Childhood Ataxia with Central Nervous System Hypomyelinization

Known as: Cree Leukoencephalopathies, CACH VWM Syndrome, Cree Leukoencephalopathy 
A rare, progressive neurological disorder inherited in an autosomal recessive pattern. It is caused by mutations in the EIF2B1, EIF2B2, EIF2B3… (More)
National Institutes of Health

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ApathyAutosomal recessive inheritanceBrainCentral Nervous System
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Papers overview

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Highly Cited
2010
Highly Cited
2010
Neuronal synchrony reveals working memory networks and predicts individual memory capacity.
Visual working memory (VWM) is used to maintain sensory information for cognitive operations, and its deficits are associated… (More)
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Highly Cited
2008
Highly Cited
2008
Orienting attention in visual working memory reduces interference from memory probes.
Given a changing visual environment, and the limited capacity of visual working memory (VWM), the contents of VWM must be in… (More)
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Review
2006
Review
2006
Vanishing white matter disease
Vanishing white matter disease (VWM) is one of the most prevalent inherited childhood leucoencephalopathies. The classical… (More)
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Highly Cited
2006
Highly Cited
2006
Distinct capacity limits for attention and working memory: Evidence from attentive tracking and visual working memory paradigms.
A hallmark of both visual attention and working memory is their severe capacity limit: People can attentively track only about… (More)
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Highly Cited
2005
Highly Cited
2005
Visual working memory for simple and complex visual stimuli.
Does the magical number four characterize our visual working memory (VWM) capacity for all kinds of objects, or is the capacity… (More)
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Highly Cited
2002
Highly Cited
2002
Mutations in each of the five subunits of translation initiation factor eIF2B can cause leukoencephalopathy with vanishing white matter.
Leukoencephalopathy with vanishing white matter is a recently defined autosomal recessive disorder. The course is chronic… (More)
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Highly Cited
2001
Highly Cited
2001
Subunits of the translation initiation factor eIF2B are mutant in leukoencephalopathy with vanishing white matter
Leukoencephalopathy with vanishing white matter (VWM) is an inherited brain disease that occurs mainly in children. The course is… (More)
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Highly Cited
1999
Highly Cited
1999
Securing ad hoc networks
Ad hoc networks are a new wireless networking paradigm far mobile hosts. Unlike traditional mobile wireless networks, ad hoc… (More)
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Highly Cited
1998
Highly Cited
1998
The role of parietal cortex in verbal working memory.
Neuroimaging studies of normal subjects and studies of patients with focal lesions implicate regions of parietal cortex in verbal… (More)
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Highly Cited
1997
Highly Cited
1997
A new leukoencephalopathy with vanishing white matter.
We identified nine children with a leukoencephalopathy of similar type according to clinical and MRI findings. The patients… (More)
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