Background
Methods and materials
Clinical studies
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 |
Neuropsychological studies
Tissue samples
Neuropathological studies
Human neural cell cultures
Real-time RT-PCR
Gene | Primer |
---|---|
ASC
| Forward 5′-GCC TGC ACT TTA TAG ACC AGC-3′ |
Reverse 5′-GCT TCC GCA TCT TGC TTG G-3′ | |
CASP1
| Forward 5′-TCC AAT AAT GGA CAA GTC AAG CC-3′ |
Reverse 5′-GCT GTA CCC CAG ATT TTG TAG CA-3′ | |
CD3e
| Forward 5′-GAT GCA GGG CAC TCA CT-3′ |
Reverse 5′-CAT TAC CAT CTT GCC CCC AA-3′ | |
GAPDH
| Forward 5′-AGC CTT CTC CAT GGT GGT GAA GAC-3′ |
Reverse 5′-CGG AGT CAA CGG ATT TGG TCG-3′ | |
HLA-DRA
| Forward 5′-GGA CAA AGC CAA CCT GGA AA-3′ |
Reverse 5′-AGG ACG TTG GGC TCT CTC AG-3′ | |
IL1b
| Forward 5′-CCA AAG AAGAAG ATG GAA AAG C-3′ |
Reverse 5′-GGT GCT GAT GTA CCA GTT GGG-3′ | |
IL18
| Forward 5′-TCT TCA TTG ACC AAG GAA ATC GG-3′ |
Reverse 5′-TCC GGG GTG CAT TAT CTC TAC-3′ | |
NLRP1
| Forward 5′-ATT CCA GTT TGT GCG AAT CCA-3′ |
Reverse 5′-GTT CCT TGG GGA GTA TTT CCA G-3′ | |
NLRP3
| Forward 5′-GAT CTT CGC TGC GAT CAA CAG-3′ |
Reverse 5′-CGT GCA TTA TCT GAA CCC CAC-3′ | |
NLRC4
| Forward 5′-TGC ATC ATT GAA GGG GAA TCT G-3′ |
Reverse 5′-GAT TGT GCC AGG TAT ATC CAG G-3′ | |
CD8
| Forward 5′-CCG ACG ATC ATG CAG AAG TA-3′ |
Reverse 5′-GGT GAA GAG GTG GAA CAG GA-3′ | |
GFAP
| Forward 5′-GAG ATC GCCACC TAC AG-3′ |
Reverse 5′-CAC ATC CTT GRG CTC CTG-3′ |