Table 1.
Detection of autoantibodies to LRP4 and other NMJ antigens in the serum and CSF from ALS patients and controls1
| Patients | No of samples | Anti-LRP4 | Anti-AChR | Anti-AChR clusters | Anti-MuSK |
|---|---|---|---|---|---|
| Greek sporadic ALS | 51 | 12 (23.5%) | 0 | 0 | 0 |
| Italian sporadic ALS | 53 | 12 (22.6%) | 0 | 0 | 02 |
| Familial ALS | 4 | 0 | 0 | 0 | 0 |
| MG (anti-AChR positive) | 40 | 1 (2.5%) | 40 | 22/22 | 0 |
| MS | 84 | 4 (4.8%) | NT | NT | NT |
| OND | 10 | 0 | NT | NT | NT |
| Healthy controls | 40 | 0 | NT | NT | NT |
| CSF from anti-LRP4 positive ALS patients | 7 | 6 | NT | NT | NT |
| CSF from anti-LRP4 negative ALS patients | 17 | 0 | NT | NT | NT |
| CSF from patients with MS or OND | 44 | 13 (2.3%) | NT | NT | NT |
MG, myasthenia gravis; MS, multiple sclerosis; OND, other neurological diseases.
Serum samples from patients in two ALS cohorts (Greek and Italian) and patients with familial ALS, MG (myasthenia gravis) with AChR antibodies, MS, and other neurological disease (OND) and healthy controls were tested in the cell-based assay at a 1/100 dilution for the presence of LRP4 or AChR cluster antibodies and in the radioimmunoprecipitation assay for the presence of AChR and MuSK antibodies. CSF samples from patients with ALS, MS, and OND were also tested, undiluted, for the presence of LRP4 antibodies.
Two sera were found ambiguous for MuSK antibodies (titer 0.02 nmol/L). Both were LRP4-negative.
This patient was one of the four LRP4-seropositive MS patients.