Table 1

Sample characterisation

ACMS
n=13
RRMS
n=9
SPMS
n=31
PPMS
n=18
Benign/subclinical MS n=3Controls n=12
Age in years46 (28–69)56 (35–69)56 (28–84)54 (28–83)69 (66–72)56 (30–97)
Female to male ratio7:67:223:811:73:08:4
Disease duration in months2.0 (0.2–7)120.0 (108–262)225.0 (72–492)204.0 (30–411)
DGM yes to no6:77:223:811:73:012:0
EDSS10.06 (2.0–7.0)8.5 (4.0–9.5)9.0 (5.0–9.5)
  • This table gives an overview of the main sample characteristics. The values represent either the median value and range or total numbers. The multiple sclerosis (MS) cohort included 13 cases of acute MS. As described by Marburg in 1906, acute MS patients (ACMS) died within 1 year after the disease onset.41 Further, nine cases of relapsing/remitting multiple sclerosis (RRMS), 31 cases of secondary progressive multiple sclerosis (SPMS) and 18 cases of primary progressive multiple sclerosis (PPMS) were included. In addition, we evaluated three cases of benign MS or subclinical MS. Subclinical MS (n=2) was diagnosed when a routine autopsy revealed MS pathology in patients with no clinical history of neurodegenerative disease. Benign MS (n=1) was diagnosed when, after 10 years of disease, the Expanded Disability Status Scale (EDSS) score was below or equal to 3. One MS case was diagnosed with progressive MS but could not be further classified. EDSS scores were evaluated 6–24 months before death. Acute cases of MS with disease durations of 0.2–7 months were evaluated as EDSS 10. If not stated explicitly in the clinical record, the EDSS was evaluated retrospectively with all of the data that were available in the clinical records. The available material included deep grey matter (DGM) in 51/75 MS cases (including the progressive MS case not further classified). Based on tissue availability, tissue quality and block size, a DGM subsample of 31 MS cases (four ACMS, two RRMS, 17 SPMS, four PPMS, three of benign/subclinical MS and one of progressive MS) and 12 controls were selected for detailed immunohistochemical analyses.