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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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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.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 102 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Spain 1 <1%
Unknown 101 99%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 21 21%
Researcher 12 12%
Student > Bachelor 12 12%
Student > Postgraduate 8 8%
Student > Master 8 8%
Other 13 13%
Unknown 28 27%
Readers by discipline Count As %
Medicine and Dentistry 21 21%
Neuroscience 14 14%
Agricultural and Biological Sciences 10 10%
Psychology 8 8%
Nursing and Health Professions 5 5%
Other 13 13%
Unknown 31 30%
Attention Score in Context

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
#13,474,769
of 22,879,161 outputs
Outputs from Orphanet Journal of Rare Diseases
#1,397
of 2,627 outputs
Outputs of similar age
#186,887
of 352,770 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#20
of 29 outputs
Altmetric has tracked 22,879,161 research outputs across all sources so far. This one is in the 39th percentile – i.e., 39% of other outputs scored the same or lower than it.
So far Altmetric has tracked 2,627 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 7.5. This one is in the 43rd percentile – i.e., 43% 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 352,770 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 45th percentile – i.e., 45% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 29 others from the same source and published within six weeks on either side of this one. This one is in the 24th percentile – i.e., 24% of its contemporaries scored the same or lower than it.