Self-perception of quality of life by adolescents with neuromuscular diseases.

PURPOSE: Little is known about quality of life of adolescents with neuromuscular diseases or the factors that influence it. We searched whether physical impairment, physical disability, and medical complications were predictors of low quality of life. METHODS: Motor function, health, orthopedic status, and rehabilitation were assessed in 43 adolescents with neuromuscular diseases (mean age, 13.8 years, standard deviation 1.7 year; sex ratio 2.9/1). Quality of life was measured with the VSP-A ("Vécu Santé Perçu par l'Adolescent"; self-perceived health state in adolescents), a validated health-related quality of life self-perception test. A mixed linear regression related quality of life to impairment, disability, and respiratory status. Comparisons were made with results from an age/sex-matched nondisabled group. RESULTS: On the average, the VSP-A scores in physically disabled adolescents were: (1) similar to those of the nondisabled group with regard to vitality, body image, relationships with parents and friends, and physical and psychological well-being; (2) higher with regard to school performance (score 68 vs. 52.8) and relationships with teachers (67.4 vs. 43.2); and (3) lower with regard to leisure activities (43.9 vs. 60.9). Physical disability and physical impairment were not negatively associated with seven of the nine VSP-A dimensions, but physical impairment was negatively associated with leisure activities and vitality (p < .001 and p < .01, respectively). Adolescents with ventilatory support did not express lower scores than adolescents not requiring ventilatory support (67.7+/-11 vs. 62.9+/-15, p=.39). CONCLUSIONS: These surprising results should lead us question our medical, educational, and rehabilitation practices. Already well-managed disabled adolescents should benefit from less compassionate but more daring and dynamic interpersonal contacts.

A motor function measure for neuromuscular diseases. Construction and validation study.

A new scale for motor function measurement has been developed for neuromuscular diseases. The validation study included 303 patients, aged 6-62 years. Seventy-two patients had Duchenne muscular dystrophy, 32 Becker muscular dystrophy, 30 limb-girdle muscular dystrophy, 39 facio-scapulo-humeral dystrophy, 29 myotonic dystrophy, 21 congenital myopathy, 10 congenital muscular dystrophy, 35 spinal muscular atrophy and 35 hereditary neuropathy. The scale comprised 32 items, in three dimensions: standing position and transfers, axial and proximal motor function, distal motor function. Agreement coefficients for inter-rater reliability were excellent (kappa=0.81-0.94) for nine items, good (kappa=0.61-0.80) for 20 items and moderate (kappa=0.51-0.60) for three items. High correlations were found between the total score and other scores: Vignos (r=0.91) and Brooke (r=0.85) grades, Functional Independence Measure (r=0.91), the global severity of disability evaluated with visual analog scales by physicians (r=0.88) and physiotherapists (r=0.91). This scale is reliable, does not require any special equipment and is well-accepted by patients. Its sensitivity to change is being assessed to permit its use in clinical trials of neuromuscular diseases.

Complete dislocation of the radial head following fracture of the radial neck in children: the Jeffery type II lesion.

The authors report four cases of Jeffery type 2 lesions in children. Despite the probable mechanism of such lesions, none of the cases reported here displayed a dislocation of the elbow. In the first observation the diagnosis was suspected only 15 days after injury. Two other cases were initially treated by closed external manipulations, one with percutaneous Kirschner wire leverage and the other with closed intramedullary pinning, but both cases displayed a 180 degree reversal of the radial head, suspected only secondarily. The first exhaustive review of the literature on the subject, shows that this complication is classical in Jeffery type 2 fractures. In fact none of the 22 cases described in the literature had successfully been reduced by external manipulations. Interposition of the lateral condyle, pathognomonic of the Jeffery type 2 fracture, hampers reduction. Attempts at reduction by external manipulation in nine cases therefore led to two non-reduction and seven reversal of the radial head. Knowledge of this particular fracture will allow an early diagnosis and an immediate surgical treatment.