Marieke van Rosmalen

Chapter 2 32 Table 2.1 Clinical characteristics Parameter Diagnosis before 2007 (n = 64) Diagnosis in or after 2007 (n = 36) p Male 46 (72%) 29 (81%) 0.34 Age at onset of symptoms 40.3 (21.4 – 53.8) 45.2 (30.1 – 67.2) < 0.001 Age at diagnosis 45.2 (25.2 – 71.1) 48.6 (30.9 – 73.5) 0.02 Time to diagnosis (months)* 42.0 (3.0 – 433.0) 27.0 (6.0 – 345.0) 0.10 Time from disease onset until treatment (months) 42.0 (3.0 – 435.9) 27.5 (3.9 – 346.0) 0.09 Maintenance treatment with immunoglobulins 55 (86%) 32 (89%) 0.67 Starting dose ivIg maintenance therapy per week (gram) 10.0 (5.0 – 33.0) 8.0 (4.0 – 12.0) < 0.001 Onset of muscle weakness Distal arm 41 (64%) 25 (70%) 0.08 Proximal arm 3 (4%) 3 (8%) Distal leg 18 (28%) 4 (11%) Proximal leg 1 (2%) 0 (0 %) Distal symmetrical 1 (2%) 4 (11%) Number of affected limbs at inclusion 0 2 (3%) 1 (3%) 0.15 1 7 (11%) 8 (22%) 2 12 (19%) 12 (33%) 3 18 (28%) 7 (20%) 4 25 (39%) 8 (22%) Electrophysiological criteria** Definite 45 (70%) 29 (81%) 0.32 Probable 15 (23%) 4 (11%) Negative 4 (6%) 3 (8%) NCS with axonal degeneration 31 (48%) 13 (36%) 0.23 Abnormalities brachial plexus MRI 22/43 (51%) 8/17 (47%) 0.77 Laboratory findings Increased CSF protein 12/16 (75%) 8/10 (80%) 0.77 Presence of anti-GM1 IgM antibodies 38/61 (62%) 17/29 (59%) 0.74 Data are shown in median (range) or number of patients (%), unless stated otherwise. * log transformed variable ** according to the EFNS/PNS criteria 5 Abbreviations: ivIg = intravenous immunoglobulins; NCS = nerve conduction studies; MRI = magnetic resonance imaging; CSF = cerebrospinal fluid.