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Retinal function in patients with the neuronal ceroid lipofuscinosis phenotype

Overview of attention for article published in Arquivos Brasileiros de Oftalmologia, January 2017
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Title
Retinal function in patients with the neuronal ceroid lipofuscinosis phenotype
Published in
Arquivos Brasileiros de Oftalmologia, January 2017
DOI 10.5935/0004-2749.20170053
Pubmed ID
Authors

Elizabeth Maria Aparecida Barasnevicius Quagliato, Daniel Martins Rocha, Paula Yuri Sacai, Sung Song Watanabe, Solange Rios Salomão, Adriana Berezovsky

Abstract

To analyze the clinical features, visual acuity, and full-field electroretinogram (ERG) findings of 15 patients with the neuronal ceroid lipofuscinosis (NCL) phenotype and to establish the role of ERG testing in NCL diagnosis. The medical records of five patients with infantile NCL, five with Jansky-Bielschowsky disease, and five with juvenile NCL who underwent full-field ERG testing were retrospectively analyzed. Progressive vision loss was the initial symptom in 66.7% of patients and was isolated or associated with ataxia, epilepsy, and neurodevelopmental involution. Epilepsy was present in 93.3% of patients, of whom 86.6% presented with neurodevelopmental involution. Fundus findings ranged from normal to pigmentary/atrophic abnormalities. Cone-rod, rod-cone, and both types of dysfunction were observed in six, one, and eight patients, respectively. In our study, all patients with the NCL phenotype had abnormal ERG findings, and the majority exhibited both cone-rod and rod-cone dysfunction. We conclude that ERG is a valuable tool for the characterization of visual dysfunction in patients with the NCL phenotype and is useful for diagnosis.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 22 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 22 100%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 4 18%
Student > Doctoral Student 2 9%
Unspecified 2 9%
Student > Master 2 9%
Professor 1 5%
Other 3 14%
Unknown 8 36%
Readers by discipline Count As %
Medicine and Dentistry 4 18%
Biochemistry, Genetics and Molecular Biology 3 14%
Unspecified 2 9%
Neuroscience 2 9%
Agricultural and Biological Sciences 1 5%
Other 2 9%
Unknown 8 36%
Attention Score in Context

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 01 October 2017.
All research outputs
#17,292,294
of 25,382,440 outputs
Outputs from Arquivos Brasileiros de Oftalmologia
#154
of 446 outputs
Outputs of similar age
#267,895
of 421,709 outputs
Outputs of similar age from Arquivos Brasileiros de Oftalmologia
#3
of 15 outputs
Altmetric has tracked 25,382,440 research outputs across all sources so far. This one is in the 21st percentile – i.e., 21% of other outputs scored the same or lower than it.
So far Altmetric has tracked 446 research outputs from this source. They receive a mean Attention Score of 2.5. This one is in the 47th percentile – i.e., 47% 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 421,709 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 27th percentile – i.e., 27% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 15 others from the same source and published within six weeks on either side of this one. This one is in the 46th percentile – i.e., 46% of its contemporaries scored the same or lower than it.