Clinical manifestation and myopathological changes in pseudohypertrophy muscular dysrtophy patients
Abstract：Objective To investigate the correlation between myopathological changes and clinical manifestations in Pseudohypertrophy Muscular Dystrophy (PMD) patients. Methods A total of 37 patients with PMD (15 DMD cases and 22 BMD cases) were recruited in Affiliated Drum Tower Hospital, Nanjing University Medical School from January 2010 to November 2016.They were assessed with Modified Gardner-Medwin and Walton (GM-W) scale and underwent muscle biopsies. The correlation between GM-W and age, course of disease, internal nuclei and the ratio of hypertrophy factor/atrophy factor was examined. Statistical analysis of pathological changes were calculated between the groups of GM-W≥4 and GM-W≤3. Results GM-W had significant correlations with age (r=0.453,P=0.005), course of disease(r=0.578,P< 0.001). 21 patients in GM-W≥ 4 group showed mild (n=1), moderate(n=6) and severe(n=14) degree of internal nuclei. The ratio of hypertrophy/atrophy factor was 2.09 (1.71, 3.41). 16 patients in GM-W≤ 3 group showed mild(n=8), moderate(n=5) and severe(n=3) degree of internal nuclei. The ratio of hypertrophy /atrophy factor was 1.77 (1.51, 2.47). GM-W was correlated with nuclear translocation and hypertrophy factor/atrophy factor (r=0.589,0.615;P<0.001). Significant differences were observed in internal nuclei and hypertrophy factor/atrophy factor between the groups of GM-W≥ 4 and GM-W≤ 3(Z=-4.860, 61.00;P≤ 0.001). Conclusions The pathological changes of patients with PMD are correlated with the severity of clinical manifestations. GM-W contributes to assess the severity and progression of PMD.
Keywords：Pseudohypertrophy muscular dysrtophyGM-WPathological changes of muscular bi-opsy
Publication Date：2017-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：5( 465-468,533 )