Physical characteristics and applicability of standard assessment methods in a total population of spinal muscular atrophy type II patients.

The aims of this study were to evaluate muscle strength, functional abilities, contractures and Forced Vital Capacity in a population of 54 spinal muscular atrophy (SMA) type II patients between the ages of 5 and 70, and to evaluate the applicability of conventional assessment methods. The patients were evaluated by means of functional scales, muscles tests, joint motion measurement and Forced Vital Capacity test. There was a significant score difference in functional tests and muscle tests as well as in the sum of contractures between younger individuals (<or= 20years) and older individuals (>or= 21years). The functional scales were not sensitive enough to differentiate among the most impaired persons. A reduced Manual Muscle Test score of the upper limbs was found to differentiate more precisely among individuals than a total score derived from testing 38 muscle groups. There is a need for clinical tools that can evaluate patients with SMA type II of all ages and with severely reduced functional abilities.