Objective. To validate manual muscle testing (MMT) for strength assessment in juvenile and adult dermatomyositis (DM) and polymyositis (PM).Methods. Patients with PM/DM (73 children and 45 adults) were assessed at baseline and reevaluated 6-9 months later. We compared Total MMT (a group of 24 proximal, distal, and axial muscles) and Proximal MMT (7 proximal muscle groups) tested bilaterally on a 0-10 scale with 144 subsets of 6 and 96 subsets of 8 muscle groups tested unilaterally. Expert consensus was used to rank the best abbreviated MMT subsets for face validity and ease of assessment.Results. The Total, Proximal, and best MMT subsets had excellent internal reliability (Total MMT r(s) = 0.91-0.98), and consistency (Cronbach's alpha = 0.78-0.97). Inter-and intrarater reliability were acceptable (Kendall's W 0.68-0.76, r(s) = 0.84-0.95). MMT subset scores correlated highly with Total and Proximal MMT scores and with the Childhood Myositis Assessment Scale, and correlated moderately with physician global activity, functional disability, magnetic resonance imaging, and axial and distal MMT scores, and, in adults, with creatine kinase level. The standardized response mean for Total MMT was 0.56 in juveniles and 0.75 in adults. Consensus was reached to use a subset of 8 muscles (neck flexors, deltoids, biceps, wrist extensors, gluteus maximus and medius, quadriceps, and ankle dorsiflexors) that performed as well as the Total and Proximal MMT, and had good face validity and ease of assessment.Conclusion. These findings aid in standardizing the use of MMT for assessing strength as an outcome measure for myositis.

Objective. Children with juvenile idiopathic inflammatory myopathies (JIIM) present with muscle inflammation and decreased strength that may affect their functional abilities. The purpose of this study was to determine the intra-rater and inter-rater reliability of the 0 to10-point manual muscle testing method for children with JIIM.Methods. For the intra-rater and inter-rater reliability studies, 10 and 9 children with JIIM participated, respectively. For intra-rater reliability, one pediatric therapist completed two assessments in one day with a one-hour break. For inter-rater reliability, four therapists assessed the same child within a single morning.Results. Spearman correlations for intra-rater reliability ranged from 0.70 to 1.00. Kendall's W coefficient for inter-rater reliability of groups of muscles (total, proximal, distal, and peripheral) ranged from 0.51 to 0.76.Conclusions. The total, proximal, and peripheral Manual Muscle Test (MMT) score, using the 0-10 point scale, has acceptable reliability in JIIM patients.

PURPOSE:Fibrotic sequelae remain the most important dose-limiting toxicity of radiation therapy to soft tissue. Functionally, this is reflected in loss of range of motion and muscle strength and the development of limb edema and pain. Tumor necrosis factor alpha and fibroblast growth factor 2 (FGF2), which are abnormally elevated in irradiated tissues, may mediate radiation fibrovascular injury.PATIENTS AND METHODS:In an open label drug trial, we studied the effects of pentoxifylline (400 mg orally tid for 8 weeks) on 30 patients who displayed late, radiation-induced fibrosis at 1 to 29 years posttreatment (40 to 84 Gy). The primary outcome measurement was change in physical impairments thought to be secondary to radiation, including active and passive range of motion (AROM and PROM), muscle strength, limb edema, and pain. Plasma levels of cytokines (tumor necrosis factor alpha and FGF2) also were measured. Twenty-seven patients completed baseline and 8-week assessments, and 24 patients completed baseline, 8-week, and 16-week assessments.RESULTS:After 8 weeks of pentoxifylline intervention, 20 of 23 patients with impaired AROM and 19 of 22 with impaired PROM improved; 11 of 19 patients with muscle weakness showed improved motor strength; five of seven patients with edema had decreased limb girth; and nine of 20 patients had decreased pain. Pretreatment FGF2 levels dropped from an average of 44.9 pg/mL to 24.0 pg/mL after 8 weeks of treatment.CONCLUSION:Patients receiving pentoxifylline demonstrated improved AROM, PROM, and muscle strength and decreased limb edema and pain. Reversal of these delayed radiation effects was associated with a decrease in circulating FGF2.

Objective: To determine whether real-tine ultrasound imaging can provide quantitative data that distinguish pathologic from healthy muscle and that correlate with strength measures.Design: Nonrandomized matched-pair, repeated-measures design.Setting: Ultrasound imaging laboratory, rehabilitation medicine department, government research hospital.Participants: Nine patients with stable active or inactive myositis, stratified into 3 groups based on their 10-point manual muscle test (MMT) scores, and 9 age- and gender-matched controls.Interventions: Maximal isometric contraction of the rectus femoris muscle in 2 knee-flexion positions (60, 90) during simultaneous ultrasound imaging and muscle force dynamometry.Main Outcomes Measures: Changes of the rectus femoris muscle in horizontal (X) and vertical (Y) diameters between relaxed and contracted states, and muscle force measurements.Results: The X diameters decreased and the Y diameters increased during isometric contraction in all participants. For each group, average changes in cross-sectional diameters were consistently higher in controls than in patients. Patients with MMT less than 8 differed significantly from controls in both X and Y dimensions. A moderately strong correlation was found between muscle force and the Y diameter during contraction at 60degrees (r=.78) and 90degrees (r=.67) knee-flexion angles.Conclusions: Ultrasonography provided a quantitative measure of change between relaxed and contracted state of muscle, which correlated with muscle force. Ultrasound identified significant differences in cross-sectional diameters between the myopathic and normal muscles sampled and may be useful for measuring muscle response to drug and exercise therapy. (C) 2004 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation.

1Chief, Rehabilitation Medicine Department Warren G. Magnusen Clinical Center National Institutes of Health Bethesda, Maryland 2Deputy Chief, Rehabilitation Medicine Department Warren G. Magnusen Clinical Center NIH Bethesda, Maryland

OBJECTIVE:To study the effects of the adenine analog, fludarabine, on patients with refractory dermatomyositis and polymyositis, and to assess variables used in following myositis patients during medical intervention.METHODS:Patients whose myositis was not controlled by prednisone and at least one other immunosuppressive medication were entered into a pilot study during which they received 6 monthly cycles of intravenous fludarabine. Patients were assessed at baseline, every other month, and at month 7 for primary outcome measures of strength and function. Other measurements including peripheral blood cell subsets, muscle enzymes, and various assessments of disease activity were followed monthly during the fludarabine infusion period and for up to 6 months post therapy.RESULTS:Of 16 patients who entered the study, 4 patients were classified as improved, and 7 patients were classified as unchanged. Five patients who withdrew before month 7 were classified as treatment failures. Fludarabine caused a significant and prolonged lymphopenia without an increase in infectious complications over that seen with other immunosuppressive agents used for myositis. A sudden death of one patient at the end of the study was not thought to be drug related. Variables followed during the study emphasized the distinction between patient functional improvement and disease remission.CONCLUSION:A subset of patients with refractory myositis may benefit from fludarabine therapy and controlled trials are indicated. Refinement and validation of variables useful for following myositis patients await larger studies.

Myopathies, regardless of their varied etiologies, are associated with muscle damage and, often, other organ system involvement causing physical impairment. The resultant adverse impact on mobility, activities of daily living, communication, and cardiorespiratory fitness results in disability, handicap, and reduced quality of life. The extent of the functional impact depends on the type of myopathy and the extent of clinical involvement caused by it, duration of the disease, time to diagnosis and treatment, and rate of progression and response to medical management. The usefulness of rehabilitation in maintaining function in muscular dystrophy has been addressed in the literature for several decades. However, the need for and efficacy of specific rehabilitation interventions and assessment tools to measure function in inflammatory myopathy have just recently emerged this decade. Although studies are few in number, they are useful. More research is needed and should be encouraged. The overall goal of rehabilitation is to enhance function and quality of life.

The primary aim of intervention in cerebral palsy is to improve the quality of walking. However, we have no means of measuring this quality or its change following treatment. In this study we set out to develop a quality score for walking which can objectively quantify quality and its change following intervention. We video recorded 60 children while walking, 55 with spastic diplegia and 5 normal children and asked 8 observers to grade the quality of their walking ability using an analogue scale. Having demonstrated the reliability of the scale, each child's quality score was then compared with their sagittal plane kinematic data and their functional data. Balance, speed, range of motion and effort emerged as the key objective and measurable features which determine the quality of walking. These four features were weighted in a formula to equate with the quality scores. The formula was tested using the final 20 patients by comparing their predicted quality scores as derived from the formula with the actual quality scores as determined by the observers.

No abstract available for this article.

Rheumatoid arthritis is a systemic rheumatoid disease that can involve many organ systems, the axial cervical spine, and almost all peripheral joints. Significant effects include fatigue, decreased aerobic capacity, muscle atrophy, diminished strength and joint motion, pain, and altered biomechanical joint function. Exercise has been proven to increase strength, joint motion, aerobic capacity, and function. Exercise programs should begin early in the disease process and should be individualized and periodically modified according to the disease stage and level of activity.

A patient with active stable idiopathic polymyositis received a 4-week supervised right quadriceps and biceps isometric strengthening program. He demonstrated a significant increase in isometric peak torque without a sustained rise in creatinine phosphokinase (CPK). A significant decrease in postexercise CPK occurred at the end of the exercise program. We conclude a randomized clinical trial using a nonexercised patient group vs an exercised patient group may be useful.

A retrospective review is presented on 145 patients who underwent limb-sparing surgery and radiation therapy (with or without adjuvant chemotherapy) for their primary soft tissue sarcomas of the extremities on protocol between 1975 and 1986. The focus on our analysis was the acute and long term toxicity of treatment on limb function. The most common acute complication was skin reaction, occurring in 52 patients (36%). Long term (occurring after more than 1 year following all treatment) treatment complications in the extremity were as follows: bone fracture = 6%; contracture = 20%; pain requiring narcotics = 7%; edema greater than 2+ = 19%; moderate to severe decrease in range of motion = 32%; moderate to severe decrease in manual muscle strength = 20%; orthotic device required = 9%; cane or crutch required = 7%; chronic infection = 9%; and tissue induration = 57%. Three amputations for treatment complications were required. Inclusion of more than 50% of the joint in the radiation portal was associated with a higher frequency of contracture. High nominal standard dose (> 1760 rets, > 63 Gy at 1.8 Gy per fraction) resulted in more painful limbs as well as limbs with increased edema, decreased manual muscle strength, decreased range of motion, and skin telangiectasias. Edema was more often noted in patients with a longer radiation portal (greater than 35 cm), as was tissue induration. Chronic ulcer or infection was more frequently seen in patients with lower extremity tumors and when more than 75% of the extremity diameter was irradiated. Although chemotherapy given concurrent with radiation therapy was associated with a higher number of acute skin reactions, this did not appear to translate into increased long term morbidity. The percentage of patients ambulating without assistive devices and with mild or no pain was 84%. Careful attention to the techniques of radiation therapy may have a significant impact on minimizing acute and long term complications of limb sparing treatment for extremity soft tissue sarcoma.

We evaluated the relationships among pain, structural deformity of the foot, 4 variables of gait, and an index of functional ambulation in 31 patients with rheumatoid arthritis. We saw significant correlations between the ambulation index and all gait variables (p less than 0.005). For the group as a whole, pain in the foot did not correlate with structural deformity. However, when patients were grouped according to the relative preponderance of pain or deformity and duration of disease, we found correlations between the sites of pain and deformity. Pain in the lower extremity, and the knee or hindfoot separately, showed correlations with functional ambulation. Foot deformity did not correlate with functional ambulation at all. Foot pain correlated well with velocity and especially stride length (p less than 0.005), and not as well with cadence and double stance time. Fewer correlations were seen between foot deformity and gait. In general, hindfoot disease was associated with greater impairment of gait and mobility than forefoot disease.

National Institutes of Health, Bethesda, Maryland, USA Current Opinion in Rheumatology 1990, 2:320-326

This self-directed learning module presents core information and new advances in the orthotic management of problems of the runner and of patients with neurovascular foot ulcers or arthritis. Additional topics covered include a comprehensive approach to positioning and splinting for burns and tone-reducing orthoses for spasticity management. It is part of the chapter on prosthetics, orthotics, and assistive devices for the Self-Directed Medical Knowledge Program Study Guide for practitioners and trainees in physical medicine and rehabilitation.

This self-directed learning module presents core information and new advances in the orthotic management of problems of the runner and of patients with neurovascular foot ulcers or arthritis. Additional topics covered include a comprehensive approach to positioning and splinting for burns and tone-reducing orthoses for spasticity management. It is part of the chapter on prosthetics, orthotics, and assistive devices for the Self-Directed Medical Knowledge Program Study Guide for practitioners and trainees in physical medicine and rehabilitation.