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A longitudinal investigation into cognition and disease progression in spinocerebellar ataxia types 1, 2, 3, 6, and 7

Overview of attention for article published in Orphanet Journal of Rare Diseases, June 2016
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  • Above-average Attention Score compared to outputs of the same age (58th percentile)
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4 tweeters

Citations

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25 Dimensions

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56 Mendeley
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Title
A longitudinal investigation into cognition and disease progression in spinocerebellar ataxia types 1, 2, 3, 6, and 7
Published in
Orphanet Journal of Rare Diseases, June 2016
DOI 10.1186/s13023-016-0447-6
Pubmed ID
Authors

Amy Moriarty, Arron Cook, Helen Hunt, Matthew E. Adams, Lisa Cipolotti, Paola Giunti

Abstract

The natural history of clinical symptoms in the spinocerebellar ataxias (SCA)s has been well characterised. However there is little longitudinal data comparing cognitive changes in the most common SCA subtypes over time. The present study provides a preliminary longitudinal characterisation of the clinical and cognitive profiles in patients with SCA1, SCA2, SCA3, SCA6 and SCA7, with the aim of elucidating the role of the cerebellum in cognition. 13 patients with different SCAs all caused by CAG repeat expansion (SCA1, n = 2; SCA2, n = 2; SCA3, n = 2; SCA6, n = 4; and SCA7, n = 3) completed a comprehensive battery of cognitive and mood assessments at two time points, a mean of 7.35 years apart. All patients were evaluated clinically using the Scale for the Rating and Assessment of Ataxia (SARA) and the Inventory of Non-Ataxia Signs (INAS). Patients underwent structural MRI imaging at follow-up. Clinical scale scores increased in all patients over time, most prominently in the SCA1 (SARA) and SCA3 (INAS) groups. New impairments on neuropsychological tests were most commonly observed with executive functions, speed, attention, visual memory and Theory of Mind. Results suggest possible differences in cognitive decline in SCA subtypes, with the most rapid cognitive decline observed in the SCA1 patients, and the least in the SCA6 patients, congruent with observed patterns of motor deterioration. Minimal changes in mood were observed, and MRI measures of atrophy did not correlate with cognitive decline. As well as increasing physical impairment, cognitive decline over time appears to be a distinct aspect of the SCA phenotype, in keeping with the cerebellar cognitive-affective syndrome. Our data suggest a trend of cognitive decline that is different for each SCA subtype, and for the majority is related to the severity of cerebellar motor impairment.

Twitter Demographics

The data shown below were collected from the profiles of 4 tweeters who shared this research output. Click here to find out more about how the information was compiled.

Mendeley readers

The data shown below were compiled from readership statistics for 56 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Spain 1 2%
Unknown 55 98%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 11 20%
Student > Bachelor 9 16%
Researcher 9 16%
Student > Master 6 11%
Student > Doctoral Student 4 7%
Other 8 14%
Unknown 9 16%
Readers by discipline Count As %
Medicine and Dentistry 15 27%
Agricultural and Biological Sciences 9 16%
Psychology 7 13%
Neuroscience 5 9%
Nursing and Health Professions 3 5%
Other 7 13%
Unknown 10 18%

Attention Score in Context

This research output has an Altmetric Attention Score of 2. 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 02 July 2016.
All research outputs
#3,395,650
of 7,976,486 outputs
Outputs from Orphanet Journal of Rare Diseases
#527
of 1,106 outputs
Outputs of similar age
#104,113
of 262,669 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#31
of 54 outputs
Altmetric has tracked 7,976,486 research outputs across all sources so far. This one has received more attention than most of these and is in the 55th percentile.
So far Altmetric has tracked 1,106 research outputs from this source. They receive a mean Attention Score of 4.8. This one is in the 49th percentile – i.e., 49% of its peers scored the same or lower than it.
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 262,669 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 58% of its contemporaries.
We're also able to compare this research output to 54 others from the same source and published within six weeks on either side of this one. This one is in the 37th percentile – i.e., 37% of its contemporaries scored the same or lower than it.