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Selective cognitive and psychiatric manifestations in Wolfram Syndrome

Overview of attention for article published in Orphanet Journal of Rare Diseases, May 2015
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Mentioned by

2 tweeters
1 peer review site


14 Dimensions

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101 Mendeley
Selective cognitive and psychiatric manifestations in Wolfram Syndrome
Published in
Orphanet Journal of Rare Diseases, May 2015
DOI 10.1186/s13023-015-0282-1
Pubmed ID

Allison N. Bischoff, Angela M. Reiersen, Anna Buttlaire, Amal Al-lozi, Tasha Doty, Bess A. Marshall, Tamara Hershey


Wolfram Syndrome (WFS) is known to involve diabetes mellitus, diabetes insipidus, optic nerve atrophy, vision loss, hearing impairment, motor abnormalities, and neurodegeneration, but has been less clearly linked to cognitive, sleep, and psychiatric abnormalities. We sought to determine whether these abnormalities are present in children, adolescents, and young adults with WFS compared to age- and gender-matched individuals with and without type 1 diabetes using standardized measures. Individuals with genetically-confirmed WFS (n = 19, ages 7-27) were compared to age- and gender- equivalent groups of individuals with type 1 diabetes (T1DM; n = 25), and non-diabetic healthy controls (HC: n = 25). Cognitive performance across multiple domains (verbal intelligence, spatial reasoning, memory, attention, smell identification) was assessed using standardized tests. Standardized self- and parent-report questionnaires on psychiatric symptoms and sleep disturbances were acquired from all groups and an unstructured psychiatric interview was performed within only the WFS group. The three groups were similar demographically (age, gender, ethnicity, parental IQ). WFS and T1DM had similar duration of diabetes but T1DM had higher HbA1C levels than WFS and as expected both groups had higher levels than HC. The WFS group was impaired on smell identification and reported sleep quality, but was not impaired in any other cognitive or self-reported psychiatric domain. In fact, the WFS group performed better than the other two groups on selected memory and attention tasks. However, based upon a clinical evaluation of only WFS patients, we found that psychiatric and behavioral problems were present and consisted primarily of anxiety and hypersomnolence. This study found that cognitive performance and psychological health were relatively preserved WFS patients, while smell and sleep abnormalities manifested in many of the WFS patients. These findings contradict past case and retrospective reports indicating significant cognitive and psychiatric impairment in WFS. While many of these patients were diagnosed with anxiety and hypersomnolence, self-reported measures of psychiatric symptoms indicated that the symptoms were not of grave concern to the patients. It may be that cognitive and psychiatric issues become more prominent later in life and/or in later stages of the disease, but this requires standardized assessment and larger samples to determine. In the relatively early stages of WFS, smell and sleep-related symptoms may be useful biomarkers of disease and should be monitored longitudinally to determine if they are good markers of progression as well. Current Clinicaltrials.gov Trial NCT02455414 .

Twitter Demographics

The data shown below were collected from the profiles of 2 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 101 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Germany 1 <1%
Unknown 100 99%

Demographic breakdown

Readers by professional status Count As %
Student > Bachelor 19 19%
Student > Master 16 16%
Other 10 10%
Student > Doctoral Student 10 10%
Researcher 8 8%
Other 19 19%
Unknown 19 19%
Readers by discipline Count As %
Medicine and Dentistry 26 26%
Psychology 24 24%
Neuroscience 9 9%
Nursing and Health Professions 7 7%
Social Sciences 3 3%
Other 9 9%
Unknown 23 23%

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 22 December 2015.
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Outputs of similar age
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Outputs of similar age from Orphanet Journal of Rare Diseases
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Altmetric has tracked 6,810,016 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 992 research outputs from this source. They receive a mean Attention Score of 4.7. This one is in the 40th percentile – i.e., 40% of its peers scored the same or lower than it.
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We're also able to compare this research output to 48 others from the same source and published within six weeks on either side of this one. This one has gotten more attention than average, scoring higher than 50% of its contemporaries.