Marieke van Rosmalen

Clinical outcomes in MMN 33 2 Figure 2.3 Correlation of MRC grade and disease duration per muscle group The boxplots provide the variability in disease duration per MRC grade (0 – 5). Disease duration is defined as years from onset of symptoms until first study visit. Abbreviations: MRC = Medical Research Council. Disability questionnaires Results of the disability questionnaires are shown in Supplemental table 2.3 . Median ODSS of the arms was 2 (range 0 – 4), of the legs 1 (range 0 – 5) and of arms and legs combined 3 (range 0 – 8). Twelve patients (12%) reported no disability of the arms and 34 patients (34%) did not experience disability of the legs. Correlates of outcome Multiple linear regression analysis showed that a lower MRC sum score correlated with longer disease duration without treatment, presence of anti-GM1 IgM antibodies and lower age at onset of symptoms ( p = 0.024, p = 0.046 and p = 0.006 respectively). Outcome measures over time Mean differences between visit 1 (2007) and visit 2 (2015 – 2016) of different outcome measures are shown in Table 2.2 . Except for ODSS, FSS and vigorimetry of the left hand, all outcome measures deteriorated over time (all p < 0.01). The difference in MRC sum score between 2015 and 2007 was significantly larger in patients with axonal damage compared to patients without axonal damage (5.2 points vs. 13.8 points; p = 0.014). Most patients indicated that their disease course was stable (25.0%) or mildly progressive (61.7%). The dose of immunoglobulin treatment significantly increased over time ( p < 0.001).