Table 3.
Clinical and paraclinical characteristics of groups A and B.
| n = 182 | Group A | % | n = 21 | Group B | % | p | |
|---|---|---|---|---|---|---|---|
| Male | 125 | 68.7 | 15 | 71.4 | 1.000 | ||
| Female | 57 | 31.3 | 6 | 28.6 | – | ||
| Typical | 128 | 70.3 | 16 | 76.2 | 0.800 | ||
| Atypical | 54 | 29.7 | 5 | 23.8 | 0.800 | ||
| Of these: MADSAM | 14 | 7.7 | 1 | 4.8 | 1.000 | ||
| Mixed | 22 | 12.1 | 2 | 9.5 | 1.000 | ||
| Pure sensory | 18 | 9.9 | 2 | 9.5 | 1.000 | ||
| MGUS | 20 | 11.0 | 3 | 14.3 | 0.714 | ||
| Diabetes | 30 | 16.5 | 6 | 28.6 | 0.223 | ||
| Age at manifestation (mean ± SD in years) | 54.6 ± 13.2 | 57 ± 14.4 | 0.427 | ||||
| Age at diagnosis (mean ± SD in years) | 57.5 ± 13.6 | 60.7 ± 14.1 | 0.313 | ||||
| Time to diagnosis (mean ± SD in years) | 2.9 ± 3.6 | 3.7 ± 4.3 | 0.377 | ||||
| ODSS at presentation (mean ± SD) | 2.41 ± 1.90 | 1.52 ± 0.93 | <0.001 | ||||
| ODSS after 1 year (mean ± SD) | 2.66 ± 1.93 | 2.05 ± 0.97 | 0.022 |
| (available n) | % | (available n) | % | p | |||
|---|---|---|---|---|---|---|---|
| Increased CSF protein | (156) | 117 | 75.0 | (18) | 13 | 72.2 | 0.779 |
| Positive nerve biopsy | (75) | 37 | 49.3 | (3) | 3 | 100.0 | (0.241) |
| Positive nerve MRI | (4) | 2 | 50.0 | (0) | 0 | – | |
| Positive SSEP/sNCS criterion | (182) | 84 | 46.2 | (21) | 10 | 47.6 | 1.000 |
| Treatment response after 1 year 1 | (181 1 ) | (21) | |||||
| EFNS defined response | 82 | 45.3 | 6 | 28.6 | 0.168 | ||
| Stabilization | 70 | 38.7 | 11 | 52.4 | 0.246 | ||
| No response | 29 | 16.0 | 4 | 19.0 | 0.755 | ||
| Treatment 2 | |||||||
| Steroids | 131 | 72.0 | 17 | 81 | 0.449 | ||
| IVIg | 136 | 74.7 | 12 | 57.1 | 0.117 | ||
| Oral Immunosuppressives | 74 | 40.7 | 8 | 38.1 | 1.000 | ||
| Escalation therapy | 29 | 15.9 | 2 | 9.5 | 0.748 | ||
Treatment with steroids was variable. Most usually administered as intravenous pulses, almost exclusive of methylprednisolone, with varying dosages and frequency (250–1000 mg/day for 3 days, every 6 to 12 weeks). Fewer patients were treated with oral prednisolone with an initial dosage of 1 mg/kg body weight followed by tapering.
Escalation therapy was considered any therapy with rituximab, cyclophosphamide, or bortezomib.
For definition of response to treatment, see methods section.
SSEP/sNCS criterion as defined by the EFNS/PNS supportive criteria. 5
MADSAM, Multifocal acquired demyelinating sensory and motor neuropathy; MGUS, Monoclonal gammopathy of undetermined significance, excluding patients with IgM gammopathy with anti‐myelin‐associated glycoprotein (MAG) antibodies; ODSS, Overall disability sum score; CSF, cerebrospinal fluid; SSEP, somatosensory evoked potential; sNCS, sensory nerve conduction studies; EFNS, European Federation of Neurological Societies; IVIg, intravenous immunoglobulin.
One patient died of unrelated causes. Significant p values marked in bold.
Maintenance treatment with IVIG was with 1 g/kg of body weight every approximately 4 weeks.