Table 1 Anti-p62 antibody-positive intra-nuclear inclusions of muscle fibers and non-muscle cells in muscle biopsy
Muscle fiber (% of all myonuclei) | Blood vessel (% of all nuclei) | Schwann cell/Pericyte (% of all nuclei) | Perineurium (% of all nuclei) | Muscle spindle (% of all nuclei) | |
|---|---|---|---|---|---|
OPMD | 11.9 ± 1.1 (n = 15) | 0 (n = 15) | 0 (n = 2) | 0 (n = 2) | 0 (n = 1) |
OPDM_LRP12 | 1.0 ± 0.3 (n = 19) | 0.7 ± 0.3 (n = 19) | 4.0 ± 1.2 (n = 7) | 4.1 ± 2.0 (n = 7) | 13.2 ± 0.7 (n = 2) |
OPDM_GIPC1 | 0.9 ± 0.4 (n = 6) | 0.9 ± 0.5 (n = 6) | 18.5 ± 8.8 (n = 2) | 8.7 ± 2.2 (n = 2) | 18.9 ± 6.9 (n = 2) |
OPDM_NOTCH2NLC | 1.5 ± 0.3 (n = 7) | 8.0 ± 1.8 (n = 7) | 13.4 ± 3.4 (n = 5) | 15.8 ± 2.8 (n = 5) | 16.3 ± 2.0 (n = 2) |
NIID | 0.2 ± 0.1 (n = 10) | 7.2 ± 1.2 (n = 10) | 8.0 ± 4.4 (n = 3) | 21.8 ± 5.2 (n = 3) | 9.1 (n = 1) |
IBM | 0.6 ± 0.3 (n = 11) | 0.5 ± 0.5 (n = 11) | 0.6 ± 0.6 (n = 4) | 0 (n = 4) | 0 (n = 3) |
GNE myopathy | 0.2 ± 0.1 (n = 11) | 0 (n = 10) | 0.8 ± 0.8 (n = 6) | 0.5 ± 0.7 (n = 6) | 5.5 ± 5.5 (n = 2) |
IBMPFD | 0.9 ± 0.2 (n = 8) | 0.2 ± 0.2 (n = 8) | 0 (n = 1) | 2.6 (n = 1) | 0 (n = 1) |
DES-related myopathy | 0.0 ± 0.0 (n = 7) | 0 (n = 7) | 0 (n = 3) | 0 (n = 3) | NA |
Control | 0.0 ± 0.0 (n = 6) | 0 (n = 6) | 0 (n = 2) | 0 (n = 2) | 0 (n = 1) |