CADASIL with Atypical Clinical Symptoms, Magnetic Resonance Imaging, and Novel Mutations: Two Case Reports and a Review of the Literature

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary microangiopathy with adult onset caused by a missense mutation in the NOTCH3 gene in chromosome 19p13. It presents with autosomal dominant arteriopathy, subcortical infarctions, and leukoencephalopathy. Its common clinical presentations are seen as recurrent strokes, migraine or migraine-like headaches, progressive dementia, pseudobulbar paralysis, and psychiatric conditions. Two patients with CADASIL syndrome, whose diagnosis was made based on clinical course, age of onset, imaging findings, and genetic assays in the patients and family members, are presented here because of new familial polymorphisms. The first patient, with cerebellar and psychotic findings, had widespread non-confluent hyperintense lesions as well as moderate cerebellar atrophy in cranial magnetic resonance scanning. The other patient, with headache, dizziness, and forgetfulness, had gliotic lesions in both cerebral hemispheres. CADASIL gene studies revealed a new polymorphism in exon 33 in the first patient. In the other patient, the NOTCH3 gene was identified as a new variant of p.H243P (c.728A > C heterozygous). By reporting a family presenting with various clinical symptoms in the presence of new polymorphisms, we emphasize that CADASIL syndrome may present with various clinical courses and should be considered in differential diagnoses. © 2019, Springer Science+Business Media, LLC, part of Springer Nature

Migrant living spaces: Religiosity and gender in a disciplinary institution

Contains fulltext : 122158.pdf (publisher's version ) (Closed access)This article looks at the production of gendered pious subjectivities at an Islamic student dorm in the Netherlands, for Turkish–Dutch female students. Following the Foucauldian framework of disciplinary power, we trace how normalising disciplinary techniques are at work in the dorm. We note, however, that members of the dorm voluntarily subject themselves to this power, as they are highly committed to Islamic self-development and living in a pious universe as a mode of being. Members perceive the disciplinary sanctions on themselves as necessary. Therefore, we argue that the constitution of gendered pious subjects is a product of the interplay between agentic subjects and the disciplinary, normalising techniques of the dorm. We want to also stress, however, that gender norms become all the more binding and stringent when people are held accountable by a disciplinary institution rather than personal ethical convictions.19 p

Development of Externalizing Behaviors in the Context of Family and non-Family Relationships

A longitudinal model was presented, that included reciprocal associations between physically harsh parenting by the mother, child externalizing problems, and support from the father, the extended family, and the neighbors. This transactional process was estimated for the years preceding school entry. The data were from a 4-years longitudinal and nationally representative study of 1009 children and their mothers in Turkey. The results indicated that concurrently, physically harsh parenting and child externalizing problems were strongly associated. Controlling for their within domain stability and cross-domain concurrent correlation, changes in harsh parenting and changes in child externalizing behaviors had significant reciprocal effects in early childhood, although these effects were small. These reciprocal effects were smaller for observer reported harsh parenting than maternal reports. There was a role of the mesosystem in this developmental process. Increases in the support from the father, and the extended family and the neighbors predicted declines in the child externalizing behaviors subsequently. Reciprocally, high child externalizing and maternal physically harsh parenting predicted subsequent declines in the support from these sources. These results were consistent with the hypotheses that negative mother–child relationships could spill over to the other relationships of the mothers, and that positive and supportive relationships of the mother could constitute positive role models for the child.This research was funded by a grant from the Turkish Institute for Scientific and Technological Research (106K347 and 109K525) and received generous support from Koc Universit