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.

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.

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.

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.

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.

Dystonia during hand activity in children with spastic unilateral cerebral palsy, an observational study.

BACKGROUND: Spasticity and dyskinesia are motor signs that co-exist in cerebral palsy (CP). It is well accepted that, in spastic bilateral CP, dystonia can be present in addition to spasticity, and equally that spasticity is often present in individuals with dyskinetic CP. In unilateral spastic CP, dystonia of the upper extremity is only rarely identified or addressed. The aim of this study was to investigate if dystonia was present in the hand of children with unilateral spastic CP, and, if present, to what extent, and when it was first noticeable. METHOD: Ninety-seven children with unilateral spastic CP, born 1999-2014, with standardized digital films of hand function from Assisting Hand Assessments (AHA), were included. Films were reviewed, and presence or absence, of dystonia and choreoathetosis were scored by three experienced raters. RESULTS: Dystonia in the hand was present during activities in 70% (68/97) of the children at a mean age of 12 years (SD 4,4). In 74% (50/68) of these children, dystonia was present more than 50% of the evaluated time. For 63% (43/68) more than one digital recording at younger ages were available. Dystonia could first clearly be observed at a mean age of 3,8 years. Choreoathetosis was observed in 7% (5/68) of the children with dystonia. Children without dystonia had significantly higher (corresponding to better function) AHA units (median: 75, 25th - 75th: 45-82) in comparison to children with dystonia (median: 57, 25th - 75th: 52-63) (p = 0.01). CONCLUSION: Dystonia in the hand is common in unilateral CP and correlates to lower hand functioning.

Gestational age and risk of intellectual disability: a population-based cohort study.

OBJECTIVE: To examine the association between gestational age at birth and risk of clinically diagnosed intellectual disability (ID) week by week to provide a detailed description of ID risk across the entire range of gestational ages and by severity of ID. METHODS: All individuals born alive in Sweden 1974-2017 were prospectively followed up from birth until 2017 using national registers. The HRs for ID according to weekly gestational age and gestational age categories were determined using Cox models. Sibling analyses were conducted to adjust for familial confounding. RESULTS: The study included 3 572 845 live births. During the follow-up, 26 596 ID cases were registered. The adjusted weekly estimates showed a gradual increase in risk of ID from week 40 to week 24 (adjusted HR37weeks=1.80 (1.74 to 1.87), aHR32weeks=3.93 (3.73 to 4.13), aHR28weeks=7.53 (6.95 to 8.16), aHR24weeks=21.58 (18.62 to 25.00)) and from week 41 onwards (aHR42weeks=1.26 (1.19 to 1.32)), with statistically significantly higher risks across the range of gestational age compared with infants born at week 40. The associations were consistent in mild, moderate and severe/profound ID but most prominent for severe/profound ID. CONCLUSION: The risk of ID increased weekly as the date of delivery moved away from 40 weeks, both preterm and post-term. The results remained robust after detailed adjustment for confounding, including familial confounding.

Impact of gestational age on risk of cerebral palsy: unravelling the role of neonatal morbidity.

BACKGROUND: The contribution of adverse consequences of preterm birth to gestational-age-related risk of cerebral palsy (CP) has rarely been studied. We aimed to assess the potential mediating roles of neonatal morbidity on the association between gestational age and risk of CP. METHODS: In this Swedish population-based study, 1 402 240 singletons born at 22-40 gestational weeks during 1998-2016 were followed from day 28 after birth for a CP diagnosis until 2017. Potential mediators included asphyxia, respiratory-related, infection-/inflammatory-related and neurological-related diseases within 0-27 days of life. Cox regression was used to estimate hazard ratios (HRs) and 95% confidence intervals (CIs). Causal mediation analysis was performed to estimate the proportion of the association mediated through pathways involving the four sequential mediators. RESULTS: We found an inverse dose-response relationship between gestational age and risk of CP, where the strongest association was observed for 22-24 weeks (HR 47.26, 95% CI 34.09-65.53) vs 39-40 weeks. Compared with non-diseased peers, children with neonatal morbidity, particularly those with neurological-related diseases (HR 31.34, 95% CI 26.39-37.21), had a higher risk of CP. The increased risk of CP was, at 24 weeks, almost entirely explained by neonatal morbidity (91.7%); this proportion decreased to 46.1% and 16.4% at 32 and 36 weeks, respectively. Asphyxia was the main mediating pathway from 22 to 34 weeks, and neurological-related neonatal diseases led the mediating pathways from 34 weeks onwards. CONCLUSION: Neonatal morbidity mediates a large proportion of the effect of preterm birth on CP, but the magnitude declines as gestational age increases.

A First Clinical Trial on Botulinum Toxin-A for Chronic Muscle-Related Pain in Cerebral Palsy.

Objective: To test if botulinum toxin-A (BoNT-A) is effective in reducing chronic muscle-related pain in adults with spastic cerebral palsy (CP), as compared to placebo. Design: A single-center, double-blind, parallel, randomized placebo-controlled trial. The design included an interim analysis to allow for confirmatory analysis, as well as pilot study outcomes. Setting: Tertiary university hospital. Participants: Adults with spastic CP and chronic pain associated with spastic muscle(s). Intervention: Treatment was one session of electromyographically guided intramuscular injections of either BoNT-A or placebo normosaline. Main Study Outcomes: The primary outcome was the proportion who achieved a reduction of pain intensity of two or more steps on the Numerical Rating Scale 6 weeks after treatment. Results: Fifty individuals were screened for eligibility, of whom 16 were included (10 female, 6 male, mean age = 32 years, SD = 13.3 years). The randomization yielded eight participants per treatment arm, and all completed the study as randomized. The study was stopped at the interim analysis due to a low probability, under a preset threshold, of a positive primary outcome. Four individuals were treatment responders in the BoNT-A group for the primary outcome compared to five responders in the placebo group (p = 1.000). Adverse events were mild to moderate. In exploratory analysis, the BoNT-A group had a trend of continuing reduction of pain at the last follow-up, after the primary endpoint. Conclusions: This study did not find evidence that BoNT-A was superior to placebo at the desired effect size (number needed to treat of 2.5) at 6 weeks after treatment. Trial registration:ClinicalTrials.gov: NCT02434549.

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.

Prevalence, identification, and interference of pain in young children with cerebral palsy: a population-based study.

OBJECTIVE: To explore the presence of pain, how pain was addressed by physicians and parents, and how pain affected everyday life in young children with cerebral palsy (CP). METHODS: Children with CP, aged 5-10 years, participated in this cross-sectional study. Data were collected from medical records spanning a period of two years and by a standardized parental interview that included six structured questions and the Pain Interference Index. RESULTS: A total of 118 children, with a mean age of 7.4 years (SD 1.5), participated in the study. The parents of 81% of these children were interviewed. Pain was reported in 52% of the children, and pain was present at all severity levels. The prescription of analgesics was documented in 25% of these children's medical records. Fifty-nine percent of the children with pain received analgesics from their parents. Pain restricted the children's everyday lives particularly concerning sleep, school work and being with friends. CONCLUSIONS: Half of this group of young children with CP were reported to have pain. Pain restricted the children's everyday lives and seemed to be under-treated. If pain can be addressed early, the children's everyday lives are likely to be improved.Implications for rehabilitationThere is a need to early identify and treat pain in young children.Important to discuss pain with parents irrespectively of the child's age and severity level.Pain interference assessment gives valuable information.Early treatment of pain might improve children's everyday life.

Childhood-onset seizures: A long-term cohort study of use of antiepileptic drugs, and drugs for neuropsychiatric conditions.

OBJECTIVE: We conducted a long-term follow-up of a cohort of children with newly diagnosed unprovoked seizures to assess treatment with antiepileptic drugs (AEDs), neuroleptics, antidepressants and medication for attention deficit hyperactivity disorder (ADHD) with special attention to the impact of comorbidities on the use of such medication. METHODS: Our study cohort comprised 769 children (28 days-18 years), living in Stockholm Sweden, with a first unprovoked seizure identified between 2001 and 2006. Information on neurodevelopmental comorbidities and Cerebral Palsy (CP) at seizure onset was collected from medical records. Information on treatment with AEDs, neuroleptics, antidepressants and ADHD medication was retrieved by linkage to the Swedish National Prescription Registry between 2005 and 2014. The association between comorbidities and drug treatments was assessed by odds ratios (OR) with 95 % confidence intervals (CI), adjusted for age and sex. RESULTS: Eight years after the index seizure, 31 % of the children were on AEDs, and this was more common among children with any of the comorbidities studied (OR; 4.0 95 % CI 2.9-5.6) compared to those without such comorbidities, and within this group of comorbidities particularly for those with CP (OR; 5.2 95 % CI: 2.9-9.3). Children with neurodevelopmental comorbidity or CP at baseline were more likely to receive neuroleptics (ORs 8 years after the index seizure; 6.9, 95 % CI: 2.4-19.8), antidepressants (OR; 2.3, 95 % CI: 1.0-5.5) and ADHD medication (OR; 3.6, 95 % CI: 1.8-7.2) than children without the studied comorbidities. CONCLUSION: Children with seizures in combination with neurodevelopmental comorbidities or CP, especially CP, have a more frequent use of AEDs, neuroleptics, antidepressants, and ADHD medication up to 13 years following the initial seizure than children without comorbidity. Our data highlight the treatment burden in children with epilepsy and comorbidities.

Reliability and Validity of the Dyskinesia Impairment Scale in Children and Young Adults with Inherited or Idiopathic Dystonia.

BACKGROUND: The Dyskinesia Impairment Scale (DIS) is a new assessment scale for dystonia and choreoathetosis in children and youth with dyskinetic cerebral palsy. Today, the Burke-Fahn-Marsden Dystonia Rating Scale (BFM) is mostly used to assess dystonia in children with inherited dystonia. The aim of this study was to assess reliability and validity of the DIS in children and youth with inherited or idiopathic dystonia. METHODS: Reliability was measured by (1) the intraclass correlation coefficients (ICCs) for inter-rater and test-retest reliability, as well as (2) standard error of measurement (SEM) and minimal detectable difference (MDD). For concurrent validity of the DIS-dystonia subscale, the BFM was administered. RESULTS: In total, 11 males and 9 females (median age 16 years and 7 months, range 6 to 24 years) were included. For inter-rater reliability, the ICCs for the DIS total score and the dystonia and choreoathetosis subscale scores were 0.83, 0.87, and 0.71, respectively. For test-retest reliability, the ICCs for the DIS total score and the dystonia and choreoathetosis subscale scores were 0.95, 0.88, and 0.93, respectively. The SEM and MDD for the total DIS were 3.98% and 11.04%, respectively. The Spearman correlation coefficient between the dystonia subscale and the BFM was 0.88 (p < 0.01). CONCLUSIONS: Good to excellent inter-rater, test-retest reliability, and validity were found for the total DIS and the dystonia subscale. The choreoathetosis subscale showed moderate inter-rater reliability and excellent test-retest reliability. The DIS may be a promising tool to assess dystonia and choreoathetosis in children and young adults with inherited or idiopathic dystonia.

Exploring sleep problems in young children with cerebral palsy - A population-based study.

OBJECTIVE: To describe and explore sleep problems in a population-based cohort of young children with cerebral palsy (CP) in Stockholm, Sweden. METHODS: All children with CP, aged 5-10 years, and living in the Northern Karolinska University Hospital's catchment area were invited to participate in a cross-sectional study. Medical records obtained in the previous two-year period were reviewed, and a pre-planned parental telephone interview that included five structured questions and the Insomnia Severity Index (ISI) was conducted. RESULTS: In total, 118 children, with a mean age of 7.4 years (SD 1.5), were included. Bilateral CP was present in 45%, unilateral in 37%, dyskinetic in 15%, and ataxic CP in 3%. Parents of 81% of the children participated in the interview. They reported sleep problems in 41% of their children, and in 80% of these children, night-time sleep was negatively affected by pain. Differences between the ISI total score in relation to CP subtypes (p < 0.025) and levels in GMFCS-E&R (p < 0.001) were detected, with increasing sleep problems for children with dyskinetic CP and children in GMFCS-E&R V. Sleep problems affected by pain were associated to the total score at ISI (rs = 0.83, p < 0.001, n = 95). CONCLUSION: The results identified that sleep problems were present in more than 40% of children with CP. Sleep problems were more frequently and extensively present in children with dyskinetic CP and children in GMFCS-E&R level V. Sleep problems were associated with the presence of pain and, in particular, in the most severely affected children.

Cognition in Children with Arachnoid Cysts.

BACKGROUND: This study aims to evaluate if children with temporal arachnoid cysts (AC) have cognitive symptoms and if neurosurgery improves these. METHODS: A prospective case series study including consecutive pediatric patients with temporal AC. The children underwent neuroradiology, neuroopthalmologic evaluation, and a standard electroencephalography (EEG). Additionally, a neuropsychologist performed a standardized set of evaluations, with a one-year follow-up consisting of Weschler Intelligence Scale for Children version IV (WISC-IV), FAS (for verbal fluency), Boston Naming Test (BNT, for visual naming ability) and NEPSY-II (Developmental NEuroPSYchological Assessment) for verbal memory. RESULTS: Fifteen children, 9 boys and 6 girls, were evaluated and 11 underwent surgery. The Full Scale IQ subscore (FSIQ) improved from M = 84.8 to M = 93.0 (p = 0.005). The preoperative Verbal Comprehension Index (VCI) was in the low average range (M = 86.7), improving to a level within the average range (M = 94.7, p = 0.001). Preoperative Perceptual Speed Index (PSI) was in the below average range (M = 81.5), improving to a level within the average range (M = 92.5, p = 0.004). CONCLUSION: ACs are a common finding in a pediatric neurosurgical setting. Our data suggest that some temporal AC have a negative effect on general cognitive ability and that this impairment can be improved by surgery. We suggest a standardized evaluation, including comprehensive and validated neuropsychological assessment tools, to thoroughly assess symptoms as well as the postoperative outcome.

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.

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.

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.