Table 2

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
ONLS3.7±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 score53.9±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)
INCAT3.0±2.0 (2.3–3.6)1.6±1.0 (1.1–2.2)//
ISS6.8±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.