Relationships between Upper Extremity Strength and the Purdue Pegboard Test and the Jebsen-Taylor Hand Function Test in Patients with Myotonic Dystrophy Type 1 (P05.187)

Abstract

Objective: To document the relationship between measures of upper extremity strength and hand function in patients with myotonic dystrophy type 1(DM1). Background Distal weakness is a common feature in patients with (DM1). The assessment of hand function in this patient population is just beginning to be explored. Measurements of upper extremity strength and hand function have demonstrated good reliability; however, the relationship between strength and function is unknown. Design/Methods: Patients with DM1 participating in a longitudinal study performed strength tests of the upper extremity including manual muscle testing (MMT) and quantitative muscles testing (QMT). MMT included shoulder abduction, elbow flexion and extension, wrist flexion and extension, and short and long finger flexors of the thumb and first two digits. QMT included shoulder abduction, elbow flexion, elbow extension and grip. Patients also performed the Purdue Pegboard Test (PPT) and the Jebsen-Taylor Hand Function Test (JTHFT). Results: Data from 71 genetically confirmed patients with DM1 were analyzed using Spearman9s rho. Statistically significant (p Conclusions: Moderate relationships exist between upper extremity strength tests and hand function tests. The PPT correlates better with MMT which included several distal hand muscles and hence captures fine motor function deficits. The JTHFT correlated better with QMT and the most significant correlation was with lifting heavy objects and this may reflect more the proximal strength deficits. Patients need to be followed longitudinally to document which of these tests is able to reflect changes over time. Supported by: Award number U54NS048843 from the National Institute of Neurological Disorders and Stroke. Disclosure: Dr. Eichinger has nothing to disclose. Dr. Dilek has nothing to disclose. Dr. Dekdebrun has nothing to disclose. Dr. Martens has nothing to disclose. Dr. Heatwole has nothing to disclose. Dr. Thornton has received personal compensation for activities with Genzyme Corporation as a consultant. Dr. Thornton has received research support from Isis Pharmaceuticals, Inc. Dr. Moxley has nothing to disclose. Ms. Pandya has received personal compensation for activities with Shire Pharmaceuticals Group.