TABLE 1.
Features of clinical variants defined according to EAN/PNS 2021 criteria.
| Features | Typical CIDP, n = 106 | Distal CIDP, n = 62 | Multifocal and focal CIDP, n = 28 | Sensory CIDP, n = 4 | Sensory‐predominant CIDP, n = 27 | Motor CIDP, n = 10 | Motor‐predominant CIDP, n = 8 | p |
|---|---|---|---|---|---|---|---|---|
| Demographics | ||||||||
| Gender male, n (%) | 72/106 (68%) | 37/62 (60%) | 24/28 (86%) | 2/2 (50%) | 22/27 (82%) | 5/10 (50%) | 5/8 (63%) | |
| Age at onset, years, median (IQR) | 50 (28) | 58 (23) | 54 (25) | 55 (29) | 61 (13) | 59 (22) | 30 (37) | 0.027 D‐T, <0.001 SP‐T, 0.023 MP‐T |
| Disease duration at enrollment, years, median (IQR) | 7 (10) | 6 (8) | 5 (6) | 7 (4) | 3 (4) | 6 (15) | 4 (10) | <0.001 MF‐T, 0.018 SP‐T |
| Disease course, n (%) | ||||||||
| Relapsing | 49/106 (46%) | 35/62 (57%) | 11/28 (39%) | 0/4 (0%) | 10/27 (49%) | 6/10 (60%) | 5/8 (63%) | |
| Acute onset | 11/106 (10%) | 3/62 (5%) | 0/28 (0%) | 0/4 (0%) | 0/24 (0%) | 0/10 (0%) | 3/8 38 (%) | |
| Symptoms, n (%) | ||||||||
| Pain | 39/106 (37%) | 22/62 (36%) | 4/28 (14%) | 1/4 (25%) | 9/27 (33%) | 6/10 (60%) | 1/8 (13%) | 0.023 MF‐T |
| Fatigue | 68/106 (64%) | 34/62 (55%) | 6/28 (21%) | 2/4 (50%) | 8/27 (30%) | 8/10 (80%) | 6/8 (75%) | <0.001 MF‐T, 0.002 SP‐T |
| Cranial nerve involvement | 30/106 (28%) | 0/62 (0%) | 6/28 (21%) | 1/4 (25%) | 0/27 (0%) | 2/10 (20%) | 1/8 (13%) | <0.001 D‐T, <0.001 SP‐T |
| Ataxia | 44/106 (42%) | 18/62 (29%) | 4/28 (14%) | 1/4 (25%) | 6/27 (22%) | 0/10 (0%) | 0/8 (0%) | 0.008 MF‐T, 0.013 M‐T, 0.022 MP‐T |
| Tremor | 17/106 (16%) | 8/62 (13%) | 3/28 (11%) | 1/4 (25%) | 0/27 (0%) | 1/10 (10%) | 1/8 (13%) | 0.026 SP‐T |
| Clinical scores at enrollment (±SD) | ||||||||
| INCAT | 3.5 (±2.3) | 2.2 (±1.7) | 2.1 (±1.3) | 0.5 (±1) | 1.3 (±1) | 3.3 (±2.8) | 2.8 (±2.5) | <0.001 D‐T, <0.001 MF‐T, <0.001 S‐T, <0.001 SP‐T |
| MRC | 52 (±10) | 55 (±7) | 56 (±4) | 60 (±0) | 60 (±0) | 55 (±3) | 53 (±8) | 0.003 D‐T, <0.001 MF‐T, <0.001 S‐T, <0.001 SP‐T |
| RODS | 32 (±12) | 35 (±9) | 37 (±10) | 35 (±11) | 41 (±6) | 36 (±9) | 34 (±11) | 0.029 D‐T, <0.001 SP‐T |
| EuroQol‐5D‐3L scale | 8.3 (±2.0) | 7.6 (±1.5) | 8.0 (±1.8) | 9.2 (±1.0) | 7.1 (±1.4) | 7.5 (±1.9) | 7.8 (±2.0) | 0.032 D‐T, 0.019 SP‐T |
| Electromyographic features | ||||||||
| Tested motor nerves, n, median (IQR) | 6 (2) | 6 (2) | 6 (3) | 7 (4) | 6 (2) | 7 (2) | 6 (4) | |
| Distal latency prolongation, n (%) | 32/106 (30%) | 24/62 (39%) | 6/28 (21%) | 0/4 (0%) | 9/27 (33%) | 1/10 (10%) | 2/8 (25%) | |
| Reduced conduction velocity, n (%) | 51/106 (48%) | 35/62 (57%) | 4/28 (14%) | 0/4 (0%) | 8/27 (30%) | 2/10 (20%) | 3/8 (38%) | 0.001 MF‐T |
| Prolonged F‐wave latency, n (%) | 19/80 (24%) | 10/48 (21%) | 1/23 (4%) | 0/4 (0%) | 8/24 (33%) | 4/10 (40%) | 3/7 (43%) | 0.040 MF‐T |
| Absent F wave, n (%) | 17/80 (21%) | 5/48 (10%) | 6/23 (26%) | 0/4 (0%) | 5/24 (21%) | 2/10 (20%) | 2/7 (29%) | |
| Conduction block, n (%) | 39/100 (39%) | 21/55 (38%) | 5/25 (20%) | 0/4 (0%) | 5/25 (20%) | 2/10 (20%) | 3/7 (43%) | |
| Temporal dispersion, n (%) | 29/60 (48%) | 14/37 (38%) | 5/16 (31%) | 0/4 (0%) | 5/18 (28%) | 2/6 (33%) | 2/3 (67%) | |
| Reduced CMAP amplitude, n (%) | 55/106 (52%) | 43/62 (69%) | 10/28 (36%) | 0/4 (0%) | 16/25 (64%) | 5/10 (50%) | 6/8 (75%) | 0.027 D‐T |
| Sural sparing pattern on sensory NCS, n (%) | 20/75 (27%) | 12/41 (29%) | 8/20 (40%) | 2/3 (66%) | 9/22 (41%) | 0/6 (0%) | 2/8 (25%) | |
| Supportive criteria | ||||||||
| Increased CSF proteins/tested, n (%) | 72/92 (78%) | 37/46 (80%) | 12/22 (55%) | 2/3 (67%) | 16/24 (67%) | 4/6 (67%) | 5/5 (100%) | 0.023 MF‐T |
| Mean CSF proteins, mg/dL (±SD) | 113 (±130) | 103 (±71) | 64 (±35) | 63 (±28) | 88 (±77) | 201 (±335) | 284 (±266) | 0.003 MF‐T |
| Demyelination on nerve biopsy/tested, n (%) | 4/6 (67%) | 2/5 (40%) | 0/1 (0%) | 1/2 (50%) | ND | 1/1 (100%) | ND | |
| Response to therapy | ||||||||
| Response IVIg/treated, n (%) | 56/88 (64%) | 36/48 (75%) | 11/21 (52%) | 2/4 (50%) | 7/11 (64%) | 6/10 (60%) | 4/6 (67%) | |
| Response steroids/treated, n (%) | 44/80 (55%) | 20/34 (59%) | 8/16 (50%) | 0/2 (0%) | 8/11 (73%) | 0/3 (0%) | 2/2 (100%) | |
| Overall response/treated, n (%) | 76/100 (76%) | 45/55 (82%) | 19/26 (73%) | 2/4 (50%) | 14/17 (82%) | 7/10 (70%) | 6/7 (86%) | |
Abbreviations: CIDP, chronic inflammatory demyelinating polyradiculoneuropathy; CMAP, compound muscle action potential; CSF, cerebrospinal fluid; D‐T, distal CIDP vs. typical CIDP; INCAT, Inflammatory Neuropathy Cause and Treatment; IQR, interquartile range; IVIg, intravenous immunoglobulin; MF‐T, multifocal CIDP vs. typical CIDP; MP‐T, motor‐predominant CIDP vs. typical CIDP; MRC, Medical Research Council; M‐T, motor CIDP vs. typical CIDP; NCS, nerve conduction studies; ND, not done; RODS, Rasch‐built Overall Disability Scale; SP‐T, sensory‐predominant CIDP vs. typical CIDP; S‐T, sensory CIDP vs. typical CIDP.