P, Freed M, Gerber M, Kaplan EL, Karchmer AW, Markowitz M, Rahimtoola SH, Shulman ST, Stollerman G, Takahashi M, Taranta A, Taubert KA, Wilson W, Durack . Guidelines for the Diagnosis of Rheumatic FeverJones Criteria, 1992 Update. JAMA.1992;268(15):2069-2073. doi:10.1001/jama.1992.03490150121036
- Gerber MA, Baltimore RS, Eaton CB, et al. 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. Circulation 2009; 119:1541.