Clinical data of patients affected by CIDP and GBS
| Typical CIDP (n=35) | Atypical CIDP (n=18) | AIDP (n=12) | Axonal GBS (n=3) | |
| Disease duration (months) | 42.0±78.0 (12.5–70.8) | 43.0±47.0 (18.2–69.1) | 0.3±0.3 (0.1–0.5) | 0.2±0.2 (0–0.5) |
| Disease activity (patients in active stage/total, %) | 26/35 (74) | 9/18 (50) | 12/12 (100) | 3/3 (100) |
| Response to treatment (patients responding to treatment/total, %) | 29/32 (90) | 8/11 (72) | 10/12 (83) | 2/3 (66) |
| Clinical scales | ||||
| ONLS | 3.7±2.2 (2.9–4.5) | 2.4±0.9 (1.9–2.9) | 5.9±3.0 (3.9–7.8) | 7.5±3.5 (5.0–10.0) |
| MRC sum score | 53.9±5.6 (52.0–55.8) | 59.8±0.6 (59.4–60.1) | 44.3±11.0 (37.2–51.2) | 45.0±9.9 (38.0–52.0) |
| INCAT | 3.0±2.0 (2.3–3.6) | 1.6±1.0 (1.1–2.2) | / | / |
| ISS | 6.8±4.2 (5.3–8.3) | 5±3 (3.4–6.5) | / | / |
| GBS disability scale | / | / | 3.2±1.0 (2.5–3.8) | 4.0±1.4 (3.0–5.0) |
Data about disease duration highlighted and confirmed the chronic phenotype of CIDP compared with AIDP. We also defined the clinical stage of the disease that was active in 100% of patients with AIDP, as expected in an acute demyelinating pathology. In typical and atypical CIDP, we estimated 74% and 50%, respectively of active patients, namely those with a clinical relapse at the moment of admission into the hospital. CIDP and GBS treatment primarily consisted of intravenous immunoglobulin and corticosteroids, less frequently plasma exchange; a large percentage of patients enrolled in the study positively responded to these treatments, displaying an improvement of at least one point in the clinical scales.
Data were expressed as mean±SD and CI (values between parentheses), unless otherwise specified.
AIDP, acute inflammatory demyelinating polyradiculoneuropathy; CIDP, chronic inflammatory demyelinating polyradiculoneuropathy; GBS, Guillain-Barré syndrome; INCAT, inflammatory neuropathy cause and treatment; ISS, INCAT sensory sum score; MRC, Medical Research Council; ONLS, Overall Neuropathy Limitations Scale.