Clinical, laboratory, and radiology features of 2 children with refractory small-vessel primary angiitis of the central nervous system before treatment with infliximab.
| Characteristics | Case 1 | Case 2 |
|---|---|---|
| Clinical features | ||
| Focal neurological deficits | Ataxia | Bilateral visual loss |
| Dysarthria | ||
| Diffuse neurological deficits | Headaches | Headaches |
| Seizures | Generalized tonic-clonic | Generalized tonic-clonic |
| Systemic features | Fever | Fever |
| Fatigue | Fatigue | |
| Weight loss | ||
| Laboratory features | ||
| ESR, mm/h | 80 | 115 |
| CRP, mg/l | < 0.6 | 24.9 |
| vWF antigen, IU/ml | 1.59 | 2.24 |
| Cerebrospinal fluid | ||
| WBC × 106/l | 60 (26% lymphocytes) | 20 (52% lymphocytes) |
| Protein, g/l | 0.59 | 0.48 |
| Oligoclonal bands | Normal | Normal |
| Radiologic features | ||
| MRI lesions | ||
| Multifocal | Yes | Yes |
| Bilateral | Yes | Yes |
| Gray matter | Yes | Yes |
| White matter | Yes | No |
Reference values: ESR 1–10 mm/h; CRP 0–8.0 mg/ml; vWF antigen 0.47–1.39 IU/ml (blood group O), 0.84–1.92 (non-blood group O); cerebrospinal fluid WBC count 0–3 × 106/l, protein 0.15–0.40 g/l. ESR: erythrocyte sedimentation rate; CRP: C-reactive protein; vWF: von Willebrand factor; WBC: white blood cell; MRI: magnetic resonance imaging.