Objective: To assess the frequency and severity of specific cognitive deficits in children with neurofibromatosis type 1 (NF1) in a large unbiased cohort. Methods: Extensive cognitive assessments were performed in 81 ...Objective: To assess the frequency and severity of specific cognitive deficits in children with neurofibromatosis type 1 (NF1) in a large unbiased cohort. Methods: Extensive cognitive assessments were performed in 81 children with NF1 ages 8 to 16 years and their performance was compared with that of 49 unaffected sibling controls. Results: Eighty-one percent of the children with NF1 had moderate to severe impairment in one or more areas of cognitive functioning. Although 51%of children with NF1 performed poorly on tasks of reading, spelling, and mathematics, specific learning disabilities (as defined by IQ achievement discrepancies) were present in only 20%of the children. Sustained attention difficulties were present in 63%of children with NF1, with 38%of children with NF1 fulfilling the diagnostic criteria for attention deficit-hyperact- ivity disorder. The NF1 neuropsychological profile is characterized by deficits in perceptual skills (visuospatial and visuoperceptual), executive functioning (planning and abstract concept formation), and attention (sustained and switching). Interestingly, both verbal and visual memory was unaffected in NF1 children, and their memory skills were in general stronger than their level of general intellectual function. Although both expressive and receptive language skills were significantly impaired in NF1 children, they appeared to be relatively better preserved than visuospatial abilities once IQ is taken into account. Conclusion: There is an extremely high frequency of cognitive problems in children with neurofibromatosis type 1, making cognitive dysfunction the most common complication to affect quality of life in these children.展开更多
Reported is the change in cognitive function after neuronal cell transplantati on as a treatment for basal ganglia stroke. Nine subjects (two controls, seven t ransplants), all over 2 years post stroke, completed a co...Reported is the change in cognitive function after neuronal cell transplantati on as a treatment for basal ganglia stroke. Nine subjects (two controls, seven t ransplants), all over 2 years post stroke, completed a comprehensive neuropsycho logical test battery prior to and 6 months after treatment. Four transplanted su bjects who had strokes in the nondominant hemisphere showed marked improvement o n the Rey Complex Figure, a test of visuospatial/constructional ability and nonv erbal memory.展开更多
Objective: To develop a quantitative method of assessing cognitive anosognosia in six cognitive and two noncognitive domains.Methods: Control (n =32) and prob able Alzheimer disease(pAD) (n = 14) subjects self-estimat...Objective: To develop a quantitative method of assessing cognitive anosognosia in six cognitive and two noncognitive domains.Methods: Control (n =32) and prob able Alzheimer disease(pAD) (n = 14) subjects self-estimated memory, attention, generative behavior, naming, visuospatial skill, limb praxis, mood,and uncorrec ted vision, both before and after these abilities were assessed. Based on this e stimate and their performance the authors calculated an anosognosia ratio (AR) b y dividing the difference between estimated and actual performance by an estimat ed and actual performance sum. With perfect awareness,AR = 0. Overestimating abi lities would yield a positive AR (≤1); underestimation would yield a negative A R (≥-1). Results: Relative to controls, pAD subjects demonstrated anosognosia. Pre-testing (off-line), pAD subjects overestimated their visuospatial skill; p ost-testing (on-line), pAD subjects overestimated their memory. Control subjec ts also made selfrating errors, underestimating their attention pre-testing and overestimating limb praxis and vision post-testing. Conclusions:This anosognos ia assessment method may allow more detailed examination of distorted self-awar eness. These results suggest that screening for anosognosia in probable Alzheime r disease (pAD) should include self-estimates of visuospatial function, and tha t, in pAD, it may be useful to assess anosognosia for amnesia both before and af ter memory testing.展开更多
文摘Objective: To assess the frequency and severity of specific cognitive deficits in children with neurofibromatosis type 1 (NF1) in a large unbiased cohort. Methods: Extensive cognitive assessments were performed in 81 children with NF1 ages 8 to 16 years and their performance was compared with that of 49 unaffected sibling controls. Results: Eighty-one percent of the children with NF1 had moderate to severe impairment in one or more areas of cognitive functioning. Although 51%of children with NF1 performed poorly on tasks of reading, spelling, and mathematics, specific learning disabilities (as defined by IQ achievement discrepancies) were present in only 20%of the children. Sustained attention difficulties were present in 63%of children with NF1, with 38%of children with NF1 fulfilling the diagnostic criteria for attention deficit-hyperact- ivity disorder. The NF1 neuropsychological profile is characterized by deficits in perceptual skills (visuospatial and visuoperceptual), executive functioning (planning and abstract concept formation), and attention (sustained and switching). Interestingly, both verbal and visual memory was unaffected in NF1 children, and their memory skills were in general stronger than their level of general intellectual function. Although both expressive and receptive language skills were significantly impaired in NF1 children, they appeared to be relatively better preserved than visuospatial abilities once IQ is taken into account. Conclusion: There is an extremely high frequency of cognitive problems in children with neurofibromatosis type 1, making cognitive dysfunction the most common complication to affect quality of life in these children.
文摘Reported is the change in cognitive function after neuronal cell transplantati on as a treatment for basal ganglia stroke. Nine subjects (two controls, seven t ransplants), all over 2 years post stroke, completed a comprehensive neuropsycho logical test battery prior to and 6 months after treatment. Four transplanted su bjects who had strokes in the nondominant hemisphere showed marked improvement o n the Rey Complex Figure, a test of visuospatial/constructional ability and nonv erbal memory.
文摘Objective: To develop a quantitative method of assessing cognitive anosognosia in six cognitive and two noncognitive domains.Methods: Control (n =32) and prob able Alzheimer disease(pAD) (n = 14) subjects self-estimated memory, attention, generative behavior, naming, visuospatial skill, limb praxis, mood,and uncorrec ted vision, both before and after these abilities were assessed. Based on this e stimate and their performance the authors calculated an anosognosia ratio (AR) b y dividing the difference between estimated and actual performance by an estimat ed and actual performance sum. With perfect awareness,AR = 0. Overestimating abi lities would yield a positive AR (≤1); underestimation would yield a negative A R (≥-1). Results: Relative to controls, pAD subjects demonstrated anosognosia. Pre-testing (off-line), pAD subjects overestimated their visuospatial skill; p ost-testing (on-line), pAD subjects overestimated their memory. Control subjec ts also made selfrating errors, underestimating their attention pre-testing and overestimating limb praxis and vision post-testing. Conclusions:This anosognos ia assessment method may allow more detailed examination of distorted self-awar eness. These results suggest that screening for anosognosia in probable Alzheime r disease (pAD) should include self-estimates of visuospatial function, and tha t, in pAD, it may be useful to assess anosognosia for amnesia both before and af ter memory testing.
