Comparisons of clinical profiles between two clusters of patients partitioned by electrodiagnostic data-driven clustering
| Variables | All patients (n=56) | Cluster 1 (n=46) | Cluster 2 (n=10) | P value |
| Gender, male | 35 (62.5%) | 29 (63.0%) | 6 (60.0%) | >0.9 |
| Age at diagnosis (years, range) | 54 (20–84) | 55.5 (22–84) | 53.0 (20–71) | 0.32 |
| Onset to diagnosis (months, range) | 7.0 (3.0–24.0) | 7.0 (3.0–24.3) | 17.0 (4.8–35.3) | 0.34 |
| Follow-up duration (months, range) | 43.5 (18.5–89.8) | 39.5 (16.8–80.8) | 57.5 (35.8–105.0) | 0.11 |
| Diabetes mellitus | 14 (25.0%) | 13 (28.3%) | 1 (10.0%) | 0.42 |
| Muscle atrophy | 21 (37.5%) | 17 (37.0%) | 4 (40.0%) | >0.9 |
| Ataxia | 31 (55.4%) | 23 (50.0%) | 8 (80.0%) | 0.16 |
| Cranial nerve involvement | 3 (5.4%) | 2 (4.3%) | 1 (10.0%) | 0.45 |
| CSF protein (mg/dL, range) | 69.0 (45.0–92.5) | 63.0 (47.0–6.0) | 74.0 (38.5–139.0) | 0.59 |
| Monoclonal gammopathy | 8 (14.3%) | 6 (14.0%) | 2 (20.0%) | 0.62 |
| MRC sum score (range) | 56 (39–60) | 56.0 (39–60) | 57.0 (50–59) | 0.85 |
| Modified Rankin scale (mRS) at diagnosis (range) | 2 (1–5) | 2 (1–5) | 2.5 (2–3) | 0.96 |
| Clinical subtypes | 0.042 | |||
| Typical | 22 (39.3%) | 19 (41.3%) | 3 (30.0%) | |
| DADS | 19 (33.9%) | 12 (26.1%) | 7 (70.0%) | |
| MADSAM | 10 (17.9%) | 10 (21.7%) | 0 | |
| Pure sensory | 5 (8.9%) | 5 (10.9%) | 0 | |
| Treatment | ||||
| Corticosteroids | 50 (89.3%) | 40 (87.0%) | 10 (100.0%) | 0.57 |
| IVIg | 40 (71.4%) | 34 (73.9%) | 6 (60.0%) | 0.43 |
| Azathioprine | 13 (23.2%) | 12 (26.1%) | 1 (10.0%) | 0.42 |
| Mycophenolate mofetil | 2 (3.5%) | 2 (4.3%) | 0 | >0.9 |
| Cyclophosphamide | 1 (1.8%) | 1 (2.2%) | 0 | >0.9 |
| Tacrolimus | 1 (1.8%) | 1 (2.2%) | 0 | >0.9 |
| Rituximab | 7 (12.5%) | 6 (13.0%) | 1 (10.0%) | >0.9 |
| Long-term outcome | 0.023 | |||
| Favourable (mRS ≤2) | 39 (69.6%) | 29 (63.0%) | 10 (100.0%) | |
| Unfavourable (mRS >2) | 17 (30.4%) | 17 (37.0%) | 0 (0.0%) |
Compared with patients of cluster 1, patients of cluster 2 had significantly prolonged distal motor latency, distal duration of compound motor action potential (CMAP), F-wave latency, and reduced nerve conduction velocity and distal CMAP amplitude (p<0.01 in all).
Median and IQR values are provided for quantitative variables, if not otherwise specified.
CSF, cerebrospinal fluid; DADS, distal acquired demyelinating symmetric polyneuropathy; IVIg, intravenous immunoglobulin; MADSAM, multifocal acquired demyelinating sensory and motor neuropathy; MRC, Medical Research Council.