Cerebellar malformations are increasingly diagnosed in the fetal period. Consequently, their consideration requires stressful and often critical decisions from both clinicians and families. This has resulted in an emergent need to understand better the impact of these early life lesions on child development. We performed a comprehensive literature search of studies describing neurodevelopmental outcomes of cerebellar malformations between January 1997 and December 2007. Overall, the data suggested that children with isolated inferior vermis hypoplasia (IVH) and mega cisterna magna (MCM) have a good developmental outcome, whereas children with molar tooth sign/Joubert syndrome, vermis hypoplasia, pontocerebellar hypoplasia (PCH) type II, and cerebellar agenesis experience moderate to severe global developmental delays. Reports for Dandy−Walker malformation (DWM) were conflicting; however, the presence of a normally lobulated vermis and the absence of associated brain anomalies were associated with a more favourable outcome. Finally, children with isolated cerebellar hypoplasia experienced fewer impairments. Important methodological limitations highlighted include a lack of standardized outcome measure use in 79% of studies and the predominant use of retrospective study designs (85%), with 40% limited to case reports or case‐series. In summary, rigorous outcome studies describing the spectrum of disabilities in survivors are urgently needed to accurately delineate the long‐term neurodevelopmental consequences of cerebellar malformations.
Risk Reconsidered: Targets of violence in the Social Networks of People with Serious Mental illness. This exploratory analysis addresses the questions: 1) Who among the members of the social network of a person diagnosed with a major psychiatric disorder is likely to become a target of violence? 2) What kind of relationships do targets have with respondents in terms of the quality and quantity of interactions?, and 3) What are the risk factors that contribute to being a target of violence for people who are in the social networks of persons with serious mental disorders? The samples of 169 people with serious mental disorders were followed for 30 months. A logistic regression model of the risk for being a target of violence among the members of the cohort's social network reveals that both target and respondent characteristics are salient, and that mothers who live with adult children who have schizophrenia and co-occurring substance abuse bear a substantially elevated risk of becoming a target of violence, compared to other social network members. Other factors that elevate the risk for being a target of violence are being an immediate family members of the respondent, more time in residence with the respondent, and whether the respondent is financially dependent on the family. Respondents with the most mental health center visits had lower odds of committing an act or threat of violence against a social network member.
Aim Advances in perinatal care and neuroimaging techniques have increased the detection of cerebellar malformations (CBMs) in the fetus and young infant. As a result, this has necessitated a greater understanding of the neurodevelopmental consequences of CBMs on child development. The aim of this study was to delineate the impact of CBMs on long‐term neurodevelopmental outcomes. Method We conducted a cross‐sectional study and systematically identified children with CBMs born between December 2000 and December 2006. We then performed follow‐up magnetic resonance imaging studies, neurologic examination, and standardized neurodevelopmental outcome testing (Mullen Scales of Early Learning, Vineland Adaptive Behavior Scale, Child Behavior Checklist, Modified Checklist for Autism in Toddlers, and the Pediatric Quality of Life Inventory). Results Our sample comprised 49 children (29 males, 20 females; mean age, 28.4mo, SD 16.4) with a CBM. Infants with evidence of acquired fetal or neonatal brain injury, intracranial birth trauma, inherited metabolic disease, or major pre‐ or postnatal cerebral ischemia were excluded. Our findings highlight that children with CBMs experience a high prevalence of neurologic, developmental, and functional disabilities including motor, cognitive, language, and social–behavioral deficits, as well as poor quality of life. The associated supratentorial anomalies, chromosomal findings, and malformations affecting the cerebellar vermis were significant independent predictors of neurodevelopmental disabilities in young children with CBMs. The associated supratentorial anomalies and chromosomal findings were also predictive of global developmental delay (p=0.01), cognitive impairment (p=0.03), gross and fine motor delay (p=0.02 and p=0.01 respectively), and positive screening for autism spectrum disorder (p=0.01). Additionally, malformations affecting the cerebellar vermis were significant independent predictors of expressive language (p=0.04) and gross motor delays (p=0.02). Interpretation Developmental surveillance and early intervention programs should be an integral part of the long‐term follow‐up of survivors of CBM.
The cerebellum has recently been recognized for its role in high-order functions, including cognition, language, and behavior. Recent studies have also begun to describe a functional topography of the mature cerebellum that includes organization on a mediolateral axis. However, no study to date has examined the relationship between regional cerebellar volume and developmental disabilities in children with cerebellar malformations. The objective of this study was to estimate the extent to which total and regional cerebellar volumes are associated with developmental disabilities in a cohort of children with cerebellar malformations. Children aged 1 to 6 years with a diagnosis of cerebellar malformation underwent standardized outcome measures and quantitative magnetic resonance scanning. The cerebellum was parcellated into seven mediolateral zones (three for each hemisphere plus the vermis) for regional volume analysis. In children with cerebellar malformations, decreased total cerebellar volume was associated with delays in global development, expressive language, cognition, as well as gross and fine motor function. Decreased volume in the right lateral cerebellar hemisphere was related to impaired cognition, expressive language, and gross motor function. Additionally, reduced vermis volume was associated with impaired global development, cognition, expressive language, and gross and fine motor skills, as well as behavior problems and a higher rate of positive autism spectrum screening test. These results begin to define the structural topography of functional outcome in children with cerebellar malformations and should lead to greater accuracy of prognostication as well as timely early developmental interventions.
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