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[Is early treatment of infants at risk of spastic diplegia warranted?].

OBJECTIVE: To test the possible link between first investigation and development of spastic diplegia. METHOD: A long-term retrospective study was carried out in the Neonatal Medicine Department of Arras Hospital, the C.A.M.S.P. of that town and with corresponding therapists. RESULTS: The study undertaken from January 1, 1991, to December 31, 1997, involved 56 children suspected of developing spastic diplegia and requiring a specialized course of treatment during the evolution of disease. In 32, evolution of disease was favourable, and in 24 spastic diplegia developed. An unfavourable evolution was significantly associated with late intervention of rehabilitation. CONCLUSION: Although few pre- or neonatal factors differentiated the two groups of children in their opposite evolution, haemorrhage during pregnancy and early detection seem to be determining factors in spastic diplegia.

Cerebral Palsy↗

A clinical and etiologic profile of spastic diplegia.

To identify the clinical and etiologic profile of children with spastic diplegia, the medical records of patients with spastic diplegia in a single practice over a 12-year period were systematically and retrospectively reviewed. Clinical factors and possible etiology based on investigations were identified. Univariate and binomial logistical regression analyses were undertaken to identify factors correlating with an etiologic determination. Chart review identified 54 children with spastic diplegia. There were 31 (57.4%) preterm children and 23 (42.6%) term children. Periventricular leukomalacia was diagnosed in 24 (44.4%) children (26.1% of term children, 58.1% of preterm children). An etiology was not identified in 25 (46.3%) children: 14 (60.9%) term children and 11 (35.5%) preterm children. Periventricular leukomalacia among all children correlated with a birth weight less than 2000 gm (P = 0.037), history of neonatal resuscitation (P = 0.004), and gestation less than 33 weeks (P = 0.001). Factors specifically associated with periventricular leukomalacia in term children were a problematic perinatal history (P = 0.011), a history of neonatal resuscitation (P = 0.011), and a history of neonatal respiratory distress (P = 0.046). Regression analysis revealed a correlation between an abnormal perinatal history and an etiology of periventricular leukomalacia among term children (odds ratio 8.67, 95% confidence interval 2.51-29.97, P = 0.001). Approximately half of all children with spastic diplegia encountered in clinical practice will have an etiology identified.

Adolescent↗

Vitamin E improves fibrinolytic activity in patients with coronary spastic angina.

INTRODUCTION: The fibrinolytic system has a major role as a defense mechanism against thrombus formation. Net fibrinolytic activity in plasma reflects the balance between tissue-type plasminogen activator and plasminogen activator inhibitor (PAI). PAI is the main factor determining overall fibrinolytic activity. MATERIALS AND METHODS: We examined the effects of oral administration of vitamin E, an antioxidant, on fibrinolytic activity and oxidative stress in patients with coronary spastic angina. Forty patients with coronary spastic angina were randomly assigned into two treatment groups, either vitamin E group (alpha-tocopherol acetate, 400 mg/day) or placebo group by means of computerized system. PAI activity and thioredoxin, a marker of oxidative stress, levels were measured before and at the end of 1 month treatment. RESULTS: Before treatment, the levels of PAI activity and thioredoxin were increased in patients with coronary spastic angina as compared with control subjects (n=17) (PAI activity levels: 13.6+/-1.4 vs. 7.6+/-2.2 IU/ml, p<0.05, thioredoxin levels: 22.8+/-1.7 vs. 16.0+/-1.4 ng/ml, p<0.05). In patients with coronary spastic angina, administration of vitamin E decreased both PAI activity and thioredoxin levels (PAI activity levels: 14.7+/-1.7 to 7.5+/-1.6 IU/ml, p<0.01, thioredoxin levels: 23.3+/-2.4 to 15.1+/-2.5 ng/ml, p<0.01), whereas placebo had no effect on these variables. CONCLUSIONS: Oral administration of vitamin E improved fibrinolytic activity and the improvement was associated with a decrease in oxidative stress. Administration of vitamin E is possible to be an effective adjunct therapy of coronary spasm in the absence of coronary atherosclerosis.

Administration, Oral↗

Developmental skills of children with spastic diplegia: functional and qualitative changes after selective dorsal rhizotomy.

OBJECTIVE: This study examined changes in muscle tone, passive range of motion, stability, and mobility in developmental skills at 6 months and 1 year after selective dorsal rhizotomy (SDR). DESIGN: Prospective outcome study of a consecutive sample. SETTING: Private children's hospital. PATIENTS: Twenty-six children with spastic diplegia: 13 independent and 13 dependent ambulators (assistive devices). RESULTS: A decrease in spasticity was seen at 6 months after SDR, with no further decrease at 1 year. Increases in passive range of motion of the hip and ankle were seen at 6 months after SDR. The ability to assume and maintain developmental positions with improved alignment and stability was seen more frequently at 6 months after SDR, whereas an improvement in the ability to perform transitional movements was seen more frequently at 1 year after SDR. CONCLUSION: SDR decreases spasticity and increases lower extremity range of motion in children with spastic diplegia and appears to be associated with the ability to assume a greater variety of developmental positions with improved alignment, thus greater stability. Improvements in the ability to perform difficult transitional movements at 1 year after SDR are most likely the result of the combined effect of maturation, SDR, and intensive therapeutic intervention.

Activities of Daily Living↗

Inhibitory casting decreases a vibratory inhibition index of the H-reflex in the spastic upper limb.

OBJECTIVE: To test the hypothesis that application of an inhibitory cast to the spastic upper limb will decrease a vibratory inhibition index (VII) of the H-reflex in the spastic upper limb. DESIGN: Prospective, nonrandomized, open-label trial. SETTING: University tertiary care center. PARTICIPANTS: Eight adults with upper limb spasticity. INTERVENTION: Fiberglass cast application spanning the wrist to the upper arm. MAIN OUTCOME MEASURE: The amplitude of the H-reflex with and without continuous 60Hz vibration to the tendon of the flexor carpi radialis was measured, and the VII was calculated using the formula: [H-reflex amplitude (vibrated)/H-reflex amplitude (control)] x 100%. RESULTS: Mean VII decreased from baseline (70.7) on day 1 (67.6, p = .699), day 2 (55.9, p =.066), and day 3 (43.5, p = .033) of casting, and increased on day 4 (89.9, p = .146) after removal of the cast. CONCLUSION: Findings lend support to the idea that during application of an inhibitory cast motor neuron excitability is decreased in the spastic upper limb.

Adult↗

The relationship between clinical and laboratory measures of spasticity.

This report examines the strength of the relationship between two clinical and two laboratory measures of spasticity. Clinical and laboratory measures of both reflex activity and resistance to movement were collected to describe spasticity in two groups of people after stroke. For both data sets, significant relationships between the clinical and laboratory measures were found only for measures of resistance to movement, not for measures of reflex activity. This finding suggests that the Ashworth scale is the best clinical tool for measuring resistance to movement. However, it is important to note that the cause of the resistance to movement graded using this tool may not be spasticity. A measurement tool which can differentiate spasticity from contracture is required for clinical practice.

Journal Article↗

Increase in plasma levels of secretory type II phospholipase A(2) in patients with coronary spastic angina.

OBJECTIVE: Plasma levels of sPLA(2) were increased in various chronic inflammatory diseases including coronary artery disease. Lipid products mediated through PLA(2) have been shown to induce impairment of endothelium-dependent dilation, contraction of smooth muscle and proliferation of smooth muscle cells, all of which might lead to coronary spasm. Thus, this study investigated whether plasma levels of secretory non-pancreatic type II phospholipase A(2) (sPLA(2)) may be increased in patients with coronary spastic angina, considering the possible link of sPLA(2) with pathogenesis of coronary artery spasm. METHODS: Plasma levels of sPLA(2) in peripheral circulation, in coronary sinus and in aortic root were measured in 57 patients with coronary spastic angina, 46 patients with stable effort angina and 53 control patients by radioimmunoassay. RESULTS: The peripheral plasma levels of sPLA(2) were increased in patients with coronary spastic angina compared with control patients. In multivariate statistical analysis, the increase in sPLA(2) levels was a significant risk for the presence of coronary spasm independent of other risk factors including C-reactive protein levels. The coronary sinus-arterial difference of plasma sPLA(2) levels, reflecting sPLA(2) released into the coronary circulation, was increased during coronary spasm induced by the intracoronary infusion of acetylcholine in patients with coronary spastic angina, but it remained unchanged both during the acetylcholine infusion and during myocardial ischemia provoked by rapid atrial pacing in patients with stable effort angina and in control patients. CONCLUSION: The increase in peripheral plasma levels of sPLA(2) is a significant risk factor for the presence of coronary spasm and it may possibly reflect inflammatory activity in spasm coronary arteries.

Acetylcholine↗

Motor strategies in standing up in leukomalacic spastic diplegia.

In spastic diplegia impaired postural control jeopardizes the organization of whole-body movements. We studied segmental motor patterns involved in standing up from a supine position in ten children with spastic diplegia associated with periventricular leukomalacia and 14 unimpaired children using a visual analysis scale previously devised for developmental research. This approach examines specific movement patterns in upper limbs, axis and lower limbs. We found that children with spastic diplegia use movement patterns described in normal children but with markedly reduced intra- and interindividual variability. One previously undescribed stereotyped lower limb pattern was observed in four patients. This approach can systematically characterize the limited repertoire of movement in patients with spastic diplegia and therefore contribute to a better understanding of motor control.

Biomechanical Phenomena↗

Nitric oxide-mediated flow-dependent dilation is impaired in coronary arteries in patients with coronary spastic angina.

OBJECTIVES: This study sought to examine whether flow-dependent dilation is impaired at the site of coronary artery spasm in patients with coronary spastic angina. BACKGROUND: Physiologic stimuli such as exercise and exposure to cold have been shown to cause an increase in coronary blood flow, leading to flow-dependent dilation of coronary arteries in normal subjects, but cause coronary constriction in patients with coronary spastic angina. METHODS: A maximal increase in blood flow was induced selectively in the left anterior descending coronary artery (LAD) by infusion of adenosine through a Doppler flow catheter tip in the midportion of the LAD in 10 patients with coronary spastic angina, all with angiographically demonstrated spasm of the LAD, and in 11 control patients. Coronary artery diameter at the proximal site of the LAD (exposed to increased flow but not to adenosine) was measured by quantitative angiography. RESULTS: Flow-dependent dilation of the proximal LAD was found to be less in spasm arteries than in control arteries. Infusion of NG-monomethyl-L-arginine (L-NMMA) in the proximal LAD suppressed flow-dependent dilation in control arteries but had no significant effect on spasm arteries. The dilator response to nitroglycerin was not impaired in spasm coronary arteries. CONCLUSIONS: Our results indicate that flow-dependent coronary dilation is impaired in spasm arteries, partly due to a deficiency in endothelial nitric oxide bioactivity, which in turn may contribute to the increase in coronary tone during physiologic stimuli in patients with coronary spastic angina.

Adenosine↗

Vitamin C attenuates abnormal vasomotor reactivity in spasm coronary arteries in patients with coronary spastic angina.

OBJECTIVES: This study sought to examine effect of vitamin C, an antioxidant, on the abnormal vasomotor reactivity in spasm coronary arteries. BACKGROUND: Oxygen free radicals generated in the arterial walls have been shown to cause endothelial vasomotor dysfunction. METHODS: Responses of the epicardial arterial diameters of the left coronary arteries to the intracoronary infusion of acetylcholine (ACh) (10 and 50 microg/min) were measured by quantitative coronary angiography before and during combined intracoronary infusion of vitamin C (10 mg/min) or saline as a placebo in 32 patients with coronary spastic angina and in 34 control subjects. RESULTS: Vitamin C infusion suppressed the constrictor response of the epicardial diameter to ACh in spasm coronary arteries but had no significant effect in the control coronary arteries (percent change in distal diameter in response to 10 microg/min of ACh [constriction (-), dilation (+), mean +/- SEM] before vitamin C: -8.2 +/- 2.9% in spasm arteries, +8.4 +/- 2.9%* in control arteries; during vitamin C: +0.2 +/- 3.8%* in spasm arteries, +7.2 +/- 1.3%* in control arteries [*p < 0.01 vs. spasm arteries before vitamin CI). The coronary sinus-arterial difference in plasma thiobarbituric acid reactive substances during ACh infusion, an indicator of lipid peroxidation in coronary circulation, was higher in patients with coronary spastic angina than in control subjects (p < 0.01) but was suppressed in patients with coronary spastic angina to comparable levels in control subjects by combined infusion of vitamin C. Saline infusion had no effect. CONCLUSIONS: The results indicate that vitamin C attenuates vasomotor dysfunction in epicardial coronary arteries in patients with coronary spastic angina. Oxygen free radicals may at least in part play a role in the abnormal coronary vasomotor reactivity in response to ACh in spasm coronary arteries.

Acetylcholine↗

Selective dorsal rhizotomy as a treatment option for children with spastic cerebral palsy.

Cerebral palsy is the most common motor disorder originating in childhood and spasticity is the most frequent manifestation. The treatment strategies to reduce spasticity and thereby ameliorate the attendant gait abnormalities have included physiotherapy, orthoses, antispastic medications, orthopaedic surgery and neurosurgery. Of these, the neurosurgical procedure known as selective dorsal rhizotomy has gained widespread exposure, and indeed acceptance, over the past two decades, despite there being some controversy as to its efficacy. In this paper we review: cerebral palsy, including classification and treatment; selective dorsal rhizotomy, including historical background, patient selection, operative procedure, clinical outcome and complications; and gait analysis studies, including temporal-distance parameters, joint kinematics, normalisation for growth, and long-term follow-up. Both the short-term (1 year) and long-term (10 years) evidence has demonstrated that selective dorsal rhizotomy not only reduces spasticity but it also provides lasting functional benefits as measured by improved range of motion during gait. Rhizotomy is not a panacea for children with spastic diplegia but it is an important treatment option for the clinician to consider. Copyright 1998 Elsevier Science B.V.

Journal Article↗

Intrarater reliability of lower limb sagittal range-of-motion measures in children with spastic diplegia.

In this study, 10 sagittal lower limb range-of-motion measures were conducted in a blinded fashion in 25 children with spastic diplegic cerebral palsy and in 25 age- and sex-matched controls. The participants comprised 22 males, mean age 10 years 8 months and 28 females, with mean age 9 years 8 months; age range 6 to 17 years. One paediatric physical therapist performed duplicate goniometric measures at zero time and 7 days later using the same sequence of measures, location, and time of day. Mean absolute differences for measures within one session ranged from 0.7 to 2.9 degrees in controls and from 1 to 4.2 degrees in children with spastic diplegia. Most intraclass correlation coefficients (ICCs) for intra-sessional measures were more than 0.90 in both groups. Measures between sessions were less reliable. Mean absolute differences between sessions were up to 7.1 degrees for children with spastic diplegia and 8.6 degrees for controls, with most ICCs being less than 0.80. Inter-sessional variation in measures was similar in both groups, suggesting that measurement variability is not influenced by the presence of spasticity. Averaging of two measures did not improve inter-sessional reliability compared with the use of a single measure. Dynamic measures (R1) were as reliable as passive measures (R2), but there were inter-sessional differences in calculations using R1 and R2 measures of up to 30 degrees.

Adolescent↗

Intrathecal baclofen therapy for adults with spinal spasticity: therapeutic efficacy and effect on hospital admissions.

A prospective trial to demonstrate the efficacy of intrathecal baclofen therapy by implanted pump for adults with spasticity due to spinal cord injury or multiple sclerosis was initiated in our hospital. Of the 140 patients assessed, 7 met the following criteria for inclusion in the study: a modified Ashworth score > 3, a spasm frequency score > 2, and an inadequate response to oral anti-spasticity drugs, (i.e., baclofen, clonidine and cyproheptadine). All patients responded to intrathecal bolus injection of baclofen in the double blind, placebo-controlled screening phase (mean bolus dose = 42.8 micrograms). Programmable Medtronic pumps were implanted in 4 patients while 3 patients received non-programmable Infusaid pumps. Post-implantation, a marked decrease in spasticity occurred with a significant reduction of the Ashworth score (mean = 1.8, p < .005), a reduced spasm score (mean = 0.8, p < .005), and an improved leg swing in the pendulum test. These effects were maintained during a follow-up of 24-41 months (average infusion dose = 218.7 micrograms/day). The gross cost-savings due to reduced hospitalizations related to spasticity was calculated by comparing the cost for the two year period before pump implantation to the same period after treatment for 6 of the 7 patients. The cost of in-hospital implantation as well as the cost of the pumps were deducted from the gross savings. There was a net cost-saving of $153,120. Our findings agree with the reported efficacy and safety of intrathecal baclofen treatment, and illustrate the cost-effectiveness of this treatment.

Adult↗

Stretch reflex latencies in spastic hemiparetic subjects are prolonged after transcutaneous electrical nerve stimulation.

Low-intensity repetitive electrical stimulation such as dorsal column and transcutaneous electrical nerve stimulation (TENS) reportedly decreases spasticity and improves voluntary motor control. However, the mechanisms mediating these effects are unclear. Recent findings suggest that spasticity may be characterized more appropriately by a decrease in the stretch reflex threshold than by an increase in gain. Our objectives were: (1) to examine possible changes in stretch reflex excitability following 45 min of TENS, (2) to map out the time course of possible post-stimulation effects via both latency and magnitude (amplitude or area) measurements, and (3) to determine the role of segmental versus non-segmental mechanisms involved in mediating these changes. The effects of 45 min of segmentally and heterosegmentally applied TENS on lower limb reflexes in ten spastic hemiparetic subjects were contrasted with those resulting from placebo stimulation. We found that both segmentally and heterosegmentally applied TENS caused an immediate increase in soleus H reflex latencies that was evident for up to 60 minutes post-stimulation in over 75% of the subjects. Similar increases for up to 60 and 40 minutes post-stimulation was noted for the stretch reflex latencies in 50% and 67% of the subjects respectively for segmental and heterosegmental stimulation. These results suggested that manipulation of segmental and heterosegmental afferents for 45 min may lead to a decrease of the otherwise augmented stretch reflex excitability accompanying hemiparetic spasticity.

Adult↗

Relief of spasticity in SCI men and women using rectal probe electrostimulation.

Although there are numerous approaches to the treatment of spasticity, many patients are still unable to find a satisfactory method of managing their spasms with acceptable side effects. In the course of our fertility studies using rectal probe electrostimulation (RPES) in SCI men to produce ejaculation, we observed that a majority of the men experienced significant relief of their spasticity for many hours. This report describes a prospective, single-blinded study of this phenomenon in six SCI men and three SCI women who underwent RPES a total of 71 times. The mean age of the subjects was 28.2 years (21-41), the mean time from injury was 6.0 years (0.5-15); there were three paraplegic and six quadriplegic persons: four were Frankel class A and five were class B. Although all subjects had moderate to severe spasticity, only four took antispasm medications; one had undergone surgery for implantation of an epidural stimulator. The effectiveness of RPES on spasticity was evaluated by each subject for frequency of spasms and interference of daily activities and by independent, blinded assessors for tone, frequency of spasms and DTRs; four patients underwent quantitative videotape analysis of the pendulum test and two underwent somatosensory evoked potentials (SSEPs) to evaluate electrical activity in the central nervous system. Treatment variables included varying probe sizes and number of stimulations. All subjects experienced good to excellent decrease in tone, frequency of spasms and interference with ADL from 3 to 24 hours depending on treatment variables used. Mean duration of relief was 8.2 hours.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Botulinum toxin type A injections in the spastic upper extremity of children with hemiplegia: child characteristics that predict a positive outcome.

Evidence is increasing to suggest that botulinum toxin type A (BTX-A) plays a role in the management of upper extremity spasticity in the paediatric population. However, little information is available on the clinical characteristics of the child that predict a response to this intervention. Our research group previously published a randomized controlled trial demonstrating that BTX-A injection improves function of the upper extremity of children with spastic hemiplegia. In the present paper, we evaluate the child characteristics that predict a positive response to the BTX-A injections in the randomized treatment group. The treatment group was divided into positive functional responders and nonresponders using a cut score of a change of 10 points on the Quality of Upper Extremity Skills Test (QUEST). A two-way analysis of variance procedure was done comparing the following baseline characteristics: function as scored on the QUEST and the Pediatric Evaluation of Disability Inventory (PEDI) self-care domain, grip strength, upper extremity spasticity and age. Grip strength was significantly higher in responders with a P-value of 0.001. Young age approached significance with a P-value of 0.05. Correlation of change scores on the QUEST with baseline characteristics in the treatment group yielded similar results. BTX-A causes a reduction in spasticity and strength; we postulate that if the hand is weak initially, BTX-A can decrease hand function. Two case reports are presented that highlight the importance of grip strength and age.

Activities of Daily Living↗

The early markers for later dyskinetic cerebral palsy are different from those for spastic cerebral palsy.

Qualitative abnormalities of spontaneous motor activity in newborns and young infants are early predictive markers for later spastic cerebral palsy. Aim of this research was to identify which motor patterns may be specific for later dyskinetic cerebral palsy. In a large, prospectively performed longitudinal study involving four European hospitals we identified twelve cases with the relatively rare condition of dyskinetic cerebral palsy and compared their early motor development with twelve spastic cerebral palsy cases and twelve controls. From birth to the fifth month post-term, all infants were repeatedly videoed and their spontaneous motor patterns, including general movements, were assessed. Until the second month post-term, the infants that later became dyskinetic displayed a poor repertoire of general movements, "arm movements in circles" and finger spreading. Abnormal arm and finger movements remained until at least five months and were then concurrent with a lack of arm and leg movements towards the midline. Later dyskinetic infants share with later spastic infants the absence of fidgety movements, a spontaneous movement pattern that is normally present from three to five months. Qualitative assessment of spontaneous motor patterns enabled us to identify infants at high risk for dyskinetic cerebral palsy early in life. Additionally, we were able to discriminate them from those infants at high risk for later spastic cerebral palsy. This is a matter of significant clinical relevance because the two types of cerebral palsy ask for different management and early intervention.

Biomarkers↗

A new locus for autosomal recessive hereditary spastic paraplegia maps to chromosome 16q24.3.

Hereditary spastic paraplegia is a genetically and phenotypically heterogeneous disorder. Both pure and complicated forms have been described, with autosomal dominant, autosomal recessive, and X-linked inheritance. Various loci (SPG1-SPG6) associated with this disorder have been mapped. Here, we report linkage analysis of a large consanguineous family affected with autosomal recessive spastic paraplegia with age at onset of 25-42 years. Linkage analysis of this family excluded all previously described spastic paraplegia loci. A genomewide linkage analysis showed evidence of linkage to chromosome 16q24.3, with markers D16S413 (maximum LOD score 3.37 at recombination fraction [theta] of .00) and D16S303 (maximum LOD score 3.74 at straight theta=.00). Multipoint analysis localized the disease gene in the most telomeric region, with a LOD score of 4.2. These data indicate the presence of a new locus linked to pure recessive spastic paraplegia, on chromosome 16q24.3, within a candidate region of 6 cM.

Adult↗