Predicting Adolescent Fear of Crime Through the Lens of General Strain Theory

This study examines the effect of neighborhood disorder and strain on adolescent fear of crime while controlling for demographic and contextual variables that have previously been demonstrated as having an association with fear of crime. Earlier work has suggested that feelings of powerlessness have a significant impact on fear of crime among adolescents; thus strain theory (derivatives of which deal with both perceptions of blocked opportunities and powerlessness to achieve positively valued goals) may impact fear of crime as well. Using data from self-report surveys of approximately 700 adolescents from a southeastern state, multiple regression analyses indicate that those adolescents who felt most “strained” had significantly higher levels of fear of crime than their counterparts. This relationship persisted even after controlling for known predictors of fear of crime. Explanations for these findings, as well as their ramifications for social policies and future research, are discussed

Beta-propeller protein-associated neurodegeneration:a new X-linked dominant disorder with brain iron accumulation

Neurodegenerative disorders with high iron in the basal ganglia encompass an expanding collection of single gene disorders collectively known as neurodegeneration with brain iron accumulation. These disorders can largely be distinguished from one another by their associated clinical and neuroimaging features. The aim of this study was to define the phenotype that is associated with mutations in WDR45, a new causative gene for neurodegeneration with brain iron accumulation located on the X chromosome. The study subjects consisted of WDR45 mutation-positive individuals identified after screening a large international cohort of patients with idiopathic neurodegeneration with brain iron accumulation. Their records were reviewed, including longitudinal clinical, laboratory and imaging data. Twenty-three mutation-positive subjects were identified (20 females). The natural history of their disease was remarkably uniform: global developmental delay in childhood and further regression in early adulthood with progressive dystonia, parkinsonism and dementia. Common early comorbidities included seizures, spasticity and disordered sleep. The symptoms of parkinsonism improved with l-DOPA; however, nearly all patients experienced early motor fluctuations that quickly progressed to disabling dyskinesias, warranting discontinuation of l-DOPA. Brain magnetic resonance imaging showed iron in the substantia nigra and globus pallidus, with a ‘halo’ of T(1) hyperintense signal in the substantia nigra. All patients harboured de novo mutations in WDR45, encoding a beta-propeller protein postulated to play a role in autophagy. Beta-propeller protein-associated neurodegeneration, the only X-linked disorder of neurodegeneration with brain iron accumulation, is associated with de novo mutations in WDR45 and is recognizable by a unique combination of clinical, natural history and neuroimaging features