Table 1.
Clinical and paraclinical characteristics of the cohort.
| n = 203 | no. | % | |
|---|---|---|---|
| Male | 140 | 69.0 | |
| Female | 63 | 31.0 | |
| Typical | 144 | 70.9 | |
| Atypical | 59 | 29.1 | |
| Of these: MADSAM | 15 | 7.4 | |
| Mixed motor & sensory | 24 | 11.8 | |
| Pure sensory | 20 | 9.9 | |
| MGUS | 23 | 11.3 | |
| Diabetes | 36 | 17.7 | |
| Age at manifestation (mean ± SD in years) | 54.8 ± 13.3 | ||
| Age at diagnosis (mean ± SD in years) | 57.8 ± 13.7 | ||
| Years to diagnosis (mean ± SD in years) | 3.0 ± 3.7 | ||
| ODSS at presentation (mean ± SD) | 2.32 ± 1.84 | ||
| ODSS after 1 year (mean ± SD) | 2.59 ± 1.86 |
| (available n) | % | ||
|---|---|---|---|
| Increased CSF protein | (174) | 130 | 74.7 |
| Positive nerve biopsy | (78) | 40 | 51.3 |
| Positive nerve MRI | (4) | 2 | 50.0 |
| Positive SSEP/sNCS criterion | (203) | 94 | 46.3 |
| Treatment response after 1 year 1 | (202 1 ) | ||
| EFNS response | 88 | 43.6 | |
| Stabilization | 81 | 40.1 | |
| No response | 33 | 16.3 |
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.
1
One patient died of unrelated causes. For definition of response to therapy, see methods section. SSEP/sNCS criterion as defined by the EFNS/PNS supportive criteria. 5