Computerized hand grip myometry reliably measures myotonia and muscle strength in myotonic dystrophy (DM1)

Abstract

The aim of this study was to develop a reliable, sensitive, quantitative measure of grip myotonia and strength and to determine whether CTG repeat length is correlated with grip myotonia and with muscle strength in myotonic dystrophy type 1 (DM1). Three maximum voluntary isometric contractions (MVICs) of the finger flexors (i.e., handgrip) were recorded on 2 successive days using a computerized handgrip myometer in 29 genetically confirmed DM1 patients and 17 normals. An automated computer program calculated MVIC peak force (PF) and relaxation times (RTs) along the declining (relaxation) phase of the force recordings at 90%, 75%, 50%, 10%, and 5% of PF. Patients also underwent quantitative strength testing (QST) manual muscle testing (MMT). The patients had longer grip RTs and lower PFs than normals. RT (90% to 5%) was above the normal mean +2.5 SD in 25 (86%) patients. In DM1, prolongation of RT was mainly in the terminal (50% to 5%), rather than the initial (90% to 50%) phase of relaxation. PFs and RTs for each patient were reproducible on consecutive days. RTs were positively correlated with leukocyte CTG repeat length, whereas measures of muscle strength, such as PF, QST, and MMT, were negatively correlated with repeat length. We conclude that computerized handgrip myometry provides a sensitive, reliable measure of myotonia and strength in DM1 and offers a method to assess natural history and response to treatment.