Table 2.
Comparison of patients with anti-Caspr1 IgG4 antibodies.
| Emilien Delmont (n = 2) | Andrea Cortese (n = 3) | Kathrin Doppler (n = 2) | Giuseppe Liberatore (n = 1) | Present report (n = 2) | |
|---|---|---|---|---|---|
| Age at onset (year) | 53 and 68 | 46 (24-57) | 30 and 69 | 57 | 44 and 59 |
| Male sex | 2/2 | 2/3 | 1/2 | 0/1 | 1/2 |
| Subacute onset | 2/2 | 2/3 | 1/2 | 1/1 | 2/2 |
| Symptoms at onset | NA | NA | Sensory deficits and severe pain, distal numbness | progressive proximal and distal motor involvement | Sensory deficits, severe pain with a cold sensation |
| Triggering infection/vaccination | 0/0 | 1/3 | 2/2 | NA | 1/2 |
| Muscle weakness | 2/2 | 3/3 | 2/2 | 1/1 | 2/2 |
| more severe in distal | 0/0 | 3/3 | 1/2 | NA | 2/2 |
| Sensory deficiency | 2/2 | 3/3 | 2/2 | 1/1 | 2/2 |
| Sensory ataxia | 1/2 | 2/3 | 0/2 | 1/1 | 1/2 |
| Tremor | 0/2 | 1/3 | 0/2 | 1/1 | 0/2 |
| Pain | 1/2 | 0/5 | 2/2 | 0/1 | 2/2 |
| Neuropathic pain accompanying cold sense | 0/2 | 0/5 | 0/2 | 0/1 | 2/2 |
| Respiratory failure | 2/2 | 0/5 | 0/2 | 0/1 | 0/2 |
| Cranial nerve involvement | 2/2 | 1/3 | 0/2 | NA | 0/2 |
| CSF protein (g/L) | 12 and 2.5 | 4.26 (3.43–5.10) | 5.2 | NA | 3.2 and 3.3 |
| Impairment | |||||
| ONLS (total) | 10 and 5 | 8 (8-9) | NA | NA | 5 and 5 |
| MRC sum score | NA | NA | 58/60 * | NA | 56 and 56 |
| NCS features | |||||
| Prolonged DML | NA | 3/3 | 2/2 | NA | 2/2 |
| Reduced CV | NA | 3/3 | 2/2 | NA | 2/2 |
| Prolonged F wave | NA | 1/3 | 2/2 | NA | 2/2 |
| Conduction blocks | NA | 1/3 | 2/2 | NA | 0/2 |
| Temporal dispersion | NA | 1/3 | 1/2 | NA | 0/0 |
| Response to IVIG | 0/2 | 2/3 | 0/1 | 0/1 | 0/1 |
| Response to steroids | 0/1 | 2/3 | 1/1 | 0/1 | 2/2 |
| Response to PEX | 0/1 | 0/1 | 1/2 | 0/1 | 0/0 |
| Response to immune suppressors | 0/0 | 1/1 | 1/1 | 0/1 | 0/0 |
CSF, cerebrospinal fluid; CV, conduction velocity; DML, distal motor latency; IVIg, intravenous immunoglobulins; NCS, nerve conduction study; PEX, plasma exchange. *: The MRC sum score of patient 2 was not found in Doppler’s article.
NA, not applicable.