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 |
CPS, complex partial seizure; EPC, epilepsia partialis continua; GCs, glucocorticoids; Gen, generalized seizures; IVIg, intravenous immune globulin; RE, Rasmussen’s encephalitis; SPT, subpial transaction.