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An epidemiological study of cerebral palsy in Western Australia, 1956-1975. I: Changes in total incidence of cerebral palsy and associated factors.

Patterns in the incidence of cerebral palsy are described over a 20-year period in Western Australia. The incidence rose to a peak between 1966 and 1970, then fell again. This pattern was particularly marked in the spastic syndromes and was seen in both sexes, in each maternal age and parity group, in each IQ group, in both multiple and single births, and in infants born in metropolitan (but not rural) areas. Improvement was more marked in heavier than in lighter infants. Since 1968 the male rate has fallen more quickly than that for females. The risk of cerebral palsy with high maternal age declined, but it remained high in relation to high parities. There were marked reductions in the proportions of older and higher-parity mothers in Western Australia over the study period, and in the proportion of multiple births. There was also a shift toward heavier babies from 1968 to 1975. Social-class information was not available. The data indicate that factors in addition to changes in perinatal care were operating to improve neonatal outcome in terms of long-term handicap in Western Australia.

Adult

A phenotypic paradigm for cerebral palsy genetics.

Cerebral palsy (CP) represents a clinically and etiologically heterogeneous group of permanent but not unchanging disorders of movement, posture, and motor function resulting from non-progressive disturbances of the developing fetal or infant brain. Pathogenic variants in Mendelian disease-associated genes can be found in a subset of individuals with CP, with variants deemed causal of CP having been published for at least 515 genes. Currently, controversy exists as to whether to interpret such pathogenic variants as causing CP, whether the diagnosis instead should be "CP mimic," or whether a clinical diagnosis of CP should coexist with the molecular diagnosis of a Mendelian disease. Accordingly, there is no universally accepted model of the genetic architecture of CP. Here, we present a statistical approach that treats CP as a phenotypic feature for which some genetic disorders confer an increased risk. Based on comprehensive literature curation, we show that the null hypothesis of no CP association can be rejected for only 89 of the 515 genes. We applied these findings to the analysis of a cohort of 460 children diagnosed with CP in the Shriner Children's network who underwent genome sequencing. We identified pathogenic or likely pathogenic (P/LP) variants in 60 genes in 15.8% of the children. Only 16 of the 60 genes had significant evidence for CP association in our literature analysis. Our results suggest that a stratified approach to attributing causality to genetic variants in CP could support precision genomic medicine for affected individuals.

Humans

Sex of a parent and knowledge about cerebral palsy.

Parents of cerebral-palsied children who attended a rehabilitation center were asked to complete both a subjective questionnaire and an objective scale to tap their knowledge about cerebral palsy. A 33 percent return was obtained. According to the results from the questionnaire, both parents tended to believe that mothers are more knowledgeable about cerebral palsy. However, an analysis of the results of the scale indicated that a parent's knowledge about the disability and how to treat it is determined or influenced by the sex of the handicapped child. Other information gained suggested that the rehabilitation center therapists are important sources of information about cerebral palsy for most parents, and the parents also suggested ways in which to improve community services to the families of children with cerebral palsy.

Adaptation, Psychological

Semicircular canal stimulation in cerebral palsied children.

Twelve cerebral palsied children were exposed to 16 sessions of horizontal and vertical semicircular canal stimulation over a four-week period in order to determine the effectiveness of this form of stimulation as a therapeutic procedure. A control group of 11 cerebral palsied children was included. A quantitative evaluation of a series of reflexes and of gross motor skills showed a highly significant degree of improvement following the four weeks of treatment. A qualitative evaluation of each subject corroborated these findings and suggested improvements in fine motor control and in social/emotional behavior. A possible central nervous system mechanism underlying the improvement in motor skills is discussed.

Cerebral Palsy

Multilocular lesions in the therapy of cerebral palsy.

The cerebral pareses occurring in early childhood or produced subsequently by trauma or spontaneous cerebral haemorrhages, present a heterogenous symptomatic picture. In the forefront we find the spastic pareses associated with extra-pyramidal motor hyperkinesia (athetosis, dystonia, ballism). 36 C.P.-patients were surveyed from 1--4 years after operative intervention. It is shown that better results can be obtained when multilocular lesions during a single session in correspondence with the dominant group of symptoms are performed (thalamic and subthalamic target points of the extrapyramidal motor nuclei and dentate nucleus). The results are presented. Continuous physical therapy after the operation and a reasonable intellectual level are essential for improvement.

Cerebral Palsy

Cerebral dominance for language in cerebral palsy.

A test of cerebral dominance for language was administered to 51 cerebral palsied adults. The test consisted of dichotically presented words and in normal speaking adults had previously demonstrated very marked right ear superiority, interpreted as left cerebral dominance. The group of cerebral palsied subjects did not show the predicted right ear superiority. This finding may represent a chance maldistribution of side of cerebral injury within the 51 subjects. If substantiated, however, it would suggest either that the cortical damage in cerebral palsy is commonly widespread or else that the inherent left cerebral dominance of neonates is less tenacious than is generally assumed.

Adolescent

Electrophysiologic behavior modification of frontal EMG in cerebral-palsied children.

Four cerebral-palsied children participated in the following ABAB design: speech and motor pre-electrophysiologic behavior modification (EMB) evaluation; frontal EMG EMB, six weeks; speech and motor post-EBM evaluation; six weeks no training; speech and motor reevaluation; EMG EBM, four weeks; speech and motor evaluation. Auditory and visual feedback of frontal EMG was monitored by cumulative integration of frontal EMG voltage. The children were shaped by setting a cumulative voltage threshold (CVT). If the child's cumulative integrated frontal EMG voltage fell below the CVT at the end of each 60-sec epoch, a reward was automatically dispensed from a Universal Feeder. Frontal EMG decreased significantly over the initial twelve trials. Correspondingly, improvement was noted for the children in speech and motor skills. Follow-up six weeks later showed increased frontal EMG voltage and deterioration of speech and motor function. Reinstitution of frontal EMG EBM produced reacquisition of low frontal EMG and some recovery of speech and motor function. Collectively, these results indicate that frontal EMG EBM shows promise as an additional treatment modality in the habilitation of cerebral palsy children with spasticity.

Behavior Therapy

[Statistical study of the relationships between the etiology and clinical picture of cerebral palsy].

846 children with cerebral palsy were examined and 642 were selected for a statistical study by correspondence analysis. The aim was to identify without any prior assumptions, the relationships between the aetiological factors and the clinical findings. The study was completed by conventional statistical analysis of 584 of the cases. Small birth weight and a history of abnormal pregnancies was associated with a cerebral palsy affecting both legs, and often with a squint. Resuscitation was associated with athetosis and abnormalities of posture and behaviour. If the resuscitation lasted for more than 15 minutes or there were certain problems during delivery, severe abnormalities of both arms and major speech difficulties were observed. Resuscitation for less than 15 minutes or for an unknown time or intractable vomiting during pregnancy was associated with quadraplegia. Hemiplegia was related to post natal events but the aetiology was not always known. Foeto-maternal incompatibility was associated with athetosis, deafness, severe speech problems and ophthalmoplegias.

Abortion, Threatened

Startle reflex habituation in children with cerebral palsy.

Two groups of children (9 with cerebral palsy and 10 normals, matched for sex and age) participated in a study of the startle reflex. Each child was instructed to press a button as soon as possible after the onset of a visual stimulus on a box on the table at which they were seated. During some of the trials, a sudden and intense auditory stimulus (85 dB) was presented concomitantly with the onset of the visual stimulus, and effects on reaction time recorded. Mean reaction time of normal children was significantly faster than that of the group with cerebral palsy. The magnitude of disruption associated with the first startle stimulus presentation was signicantly greater for cerebral palsied children. The course between groups of habituation to the startle stimuli was not significantly different. Data support the hypothesis that startle reflexes of children with cerebral palsy are more marked than are those of normal children.

Acoustic Stimulation

Knee flexion deformities and genu recurvatum in cerebral palsy: roentgenographic findings.

Patients with cerebral palsy often have recurvatum or flexion deformities at the knee. To determine whether these are due to a bony or soft-tissue abnormality, four angles were measured on lateral roentgenograms of the knee: the femur-physis angle (angle 1); femur-Blumensaat's line angle (angle 2); tibia-plateau angle (angle 3); and tibia-physis angle (angle 4). These angles were measured for 45 patients with knee flexion deformities and for 31 with genu recurvatum. Values were also obtained from 204 normal lateral knee reoentgenograms. The age range for the whole series was from one to 22 years. Angles 2, 3 and 4 decreased from normal values with increasing age. The angulation of the proximal tibia, as measured by angle 3, is abnormal in patients with flexion deformities. Angles 2 and 3 were abnormal in cerebral-palsied patients with flexion deformities, while patients with genu recurvatum showed no significant bony abnormalities. These results suggest that treatment of flexion and recurvatum should be based on etiology.

Adolescent