Table 1 The clinical features of the two cases
No | Age (years) | Sex | Episodes | Chief complaint | Brian MRI | VEEG | CSF Cell count | CSF protein | MOG-IgG | Treatments | Follow-up |
|---|---|---|---|---|---|---|---|---|---|---|---|
1 | 0.5 | boy | 1 | Fever and drowsiness | Symmetry lesions of extensive and confluent bilateral white matter | Normal | 49 × 106/L | Normal | 1:32 | IV methylprednisolone (20 mg/kg for 3 days, halved every 3 days) and oral prednisone tapering gradually until stop taking in ten months | Fully recovered |
1.5 | 2 | Seizure | Bilateral white matter lesions were reduced and new lesions were found in the right occipital cortex | Spiky waves and spiky slow waves during both waking and sleeping periods and one focal attack were detected | Normal | Normal | 1:100 | IV methylprednisolone (20 mg/kg*3d, halved every 3 days) and gamma globulin (1 g/kg*2d), and oral prednisone tapering until 1.5 mg/kg daily | Epilepsy | ||
2.3 | 3 | Seizure, unresponsive and language barriers | Extensive leukodystrophy-like lesions, and the lesions were reduced during follow-up | Diffuse slow waves and spinous slow waves | Normal | Normal | 1:32 | Rituximab (375 mg/m2 on Days 1, 7, 15, and 22) was treated every 6 months, and oxcarbazepine and levetiracetam simultaneously | Epilepsy, cognitive degradation | ||
2 | 3.0 | boy | 1 | Fever, alteration of consciousness | Multiple white matter, basal ganglia and cortex affected (ADEM) | Diffuse slow waves(2-4 Hz) | 67 × 106/L | 0.62 g/L | 1:32 | IV methylprednisolone (20 mg/kg for 3 days, halved every 3 days) and oral prednisone tapering until 1.5 mg/kg daily | Fully recovered |
3.7 | 2 | Ataxia | Bilateral cerebellar hemispheres suffered, and multiple lessions were absorbed compared with last time | Normal | Normal | Normal | 1:32 | IV methylprednisolone (20 mg/kg for 5 days, halved every 3 days) and oral prednisone tapering until 2.0 mg/kg daily | Slight claudication | ||
4.2 | 3 | Epileptic status and poor memory | Leukodystrophy-like lesions, and the lesions were reduced during follow-up | Diffuse slow waves, spinous slow and cusp slow waves | Not done | Not done | 1:32 | Rituximab (375 mg/m2 on Days 1, 7, 15, and 22) was treated every 6 months, and levetiracetam was added orally | Cognitive impairment, dyspraxia and epilepsy |