Cognitive skills in young boys with Duchenne muscular dystrophy.
Item
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Title
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Cognitive skills in young boys with Duchenne muscular dystrophy.
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Identifier
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AAI3231986
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identifier
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3231986
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Creator
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Cyrulnik, Shana E.
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Contributor
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Adviser: Jeffrey M. Halperin
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Date
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2006
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Language
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English
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Publisher
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City University of New York.
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Subject
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Psychology, Cognitive | Psychology, Psychobiology
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Abstract
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Duchenne muscular dystrophy (DMD) is a genetic disorder that causes progressive, and ultimately fatal, muscular weakness. While known primarily for its devastating motor effects, DMD is also associated with cognitive deficits. These cognitive deficits have been well documented in older children and adolescents with DMD. However, there are little data examining the development of cognitive skills in young male children with this disorder. The goal of the current dissertation was to focus directly on the early development of cognitive skills in young boys with DMD. To that end, two studies were conducted. First, early development was assessed indirectly via retrospective parental report in a large sample of children with DMD. Second, cognitive skills were assessed directly in a smaller sample of children with DMD aged three to six. This two-step investigation allowed for the capture of both the global manifestation of early developmental delay and the more detailed explication of cognitive skills as they emerged in young children. In addition to these two research studies, the author presents a hypothesis as to the neural substrates of these cognitive deficits. Many investigators have linked cognitive impairments in DMD to the absence of dystrophin, a protein product which is normally found in multiple tissues throughout the body, but is absent in DMD. The current dissertation advances the hypothesis that it is the absence of dystrophin in the cerebellum which is responsible for the cognitive deficits observed. Specifically, the author contends that brain pathways in the cerebellum (e.g., cerebro-cerebellar loops) which develop without dystrophin may result in altered brain function presenting as cognitive deficits in DMD.
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Type
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dissertation
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Source
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PQT Legacy CUNY.xlsx
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degree
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Ph.D.
