Comparison of clinical findings in LMNA-related muscular dystrophy
| L-CMD (n=41) | EDMD (n=32) | LGMD1B (n=11) | P value | |
| Mean age of onset, years, ±SD, range | 0.3±0.5, 0–2.0 | 2.2±1.7, 1.0–10.0 | 2.6±3.0, 1.0–11.0 | <0.001 |
| Mean age of last review, years, ±SD, range | 6.5±3.3, 1.5–16.0 | 10.6±9.0, 1.5–43.0 | 11.4±13.1, 2.5–38.0 | 0.04 |
| Spinal deformities | 23 (56.1%) | 17 (53.1%) | 0 (0%) | 0.003 |
| Contractures | 21 (51.2%) | 24 (75.0%) | 2 (18.2%) | 0.003 |
| Cardiac involvement | 5 (12.1%) | 14 (43.8%) | 1 (9.1%) | 0.003 |
| Mean CK level | 1142.0±724.4, 195.0–3766.0 | 1047.4±787.0, 223.0–3494.0 | 994.9±816.5, 162.0–2623.0 | 0.797 |
CK, creatine kinase; EDMD, Emery-Dreifuss muscular dystrophy; L-CMD, LMNA-related congenital muscular dystrophy; LGMD1B, limb-girdle muscular dystrophy type 1B.