Table 1 Clinical features of RE patients
Patient 1 | Patient 2 | Patient 3 | Patient 4 | |
|---|---|---|---|---|
Age of onset, gender | 3 years, female | 8 years, female | 14 years, male | 12 years, female |
Seizure type | CPS, Gen, right face/hand EPC | CPS, right face/hand EPC | CPS, right face/hand EPC | CPS, right face/hand EPC |
Hemisphere | Left | Left | Bilateral | Left |
Neurologic deficit(s) | Right hemiparesis | Right hemiparesis | Right hemiparesis, aphasia | Right hemiparesis |
Neuroimaging | Cortical and white matter signal | Cortical and white matter signal | Cortical and white matter signal | Cortical and white matter signal |
Surgery, age | Left hemispherectomy, 4 years | Left hemispherectomy, 11 years | SPT, 17 years; left face/hand area resection, 17 years | Left face/hand area resection, 18 years |
Neuropathology | Mononuclear cell infiltration with gliosis | Mononuclear cell infiltration with gliosis | Mononuclear cell infiltration with gliosis | Mononuclear cell infiltration with gliosis |
Clinical outcome | Engel class I, seizure-free (at 14 months) | Engel class I, seizure-free (at 24 months) | Engel class IV, death | Engel class III, 2 years (lost to follow-up) |
Treatment(s) | GCs, no benefit | GCs, good response | GCs, IVIg, tacrolimus, cyclophosphamide (50 mg/kg) | GCs, IVIg, methotrexate, no benefit |