神经肌肉病患者功能评定的临床应用进展

大部分神经肌肉病属于罕见病,其中相对较为常见的是Duchenne型肌营养不良和脊髓性肌萎缩症。神经肌肉病的一个重要特征是进行性残疾,因此功能评定对评价神经肌肉病患者的疾病严重程度、运动功能以及活动能力非常重要。目前功能评定包括肌力、关节活动度、姿势、活动能力等方面,涉及多种评定工具,但尚未形成统一标准。精确、合理的评定方法对多学科管理团队为患者制定个体化治疗方案具有重要意义。     

Contractures in neuromuscular disease.

The percentage of subjects with contractures, mean maximal loss of range, and relative contracture indices are reported in 230 patients, with 11 diseases seen in a neuromuscular disease clinic during a five-year period. The highest percentage of contractures occurred in patients with Duchenne muscular dystrophy. The number of contractures was significantly greater (p less than .001) (1) in the lower than in the upper extremities; (2) in diseases considered myopathic than in those considered neuropathic; (3) in diseases that are X-linked than in those that are not; and (4) in rapidly progressive than in slowly progressive diseases.     

The effect of botulinum toxin type-A injection on spasticity, range of motion and gait patterns in children with spastic diplegic cerebral palsy: an Egyptian study

Spasticity is defined as increased resistance to passive movement, secondary to hyperreflexia after an upper motor neuron lesion. In children with cerebral palsy (CP), it can interfere with mobility and self-care and can contribute to development of fixed myostatic contractures. This study investigated the efficacy of botulinum toxin type-A, a neuromuscular blocking agent that reduces muscle tone, in a variety of neuromuscular disorders, injections in a prospective, 3-month, controlled study involving 40 children with spastic diplegic CP. The patients were divided into two groups: Group 1 (20 patients) entered a botulinum toxin type-A injection+physiotherapy rehabilitation program; Group 2 (20 patients) were given the physiotherapy rehabilitation program only. Patients were assessed at 4, 8 and 12 weeks post-treatment using the Modified Ashworth Scale (MAS), dynamic gait pattern, ankle range-of-motion measurements and quantification of muscle denervation by nerve conduction techniques. The botulinum toxin type-A group demonstrated statistically significantly decreased spasticity, improved gait function and improved range of motion with evidence of partial denervation of the injected muscle compared to the control group. In conclusion, botulinum toxin type-A injections are a well-tolerated, non-surgical technique that can improve overall response to physiotherapy.     

Customized adjustable orthoses: their use in spasticity

Spastic contractures are a common problem which frequently requires the use of splinting or casting. Customized adjustable orthoses can reduce contractures without the problems often associated with these procedures. These devices reduced contractures in a head-injured patient's severely spastic wrist and ankles. As a wrist contracture decreased, the patient increasingly used her arm and hand for feeding, grooming, and leisure activities. The plastic orthoses, molded to the individual limb, incorporate Klenzak or Lehrman fracture brace joints. Custom fit positions spastic limbs and promotes improved range of motion. Small brace joint adjustments are done at intervals to promote slow stretch of spastic limbs; since the device remains on during the adjustment, there is no loss of range of motion. The device may be removed for skin monitoring. The patient maintains joint range, as the orthosis becomes a permanent part of the home program. High cost of the device is potentially offset by the advantages of decreased skin morbidity, reduced therapist time in making adjustment, and maintenance of gains in range of motion. Such orthoses also have been used for elbow contractures.     

Continuous passive motion device for hand rehabilitation

Prototypes have been designed and manufactured for a lightweight portable device which continually moves all the joints of the hand passively through a range of motion. Inflatable bag modules in the palm of the hand are ganged together to create the required range of motion. A battery-operated air pump cycles compressed air to the modules. Initial clinical experience with patients who have flexion contractures caused by burns, trauma, and Dupuytren's contractures has shown the device to be well tolerated and effective.     

A novel splint for proximal interphalangeal joint contractures: a case report

Wollstein R, Rodgers J, Ogden T, Loeffler J, Pearlman J. A novel splint for proximal interphalangeal joint contractures: a case report. Proximal interphalangeal (PIP) joint contractures are notoriously difficult to treat. Best results are obtained with early mobilization and splinting, though a high level of adherence is critical for a good outcome. A new roll-on splint that aims to increase motion with minimal difficulty was used. The patient described here with moderate PIP joint contractures (30°-60°) was treated successfully using this splint. The splint design and therapy protocol are described. The patient was treated for 12 weeks with good adherence to therapy and splinting. Total active motion increased by 87% in the index finger and 108% in the ring finger. Grip, pinch, and tip-pinch strengths increased. The Disabilities of the Arm, Shoulder and Hand score improved from 26.7% to 2.5%. At 3 months, the patient returned to work. Though this case illustrates some of the advantages and disadvantages of the new splint, further study is necessary to evaluate the splint and compare it with other existing forms of treatment for PIP joint contractures.     

Effect of joint mobilization using KEOMT and PNF on a patient with CLBP and a lumbar transitional vertebra: a case study

[Purpose] The purpose of this case study was to identify the effects of joint mobilization using Kaltenborn-Evjenth orthopedic manual therapy (KEOMT) and proprioceptive neuromuscular facilitation (PNF) techniques on a patient with chronic low back pain (CLBP) and a lumbar transitional vertebra. [Methods] The intervention methods were joint mobilization using KEOMT and PNF techniques. The program consisted of 40-min sessions 3 days a week for 4 weeks. The spinal motion (thoracic and lumbar vertebrae), pain, and thickness of the multifidus were measured. [Results] The angle of spinal curvature increased, and the range of motions (ROMs) flexion and extension increased in the thoracic and lumbar vertebrae. The pain score as measured on a visual analogue scale (VAS) and the Oswestry disability index (ODI) score decreased. The thickness of the multifidus (L4) increased on the left and right sides. [Conclusion] These results suggest that joint mobilization using KEOMT and PNF techniques had a positive effect on the spinal motion, pain, and thickness of the multifidus of a patient with chronic low back pain and a lumbar transitional vertebra.Key words: Lumbar transitional vertebra, Joint mobilization, Proprioceptive neuromuscular facilitation     

Alterations in functional movement after axillary burn scar contracture: a motion analysis study

Children with axillary burns often develop scar contractures that restrict shoulder movement. Objective data on functional movement patterns after contracture formation is sparse. The purpose of this study was to determine how axillary contractures affect shoulder movement during activities of daily living (ADLs). This was a prospective study of children with axillary contractures scheduled for surgical release. Three-dimensional upper extremity kinematic analysis was used to assess shoulder, elbow, and trunk motion during two ADLs: high reach and hand to back pocket. Results were compared with a pool of 49 normal age-matched controls. Eleven children with axillary contractures were compared with controls. During high reach, significant decreases in shoulder flexion, shoulder internal rotation, arm pronation, and trunk extension occurred. Elbow flexion increased significantly. In the hand to back pocket task, shoulder extension and elbow flexion decreased and shoulder abduction increased. Axillary contractures result in quantifiable movement changes during ADLs. Aggressive rehabilitation is required to prevent contracture formation. Three-dimensional motion analysis is a unique tool for the quantification of functional limitations and provides an objective method to evaluate treatment efficacy in patients with axillary contractures.     

Percutaneous intramuscular neuromuscular electric stimulation for the treatment of shoulder subluxation and pain in patients with chronic hemiplegia: a pilot study

OBJECTIVE: To investigate the feasibility of percutaneous intramuscular neuromuscular electric stimulation (perc-NMES) for treating shoulder subluxation and pain in patients with chronic hemiplegia. DESIGN: Before-after trial. SETTING: University-affiliated tertiary care hospital. PARTICIPANTS: A convenience sample of 8 neurologically stable subjects with chronic hemiplegia and shoulder subluxation. INTERVENTION: Six weeks of perc-NMES to the subluxated shoulder. MAIN OUTCOME MEASURES: Shoulder subluxation (radiograph), shoulder pain (Brief Pain Inventory), motor impairment (Fugl-Meyer score), shoulder pain-free external rotation (handheld goniometer), and disability (FIM instrument) were assessed before treatment (T1), after 6 weeks of neuromuscular stimulation (T2), and at 3-month follow-up (T3). A 1-way, repeated-measures analysis of variance using the generalized estimating equation approach was used to evaluate differences from T1 to T2 and from T1 to T3 for all outcome measures. RESULTS: Subluxation (p =.0117), pain (p =.0115), shoulder pain-free external rotation (p <.0001), and disability (p =.0044) improved significantly from T1 to T2. Subluxation (p =.0066), pain (p =.0136), motor impairment (p <.0001), shoulder pain-free external rotation (p =.0234), and disability (p =.0152) improved significantly from T1 to T3. CONCLUSIONS: Perc-NMES is feasible for treating shoulder dysfunction in hemiplegia and may reduce shoulder subluxation, reduce pain, improve range of motion, enhance motor recovery, and reduce disability in patients with chronic hemiplegia and shoulder subluxation. Further investigation is warranted.     

Balance-Retraining

Approximately 30% of people over 65 years of age and living in their own home fall at least once each year and the percentage is substantially higher in institutions. Falls result for multifactorial reasons and loss of mobility, various diseases and medications are risk factors. Aging itself contributes to decrement in neuromuscular performance and balance ability. A physically active lifestyle positively affects neuromuscular functions at any age. Older individuals who remain physically active may show a muscular performance that equals or exceeds those of younger sedentary adults. Regular exercise aimed at maintaining or increasing the range of motion, muscle mass, neuromuscular power and balance substantially reduces the risk of falls. The progressive increase of any exercise should involve a gradual reduction of the amount of support for standing.     

Progression and direction of contractures of knee joints following spinal cord injury in the rat

Joint contractures following central nervous system injuries remain a prevalent and significant complication, but no reports are available on evidence of contracture formation over time. The objective of this study was to determine the rate of contracture progression and the direction of loss in joint movement following spinal cord injuries (SCI). Forty-eight female Wistar rats were used. Twenty-four experimental rats underwent a spinal cord transection at the level of T8 and 24 control rats underwent a sham-operation. The animals were studied at each of 5 time points: 2, 4, 8, 12, 16, and 24 weeks after surgical intervention. The degree of contractures was assessed by measuring the femorotibial angle on both hindlimbs with the use of a goniometer. Knee joint motion was measured for flexion and extension direction. Knee flexion contractures developed in all experimental rats. The restriction in motion progressed during the first 12 weeks and plateaued thereafter. The contractures were produced almost exclusively by a loss in the extension range of motion. This study defined the time course that contracture progression was more rapid in the early stage after SCI and stabilized in the later stage of injury. Contractures following SCI occurred in flexion at the knees and resulted from a loss of extension. These findings should help guide timely treatment and provide a better understanding of contracture development.     

Use of a static adjustable ankle-foot orthosis following tibial nerve block to reduce plantar-flexion contracture in an individual with brain injury

BACKGROUND AND PURPOSE: Ankle plantar-flexion contractures are a common complication of brain injuries and can lead to secondary limitations in mobility. CASE DESCRIPTION: The patient was a 44-year-old woman with left hemiplegia following a right frontal arteriovenous malformation resection. She had a left ankle plantar-flexion contracture of -31 degrees from neutral. After a tibial nerve block, an adjustable ankle-foot orthosis was applied 23 hours a day for 27 days. Adjustments of the orthosis were made as the contracture was reduced. The patient received physical therapy during the 27-day period for functional mobility activities and stretching the plantar flexors outside of the orthosis. OUTCOMES: The patient's dorsiflexion passive range of motion increased from -31 degrees to +10 degrees. DISCUSSION: The application of an adjustable ankle-foot orthosis following a tibial nerve block, as an addition to a physical therapy regimen of stretching and mobility training, may reduce plantar-flexion contractures in patients with brain injury.     

The extremity function index (EFI), a disability severity measure for neuromuscular diseases: psychometric evaluation

Objective: To adapt and to combine the self-report Upper Extremity Functional Index and Lower Extremity Function Scale, for the assessment of disability severity in patients with a neuromuscular disease and to examine its psychometric properties in order to make it suitable for indicating disease severity in neuromuscular diseases.

Design: A cross-sectional postal survey study was performed among patients diagnosed with a neuromuscular disease.

Methods: Patients completed both adapted extremity function scales, questionnaires for psychometric evaluation, and disease-specific questions. Confirmatory factor analysis was performed, and reliability and validity were examined.

Results: Response rate was 70% (n?=?702). The Extremity Function Index model with a two-factor structure – for upper and lower extremities – showed an acceptable fit. The Extremity Function Index scales showed good internal consistency (alphas: 0.97–0.98). The known-groups validity test confirmed that Extremity Function Index scales discriminate between categories of “Extent of limitations” and “Quality of Life.” Convergent and divergent validity tests confirmed that Extremity Function Index scales measure the physical impact of neuromuscular diseases. Relative validity tests showed that the Extremity Function Index scales performed well in discriminating between subgroups of patients with increasing “Extent of limitations” compared to concurrent measurement instruments.

Conclusion: The Extremity Function Index proved to be a sound and easy to apply self-report disability severity measurement instrument in neuromuscular diseases.

• Implications for rehabilitation

• The Extremity Function Index reflects the functioning of all muscles in the upper and lower extremities involved in activities of daily living.

• The Extremity Function Index is an easy to administer and patient-friendly disability severity measurement instrument that has the ability to evaluate differences in disability severity between relevant neuromuscular disease subgroups.

• The Extremity Function Index is a valid and reliable disability severity measurement instrument for neuromuscular diseases.

     

Continuous passive motion in the treatment of knee flexion contractures. A case report

Joint contractures are a problem frequently encountered by therapists. Methods of treating joint contractures traditionally have consisted of range-of-motion and stretching exercises. The medical literature suggests that, to be effective, ROM and stretching exercises must be performed with well-controlled speed, force, and duration. The patient in our case study used continuous passive motion machines after conventional methods of exercise failed. He subsequently demonstrated a 55-degree, bilateral reduction in knee flexion contractures. The purpose of this case report is to illustrate the use of continuous passive motion machines in the treatment of severe knee flexion contractures.     

Extent and direction of joint motion limitation after prolonged immobility: an experimental study in the rat

OBJECTIVES: To test the hypotheses that contractures progress at different rates in relation to the time after immobilization, that immobilization in flexion leads to loss of extension range of motion, and that joints of sham-operated animals are better controls than the contralateral joint of experimental animals. STUDY DESIGN: Experimental, controlled study in which 40 adult rats had one knee joint immobilized at 135 degrees of flexion for up to 32 weeks and 20 animals underwent a sham procedure. At intervals of 2, 4, 8, 16, and 32 weeks, 8 experimental and 4 sham-operated animals were killed and their knee motion measured in flexion and extension. RESULTS: In the experimental group, the range of motion decreased in the first 16 weeks of immobility at an average rate of 3.8 degrees per week (p<.0001) to reach 61.1 degrees of restriction. A plateau was then observed from which the contracture did not progress further. The loss in range of motion occurred in extension, not in flexion. CONCLUSION: This study defined an acute stage of contractures starting at the onset of immobility and lasting 16 weeks, during which the range of motion was progressively restricted, and a chronic stage during which no additional limitation was detected. The loss in motion was attributed to posterior knee structures not under tension during immobilization in flexion. Contrary to the hypothesis, the contralateral joint was validated as a control choice for range-of-motion experiments.     

Opportunities and challenges for the development of antisense treatment in neuromuscular disorders

INTRODUCTION: Neuromuscular disorders are diseases of the musculature and/or the nervous system, generally leading to loss of muscle function. They are a frequent cause of disability and treatment options are often only symptomatic. Interestingly, for a number of neuromuscular disorders the application of antisense oligonucleotides has therapeutic potential. AREAS COVERED: The authors describe how this approach is exploited for different neuromuscular diseases, focusing on literature published in the past 10 years. For each disease the opportunities of this approach, the state of the art, and current challenges are described. EXPERT OPINION: A lot of progress has been made in the development of antisense-mediated approaches during recent years and they may become clinically applicable in the near future.     

Multimodal versus progressive treatment techniques to correct burn scar contractures

The treatment of burn scar contractures is a major emphasis in the rehabilitation of patients with burn injuries. Many treatment techniques have been used successfully but without a critical investigation of the best practice of care. In this study, we compared the outcomes for pediatric and adult patients treated with a multimodal therapy approach to treatment techniques that are considered to be progressive to determine if differences existed in the techniques. The medical records of 52 patients with documented burn scar contractures were reviewed for patient and rehabilitation treatment parameters. Included were population demographic information and type of treatment intervention used to correct the scar contracture. In particular, the postburn day when the contracture appeared, the percentage of range of motion deficit, the day when definitive treatment that eventually corrected the contracture was begun, and the days required to correct the contracture were noted. With equal range-of-motion deficits identified, the burn scar contractures of patients in the progressive treatment group were corrected in less than half the time of the burn scar contractures of the patients in the multimodal treatment group. This result occurred despite scar contractures that appeared significantly earlier and later initiation of definitive treatment.     

Compartment syndrome.

Compartment syndrome results from increased pressure in the closed compartments of the lower or upper extremities. Although it is not a common occurrence, it should be considered a possible complication whenever a patient experiences acute arterial occlusion or revascularization. If left untreated, compartment syndrome can lead to the loss of neuromuscular function, contractures, myoglobinuric renal failure, and amputation. Nurses play an integral role in the detection and treatment of compartment syndrome. Treatment is aimed at minimizing tissue damage and loss of function.     

Late onset polio sequelae: disabilities and handicaps in a population-based cohort of the 1956 poliomyelitis outbreak in The Netherlands.

OBJECTIVE: To investigate the prevalence of new neuromuscular symptoms, disabilities, and handicaps in a group of polio survivors. DESIGN: A self-constructed health questionnaire about neuromuscular complaints and disability and handicap levels during the stable period after recovery from polio and at present. SUBJECTS: Three hundred fifty subjects, derived from the 1,784 polio cases registered during the 1956 polio outbreak in The Netherlands. RESULTS: Respondents totaled 260 (74%), 27 of whom denied or did not recall having had paralytic poliomyelitis. The remaining 233 subjects comprised the study group (mean age, 44yrs; range, 39 to 77; SD = 6.3). Frequency of all neuromuscular complaints at present time was significantly higher than that during the stable period after polio (range in p of .001 to .004). Fifty-eight percent of cases reported an increase in muscle weakness in comparison with muscle condition during the stable period. Fifty-six percent reported an increase in disabilities, mainly a restriction in gait functions. Fifty-three percent reported increased handicaps with regard to occupation and social integration, and there was an increased need for adaptive measures and devices. CONCLUSION: Nearly 60% of a sample of Dutch survivors of the 1956 polio outbreak experience late onset polio sequelae, resulting in increased severity of disabilities and handicaps.     

Basic Principles Regarding Strength,Flexibility, and Stability Exercises

Strength, flexibility, and stability are physiologic parameters associated with health-related physical fitness. Each of these domains affects health in general, the risk of injury, how an injury is treated, and performance in activities of daily living and sports. These domains are affected by individual phenotype, age, deconditioning, occupational activity, and formal exercise. Deficits or loss of strength, flexibility, and stability can be prevented or reduced with exercise programs. Normal muscle strength has been associated with general health benefits, increased life expectancy, psychological benefits, prevention of illness, and reduction of disability in older adults. Static flexibility programs have been shown to improve joint range of motion and tolerance to stretch but do not appear to reduce the risk of musculoskeletal injury and may impair muscle performance immediately after a static stretch. Dynamic flexibility, on the other hand, may enhance power and improve sports-specific performance. Stability training leads to improved balance and neuromuscular control, may prevent injury to the knee and ankle joints, and can be used for treatment of patients with low back pain.