Table 1.
Antibody findings and syndromes
| All patients | Abs against cell surface antigensa | Abs against intracellular antigens | Hashimoto encephalopathy/SREAT | |
|---|---|---|---|---|
| Number of cases (% per subgroup) | 145 (100%) | 80 (55%) | 15 (10%) | 50 (34%) |
| Females (%) | 93 (64%) | 49 (61%) | 9 (60%) | 35 (70%) |
| Males (%) | 52 (36%) | 31 (39%) | 6 (40%) | 15 (30%) |
| Age ± s.d. in years (range) | 43.9 ± 22.1 (from 2–91 years) | 40.7 ± 22.1 (from 2–91 years) | 56.9 ± 20.6 (from 19–85 years) | 45.1 ± 21.2 (from 10–84 years) |
| Antibody subtypes (% per subgroup) | Abs against cell surface antigens: 80 (55%) Abs against intracellular antigens: 15 (10%) Hashimoto encephalopathy: 50 (34%) |
Anti-NMDA-R: 46 (58%) Anti-VGKC: 18 (23%) LGI1: 4 (5%) CASPR2: 0 (0%) Anti-AMPA: 12 (15%) Anti-GABAA: 1 (1%) Anti-DPPX: 1 (1%) Anti-AQP4: 1 (1%) Unknown: 1 (1%) |
Anti-GAD65: 1 (7%) Anti-Ca/ARHGAP26 ab: 1 (7%) Anti-Ma1/2: 3 (20%) Anti-Ri: 2 (13%) Anti-Hu: 1 (7%) Anti-Yo: 1 (7%) Anti-AK5: 5 (33%) Anti-BRSK2: 1 (7%) |
Anti-TPO: 22 (44%) Anti-TG: 2 (4%) Anti-TPO + TG: 25 (50%) Unclear: 1 (2%) (‘antithyroid antibody’) |
| Antibody investigation in serum/CSF | Serum: 135 (93%) CSF: 69 (48%) |
Serum: 70 (88%) CSF: 47 (59%) |
Serum: 15 (100%) CSF: 8 (53%) |
Serum: 50 (100%) CSF: 14 (28%) |
| Antibody detection in serum/CSF | Serum: 119 from 135 (88%) CSF: 52 from 69 (75%) Unclear: 6 |
Serum: 57 from 70 (81%) CSF: 38 from 47 (81%) Unclear: 6 |
Serum: 12 from 15 (80%) CSF: 8 from 8 (100%) Unclear: 0 |
Serum: 50 from 50 (100%) CSF: 6 from 14 (43%) Unclear: 0 |
| Syndromes (% per subgroup) | ||||
| Schizophreniform | 50 (34%) | 29 (36%) | 3 (20%) | 18 (36%) |
| Amnestic/dementia-like | 57 (39%) | 28 (35%) | 12 (80%) | 17 (34%) |
| Confusional | 16 (11%) | 11 (14%) | 0 (0%) | 5 (10%) |
| Depressive | 11 (8%) | 5 (6%) | 0 (0%) | 6 (12%) |
| Manic | 5 (3%) | 3 (4%) | 0 (0%) | 2 (4%) |
| Tic | 1 (1%) | 0 (0%) | 0 (0%) | 1 (2%) |
| Sleep-disturbance | 2 (1%) | 2 (3%) | 0 (0%) | 0 (0%) |
| Autistic | 2 (1%) | 2 (3%) | 0 (0%) | 0 (0%) |
| Anxiety-compulsive | 1 (1%) | 0 (0%) | 0 (0%) | 1 (2%) |
SD, standard deviation; CSF, cerebrospinal fluid; SREAT, steroid-responsive encephalopathy associated with autoimmune thyroiditis.
More than one antibody (Ab) could have been positive in individual cases, in these cases, only the predominant Ab was mentioned here. From the anti-VGKC Ab positive patients, seven cases were screened for anti-LGI1 and five cases were screened for anti-CASPR2 subtype. One patient with intracellular Abs also had anti-VGKC-Abs. Five patients with antineuronal Abs against cell surface, intracellular Abs and anti-thyroid Abs also had other intracellular Abs. Five patients with antineuronal Abs against cell surface and intracellular antigens also had reported anti-thyroid Abs.
Patients with anti-AQP4 Abs were added to the group of patients with Abs against cell surface antigens for subgroup analyses.