Severe Spastic Cerebral Palsy Research Articles

Placebo controlled utility and feasibility study of the H-reflex and flexor reflex in spastic children treated with intrathecal baclofen

ObjectiveTo evaluate feasibility and utility of the soleus H-reflex and tibialis anterior flexor reflex (FR) in identifying spinal cord neuronal response to intrathecal baclofen (ITB) in children with severe spastic cerebral palsy. MethodsDuring a randomized, double-blind, placebo-controlled dose-escalation test treatment, maximum H amplitude/maximum M amplitude (H/M ratio) and FR parameters were bilaterally recorded at baseline and 2–3h after intrathecal bolus administration of placebo and increasing doses of baclofen until both an improvement in the individual treatment goal(s) and a one-point reduction on the Ashworth scale were observed. ResultsElectrophysiological data of 14 children were studied. The H-reflex was feasible in 13 children, the FR threshold area in 9 and the FR, elicited with supramaximal stimulation, in only one child. After ITB, the H/M ratio significantly decreased (left: 0.67±0.47 to 0.15±0.18, P=0.005; right: 0.55±0.32 to 0.14±0.19, P=0.002) without placebo effect. FR threshold area after ITB, only decreased significantly in children not taking oral baclofen (left: 146±53 to 41±54mVms, P=0.000; right: 156±80 to 66±48mVms, P=0.002). ConclusionsThis is the first randomized, double-blind, placebo-controlled dose-escalation study in spastic children demonstrating the soleus H-reflex to be a feasible and objective measure to quantify the decreasing motoneuron excitability in response to ITB bolus administration. Only in children not taking oral baclofen, FR threshold area can also be used as an objective outcome measure, yet feasibility is limited. SignificanceWe suggest introducing the H-reflex as the electrophysiological gold standard for the evaluation of the effect of ITB in spastic children.

Botulinum Toxin Type A Injections to Salivary Glands: Combination With Single Event Multilevel Chemoneurolysis in 2 Children With Severe Spastic Quadriplegic Cerebral Palsy

Kim H, Lee Y, Weiner D, Kaye R, Cahill AM, Yudkoff M. Botulinum toxin type A injections to salivary glands: combination with single event multilevel chemoneurolysis in 2 children with severe spastic quadriplegic cerebral palsy. We describe 2 children with severe spastic quadriplegic cerebral palsy (CP) who have significant drooling and frequent aspiration pneumonia. They underwent simultaneous botulinum toxin type A (BTX-A) injections to salivary glands for drooling and prevention of aspiration pneumonia along with single-event multilevel chemoneurolysis (SEMLC) with BTX-A and 5% phenol for severe diffuse spasticity. There was significant improvement in drooling, frequency of aspiration pneumonia, and spasticity without adverse effect. BTX-A injections into the salivary glands, in addition to SEMLC, for these 2 children with medically complicated severe spastic quadriplegic CP, were safe and highly successful procedures, which improved their health-related quality of life.

Longitudinal Changes in Bone Density in Children and Adolescents with Moderate to Severe Cerebral Palsy

To assess the natural history of "growth" in bone mineral density (BMD) in children and adolescents with moderate to severe cerebral palsy (CP). A prospective, longitudinal, observational study of BMD in 69 subjects with moderate to severe spastic CP ages 2.0 to 17.7 years. Fifty-five subjects were observed for more than 2 years and 40 subjects for more than 3 years. Each evaluation also included assessments of growth, nutritional status, Tanner stage, general health, and various clinical features of CP. Lower BMD z-scores at the initial evaluation were associated with greater severity of CP as judged by gross motor function and feeding difficulty, and with poorer growth and nutrition as judged by weight z-scores. BMD increased an average of 2% to 5%/y in the distal femur and lumbar spine, but ranged widely from +42%/y to -31%. In spite of increases in BMD, distal femur BMD z-scores decrease with age in this population. Children with severe CP develop over the course of their lives clinically significant osteopenia. Unlike elderly adults, this is not primarily from true losses in bone mineral, but from a rate of growth in bone mineral that is diminished relative to healthy children. The efficacy of interventions to increase BMD can truly be assessed only with a clear understanding of the expected changes in BMD without intervention.

Agreement of aspiration tests using barium videofluoroscopy, salivagram, and milk scan in children with cerebral palsy

To study the agreement between three tests for aspiration, barium videofluoroscopy, salivagram, and milk scan we studied 63 children with severe non-ambulant spastic quadriplegic cerebral palsy (CP) aged 14 months to 16 years (32 males, 31 females). The salivagram was most frequently positive (56%, 95% confidence interval 43 to 68%); the next most frequently positive was barium videofluoroscopy when aspiration was defined as the presence of either laryngeal penetration of material or frank aspiration (39%, 95% confidence interval 26 to 53%). The milk scan was rarely positive (6%, 95% confidence interval 2 to 16%). Agreement between the tests of aspiration was poor. The maximum agreement (kappa=0.20) was between aspiration as diagnosed with the salivagram and by barium videofluoroscopy. Positive tests for aspiration are frequent in children with severe CP. Frequency varies widely depending on the investigation used. There is poor agreement between tests used for the diagnosis of aspiration. This information is of importance in assessing the significance of test results.

Predicting low bone density in children and young adults with quadriplegic cerebral palsy.

Many children and young adults with cerebral palsy (CP) have diminished bone mineral density (BMD) and a propensity to fracture with minimal trauma. The aim of this study was to identify variables which are routinely assessed as part of standard clinical care and that might be used to identify those individuals with CP who are most likely to have low BMD. One hundred and seven participants (ages 2 years 1 month to 21 years 1 month; mean age 10 years 11 months, SD 4 years 2 months) with moderate to severe spastic CP were assessed in detail. This included gathering clinical data, taking anthropometric measures of growth and nutrition, as well as dual energy X-ray absorptiometry measures of BMD. Seventeen participants were ambulatory with assistance (Gross Motor Function Classification System [GMFCS] level III), and 90 were capable of little or no ambulation even with assistance (26 GMFCS level IV and 64 GMFCS level V). Weight z score proved to be the best predictor of BMD z score. Declining BMD z scores also correlated with increasing age and greater severity of involvement. It can be predicted, with reasonable reliability, that a 10-year-old non-ambulatory child with quadriplegic CP and a 'typical' weight z score of -2 will have a BMD z score that is at best -2. Prior fractures, use of anticonvulsants, and feeding difficulties further reduce predicted BMD.

Including a Child with Severe Cerebral Palsy in Physical Education: A Case Study

The purpose was to examine an inclusive physical education kindergarten class containing a child with severe spastic diplegic cerebral palsy. An adapted physical educator served as a human resource. Participants were a kindergartner (6 years of age) with severe disability, 20 nondisabled peers (5-6 years of age), an adapted physical educator, and a regular physical educator. The research method was case study. Data were collected periodically by systematic observation and by interview during an 18-week period in the fall school semester. Twenty percent, or approximately one class per week (n = 19) were analyzed that were movement exploration in nature. Results indicated that inclusion classes were highly effective in time engagement and management, and the qualitative nature of inclusion was one of widespread social acceptance and successful motor participation. It was concluded that the use of a people resource model, with an adapted educator, is a highly effective educational practice.

Elevated Cytokines and Cerebral Palsy

Neurology| September 01 1999 Elevated Cytokines and Cerebral Palsy AAP Grand Rounds (1999) 2 (3): 30–31. https://doi.org/10.1542/gr.2-3-30 Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Facebook Twitter LinkedIn MailTo Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation Elevated Cytokines and Cerebral Palsy. AAP Grand Rounds September 1999; 2 (3): 30–31. https://doi.org/10.1542/gr.2-3-30 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search toolbar search search input Search input auto suggest filter your search All PublicationsAll JournalsAAP Grand RoundsPediatricsHospital PediatricsPediatrics In ReviewNeoReviewsAAP NewsAll AAP Sites Search Advanced Search Topics: cerebral palsy, cytokine, interferons Source: Grether JK, Nelson KB, Dambrosia JM, Phillips TM. Interferons and cerebral palsy. J Pediatr. 1999;134:324–332. In a retrospective study, Grether et al investigated the association between spastic cerebral palsy (CP) and serum levels of neonatal interferons (IFNs), other inflammatory cytokines, thyroid hormones, complement and coagulation factors. The subjects were selected from a population based case-control study of CP risk factors conducted in the San Francisco area between 1983 and 1985. One hundred and ninety-two children were identified with CP at 3 years of age. From this group, a subset of children with moderate to severe spastic CP was selected based upon the availability of archived blood from newborn metabolic screening and neonatal exposure to intrauterine infection, asphyxia or maternal problems in the second half of pregnancy. Also included was a small group of children with no apparent cause for their CP. The final study cohort included 31 mostly term children with spastic CP for whom neonatal blood spots were available, and 65 children of unknown gestational age and weight randomly selected from the control group. Blood was eluted from blood spots that had been stored over time at −15°F and analyzed for various constituents. Fourteen of the 31 children with spastic CP were found to have levels of all 3 IFNs higher than any child in the control group and were at or above the maximum adult normal values. IL-1, 6, 8, TNF alpha and other inflammatory mediators were also high in this group. In contrast, thyroid hormones, total T4, T3 and complement proteins C3, 4 and 9 were lower than in the controls. Seven of the 9 children with increased IFNs had spastic diplegia, a subtype of CP. In the remaining 17 of the cases, IFNs were low and in the range observed in the control group. The concentration of other cytokines, thyroid hormones and complement were also within the normal range. Although these children with CP had no evidence of inflammation, 70% of them had one or more coagulation factors that were abnormal. The most frequent form of CP in this group was spastic hemiplegia. The etiology of CP remains elusive in the majority of children. Recently, strong epidemiological evidence has been presented linking maternal intrauterine infection with neonatal white matter disease or periventricular leukomalacia (PVL) in the preterm infant. Yoon reported a correlation between elevated cytokines in amniotic fluid, histologic chorioamnionitis, PVL on neurosonography and CP.1 In children who died with PVL, immunohistochemical studies have shown an overproduction of cytokines in hypertrophic astrocytes and microglial cells of the CNS.2,3 Sixty to 100% of neonates with PVL develop CP, most commonly spastic diplegia. Cytokine mediated inflammatory reactions appear to be involved in the evolution of brain damage. In this small (N=31) retrospective exploratory study, Grether et al found that nearly half of the 31 children with CP had evidence of inflammation during the neonatal period with markedly elevated serum IFNs and other inflammatory mediators. This is the first report... You do not currently have access to this content.

Antenatal and Perinatal Antecedents of Moderate and Severe Spastic Cerebral Palsy

Dite, Gillian S.; Bell, Robin; Reddihough, Dinah S.; Bessell, Christine; Brennecke, Shaun; Sheedy, Mary Author Information

Selection of Neonates for Neuroprotective Therapies

To estimate the proportion of children with cerebral palsy (CP) who had signs of birth asphyxia" in the early hours of life, and to examine the nature of the illnesses in those infants. Population-based case-control study. All births in 4 northern California counties, 1983 through 1985. Eighty-four full-term singleton children surviving to age 3 years with spastic CP and 366 full-term control children. Moderate or severe spastic CP. Of 84 full-term children with spastic CP, 18 had 5-minute Apgar scores of less than 6, 20 required intubation for ventilation in the delivery room, and 5 had an initial blood pH of 7.00 or less. Three (3.6%) of the 84 children had all 3 signs evaluated, a prevalence of 0.019 per 1000 survivors. All 3 had neonatal seizures. When we relaxed the pH cutoff to 7.10 or less, there were 19 children with CP who met at least 2 criteria. Eight of these 19 infants were born in level I facilities. In these children there was evidence of maternal or infant infection (n = 7), abnormal coagulation factor, thrombosis, or thrombocytopenia (n = 8); or other complication predating birth (n = 9). Neuroprotective therapy offered to neonates with these early characteristics, even if perfectly effective, would be unlikely to prevent most CP. Most of these depressed infants with CP had nonasphyxial conditions that may have contributed to adverse neurological outcome.

Antenatal and perinatal antecedents of moderate and severe spastic cerebral palsy.

Routinely collected perinatal morbidity data were abstracted for 204 cases of moderate and severe spastic cerebral palsy and 816 matched controls. Separate analyses were conducted for cases with birth-weight > or = 2,500 g and birth-weight < 2,500 g. The presence of a congenital abnormality was an important risk factor for cerebral palsy in both groups and further analyses were conducted after dividing the groups according to presence or absence of a congenital abnormality. In the < 2,500 g group, resuscitation needed was clearly identified as a risk factor for cerebral palsy in the group with no congenital abnormalities (adjusted OR=3.4; 95% CI=1.6-7.5) while in the group with congenital abnormalities, none of the risk factors were clearly associated with an increased risk of cerebral palsy. Among the cases with birth-weight > or = 2,500 g, intrauterine hypoxia/birth asphyxia was clearly associated with an increased risk of cerebral palsy (adjusted OR=18.1; 95% CI=1.8-186) in the group with no congenital abnormalities while in the group with congenital abnormalities, none of the factors were clearly associated with an increased risk of cerebral palsy.

Natural history of scoliosis in spastic cerebral palsy

Although the frequent occurrence of scoliosis in patients who have spastic cerebral palsy is well known and surgical treatment has often been recommended for these patients, little is known about the natural history of scoliosis in this population. We aimed to clarify the natural history of scoliosis from childhood through to adulthood and provide objective data on proper surgical indications for such patients. The participants were 37 institutionalised patients with severe spastic cerebral palsy and scoliosis. All the participants had a series of radiographs taken, starting at a mean age of 7.8 years; they were followed up for an average of 17.3 years. We retrospectively reviewed radiographs and assessed the effect of five factors on progression of scoliosis: sex, degree of spasticity, initial physical capability, pattern of spinal curve, and location of curve. Scoliosis usually started before the age of 10 years and progressed rapidly during the growth period. In many cases, even after growth had ended, continuous progression was seen. The mean magnitude of the curves at final examination was 55 degrees (Cobb angle). In 11 (85%) of 13 patients who had a spinal curve of more than 40 degrees before age 15 years, the scoliosis progressed to more than 60 degrees by the time of the final examination. Meanwhile, in only three (13%) of 24 patients who had a curve of less than 40 degrees at age 15 years, did the scoliosis progress to more than 60 degrees. Severe scoliosis (> or = 60 degrees) developed predominantly in those who had total body involvement (67%), were bedridden (100%), or had throacolumbar curves (57%). The risk factors for progression of scoliosis in spastic cerebral palsy are: having a spinal curve of 40 degrees before age 15 years; having total body involvement; being bedridden; and having a thoracolumbar curve. Patients with these risk factors might benefit from early surgical intervention to prevent progression to severe scoliosis.

A comparative longitudinal study of gastrostomy devices in children.

Long-term gastrostomy is a predictable intervention to ameliorate the effects of feeding and swallowing difficulties among children with severe spastic cerebral palsy. The evidence evaluating the efficacy and implications of the available gastrostomy devices in common use has focused primarily on the operative phase, ignoring the long-term effects that may be critical from a nurse's or family caregiver's point of view. In this study, the authors describe a sample of children with gastrostomy, comparing skin-level and tube devices on measures of nutritional outcome, complications, and caregiver satisfaction. In contrast to manufacturer's claims about the superiority of skin-level devices, the results presented here reveal few differences between the devices. The authors conclude, therefore, that device selection should be determined by individualized comprehensive assessment of the child and family circumstances.

Increases in the Communicative Behaviours of Students with Cerebral Palsy as a Result of Feedback to, and the Selection of Goals by, Paraprofessionals

The employment of untrained paraprofessionals to support children with disabilities in educational settings is not uncommon. Although urgently needed, research on effective methods of increasing the skills of these paraprofessionals is scarce. This study, which was conducted over 3 academic years, assessed the effectiveness of incorporating naturalistic prompting strategies by paraprofessionals for increasing target communicative behaviours of students with severe spastic quadriplegic cerebral palsy. A concurrent multiple-baseline design replicated across 3 paraprofessionals was used to determine the effectiveness of verbal and written feedback regarding their attainment of self-selected goals for prompting target communicative behaviours. The results indicate that, after the introduction of the intervention, the prompting behaviours of all paraprofessionals increased, as did the target behaviours of all students. Increases maintained 5 months after intervention for all students observed and for 2 of the 3 paraprofessionals observed. The implications of designing a program to include specific performance feedback and active involvement by staff are discussed.

Videofluoroscopic assessment in children with severe cerebral palsy presenting with dysphagia.

In this study 16 patients with severe spastic cerebral palsy with an age range between 6 months and 16 years were examined using videofluoroscopy and a modified barium meal. All patients were slow, inefficient eaters. Silent aspiration was demonstrated in five cases. The latter five patients demonstrated a delayed swallow reflex but there was little correlation between aspiration and the oral phase of deglutition. Our data confirms the impression that early diagnostic workup including videofluoroscopy is helpful in managing the feeding difficulties in these children, and may prevent chronic aspiration and malnutrition.

Videofluoroscopic assessment of dysphagia in children with severe spastic cerebral palsy.

Very little has been published about the characteristics and sequelae of dysphagia in children with neurological impairment. The swallowing difficulties encountered by children with spastic cerebral palsy are particularly debilitating and potentially lethal. However, aggressive evaluation and management of their feeding is typically deferred until they are medically or nutritionally compromised. Reports of the use of videofluoroscopy to analyze the swallowing patterns and presence or absence of aspiration in such children are rare. This paper describes the histories and analyzes the videofluorographic swallow studies of 22 patients with the primary diagnosis of severe spastic cerebral palsy. The ages of the subjects ranged from 7 months to 19 years. All had severe dysphagia and were slow, inefficient eaters. Fifteen patients (68.2%) demonstrated significant silent aspiration during their swallow study. Analysis of specific features of their swallowing patterns indicated that decreased or poorly coordinated pharyngeal motility was predictive of silent aspiration. Moderately to severely impaired oral-motor coordination was indicative of severity of feeding complications. Our data suggest that early diagnostic workup, including baseline and comparative videofluoroscopic swallow studies, could be helpful in managing the feeding difficulties in these children and preventing chronic aspiration, malnutrition, and unpleasant lengthy mealtimes.

Control of Drooling with Transdermal Scopolamine in a Child with Cerebral Palsy

Transdermal scopolamine patches (1.5 mg) were used to control drooling in a two-year-old boy with severe spastic quadriparetic cerebral palsy and developmental delay. He responded well to the continuous scopolamine therapy, with a decrease in drooling, and a secondary decrease in respiratory distress and frequency of suctioning. No significant side-effects were noted.

Dandy-Walker syndrome revisited.

The Dandy-Walker syndrome is said to be associated with a high incidence of mental retardation and motor dysfunction leading some to suggest termination of the affected fetus in utero. Since this view seemed contrary to our experience, we reviewed 19 patients with the Dandy-Walker syndrome diagnosed from 1966 to 1983. Thirteen patients with Dandy-Walker syndrome were diagnosed before 6 months of age and followed for more than 2 years (mean: 10 years). In the absence of other associated major abnormalities, 7 of 8 (88%) are functioning well and have attended regular schools. Two of these seven have had special help with isolated learning problems. None of these patients have significant motor disability. One additional patient with normal intellectual and motor function died of acute shunt malfunction. In the remaining 4 patients with Dandy-Walker-associated abnormalities, 3 (75%) have severe intellectual retardation and spastic cerebral palsy. Five of six patients (83%) with Dandy-Walker syndrome diagnosed after 6 months of age are normal. The outcome of patients with Dandy-Walker syndrome appears far better than previously reported.