Video 1
Clinical examination on admission in the hospital. The video shows left hemichoreic and ballistic involuntary movements.
Figure 1
Brain magnetic resonance imaging (MRI) of the patient. A: T1-weighted axial image, and B: fluid attenuated inversion recovery (FLAIR) axial image, showing normal findings in the basal ganglia and Fazekas 1 leucopathy.
Table 1
Main results of biological, imaging and functional explorations (excluding brain imaging, detailed in Figures 1 and 2).
| INVESTIGATION | MAIN RESULTS | |
|---|---|---|
| Biological analyses | Routine blood test | Lymphopenia (0.82 G/L lymphocytes, N: 1–4), INR: 3.33 (patient treated with warfarine), normal electrolytes, normal ammonium level, urea: 12.2 mmol/L (N 2;5–7.6), creatinine: 122 µmol/L (N 80–110) CRP: 15 mg/L (N < 6), liver function tests: AST: 1.5N, normal ALT, GGT: 7N, ALP: 1.5N, serum electrophoresis: hypogammaglobulinemia (5.4 g/L; N: 6–15), hypoalbuminemia (31.7 g/L; N:35–52), normal immunofixation normal TSH, T3 and T4 levels |
| Metabolism | Glycemia, HbA1c 5.3% (N < 6.5) homocysteinemia, folate and B12: Normal levels | |
| Infectious disease | HIV, HBV, HCV, Lyme, syphilis: negative serologies | |
| Auto-immunity | ANAs, ENAs, dsDNA, lupus anticoagulant, B2 glycoprotein antibody, anti-cardiolipin antibody, onconeuronal antibodies (anti-Hu, anti-Ri, anti-Yo, anti-Gad, anti-CV2, anti-Tr, anti-NMDAR, anti-AMPA1 receptor, anti-AMPA2 receptor, anti-LGI1, anti-CASPR2, anti-GABArb receptor), anti TPO antibody, anti-thyroglobulin antibody: all negative. C3, C4, CH50: normal levels, C3Nef (to be controlled in 6 months) | |
| Biology-others | Negative JAK2 mutation | |
| CSF | Clear appearance, 40/mm3 RBCs, WBC 0/mm3 (N < 5) protein: 0.3 g/L (N < 0.5), glucose 3.2 mmol/L (N < 6), presence of oligoclonal bands, IgG index 0.46 (N 0–0.25), microbiology: sterile, viral PCR (CMV, HSV, VZV, EBV): negative; onconeuronal antibodies (anti-NMDAR, anti-AMPA1 receptor, anti-AMPA2 receptor, anti-LGI1, anti-CASPR2, anti-GABArb receptor): negative. | |
| EEG | Normal | |
| Doppler ultrasound of supra-aortic trunks | Atherosclerosis without stenosis | |
| Liver ultrasound | Chronic hepatopathy, normal biliary tracts | |
[i] ALP: alkaline phosphatase; ALT: alanine aminotransferase; ANA: antinuclear antibody; AST: Aspartate aminotransferase; C3, C4, CH50: complement; C3Nef: C3 nephritic factor; CMV: cytomegalovirus; CRP: C-reactive protein; CSF: cerebrospinal Fluid; dsDNA: double-stranded DNA antibody; EBV: Epstein-Barr virus; EEG: electroencephalogram; ENA: extractable antigen antibody; GGT: gammaglutamyl transferase; HSV: herpes simplex virus; INR: International Normalized Ratio; MRI: magnetic resonance imaging; PCR: polymerase chain reaction; PET-FDG: 18-F-fluorodeoxyglucose-positron emission tomography; RBC: red blood cell; SUV: standardized uptake value; TPO: thyroperoxydase; TSH: thyroid stimulating hormone; VZV: varicella zoster virus; WBC: white blood cell.
Figure 2
[18F]-fluorodeoxyglucose-positron emission tomography (FDG-PET) of the patient. A: FDG-PET before corticosteroid treatment, showing increased metabolism of the right putamen as compared to the left side (standardized uptake value (SUV) of 10.19 on the right side versus 8.77 SUV on the left, i.e. a difference of 16.2%); B: FDG-PET after corticosteroid treatment, showing resolution of the right putamen increased metabolism (12.96 SUV on the right side versus 12.17 on the left side, i.e. a difference of 6.5%).
Video 2
Clinical examination the day after treatment with corticosteroids. The video shows a dramatic improvement of the abnormal movements shown in Video 1.