Effects of Prednisone on a Patient with Dysferlinopathy Assessed by Maximal Voluntary Isometric Contraction: Alternate-Day Low-Dose Administration for a 17-Year Period

Abstract

Glucocorticoids are candidates for the pharmacological treatment of dysferlinopathy. Deflazacort, however, showed a worse effect on muscle strength than placebo. Alternate-day low-dose prednisone may have beneficial effects with fewer adverse effects. The outcomes for a female patient with dysferlinopathy (limb-girdle muscular dystrophy type 2B) were assessed by maximal voluntary isometric contraction (MVIC) using a newly devised chair and arm table with push-pull type strain gauges. Grip strength was also measured isometrically. Prednisone 15 mg was started orally at the age of 24 years and was taken every other day in the morning until 41 years of age. The MVIC of flexion of the knees and elbows increased gradually and significantly. The MVIC of extension of the knees and elbows increased to a lesser extent. Isometric grip strength showed no remarkable increase, but strength was sustained over 10 years. Muscle fiber types account for these differences. The beneficial effects of alternate-day prednisone treatment on dysferlinopathy are reported.

Keywords

• Dysferlinopathy
• Limb-girdle muscular dystrophy type 2B
• Prednisone
• Alternate-day low-dose administration
• Isometric muscle strength