Cognition in children with arachnoid cysts - A five-year follow-up after microneurosurgical fenestration.

BACKGROUND AND PURPOSE: In recent years there has been a re-evaluation regarding the clinical implications of temporal lobe arachnoid cysts (temporal arachnoid cysts) in children. These cysts have often been considered asymptomatic, or if symptomatic, only causing focal neurological symptoms or signs of increased intracranial pressure. However, several studies have more recently reported on cognitive symptoms improving after surgery. This study aimed to evaluate if reported cognitive improvement after surgery of temporal arachnoid cysts were stable after five years. METHOD: Ten consecutive children (m = 14.65; range 12.1-19.415 were assessed cognitively five years after micro-neurosurgical fenestration of a temporal arachnoid cyst. Results were compared to results from their pre- and post-surgical evaluations. Evaluations included the Wechsler-scales, Boston Naming Test (BNT), Rey Auditory Verbal Learning Test (RAVLT), verbal fluency test (FAS) and Rey Complex Figure Test (RCFT). RESULTS: The analysis revealed significant postsurgical improvement compared to baseline on the Wechsler-scales measures of general intelligence (FSIQ), verbal abilities (VCI) and processing speed (PSI). Mean differences after surgery were 8.3 for FSIQ, (p = 0.026), 8.5 for VI (p = < .01) and 9.9 for PSI (p = 0.03). There were no significant differences in mean test results when comparing postsurgical scores with scores five years after surgery, indicating long-term stability of improvements. CONCLUSION: The results indicate that affected cognitive functions in children with temporal arachnoid cysts improve after surgery and that the improvements remain stable five years later. The improvements and long term stability were also consistent with the experience of both parents and children. The findings provide a strong argument for neurosurgical fenestration of temporal arachnoid cysts in children.

The Dyskinesia Impairment Scale, Second Edition: Development, construct validity, and reliability.

AIM: To create a shortened, more user-friendly Second Edition of the Dyskinesia Impairment Scale (DIS-II) to assess dystonia and choreoathetosis, and evaluate its construct validity and reliability. METHOD: Scale development included an online expert meeting (n = 21) and iterative discussions within the research group (n = 6). A Rasch measurement model analysis on DIS scores from individuals with dyskinetic cerebral palsy or inherited/idiopathic dystonia (n = 123, 74 males, mean age 14 years, SD 5 years) was performed to evaluate the construct validity and reliability of the DIS-II. RESULTS: The DIS-II evaluates dystonia and choreoathetosis in action and rest in 11 body regions, with action items scored from 0 to 3 and rest items 0 to 2. The number of videos to record are reduced from 26 to 14 and the items to score are reduced from 144 to 88. Rating scale functioning, goodness-of-fit evaluation, principal component analysis, and targeting met the predefined quality criteria of the study and construct validity was therefore considered good. Furthermore, person reliability indicated that the DIS-II can separate individuals into eight distinct ability levels. INTERPRETATION: The DIS-II provides valid and reliable measures for dystonia and choreoathetosis, and reduces the administration and scoring time compared with the DIS. The DIS-II logit scores (interval level data) enhance comparison over time and between individuals in clinical practice and research. WHAT THIS PAPER ADDS: Compared with the Dyskinesia Impairment Scale (DIS), the shortened edition (DIS-II) requires half of the number of videos to be scored. The DIS-II has a simplified rating scale, requiring scoring of 88 instead of 144 items. The DIS-II has shown excellent reliability and good construct validity. The interval properties of the DIS-II are superior to the ordinal level outcome measures of the DIS.

Long-term neurological morbidity among children delivered by vacuum extraction - a national cohort study.

INTRODUCTION: This is the first nationwide cohort study of vacuum extraction (VE) and long-term neurological morbidity. We hypothesized that VE per se, and not only complicated labor, can cause intracranial bleedings, which could further cause neurological long-term morbidity. The aim of this study was to investigate the risk of neonatal mortality, cerebral palsy (CP), and epilepsy among children delivered by VE in a long-term perspective. MATERIAL AND METHODS: The study population included 1 509 589 term singleton children planned for vaginal birth in Sweden (January 1, 1999 to December 31, 2017). We investigated the risk of neonatal death (ND), CP, and epilepsy among children delivered by VE (successful or failed) and compared their risks with those born by spontaneous vaginal birth and emergency cesarean section (ECS). We used logistic regression to study the adjusted associations with each outcome. The follow-up time was from birth until December 31, 2019. RESULTS: The percentage and total number of children with the outcomes were ND (0.04%, n = 616), CP (0.12%, n = 1822), and epilepsy (0.74%, n = 11 190). Compared with children delivered by ECS, those born by VE had no increased risk of ND, but there was an increased risk for those born after failed VE (adj OR 2.23 [1.33-3.72]). The risk of CP was similar among children born by VE and those born spontaneously vaginally. Further, the risk of CP was similar among children born after failed VE compared with ECS. The risk of epilepsy was not increased among children born by VE (successful/failed), compared with those who had spontaneous vaginal birth or ECS. CONCLUSIONS: The outcomes ND, CP, and epilepsy are rare. In this nationwide cohort study, children born after successful VE had no increased risk of ND, CP or epilepsy compared with those delivered by ECS, but there was an increased risk of ND among those born by failed VE. Concerning the studied outcomes, VE appears to be a safe obstetric intervention; however, it requires a thorough risk assessment and awareness of when to convert to ECS.

Anorexia Nervosa With Comorbid Severe Depression: A Systematic Scoping Review of Brain Stimulation Treatments.

ABSTRACT: Major depressive disorder (MDD) is highly prevalent in individuals with anorexia nervosa (AN) and is a predictor of greater clinical severity. However, there is a limited amount of evidence supporting the use of psychotropic medications for its management. A systematic scoping review was conducted to assess the current literature on brain stimulation treatments for AN with comorbid MDD, with a specific focus on MDD treatment response and weight restoration. This review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, and the PubMed, PsycInfo, and MEDLINE databases were searched until July 2022 using specific key words related to AN and brain stimulation treatments. A total of 373 citations were identified, and 49 treatment studies that met the inclusion criteria were included in the review. The initial evidence suggests that electroconvulsive therapy, repetitive transcranial magnetic stimulation, and deep-brain stimulation may be effective in managing comorbid MDD in AN. Emerging evidence suggests that transcranial direct current stimulation may have a positive effect on body mass index in individuals with severe to extreme AN. However, there is a need for the development of better measurement techniques for assessing the severity of depression in the context of AN. Controlled trials that are adequately designed to account for these limitations are highly warranted for deep-brain stimulation, electroconvulsive therapy, and repetitive transcranial magnetic stimulation and hold promise for providing clinically meaningful results.

Deep brain stimulation in Lesch-Nyhan disease: outcomes from the patient's perspective.

AIM: To provide insight into outcome and long-term safety and efficacy of deep brain stimulation (DBS), from the perspective of individuals with Lesch-Nyhan disease (LND) and their families. METHOD: We used patient-centered outcome measures to assess long-term outcomes of DBS for 14 individuals (mean [SD] age 10y 10mo [5y 6mo], range 5-23y, all males) with LND, after an average duration of 5y 6mo (range 11mo-10y 5mo) after surgery. We compared these results with a comprehensive review of previously published cases. RESULTS: Patients and their families reported that DBS of the globus pallidus can be effective both for motor and behavioral disturbances in LND. However, outcome measures were often not significantly changed owing to substantial variability among individuals, and were overall less positive than in previous reports based on clinician assessments. In addition, there was an unexpectedly high rate of adverse events, tempering overall enthusiasm for the procedure. INTERPRETATION: Although DBS might be an effective treatment for LND, more research is needed to understand the reasons for response variability and the unusually high rates of adverse events before DBS can be recommended for these patients. What this paper adds Individuals with Lesch-Nyhan disease and their families report variable efficacy of deep brain stimulation. Long-term outcomes are associated with a high adverse event rate.

Risk of intellectual disability in children born appropriate-for-gestational-age at term or post-term: impact of birth weight for gestational age and gestational age.

Children born small for gestational age have a higher risk of intellectual disability. We investigated associations of birth weight for gestational age percentile and gestational age with risk of intellectual disability in appropriate-for-gestational-age (AGA) children. We included 828,948 non-malformed term or post-term AGA singleton children (including 429,379 full siblings) born between 1998 and 2009 based on data from the Swedish Medical Birth Register. Diagnosis of intellectual disability after 3 years of age was identified through the Patient Register. Using Cox regression models, we calculated hazard ratios (HRs) with 95% confidence intervals (CIs) of intellectual disability among children with different birth weight percentiles and gestational age in the whole population and in a subpopulation of full siblings. A total of 1688 children were diagnosed with intellectual disability during follow-up. HRs (95% CIs) of intellectual disability for the low birth weight percentile groups (10th-24th and 25th-39th percentiles, respectively) versus the reference group (40th-59th percentiles) were 1.43 (1.22-1.67) and 1.28 (1.10-1.50) in population analysis and 1.52 (1.00-2.31) and 1.44 (1.00-2.09) in sibling comparison analysis. The increased risk for low birth weight percentiles in population analysis was stable irrespective of gestational age. A weak U-shaped association between gestational age and intellectual disability was observed in population analysis, although not in sibling comparison analysis. These findings suggest that among AGA children born at term or post-term, lower birth weight percentiles within the normal range are associated with increased risk of intellectual disability, regardless of gestational age.

Long-term effects of selective dorsal rhizotomy in children with cerebral palsy: a systematic review.

AIM: To evaluate the long-term effects of selective dorsal rhizotomy (SDR) 10 years or more after the procedure and complications observed any time after SDR in children with cerebral palsy (CP). METHOD: Embase, PubMed, and the Cochrane Library were searched from their individual dates of inception through 1st June 2018 for full-text original articles in English that described long-term follow-up after SDR in children with CP. The authors independently screened publications to determine whether they met inclusion criteria; thereafter all authors extracted data on patient characteristics, the proportion of the original cohort being followed-up, and the reported outcomes. RESULTS: Of the 199 studies identified, 16 were included in this evaluation: 14 were case series and two studies reported a retrospectively assigned comparison group. Evidence concerning function was limited by study design differences, clinical variability, loss to follow-up, and heterogeneity across trials. INTERPRETATION: At 10 years or more follow-up, available studies generate low-level evidence with considerable bias. No functional improvement of SDR over routine therapy is documented. Furthermore, the long-term effects of SDR with respect to spasticity reduction is unclear, with many studies reporting a high amount of add-on spasticity treatment. More long-term follow-up using robust scientific protocols is required before it can be decided whether the use of SDR as routine therapy for children with CP is to be recommended or not. WHAT THIS PAPER ADDS: Ten years after selective dorsal rhizotomy, available studies supply inconclusive evidence on functional outcomes. The long-term effect on spasticity is uncertain, studies reported a substantial need for add-on treatment. Short- and long-term complications seem frequent but are not reported in a consistent manner.

Health-related quality of life, pain, and fatigue in young adults with cerebral palsy.

AIM: To describe health-related quality of life (HRQoL), pain, fatigue, and other health variables in young adults with cerebral palsy (CP), and to explore associations with the Gross Motor Function Classification System - Expanded and Revised (GMFCS-ER) and physical activity. METHOD: This was a cross-sectional study of 61 young adults at a mean age of 21 years 2 months (standard deviation 8mo, range 20-22y) with CP, from a geographically defined area. Data collection included: Short Form 36 version 2 for HRQoL, Brief Pain Inventory - Short Form, Fatigue Severity Scale, level of physical activity, medical history, and physical examination. RESULTS: Overall HRQoL equalled that of population norms; however self-reported physical health was lower in GMFCS-ER levels III to V compared to GMFCS-ER levels I to II. Self-reported mental health was, inversely, lower in GMFCS-ER levels I to II compared to GMFCS-ER levels III to V. Pain prevalence was 49%, and pain was present across all GMFCS-ER levels. Fatigue, as well as sleep problems, had 41% prevalence, with fatigue severity decreasing with increasing level of physical activity. INTERPRETATION: General HRQoL in young adults with CP was comparable to population norms. Pain and fatigue are important to address in high motor-functioning individuals also. Physical activity could be a possible protective factor against fatigue. WHAT THIS PAPER ADDS: Health-related quality of life in young adults with cerebral palsy (CP) was comparable to population norms. Pain, fatigue, and sleep problems occurred at all Gross Motor Function Classification System levels. There is a possible protective effect of physical activity on fatigue.

Benign paroxysmal torticollis of infancy does not lead to neurological sequelae.

AIM: To elucidate the natural course of benign paroxysmal torticollis, the relationship of this disorder to migraine and other paroxysmal diseases, and to analyse candidate genes. METHOD: This was a case series of children with benign paroxysmal torticollis of infancy (BPTI) diagnosed from 1998 to 2005, at Astrid Lindgren Children's Hospital, Stockholm, Sweden. A neurological examination and a formalized motor assessment were performed from 2005 to 2007. At a second follow-up, in 2014 to 2015, the children and their parents were interviewed and candidate genes analysed. RESULTS: The mean age of the eight females and three males included in the second follow-up was 13 years 9 months (SD 2y 2mo). All motor assessments were normal. Five had developed migraine, abdominal migraine, and/or cyclic vomiting. Prophylactic treatment or migraine-specific medication during attacks were not needed. No paroxysmal tonic upgaze, benign paroxysmal vertigo, epilepsy, episodic ataxia, or paroxysmal dyskinesia was reported. Rare genetic variants in CACNA1A and ATP1A2 were found in two children. Five had a family history of migraine. INTERPRETATION: BPTI is transient and does not lead to neurological sequelae. Most children afflicted experience either a mild migraine or no paroxysmal disorder at all in their adolescence. Genetic variants in candidate genes were few, indicating potential genetic heterogeneity. WHAT THIS PAPER ADDS: After resolution of their benign paroxysmal torticollis of infancy (BPTI), children display no gross motor delay. Most adolescents who previously had BPTI have not developed migraine. No mutations in candidate genes, known to cause hemiplegic migraine, were found. Associated symptoms are often lacking during episodes of torticollis.

Association Between Maternal Body Mass Index in Early Pregnancy and Incidence of Cerebral Palsy.

Importance: Maternal overweight and obesity are associated with increased risks of preterm delivery, asphyxia-related neonatal complications, and congenital malformations, which in turn are associated with increased risks of cerebral palsy. It is uncertain whether risk of cerebral palsy in offspring increases with maternal overweight and obesity severity and what could be possible mechanisms. Objective: To study the associations between early pregnancy body mass index (BMI) and rates of cerebral palsy by gestational age and to identify potential mediators of these associations. Design, Setting, and Participants: Population-based retrospective cohort study of women with singleton children born in Sweden from 1997 through 2011. Using national registries, children were followed for a cerebral palsy diagnosis through 2012. Exposures: Early pregnancy BMI. Main Outcomes and Measures: Incidence rates of cerebral palsy and hazard ratios (HRs) with 95% CIs, adjusted for maternal age, country of origin, education level, cohabitation with a partner, height, smoking during pregnancy, and year of delivery. Results: Of 1 423 929 children included (mean gestational age, 39.8 weeks [SD, 1.8]; 51.4% male), 3029 were diagnosed with cerebral palsy over a median 7.8 years of follow-up (risk, 2.13 per 1000 live births; rate, 2.63/10 000 child-years). The percentages of mothers in BMI categories were 2.4% at BMI less than 18.5 (underweight), 61.8% at BMI of 18.5 to 24.9 (normal weight), 24.8% at BMI of 25 to 29.9 (overweight), 7.8% at BMI of 30 to 34.9 (obesity grade 1), 2.4% at BMI of 35 to 39.9 (obesity grade 2), and 0.8% at BMI 40 or greater (obesity grade 3). The number of cerebral palsy cases in each BMI category was 64, 1487, 728, 239, 88, and 38; and the rates per 10 000 child-years were 2.58, 2.35, 2.92, 3.15, 4.00, and 5.19, respectively. Compared with children of normal-weight mothers, adjusted HR of cerebral palsy were 1.22 (95% CI, 1.11-1.33) for overweight, 1.28 (95% CI, 1.11-1.47) for obesity grade 1, 1.54 (95% CI, 1.24, 1.93) for obesity grade 2, and 2.02 (95% CI, 1.46-2.79) for obesity grade 3. Results were statistically significant for children born at full term, who comprised 71% of all children with cerebral palsy, but not for preterm infants. An estimated 45% of the association between maternal BMI and rates of cerebral palsy in full-term children was mediated through asphyxia-related neonatal morbidity. Conclusions and Relevance: Among Swedish women with singleton children, maternal overweight and obesity were significantly associated with the rate of cerebral palsy. The association was limited to children born at full term and was partly mediated through asphyxia-related neonatal complications.

The role of visual stimuli on standing posture in children with bilateral cerebral palsy.

BACKGROUND: In children with bilateral cerebral palsy (CP) maintaining a standing position can be difficult. The fundamental motor task of standing independently is achieved by an interaction between the visual, somatosensory, and vestibular systems. In CP, the motor disorders are commonly accompanied by sensory and perceptual disturbances. Our aims were to examine the influence of visual stimuli on standing posture in relation to standing ability. METHODS: Three dimensional motion analysis with surface electromyography was recorded to describe body position, body movement, and muscle activity during three standing tasks: in a self-selected position, while blindfolded, and during an attention-demanding task. Participants were twenty-seven typically-developing (TD) children and 36 children with bilateral CP, of which 17 required support for standing (CP-SwS) and 19 stood without support (CP-SwoS). RESULTS: All children with CP stood with a more flexed body position than the TD children, even more pronounced in the children in CP-SwS. While blindfolded, the CP-SwS group further flexed their hips and knees, and increased muscle activity in knee extensors. In contrast, the children in CP-SwoS maintained the same body position but increased calf muscle activity. During the attention-demanding task, the children in CP-SwoS stood with more still head and knee positions and with less muscle activity. CONCLUSIONS: Visual input was important for children with CP to maintain a standing position. Without visual input the children who required support dropped into a further crouched position. The somatosensory and vestibular systems alone could not provide enough information about the body position in space without visual cues as a reference frame. In the children who stood without support, an intensified visual stimulus enhanced the ability to maintain a quiet standing position. It may be that impairments in the sensory systems are major contributors to the difficulties to stand erect in children with CP.

Efficacy of the small step program in a randomised controlled trial for infants below age 12 months with clinical signs of CP; a study protocol.

BACKGROUND: Children with cerebral palsy (CP) have life-long motor disorders, and they are typically subjected to extensive treatment throughout their childhood. Despite this, there is a lack of evidence supporting the effectiveness of early interventions aiming at improving motor function, activity, and participation in daily life. The study will evaluate the effectiveness of the newly developed Small Step Program, which is introduced to children at risk of developing CP during their first year of life. The intervention is based upon theories of early learning-induced brain plasticity and comprises important components of evidence-based intervention approaches used with older children with CP. METHOD AND DESIGN: A two-group randomised control trial will be conducted. Invited infants at risk of developing CP due to a neonatal event affecting the brain will be randomised to either the Small Step Program or to usual care. They will be recruited from Astrid Lindgren Children's Hospital at regular check-up and included at age 3-8 months. The Small Step Program was designed to provide individualized, goal directed, and intensive intervention focusing on hand use, mobility, and communication in the child's own home environment and carried out by their parents who have been trained and coached by therapists. The primary endpoint will be approximately 35 weeks after the start of the intervention, and the secondary endpoint will be at 2 years of age. The primary outcome measure will be the Peabody Developmental Motor Scale (second edition). Secondary assessments will measure and describe the children's general and specific development and brain pathology. In addition, the parents' perspective of the program will be evaluated. General linear models will be used to compare outcomes between groups. DISCUSSION: This paper presents the background and rationale for developing the Small-Step Program and the design and protocol of a randomized controlled trial. The aim of the Small Step Program is to influence development by enabling children to function on a higher level than if not treated by the program and to evaluate whether the program will affect parent's ability to cope with stress and anxiety related to having a child at risk of developing CP. TRIAL REGISTRATION: ClinicalTrials.gov Identifier NCT02166801 . Registered June 12, 2014.

Muscle strength does not explain standing ability in children with bilateral spastic cerebral palsy: a cross sectional descriptive study.

BACKGROUND: In bilateral cerebral palsy (CP) muscle strength is considered important for development of gross motor functions, but its influence on standing ability has not been explored. Our aims were to examine muscle strength with respect to the ability to stand with (SwS) or without (SwoS) hand support, asymmetrical weight bearing (WB), and whether the ability to produce strength was influenced by different seated conditions. METHODS: In this cross sectional descriptive study standing posture was recorded with 3D motion analysis, and muscle strength was measured with a hand-held dynamometer, in 25 children with bilateral CP, GMFCS levels II-III, SwS (n = 14, median age 11.4 years), or SwoS, (n = 11, median age 11.4 years). Strength measurements were taken in the hip flexors, knee extensors, dorsiflexors and plantarflexors, in two seated conditions; a chair with arm- and backrests, and a stool. RESULTS: Compared to SwoS, children SwS stood with a more flexed posture, but presented with equal strength in the hip flexors, dorsiflexors and plantarflexors, and with somewhat more strength in the knee extensors. Despite asymmetric WB during standing, both limbs were equally strong in the two groups. No differences in strength were measured between the two seated conditions. CONCLUSIONS: Despite challenges measuring muscle strength in CP, the lower limb muscle strength cannot be considered an explanatory factor for variations in standing in this group of children with bilateral CP. The findings rather strengthen our hypothesis that deficits in the sensory systems could be as determinant for standing as muscle weakness in children with bilateral spastic CP.

A prospective cohort study investigating gross motor function, pain, and health-related quality of life 17 years after selective dorsal rhizotomy in cerebral palsy.

AIM: The aim of this study is to evaluate the long-term effects of selective dorsal rhizotomy (SDR), 15 to 20 years after surgery in patients with cerebral palsy. METHOD: Eighteen children (four females, 14 males; mean age at SDR 4y 7mo, SD 1y 7mo) with bilateral spastic cerebral palsy (CP), were prospectively assessed after SDR. This study focuses on the outcome 15 to 20 years after the procedure. The assessments include the Modified Ashworth Scale for spasticity, the Gross Motor Function Measure (GMFM-88), the Wilson Mobility Scale, The Health-Related Quality of Life Health Survey, SF-36v2, and the Brief Pain Inventory. RESULTS: The effect of normalized muscle tone in lower extremities after SDR was sustained after a median of 17 years. The best gross motor function capacity, according to the GMFM score, was seen at the 3-year follow-up, thereafter a gradual decline followed. Half of the individuals reported low intensity pain and interference. Compared to a norm sample the physical health component of SF-36v2 was slightly lower and the mental health component slightly higher. INTERPRETATION: The spasticity-reducing effect of SDR does not improve long-term functioning, nor prevent contractures, but it can possibly reduce the pain often experienced by individuals with CP.

Introduction of the gross motor function classification system in Venezuela--a model for knowledge dissemination.

BACKGROUND: A current worldwide common goal is to optimize the health and well-being of children with cerebral palsy (CP). In order to reach that goal, for this heterogeneous group, a common language and classification systems are required to predict development and offer evidence based interventions. In most countries in Africa, South America, Asia and Eastern Europe the classification systems for CP are unfamiliar and rarely used. Education and implementation are required. The specific aims of this study were to examine a model in order to introduce the Gross Motor Function Classification System (GMFCS-E&R) in Venezuela, and to examine the validity and the reliability. METHODS: Children with CP, registered at a National child rehabilitation centre in Venezuela, were invited to participate. The Spanish version of GMFCS-E&R was used. The Wilson mobility scale was translated and used to examine the concurrent validity. A structured questionnaire, comprising aspects of mobility and gross motor function, was constructed. In addition, each child was filmed. A paediatrician in Venezuela received supervised self-education in GMFCS-E&R and the Wilson mobility scale. A Swedish student was educated in GMFCS-E&R and the Wilson mobility scale prior to visiting Venezuela. In Venezuela, all children were classified and scored by the paediatrician and student independently. An experienced paediatric physiotherapist (PT) in Sweden made independent GMFCS-E&R classifications and Wilson mobility scale scorings, accomplished through merging data from the structured questionnaire with observations of the films. Descriptive statistics were used and reliability was presented with weighted Kappa (Kw). Spearman's correlation coefficient was calculated to explore the concurrent validity between GMFCS-E&R and Wilson mobility scale. RESULTS: Eighty-eight children (56 boys), mean age 10 years (3-18), with CP participated. The inter-rater reliability of GMFCS-E&R between; the paediatrician and the PT was Kw = 0.85 (95% CI: 0.75-0.88), the PT and student was Kw = 0.91 (95% CI: 0.86-0.95) and the paediatrician and student was Kw = 0.85 (95 % CI: 0.79-0.90). The correlations between GMFCS-E&R and Wilson mobility scale were high rs =0.94-0.95 (p < 0.001). CONCLUSIONS: In a setting with no previous knowledge of GMFCS-E&R, the model with education, supervised self-education and practice was efficient and resulted in very good reliability and validity.

The accommodative process in children with cerebral palsy: different strategies to obtain clear vision at short distance.

AIM: Accommodation is the ability of the eye to change focus in order to maintain a sharp image of objects at various distances. The accommodative process is largely unknown in children and requires new assessment techniques. The aim of the study was to investigate this process in children with and without cerebral palsy (CP). METHOD: In a descriptive case-control study, children with CP (n=15; nine females, six males; median age 14y) and 21 typically developing children (11 females, 10 males; median age 12y) underwent standard ophthalmological examination and examination by the PowerRefractor. Six of the children had spastic bilateral CP, five had spastic unilateral CP, and four had dyskinetic CP. The children's Gross Motor Function Classification System (GMFCS) levels were as follows: level I, seven children; level II, two children; level III, three children; and level IV, three children. The PowerRefractor measures accommodation in response to minus lens stimuli. Continuous measurements of refraction/accommodation, eye position, and pupil size are obtained. The Kruskal-Wallis analysis of variance (ANOVA) and Mann-Whitney U test were used for between-group analysis (α=0.05), and Friedman ANOVA was used for within-group analysis. RESULTS: The stimuli-response gain (input/output) was approximately 80% in typical children inducing a focusing error (0.2-0.5D) increasing with minus lens power. Children with CP accommodated significantly less (gain: ~30%; p<0.001), inducing a larger focusing error (1.1-1.7D) increasing with minus lens power. The accommodative response was slower and more variable in children with CP. The pupil response did not mirror the accommodative response. INTERPRETATION: Children with CP exhibit problems in generating an appropriate accommodative response. This can affect everyday living and reading skills.

Efficacy of baby-CIMT: study protocol for a randomised controlled trial on infants below age 12 months, with clinical signs of unilateral CP.

BACKGROUND: Infants with unilateral brain lesions are at high risk of developing unilateral cerebral palsy (CP). Given the great plasticity of the young brain, possible interventions for infants at risk of unilateral CP deserve exploration. Constraint-induced movement therapy (CIMT) is known to be effective for older children with unilateral CP but is not systematically used for infants. The development of CIMT for infants (baby-CIMT) is described here, as is the methodology of an RCT comparing the effects on manual ability development of baby-CIMT versus baby-massage. The main hypothesis is that infants receiving baby-CIMT will develop manual ability in the involved hand faster than will infants receiving baby-massage in the first year of life. METHOD AND DESIGN: The study will be a randomised, controlled, prospective parallel-group trial. Invited infants will be to be randomised to either the baby-CIMT or the baby-massage group if they: 1) are at risk of developing unilateral CP due to a known neonatal event affecting the brain or 2) have been referred to Astrid Lindgren Children's Hospital due to asymmetric hand function. The inclusion criteria are age 3-8 months and established asymmetric hand use. Infants in both groups will receive two 6-weeks training periods separated by a 6-week pause, for 12 weeks in total of treatment. The primary outcome measure will be the new Hand Assessment for Infants (HAI) for evaluating manual ability. In addition, the Parenting Sense of Competence scale and Alberta Infant Motor Scale will be used. Clinical neuroimaging will be utilized to characterise the brain lesion type. To compare outcomes between treatment groups generalised linear models will be used. DISCUSSION: The model of early intensive intervention for hand function, baby-CIMT evaluated by the Hand Assessment for Infants (HAI) will have the potential to significantly increase our understanding of how early intervention of upper limb function in infants at risk of developing unilateral CP can be performed and measured. TRIAL REGISTRATION: SFO-V4072/2012, 05/22/2013.

Maternal type 1 diabetes, preterm birth, and risk of intellectual disability in the offspring: A nation-wide study in Sweden.

OBJECTIVE: There are few data on long-term neurological or cognitive outcomes in the offspring of mothers with type 1 diabetes (T1D). The aims of this study were to examine if maternal T1D increases the risk of intellectual disability (ID) in the offspring, estimate the amount of mediation through preterm birth, and examine if the association was modified by maternal glycated hemoglobin (HbA1c). DESIGN: Population-based cohort study using population-based data from several national registries in Sweden. SETTING AND PARTICIPANTS: All offspring born alive in Sweden between the years 1998 and 2015. MAIN OUTCOME MEASURE: The risk of ID was estimated through hazard ratios with 95% confidence intervals (HR, 95% CI) from Cox proportional hazard models, adjusting for potential confounding. Risks were also assessed in mediation analyses and in subgroups of term/preterm births, in relation to maternal HbA1c and by severity of ID. RESULTS: In total, 1,406,441 offspring were included. In this cohort, 7,794 (0.57%) offspring were born to mothers with T1D. The risk of ID was increased in offspring of mothers with T1D (HR; 1.77, 1.43-2.20), of which 47% (95% CI: 34-100) was mediated through preterm birth. The HRs were not modified by HbA1c. CONCLUSION: T1D in pregnancy is associated with moderately increased risks of ID in the offspring. The risk is largely mediated by preterm birth, in particular for moderate/severe cases of ID. There was no support for risk-modification by maternal HbA1c.

Responses to Sensory Events in Daily Life in Children with Cerebral Palsy from a Parent Reported Perspective and in a Swedish Context.

The motor disorders of cerebral palsy (CP) are often accompanied by sensory disturbances, but knowledge of their relationship to motor functioning is sparse. This study explored responses to sensory events in relation to spastic subtype and motor functioning in children with CP. Parents of 60 children with CP (unilateral: 18, bilateral: 42) with GMFCS levels I:29, II:13, III:15 and IV:3 of mean age 12.3 years (3.7 SD) participated. The parents (n = 55) rated their children´s responses with the norm-referenced questionnaire Child Sensory Profile-2© (CSP-2©), Swedish version, incorporating nine sections and four sensory processing patterns/quadrants, and replied (n = 57) to two additional questions. On the CSP-2©, thirty (55%) of the children were reported to have responses "much more than others" (>2 SD) in one or more of the sections and/or quadrants and 22 (40%) in the section of Body Position, overrepresented by the children with bilateral CP. The additional questions revealed that a greater proportion of children at GMFCS levels III-IV compared to level I frequently were requested to sit/stand up straight (14/17 versus 6/26, p < 0.001) and were sound sensitive at a younger age (14/17 versus 10/26, p = 0.005). The findings of this study highlight the sensory aspects of motor functioning in children with spastic CP.

Mapping length of inpatient treatment duration and year-wise relapse rates in eating disordered populations in a well-defined Western-European healthcare region across 1998-2020.

OBJECTIVES: Updated international guideline recommendations for AN inpatient care rely on expert opinions/observational evidence and promote extended inpatient stays, warranting investigation using higher-level ecological evidence. METHODS: The study was conducted according to Guidelines for Accurate and Transparent Health Estimates Reporting (GATHER). Data encompassing 13,885 ED inpatients (5336 adolescents and 8549 adults) was retrieved from Swedish public health registries. Variables analyzed included (1) ED inpatient care opportunities, (2) unique number of ED inpatients and (3) mean length of ED-related inpatient stays in age groups 15-19 and 20-88+, across 1998-2020. RESULTS: Mean length of inpatient stays was inversely correlated to relapse to ED-related inpatient care within the same year (p < 0.001, R-squaredadj  = 0.5216 and p < 0.00001, R-squaredadj  = 0.5090, in the 15-19 and 20-88+ age groups, respectively), independent of number of ED inpatients treated within a year in both age groups. Extending mean adolescent inpatient duration from 35 to 45 days was associated with a ∼30% reduction in the year-wise relapse rate. CONCLUSIONS: Mean length of ED-related inpatient treatment stays was associated with reduced relapses to inpatient care within the same year, which could be interpreted as support for recommendations to include a stabilization phase in inpatient ED treatment.