Intervention with the CO-OP Approach leads to a transfer effect over time to untrained goals for children with cerebral palsy or spina bifida.

PURPOSE: This study aims to investigate whether the treatment effects, in terms of goal attainment, transfer effects and impact on executive functions, of an intervention in children with cerebral palsy or spina bifida using the Cognitive Orientation to daily Occupational Performance (CO-OP) Approach are maintained over time, from immediately after the intervention to three months afterwards. METHOD: A three-month follow-up study, from an intervention using CO-OP. Thirty-four children (7-16 years) each identified four goals (one untrained to examine transfer) and participated in an eleven-session intervention. Assessments were performed at baseline, immediately after the intervention and at a three-month follow-up using the Canadian Occupational Performance Measure and the Performance Quality Rating Scale. Executive function and self-rated competence were assessed at the same timepoints. RESULTS: Statistically significant and clinically relevant improvements in goal achievement were demonstrated for both trained and untrained goals after the intervention and were maintained at follow-up. The clinically relevant improvement in untrained goals continued to increase until follow-up. Self-rated competence increased after the intervention and was maintained at follow-up. CONCLUSION: The CO-OP intervention was effective in achieving and maintaining the children's own goals over time. The transfer effect was confirmed by higher goal attainment for the untrained goals.

The children's self-defined goals were achieved after the Cognitive Orientation to daily Occupational Performance (CO-OP) intervention and remained so at the three-month follow-up.The CO-OP Approach is useful for children with cerebral palsy or spina bifida.A transfer effect was demonstrated for untrained goals by both subjective and objective assessments.Using children's self-defined goals is effective.

The Cognitive Orientation to daily Occupational Performance (CO-OP) Approach is superior to ordinary treatment for achievement of goals and transfer effects in children with cerebral palsy and spina bifida - a randomized controlled trial.

PURPOSE: Children with cerebral palsy (CP) or spina bifida (SB) often have executive dysfunction affecting activity performance. With the Cognitive Orientation to daily Occupational Performance (CO-OP) Approach, children find their own way to perform activities, using problem-solving strategies and meta-cognitive thinking. The present study aimed to investigate the effectiveness of the CO-OP Approach in children with CP or SB, compared with conventional rehabilitation, in achieving self-identified activity goals, and to explore any generalization and transfer effects. METHOD: Randomized controlled trial, CO-OP versus treatment as usual, 38 children (7-16 years) participated. Each child identified four goals (to study generalization and transfer, one remained untrained). Primary outcomes: Canadian Occupational Performance Measure (COPM) and Performance Quality Rating Scale (PQRS). Secondary outcomes assessed executive functions and self-rated everyday-life competence. RESULTS: Self-rated goal attainment (COPM) was significantly greater for both trained and untrained goals in the CO-OP group compared with the control group. The rating of observed performance (PQRS) was significantly higher for trained goals in the CO-OP group. The CO-OP group experienced fewer problems in everyday life after treatment. Executive functions did not differ significantly between groups. CONCLUSION: CO-OP is more effective than ordinary treatment in achieving both trained and untrained goals.IMPLICATIONS FOR REHABILITATIONCO-OP enables children with CP (MACS levels I-III) or SB without intellectual disabilities to reach self-identified goals.CO-OP shows transfer effects to new activities and situations, which may enhance children's self-efficacy.CO-OP is an important complement to conventional rehabilitation services for children with CP and SB.

A scoping review of cognition in spina bifida and its consequences for activity and participation throughout life.

AIM: The aim of this scoping review was to summarise findings concerning cognitive characteristics in people with spina bifida and explain how cognitive factors influence activities and participation in different areas and stages of life. METHODS: PubMed, Psych INFO, ERIC, Scopus, CINAHL and the Cochrane Library were searched for English language papers published in 2000-2018. A total of 92 papers were selected and quality was assessed according to the McMaster criteria. The results were presented related to body functions, activity and participation from the International Classification of Function and Health, ICF. RESULTS: People with spina bifida tended to have a lower IQ than those without. The majority also had cognitive difficulties manifested in problems with language, perception, memory, executive and attentional functions. Those difficulties affected activity and participation in all life domains in ICF. This may affect medical adherence and responsibility and by extension the prevention of secondary complications. CONCLUSION: It is important for caregivers, professionals and especially individuals with spina bifida themselves to understand and handle both physical and cognitive consequences in all life circumstances. Having insight into one's own assets and difficulties paves the way to managing life challenges, which could enhance health, self-management and participation in society.

Systematic Monitoring of Cognition for Adults With Cerebral Palsy-The Rationale Behind the Development of the CPCog-Adult Follow-Up Protocol.

Cerebral palsy (CP) comprises a heterogeneous group of conditions recognized by disturbances of movement and posture and is caused by a non-progressive injury to the developing brain. Birth prevalence of CP is about 2-2.5 per 1,000 live births. Although the motor impairment is the hallmark of the diagnosis, individuals with CP often have other impairments, including cognitive ones. Cognitive impairments may affect communication, education, vocational opportunities, participation, and mental health. For many years, CP has been considered a "childhood disability," but the challenges continue through the life course, and health issues may worsen and new challenges may arise with age. This is particularly true for cognitive impairments, which may become more pronounced as the demands of life increase. For individuals with CP, there is no one-to-one correlation between cognition and functioning in other areas, and therefore, cognition must be individually assessed to determine what targeted interventions might be beneficial. To facilitate this for children with CP, a systematic follow-up protocol of cognition, the CPCog, has been implemented in Norway and Sweden. However, no such protocol currently exists for adults with CP. Such discontinuity in healthcare services that results from lack of follow-up of cognitive functioning and subsequent needs for adjustments and interventions makes transition from pediatric to adult healthcare services challenging. As a result, a protocol for the surveillance of cognition in adults with CP, the CPCog-Adult, has been developed. It includes assessment of verbal skills, non-verbal reasoning, visual-spatial perception, and executive functioning. It is recommended to perform these assessments at least once in young adulthood and once in the mid-fifties. This report describes the process of developing the CPCog-Adult, which has a three-fold purpose: (1) to provide equal access to healthcare services to enable the detection of cognitive impairments; (2) to provide interventions that increase educational and vocational participation, enhance quality of life, and prevent secondary impairments; and (3) to collect systematic data for research purposes. The consent-based registration of data in the well-established Swedish and Norwegian national CP registries will secure longitudinal data from childhood into adulthood.

Pain and health status in adults with myelomeningocele living in Sweden.

PURPOSE: To increase knowledge about pain and general health in adults with myelomeningocele, a health condition with several risk factors for pain such as musculoskeletal deformities, shunt dysfunctions, bowel problems, and urinary tract infections/stones. METHODS: Descriptive correlational pilot study (N= 51, 53% males). Chi-square tests were used to test associations among presence, impact, severity, and pain site in relation to sex and age. ANOVA was used to analyze associations between sex, age, and general health (today). RESULTS: Seventy-three percent reported pain in the past four weeks. No significant sex or age differences were associated with the presence of pain. Women were significantly more likely to report that pain interfered with work, χ2(1, N= 41) = 5.53, p= 0.02. There were significant main effects for sex and age on general health (today), F(2, 44) = 5.63, p= 0.007, adjusted R2= 0.17. Women scored lower on general health (today) (mean = 63.58) than men (mean = 76.33). Older individuals reported worse general health (today) than did younger (B=-0.89, t=-2.79, p= 0.008). CONCLUSIONS: Pain was frequent, and pain sites differed widely. Women were more likely to report that pain interfered with work, and scored lower on health, as did older persons.

Effect of Delayed Cord Clamping on Neurodevelopment at 4 Years of Age: A Randomized Clinical Trial.

IMPORTANCE: Prevention of iron deficiency in infancy may promote neurodevelopment. Delayed umbilical cord clamping (CC) prevents iron deficiency at 4 to 6 months of age, but long-term effects after 12 months of age have not been reported. OBJECTIVE: To investigate the effects of delayed CC compared with early CC on neurodevelopment at 4 years of age. DESIGN, SETTING, AND PARTICIPANTS: Follow-up of a randomized clinical trial conducted from April 16, 2008, through May 21, 2010, at a Swedish county hospital. Children who were included in the original study (n = 382) as full-term infants born after a low-risk pregnancy were invited to return for follow-up at 4 years of age. Wechsler Preschool and Primary Scale of Intelligence (WPPSI-III) and Movement Assessment Battery for Children (Movement ABC) scores (collected between April 18, 2012, and July 5, 2013) were assessed by a blinded psychologist. Between April 11, 2012, and August 13, 2013, parents recorded their child's development using the Ages and Stages Questionnaire, Third Edition (ASQ) and behavior using the Strengths and Difficulties Questionnaire. All data were analyzed by intention to treat. INTERVENTIONS: Randomization to delayed CC (≥180 seconds after delivery) or early CC (≤10 seconds after delivery). MAIN OUTCOMES AND MEASURES: The main outcome was full-scale IQ as assessed by the WPPSI-III. Secondary objectives were development as assessed by the scales from the WPPSI-III and Movement ABC, development as recorded using the ASQ, and behavior using the Strengths and Difficulties Questionnaire. RESULTS: We assessed 263 children (68.8%). No differences were found in WPPSI-III scores between groups. Delayed CC improved the adjusted mean differences (AMDs) in the ASQ personal-social (AMD, 2.8; 95% CI, 0.8-4.7) and fine-motor (AMD, 2.1; 95% CI, 0.2-4.0) domains and the Strengths and Difficulties Questionnaire prosocial subscale (AMD, 0.5; 95% CI, >0.0-0.9). Fewer children in the delayed-CC group had results below the cutoff in the ASQ fine-motor domain (11.0% vs 3.7%; P = .02) and the Movement ABC bicycle-trail task (12.9% vs 3.8%; P = .02). Boys who received delayed CC had significantly higher AMDs in the WPPSI-III processing-speed quotient (AMD, 4.2; 95% CI, 0.8-7.6; P = .02), Movement ABC bicycle-trail task (AMD, 0.8; 95% CI, 0.1-1.5; P = .03), and fine-motor (AMD, 4.7; 95% CI, 1.0-8.4; P = .01) and personal-social (AMD, 4.9; 95% CI, 1.6-8.3; P = .004) domains of the ASQ. CONCLUSIONS AND RELEVANCE: Delayed CC compared with early CC improved scores in the fine-motor and social domains at 4 years of age, especially in boys, indicating that optimizing the time to CC may affect neurodevelopment in a low-risk population of children born in a high-income country. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT01581489.

Quality of life in adults treated in infancy for hydrocephalus.

PURPOSE: The objective was to analyze quality of life in a very long-term follow-up study of now adult individuals, treated for hydrocephalus (without spina bifida) during infancy. METHODS: The entire series was population-based, and the subgroup under study consisted of the 29 individuals without intellectual disability, who consented to participate. About one third had concomitant mild cerebral palsy or epilepsy or both. A Finnish validated questionnaire, the 15D, was used to measure quality of life. RESULTS: There was no significant difference between the study group and the controls with regard to the total quality of life score. Individuals with associated cerebral palsy and/or epilepsy had a lower total score compared with both those without associated impairments and controls. Most participants differed from controls in the dimension of mental/memory function which pertains to executive functions, an ability of considerable importance for daily life skills. CONCLUSION: It is important to follow children with hydrocephalus over time--due to the different etiological panorama, interventions, and associated impairments this group displays. This is the only way to learn more about critical factors that require attention and that predict quality of life in adulthood.

Very long-term follow-up of adults treated in infancy for hydrocephalus.

PURPOSE: The purpose of this study is to perform a population-based, very long-term follow-up of adults who had been shunt treated for hydrocephalus in infancy. METHODS: The 72 children with hydrocephalus born in 1967-1978 in western Sweden, who had participated in a follow-up at school age, were re-examined at 30-43 years of age. The 29 with mental retardation were described in terms of developmental level and survival, whereas the remaining 43 were invited to take part in a follow-up and 28 accepted. The assessments included a semi-structured interview pertaining to medical issues, academic achievements and social function. RESULTS: Six children had died, i.e. a mortality rate of 8%. Mental retardation was present in 29 (40%), severe (IQ <50) in 13 and mild (IQ 50-70) in 16. Four of the 28 (14%) had cerebral palsy and 8 (28%) had other motor problems. Five (18%) had epilepsy and nine (32%) had visual impairments. A total of 20 (71%) reported some kind of health problem. Repeated revisions of the shunt had been performed in 23 (82%). Many worried about their shunt and requested a systematic medical follow-up. Nineteen subjects (68%) lived with a partner and 16 (57%) were parents. The majority had completed secondary school and 9 (32%) had completed university studies, while 18 (64%) worked full time, equal to the general population. CONCLUSION: In general, the group of normally gifted individuals with hydrocephalus, who had been shunt treated during infancy, was functioning well as adults and participated in society to the same extent as other people.

Very long-term follow-up of cognitive function in adults treated in infancy for hydrocephalus.

PURPOSE: The aim was to investigate the very long-term cognitive outcome in adults who had been shunt treated for hydrocephalus during their first year of life. METHODS: In a population-based series of 72 children born in 1967-1978 and shunt treated for infantile hydrocephalus, 43 were found to have a normal cognitive function when assessed at 6-17 years of age. Twenty-five of them agreed to participate in a follow-up study of cognition at a mean age of 35 years (range, 30-41 years). The Wechsler Adult Intelligence Scale (WAIS-III) was used. RESULTS: The median full-scale IQ was 101 (range, 83-120), median verbal IQ was 104 (81-115) and performance IQ was 99 (80-127). The corresponding IQs in childhood in the 16 subjects who had been tested previously with the WISC were 101 (84-124), 108 (86-135), and 101 (73-124). Specific cognitive deficits were found for working memory and processing speed. Shunt complications did not affect IQ. CONCLUSION: This very long-term follow-up study of normally gifted children with hydrocephalus revealed that, as adults, they still had preserved cognitive functions despite recurrent shunt dysfunction. The results are encouraging and represent a tribute to neurosurgical intervention. Continued follow-up studies are needed since the etiological panorama and treatment procedures of children with hydrocephalus are changing over time.

Cognitive functions in children with myelomeningocele without hydrocephalus.

OBJECTIVE: The aim of this study was to explore the separate effects of myelomeningocele (MMC) and hydrocephalus on intelligence and neuropsychological functions in a population-based series of children. MATERIAL AND METHODS: Of the 69 children with MMC born in 1992-1999 in western Sweden, nine did not develop hydrocephalus. Eight of them participated in this study and were compared with age- and gender-matched children with MMC in combination with hydrocephalus and with controls. RESULTS: Children with only MMC had an IQ of 103 compared with 75 in those with hydrocephalus added to the MMC and they had significantly better immediate and long-term memory and executive functions. When compared with controls, they had difficulty with learning and executive functions, but when the two children with an IQ of <70 were excluded, those with only MMC performed just as well as the controls. CONCLUSION: Hydrocephalus rather than MMC in itself appeared to cause the cognitive deficits found in children with MMC.

Learning, memory and executive functions in children with hydrocephalus.

AIMS: To explore learning, memory and executive abilities in children with hydrocephalus without learning disabilities, and to find out whether children with an isolated hydrocephalus differed from those with hydrocephalus in combination with myelomeningocele (MMC). METHODS: Thirty-six children with an intelligence quotient (IQ) of >or=70 from a population of all the 107 children with hydrocephalus born in western Sweden in 1989-1993 were examined and compared with age- and gender-matched controls. The neuropsychological assessment of the school-aged child (NIMES) test battery was used. RESULTS: The children with hydrocephalus differed significantly from controls in all functions apart from registration skills and recognition. Learning, memory and executive functions were all impaired. Twenty children with infantile hydrocephalus did not differ from those with hydrocephalus associated with MMC. Also, children with an IQ of >84 performed significantly worse than controls. CONCLUSIONS: Despite an IQ of >or=70, children with hydrocephalus had significantly impaired learning, memory and executive functions. When major brain lesions resulting in learning disability had been excluded, the hydrocephalus, rather than the underlying aetiology, was most important for the development of cognitive functions.

Vision in children with hydrocephalus.

Hydrocephalus in children has many aetiologies, and can cause multiple ophthalmic and visual disorders. This study sets out to detect and quantify visual and visuoperceptual dysfunction in children who have received surgical treatment for hydrocephalus with and without myelomeningocele, and to relate the results to the associated diagnoses and results from a comparison group. Seventy-five school-aged children (41 males, 34 females) with surgically-treated hydrocephalus and 140 comparison children (76 males, 64 females) matched for age and sex underwent comprehensive ophthalmologic examination. Median age at examination was 9 years and 4 months (range 7 y 4 mo-12 y 10 mo). Visual function deficits were identified in 83% (62/75) of the children with hydrocephalus. Visual impairment (binocular visual acuity <0.3) was found in 15% (11/73; comparison group 0%) but in none with myelomeningocele. Strabismus was found in 69% (51/74; comparison group 4% [5/140], p < 0.001), and refractive errors were found in 67% (47/70; comparison group 20% [28/140], p < 0.001). Cognitive visual dysfunction was identified in 59% (38/64; comparison group 3% [4/140], p < 0.001). These disorders were identified in various combinations and comprised impaired ability to plan movement through depth (e.g. going down a stair), impaired simultaneous perception, impaired perception of movement, impaired orientation, and (least frequently) impaired recognition. In this study, children with hydrocephalus associated with myelomeningocele were least commonly affected. Visual disorders were most frequent in those with epilepsy, cerebral palsy, and/or cognitive disability.

Behavioural problems and autism in children with hydrocephalus : a population-based study.

OBJECTIVE: To investigate the prevalence of behavioural problems and autism in a population-based group of children with hydrocephalus and to see whether learning disabilities, cerebral palsy (CP), epilepsy, myelomeningocele (MMC) or preterm birth increase the risk of these problems. METHOD: In the 107 children with hydrocephalus born in western Sweden in 1989-1993, behaviour was assessed using the Conners' parent rating scales in 66 and the teacher's rating scales in 57. Autism was investigated using the Childhood Autism Rating Scale. RESULTS: Parents rated 67% of the children and teachers 39% of the children as having behavioural problems (>1.5 SD, or T score >65). Learning disabilities increased the risk significantly and almost all the children with CP and/or epilepsy had behavioural problems. Autism was present in nine children (13%), in 20% of those without MMC and in one of 26 with MMC. Autism was significantly more frequent in children with learning disabilities (27% vs. 7%) and in children with CP and/or epilepsy (33% vs. 6%). CONCLUSION: The majority of children with hydrocephalus have behavioural problems and many have autism. It is therefore important to assess and understand all the aspects of cognition and behaviour in these children in order to minimise disability and enhance participation for the child.

Learning disabilities in a population-based group of children with hydrocephalus.

AIM: To assess cognitive functions in a population-based group of children with hydrocephalus and to analyse differences between children with and without myelomeningocoele (MMC); with hydrocephalus already present at birth and those who developed it later; children born at full term and those born preterm. METHODS: Of 103 children with hydrocephalus born in the western-Swedish region in 1989-1993, 73 were assessed using the Wechsler Intelligence Scales or the Griffith Developmental Scales. RESULTS: One-third of the children were normally gifted (IQ >85), another 30% had a low average IQ of 70-84 and 37% had learning disabilities with an IQ of <70. The median IQ was 75, verbal IQ 90 and performance IQ 76 (p<0.001). An IQ of <70 was found in 19 of 45 (42%) children without MMC and in 8 of 28 (29%) of those with MMC. Children born preterm had a lower IQ of 68 than those born at full term with an IQ of 76, while children with hydrocephalus present at birth but without MMC had a lower IQ of 60 than the others with an IQ of 77. Children with cerebral palsy and/or epilepsy (n=22) had a lower IQ of 66 than the IQ of 78 in those without these impairments (p<0.01). CONCLUSION: Cognitive functions in children with hydrocephalus need to be carefully assessed before school age to ensure adequate support and education. Even the one-third near normally gifted children with an IQ of 70-85 require special attention, due to the profile of relatively well-preserved verbal functions but greatly impaired perceptual and non-verbal abilities.