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Mendeley readers
Attention Score in Context
| Title |
Evolution of structural neuroimaging biomarkers in a series of adult patients with Niemann-Pick type C under treatment
|
|---|---|
| Published in |
Orphanet Journal of Rare Diseases, February 2017
|
| DOI | 10.1186/s13023-017-0579-3 |
| Pubmed ID | |
| Authors |
Marion Masingue, Isaac Adanyeguh, Yann Nadjar, Frédéric Sedel, Damien Galanaud, Fanny Mochel |
| Abstract |
Niemann-Pick type C (NPC) disease is a lysosomal storage disorder characterized by a wide clinical spectrum and non-specific conventional magnetic resonance imaging (MRI) signs. As substrate reduction therapy with miglustat is now used in almost all patients, its efficacy and the course of the disease are sometimes difficult to evaluate. Neuroimaging biomarkers could prove useful in this matter. We first performed a retrospective analysis of volumetric and diffusion tensor imaging (DTI) data on 13 adult NPC patients compared to 13 controls of similar age and sex. Eleven NPC patients were then studied using the same neuroimaging modalities over a mean of 5 years. The NPC composite score was used to evaluate disease severity. NPC patients showed atrophy in basal ganglia - pallidum (p = 0.029), caudate nucleus (p = 0.022), putamen (p = 0.002) and thalamus (p < 0.001) - cerebral peduncles (p = 0.003) and corpus callosum (p = 0.006), compared to controls. NPC patients also displayed decreased fractional anisotropy (FA) in several regions of interest - corona radiata (p = 0.015), internal capsule (p = 0.007), corpus callosum (p = 0.032) and cingulate gyrus (p = 0.002) - as well as a broad increase in radial diffusivity (p < 0.001), compared to controls. Over time, 3 patients worsened clinically, including 2 patients who interrupted treatment, while 8 patients remained stable. With miglustat, no significant volumetric change was observed but FA improved after 2 years in the corpus callosum and the corona radiata of NPC patients (n = 4; p = 0.029) - although that was no longer observed at further time points. This is the first study conducted on a series of adult NPC patients using two neuroimaging modalities and followed under treatment. It confirmed that NPC patients displayed cerebral atrophy in several regions of interest compared to controls. Furthermore, miglustat showed an early effect on diffusion metrics in treated patients. DTI can detect brain microstructure alterations caused by neurometabolic dysfunction. Its potential as a biomarker in NPC shall be further evaluated in upcoming therapeutic trials. |
Mendeley readers
The data shown below were compiled from readership statistics for 44 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 44 | 100% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Researcher | 7 | 16% |
| Other | 6 | 14% |
| Student > Ph. D. Student | 6 | 14% |
| Student > Postgraduate | 5 | 11% |
| Student > Bachelor | 4 | 9% |
| Other | 4 | 9% |
| Unknown | 12 | 27% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 17 | 39% |
| Neuroscience | 5 | 11% |
| Biochemistry, Genetics and Molecular Biology | 3 | 7% |
| Nursing and Health Professions | 1 | 2% |
| Pharmacology, Toxicology and Pharmaceutical Science | 1 | 2% |
| Other | 2 | 5% |
| Unknown | 15 | 34% |
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 13 February 2018.
All research outputs
#15,492,327
of 23,023,224 outputs
Outputs from Orphanet Journal of Rare Diseases
#1,818
of 2,640 outputs
Outputs of similar age
#257,114
of 420,603 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#37
of 55 outputs
Altmetric has tracked 23,023,224 research outputs across all sources so far. This one is in the 22nd percentile – i.e., 22% of other outputs scored the same or lower than it.
So far Altmetric has tracked 2,640 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 7.6. This one is in the 22nd percentile – i.e., 22% 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 420,603 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 30th percentile – i.e., 30% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 55 others from the same source and published within six weeks on either side of this one. This one is in the 29th percentile – i.e., 29% of its contemporaries scored the same or lower than it.