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Patterns of motor signs in spinocerebellar ataxia type 3 at the start of follow-up in a reference unit

Overview of attention for article published in Cerebellum & Ataxias, February 2016
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Patterns of motor signs in spinocerebellar ataxia type 3 at the start of follow-up in a reference unit
Published in
Cerebellum & Ataxias, February 2016
DOI 10.1186/s40673-016-0042-6
Pubmed ID

Irene Pulido-Valdeolivas, David Gómez-Andrés, Irene Sanz-Gallego, Estrella Rausell, Javier Arpa


Spinocerebellar ataxia type 3 (SCA3) is a neurodegenerative disorder that affects the cerebellar system and other subcortical regions of the brain. As for other cerebellar diseases, the severity of this type of ataxia can be assessed with the Scale for Assessment and Rating of Ataxia (SARA) which gives a total score that reflects functional impairment out of 8 cerebellar function tests. SCA3 patients score profile is heterogeneous on at the start of follow up. This study investigates possible patterns in those profiles and analyses the impact of other usually concurrent signs of impairment of extracerebellar motor systems in that profile variability by means of multivariate statistical approaches. Seventeen patients with SCA3 underwent systematic anamnesis, neurological and SARA assessment, visual evaluation of (123)I-Ioflupane (DaTSCAN) single-photon emission computed tomography (SPECT) imaging and electrophysiological studies (nerve conduction and electromyography). Patterns in the profiles of SARA item scores were investigated by hierarchical clustering after multivariate correspondence analysis. A network analysis was used to represent relationships between SARA item scores, clinical, genetic and neurological examination parameters as well as abnormalities of DaTSCAN SPECT imaging and electrophysiological studies. The most frequently altered SARA items in all patients are gait and stance, and three profiles of SCA3 patients can be distinguished depending mainly on their degree of impairment in those two items. Other SARA items like the score on heel-shin slide contribute less to the classification. Network analysis shows that SARA item scores configure a single domain that is independent of the size of the mutated expanded allele and age of onset, which are, in turn closely and inversely correlated. The severity of cerebellar dysfunction is correlated with longer disease duration, altered visual evaluation of DaTSCAN SPECT imaging and decreased patellar reflexes. Neither the presence of pyramidal or extrapyramidal signs nor the intensity of polyneuropathy is correlated with the SARA items scores. Pattern recognition approaches are useful tools to describe clinical phenotypes of ataxias and to identify particular configurations of cerebellar signs.

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Mendeley readers

The data shown below were compiled from readership statistics for 16 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 16 100%

Demographic breakdown

Readers by professional status Count As %
Student > Bachelor 2 13%
Other 2 13%
Student > Master 2 13%
Student > Ph. D. Student 2 13%
Librarian 1 6%
Other 3 19%
Unknown 4 25%
Readers by discipline Count As %
Medicine and Dentistry 4 25%
Nursing and Health Professions 2 13%
Psychology 1 6%
Biochemistry, Genetics and Molecular Biology 1 6%
Neuroscience 1 6%
Other 1 6%
Unknown 6 38%

Attention Score in Context

This research output has an Altmetric Attention Score of 1. 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 24 February 2016.
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