Table 2.
Demographic, clinical, and paraclinical features, and long‐term outcomes of antibody‐positive epilepsy patients
| Case no./timing of sample (from onset of epilepsy, days) | Age and sex | Seizure type at onset | Type of epilepsy/etiology | Associated clinical features | Treatment history first 5 years/at end of follow‐up (FU, years) | Epilepsy course/seizure type at end of 5 year follow‐up/outcome (TR, years) | Ab positivity (pm/titers) |
|---|---|---|---|---|---|---|---|
| 1 (33) | 4 F | CPS | Localization related symptomatic/remote symptomatic | Right hemiplegia Learning difficulties |
4 AEDS: no fast response/off AED at final FU (14.6) | Improving course SPS, SE, unclear seizures TR = 10.2 |
VGKC (712 pm)a 252 pm at 12 months |
| 2 (0) | 1.7 M | TC | Localization‐related symptomatic/cryptogenic | Mental retardation, not progressive | 3 AEDs: no fast response/on AED at final contact (14.7) |
Poor course TC, SPS, CPS, unclear seizures TR < 1 year, intractable |
VGKC (480 pm)a 25 pm at 12 months |
| 3 (2,333) | 7.4 F | TC | Generalized idiopathic/idiopathic | Mild developmental delay | No AEDs; not on AED at final contact (15.3) |
Improving course TC, unclear seizures TR = 1.5 |
VGKC (414 pm) |
| 4 (365) | 7.4 M | Absences | Generalized idiopathic childhood absence epilepsy/idiopathic | – | 2 AEDs: fast response/off AED at final contact (15.7) |
Good course absences TR = 15.6 |
NMDAR (1 in 100) 1 in 20 at 12 months |
| 5 (106) | 1.5 F | Minor motor and absences | Generalized idiopathic childhood absence epilepsy/idiopathic | – | 2 AEDs: no response (bad compliance)/on AED at final contact (13.5) |
Improving course Minor motor and absences TR = 1.7 |
NMDAR (1 in 100) Negative at 12 months |
| 6 (4) | 12.6 F | TC | Generalized idiopathic with photosensitivity/remote symptomatic | Mild learning difficulties (LD) –attended regular school | 1 AED: fast response/on AED at final contact (13.1) |
Good course TC TR = 13.1 |
NMDAR (1 in 20) Negative at 12 months |
| 7 (0) | 12 M | SE | Localization related symptomatic/remote symptomatic |
Learning difficulties, IQ < 50 Tetraparesis |
2 AEDs: no fast response/on AED at death (died 1.4 years after enrollment) |
SE, unclear small seizures TR = 1 |
NMDAR (1 in 20)a |
| 8 (11) | 3.6 F | Atonic seizures | Generalized Lennox‐Gastaut syndrome/remote symptomatic | Mild global delay | 2 AEDs: no fast response/on AED at final FU (12.7) |
Deteriorating course Atonic seizures, TC TR = 0.5 |
NMDAR (1 in 20)a
Negative at 6 months |
| 9 (89) | 6.6 F | Absences | CAE/idiopathic | – | 1 AED: fast response/off AED at final FU (14.8) |
Good course Absences TR = 14.8 |
NMDAR (1 in 20)a
Negative at 12 months |
| 10 (0) | 15.5 F | TC | IGE/idiopathic | – | No AED/off AED at final FU (2) |
Lost to follow‐up after 2 years TC TR = 2 |
NMDAR (1 in 20) |
| 11 (46) | 4.1 M | SE | Localization related symptomatic/RS including MR | Mild learning difficulties, autism spectrum disorders | 1 AED: fast response/off AED at final FU (15.9) |
Good course SE TR 12.1 |
Contactin‐2 (1 in 100)a |
| 12 (124) | 4.2 M | Unclear seizures | Remote symptomatic including MR | Global mental retardation, spasticity, visual problems | 1 AED: fast response/on AED at final FU (5) |
Improving course TC with focal onset TR > 2 |
Contactin‐2 (1 in 100) |
| 13 (271) | 8.8 F | Atonic, astatic | BECTS/idiopathic | – | 3 AED: no fast response/off AED at final FU (5) |
Improving course SPS with generalization TR > 2 |
Contactin‐2 (1 in 100) |
| 14 (31) | 12.5 M | SPS | Benign partial epilepsy/idiopathic | – | Never used AED |
Good course SPS with generalization TR > 5 |
CASPR2 (1 in 100)a |
| 15 (49) | 8.9 M | TCS | IGE/idiopathic | – | 1 AED: fast response/no AED at final FU (14) |
Good course TCS TR > 5 |
CASPR2 (1 in 100)a |
| 16 (1) | 10.5 M | PS with secondary generalization | Localization related cryptogenic/cryptogenic | – | 3 AED: no fast response, polytherapy/on AED at final FU (13.3) |
Poor course, intractable Clustered PS with gen leading to hospitalization TR < 0.1 |
CASPR2 (1 in 100) |
| 17 (130) | 0.6 M | CPS, myoclonic, atonic | Secondary generalized multifocal with atonic and atypical absence seizures/RS including MR |
Severe mental retardation Febrile seizures during FU |
4 AED: no fast response/on AED at final FU (16.5) |
Improving course. Intractable at 5 years – bad compliance. TCS TR > 5 |
CASPR2 (>1 in 100) |
AED, antiepileptic drug; BECTS, benign epilepsy with centrotemporal spikes; CAE, childhood absence epilepsy; CPS, complex partial seizures; CT, computed tomography; EEG, electroencephalography; FU, follow‐up; IGE, idiopathic generalized epilepsy; MJ, myoclonic jerks; PS, partial seizure; RS, remote symptomatic; SPS, simple partial seizures; SWD, spike‐wave discharge; TC, tonic–clonic; TCS, tonic–clonic seizure; TR, terminal remission; SE, status epileptic.
a
Positive serum staining on the surface of hippocampal neurons in vitro.