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At least 397 records · Page 22Linked to original sources

Dramatically different phenotypes in mouse models of human Tay-Sachs and Sandhoff diseases.

We have generated mouse models of human Tay-Sachs and Sandhoff diseases by targeted disruption of the Hexa (alpha subunit) or Hexb (beta subunit) genes, respectively, encoding lysosomal beta-hexosaminidase A (structure, alpha) and B (structure, beta beta). Both mutant mice accumulate GM2 ganglioside in brain, much more so in Hexb -/- mice, and the latter also accumulate glycolipid GA2. Hexa -/- mice suffer no obvious behavioral or neurological deficit, while Hexb -/- mice develop a fatal neurodegenerative disease, with spasticity, muscle weakness, rigidity, tremor and ataxia. The Hexb -/- but not the Hexa -/- mice have massive depletion of spinal cord axons as an apparent consequence of neuronal storage of GM2. We propose that Hexa -/- mice escape disease through partial catabolism of accumulated GM2 via GA2 (asialo-GM2) through the combined action of sialidase and beta-hexosaminidase B.

Animals↗

Effect of tone-inhibiting casts and orthoses on gait. A case report.

In the adult case presented, inhibitory casting proved successful in decreasing the positive support reaction. When casting was discontinued, the positive reaction returned. To provide a long-term solution for this problem, a polypropylene tone-inhibiting AFO was fabricated. It incorporated the same principles as the casts. The success achieved with casting was rapidly regained and then surpassed with the use of the tone-inhibiting AFO. Natural recovery might be cited by some as the cause for improvement in the patient presented. However, the loss of improvement during the interval between the removal of the inhibitory casts and the initiation of the use of the tone-inhibiting AFO demonstrates that the casting and the position provided by the casts had a positive effect on our patient. This notion is further supported by the fact that our patient still required use of the tone-inhibiting AFO as late as March 1980. We have no explanation for the rapid improvement following the cranioplasty. Suggestions for further research would include a similar study with a larger number of subjects and the use of cinematography and kinesiological EMG during walking with and without tone-inhibiting casts and orthoses to determine the effect of the devices on spastic muscle groups and gait patterns.

Adult↗

Electromyographic biofeedback applications to the hemiplegic patient. Changes in upper extremity neuromuscular and functional status.

The effect of a specific EMG biofeedback treatment protocol on quantified changes in neuromuscular measures and functional activities was examined among the upper extremities of 22 chronic stroke patients who each received 60 feedback training sessions. These data were compared with changes measured from a Control Group of 9 (no treatment) patients. Those patients receiving feedback training showed significant improvements in numerous neuromuscular measures but not in functional measures. When the Experimental Group was subdivided into two groups (hand, n = 5; no hand, n = 17) on the basis of acquiring a specific hand function, significant pretreatment differences in neuromuscular status emerged. Based upon these pretreatment differences and outcome measures, characteristics possibly predictive of beneficial outcomes from EMG biofeedback training were exposed. Chronic stroke patients who gained maximal functional benefits from the biofeedback intervention initially had greater active range of motion at all major upper extremity joints and comparatively less hyperactivity within typically "spastic" muscles. Electromyographic biofeedback can lead to substantial improvements among select chronic stroke patients and can be of considerable functional benefit to others.

Adult↗

Effect of intermittent, supine cervical traction on the myoelectric activity of the upper trapezius muscle in subjects with neck pain.

This study was undertaken to compare the myoelectric activity of the upper trapezius muscle before, during, and after intermittent, supine cervical traction. Twelve people with diagnosed disease or injury of the cervical spine served as subjects. Electromyographic recordings were taken from the upper trapezius muscle with bipolar surface electrodes. The subjects were treated with 20 minutes of intermittent, cervical traction at a force of 8% of their body weight. Recordings were taken with the subjects in the supine position before the traction, during one pull and release phase of the 10th and 20th minutes of traction, and after completion of the traction treatment. An analysis of variance with repeated measures showed no significant differences in the myoelectrical activity during the six time periods measured. The results of this study do not support the clinical use of intermittent, supine traction to produce cervical muscle relaxation.

Adolescent↗

Force-time measurements of knee muscle functions of subjects with multiple sclerosis.

The purpose of this study was to determine whether the time-rate of muscle tension development, the muscle tension-maintaining capacity, and the reciprocal inhibition time of muscles in subjects with multiple sclerosis were significantly different from those of healthy subjects. An isokinetic dynamometer was used to evaluate the quadriceps femoris and hamstring muscles of 15 Experimental Group subjects with multiple sclerosis and 17 healthy Control Group subjects. The muscles were tested isometrically at 45 degrees of knee flexion and isokinetically at speeds of 30 degrees/sec and 90 degrees/sec. The subjects with multiple sclerosis demonstrated a significant (p less than .001) slowing of the time-rate of muscle tension development and a significant (p less than .005) decrease in muscle tension-maintaining capacity when compared with healthy subjects. The difference in the reciprocal inhibition time of muscles of the Experimental and Control Groups was not significant. When the reciprocal inhibition time of muscles was expressed as a percentage of the total torque curve, however, a significant prolongation (p less than .001) was observed in the subjects with multiple sclerosis.

Adolescent↗

Clinical investigations of muscle contractility.

The use of tests of muscle contractility based on electrically stimulated contractions means that information obtained from the more traditional tests of voluntary muscular performance and strength may be complemented by indices which are independent of volition and independent of muscle mass. Such tests are no longer limited to peripheral muscles but may now be applied to a large proximal muscle--the quadriceps femoris. This report describes the in vivo testing of a drug's pharmacological activity on the quadriceps muscle by a study of the relative forces produced by different frequencies of stimulation. It also illustrates that hypothyroidism is not the only endocrine disorder whose treatment may be monitored by measuring the speed of muscle relaxation. Finally, it describes a patient whose quadriceps weakness was explained by the demonstration of the failure of the muscle to maintain tension during prolonged stimulation.

Clinical Trials as Topic↗

Silicone molar block used as a fulcrum in condylar surgery.

The narrowness of the temporomandibular joint (TMJ) can cause difficulty in open reduction and fixation of condylar fracture. The authors designed a silicone block that acts as a fulcrum to create more space around the TMJ. The block is made of high-quality silicone that is firm, elastic, nontoxic, autoclave tolerant, and harmless to dental tissue. This block has been used successfully in facilitating reduction and fixation of condylar fractures in 27 patients, and for releasing spastic muscles during closed reduction in 2 patients with prolonged TMJ dislocation. In edentulous patients, the silicone molar block can be used as a mouth opener to facilitate most intraoral procedures without injury to the gum.

Adult↗

Choosing upper limb muscles for focal intervention after traumatic brain injury.

The upper motoneuron syndrome (UMNS) resulting from lesions of corticospinal pathways is an important source of disability after traumatic brain injury (TBI). Classic expressions of motor behavior in UMNS are of 2 kinds: (1) manifestation of muscle underactivity, termed negative signs, and (2) manifestation of a variety of forms of muscle overactivity, termed positive signs. Combinations of negative and positive signs give rise to clinical patterns of movement dysfunction such as the flexed elbow, the clenched fist, and the thumb-in-palm deformity. These clinical patterns can be viewed as reflecting a net balance of muscle forces acting across the joints of a limb. Individual muscles are amenable to a variety of focal interventions such as neurolysis, chemodenervation, or surgery. Since more than one muscle acts across most joints, choices among muscles for focal intervention are many. This article will focus on focal interventions of upper limb muscles of patients with TBI who have UMNS and will explore the theme of choosing upper limb muscles for focal interventions after TBI.

Brain Injuries↗

Modulation of spinal cord excitability by subthreshold repetitive transcranial magnetic stimulation of the primary motor cortex in humans.

Repetitive transcranial magnetic stimulation (rTMS) allows the modulation of intra-cortical excitability and may therefore affect the descending control of spinal excitability. We applied rTMS at subthreshold intensity and 1 Hz frequency for 10 min to the left primary motor cortex representation of the flexor carpi radialis muscle (FCR) in 10 subjects and assessed the H and M responses to median nerve stimulation before and after the rTMS. Following rTMS, H wave thresholds significantly reduced by approximately 20%. Maximal H but not M wave amplitude significantly increased over the baseline, so that H/M amplitude ratio was increased by 41%. Sham stimulation did not induce any noticeable change in M or H waves. Slow rTMS might facilitate monosynaptic spinal cord reflexes by inhibiting the cortico-spinal projections modulating spinal excitability.

Adult↗

Neural factors underlying reduced postural adaptability in children with cerebral palsy.

This study aimed to test mechanisms underlying impairments in balance control in children with cerebral palsy. We hypothesized that balance loss during large/fast perturbations could be due to reduced contraction of agonist muscles, delay in muscle contraction or simultaneous contraction of antagonist muscles, reducing the efficiency of the agonist burst. Electromyograms were recorded as children recovered from balance threats of varying magnitudes/velocities. In typically developing children muscle response magnitudes increased with larger/faster perturbations, while in children with cerebral palsy they did not. There was no difference in muscle onset latency or antagonist co-contraction between groups. Thus the primary constraint on balance recovery in these children is insufficient levels of contraction of agonist postural muscles.

Adaptation, Physiological↗

Localization of the motor nerve branches and motor points of the triceps surae muscles in korean cadavers.

OBJECTIVE: To identify the precise locations of the muscular branches (MBs) and motor points (MPs) of triceps surae muscles in relation to the bony landmarks. DESIGN: Thirty-six limbs from 22 adult cadavers were anatomically dissected. The location and the number of MPs and MBs from the tibial nerve to the triceps surae muscles were defined relative to four bony landmarks. Locations of the MBs and the MPs were expressed as a percentage of the lower leg length and the percentage of the lower leg width. RESULTS: One MB was identified from the posterior tibial nerve to the soleus muscle, one or two branches to the medial gastrocnemius muscle, and up to four branches to the lateral gastrocnemius muscle. One to four MPs were identified in the triceps surae muscles. CONCLUSION: Estimation of the locations of the MBs and the MPs, when combined with our anthropometric observations, could increase the ease and accuracy with which MB or MP blocks can be applied to the triceps surae muscles.

Adult↗

A focused review of the use of botulinum toxins for low back pain.

Chronic low back pain is the second most common illness reported by patients in the United States and accounts for substantial morbidity and health-care resource utilization. Many back and spine stressors can contribute to tissue injury, resulting in acute or chronic pain. In response to injury, biochemical processes that cause inflammation and nerve sensitization increase pain levels and contribute to a cycle of reactivity that further heightens patients' sensitivity to pain stimuli. Treatment of back pain depends on its severity, duration, and underlying cause. Traditional therapeutic options include exercise, oral anti-inflammatory or analgesic medication, antidepressants, physical therapy and, in severe cases, surgery. Unfortunately, dissatisfaction with treatment of back pain is common. Oral medications may not completely alleviate symptoms, and opioid analgesics must be used with caution because of their addictive properties. Surgery does not always produce relief and, in some cases, may even exacerbate the problem. Botulinum toxin, which has already been shown to alleviate pain associated with cervical dystonia and other conditions characterized by muscle spasticity, is now being studied for the treatment of back pain. Preliminary evaluations have shown that this treatment is safe and has the advantage of providing local relief directly to the site of injury or pain, without causing systemic side effects. Initial data from small trials also suggest that botulinum toxin is effective, alleviating back pain in selected patients. On the basis of these promising results, additional study in larger trials is warranted.

Animals↗

Predictive value of the Duncan-Ely test in distal rectus femoris transfer.

Fifty-six patients who underwent 94 distal rectus femoris transfers and pre- and postoperative gait analyses were retrospectively reviewed. The patients were divided into three groups based on pre- and postoperative Duncan-Ely tests. Group A (34 limbs) had positive tests both before and after surgery. Group B (46 limbs) had positive tests before surgery and negative tests after surgery. Group C (13 limbs) had negative tests both before and after surgery. One limb had a negative test before surgery and a positive test after surgery and was not included in any group. Knee arc increased significantly in both groups with positive preoperative Duncan-Ely tests (groups A and B), but not in the group with negative preoperative tests (group C). The timing of peak knee flexion in swing improved in all groups, but the change was smaller and not statistically significant in the group with negative preoperative tests (group C). The findings of the current study indicate that the Duncan-Ely test may be a helpful predictor of outcome in children for whom distal rectus femoris transfer is being considered. Caution should be exercised when patients have weak quadriceps and a negative Duncan-Ely test before surgery, particularly when concurrent calf lengthening procedures are planned.

Adolescent↗