COMPLEMENTARY STUDY | PATIENT 1 (8 years old) | PATIENT 2 (10 years old) |
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Blood test | Normal results for: blood count (hemoglobin 12.9 mg/dL, leukocytes 6300/mm3, platelets 302,000/mm3), biochemistry, liver enzymes, folic acid, vitamin B12, creatine kinase, ammonium, homocisteyne, copper, ceruloplasmin and thyroid hormones. CRP 1.1 mg/dL, ESR 20 mm/h, Anti-streptolysin O 458 IU/mL (ULN for age 265 IU/mL) [10] | Normal results for: blood count (hemoglobin 12.8 mg/dL, platelets 318,000/ mm3) except leucocytes 16,010/mm3 and 70% neutrophils, biochemistry, liver enzymes, copper, ceruloplasmin and thyroid hormones. CRP 12 mg/dL, ESR 96 mm/h, Anti-streptolysin O 956 IU/Ml (ULN for age 276 IU/mL) [10], Anti DNAse B > 1600 IU/mL (ULN for age 499 IU/mL) [10] |
Auto-antibodies | ANA, anti-transglutaminase antibodies, anti-gliadin antibodies, anti-phospholipid antibodies, anti-thyroid antibodies: negative. | ANA, anti-double stranded DNA antibodies, rheumatoid factor: negative |
Cerebrospinal fluid (CSF) test | Clear and colourless, red blood cells 0/μL, leukocytes 4/μL, protein 61 mg/dL, glucose 16 mg/dL | |
Neurotropic virus polymerase chain reaction in CSF | Varicella-zoster virus, herpes simplex 1 and 2 virus, enterovirus, Epstein-Barr virus, citomegalovirus, human herpesvirus 6, 7 and 8: negative | |
Electroencephalogram | Normal | Normal |
Cranial computerized tomography without contrast | Normal | Not interpretable because of the chorea movements |
Brain magnetic resonance imaging (with and without contrast) | No anatomic alterations. Ventricular system with normal morphology and size. There are no white matter signal intensity alterations suggesting pathology. No restriction areas are observed in diffusion sequences. | Anatomy of supratentorial structures and posterior fossa are normal. Normal craniocervical junction. Ventricular system with normal morphology and size. There are no white matter signal intensity alterations suggesting pathology. No restriction areas are observed in diffusion sequences. |
S. pyogenes pharyngeal culture | Negative | Negative |
Chest x-ray | Not performed | Normal |
Electrocardiogram | Normal (PR 130 ms, no block heart) | Normal sinus rhythm, heart rate 100 bpm. QRS axis 75°. PR interval 136 ms. QTc: 440 ms. No hypertrophy signs. |
Echocardiogram | Leaflet thickening anterior mitral valve with mild mitral insufficiency and mitral valve prolapse. Aortic valve with 16.7 mm diameter of the aortic annulus and thickening resulting in mild aortic insufficiency. | Mild-moderate mitral insufficiency. Dilation of the left ventricle. |