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A cross-sectional controlled developmental study of neuropsychological functions in patients with glutaric aciduria type I

Overview of attention for article published in Orphanet Journal of Rare Diseases, December 2015
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  • Above-average Attention Score compared to outputs of the same age and source (56th percentile)

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Title
A cross-sectional controlled developmental study of neuropsychological functions in patients with glutaric aciduria type I
Published in
Orphanet Journal of Rare Diseases, December 2015
DOI 10.1186/s13023-015-0379-6
Pubmed ID
Authors

Nikolas Boy, Jana Heringer, Gisela Haege, Esther M. Glahn, Georg F. Hoffmann, Sven F. Garbade, Stefan Kölker, Peter Burgard

Abstract

Glutaric aciduria type I (GA-I) is an inherited metabolic disease due to deficiency of glutaryl-CoA dehydrogenase (GCDH). Cognitive functions are generally thought to be spared, but have not yet been studied in detail. Thirty patients detected by newborn screening (n = 13), high-risk screening (n = 3) or targeted metabolic testing (n = 14) were studied for simple reaction time (SRT), continuous performance (CP), visual working memory (VWM), visual-motor coordination (Tracking) and visual search (VS). Dystonia (n = 13 patients) was categorized using the Barry-Albright-Dystonia Scale (BADS). Patients were compared with 196 healthy controls. Developmental functions of cognitive performances were analysed using a negative exponential function model. BADS scores correlated with speed tests but not with tests measuring stability or higher cognitive functions without time constraints. Developmental functions of GA-I patients significantly differed from controls for SRT and VS but not for VWM and showed obvious trends for CP and Tracking. Dystonic patients were slower in SRT and CP but reached their asymptote of performance similar to asymptomatic patients and controls in all tests. Asymptomatic patients did not differ from controls, except showing significantly better results in Tracking and a trend for slower reactions in visual search. Data across all age groups of patients and controls fitted well to a model of negative exponential development. Dystonic patients predominantly showed motor speed impairment, whereas performance improved with higher cognitive load. Patients without motor symptoms did not differ from controls. Developmental functions of cognitive performances were similar in patients and controls. Performance in tests with higher cognitive demand might be preserved in GA-I, even in patients with striatal degeneration.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 46 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 46 100%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 11 24%
Student > Master 9 20%
Student > Doctoral Student 5 11%
Student > Bachelor 5 11%
Other 3 7%
Other 7 15%
Unknown 6 13%
Readers by discipline Count As %
Psychology 10 22%
Medicine and Dentistry 7 15%
Nursing and Health Professions 7 15%
Neuroscience 6 13%
Agricultural and Biological Sciences 3 7%
Other 5 11%
Unknown 8 17%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 4. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 01 August 2016.
All research outputs
#7,932,336
of 25,371,292 outputs
Outputs from Orphanet Journal of Rare Diseases
#1,129
of 3,079 outputs
Outputs of similar age
#116,808
of 403,753 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#28
of 69 outputs
Altmetric has tracked 25,371,292 research outputs across all sources so far. This one has received more attention than most of these and is in the 67th percentile.
So far Altmetric has tracked 3,079 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 8.2. This one has gotten more attention than average, scoring higher than 61% of its peers.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 403,753 tracked outputs that were published within six weeks on either side of this one in any source. This one has gotten more attention than average, scoring higher than 70% of its contemporaries.
We're also able to compare this research output to 69 others from the same source and published within six weeks on either side of this one. This one has gotten more attention than average, scoring higher than 56% of its contemporaries.