Gender Differences in the Behavioral Symptom Severity of Prader-Willi Syndrome

Gito, Masao; Ihara, Hiroshi; Ogata, Hiroyuki; Sayama, Masayuki; Murakami, Nobuyuki; Nagai, Toshiro; Ayabe, Tadayuki; Oto, Yuji; Saito, Kazutaka

doi:10.1155/2015/294127

Cited by 14 publications

(7 citation statements)

References 55 publications

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“…Girls scored higher than boys for food-related obsessive speech and compulsive behaviors including hiding or hoarding. At least one other study has found that food-related behaviors differ based on both sex and genetic subtype of PWS, with less severe behavior reported in males within the UPD subtype [37]. More research studies focused on potential sex-based differences in behavioral phenotypes of PWS are needed.…”

Section: Discussionmentioning

confidence: 99%

Food and Non-Food-Related Behavior across Settings in Children with Prader–Willi Syndrome

Gantz¹,

Andrews²,

Wheeler³

2020

Genes

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This study sought to describe food- and non-food-related behaviors of children aged 3 to 18 years with Prader–Willi syndrome (PWS) in home and school settings, as assessed by 86 parents and 63 teachers using 7 subscales of the Global Assessment of Individual’s Behavior (GAIB). General Behavior Problem, Non-Food-Related Behavior Problem, and Non-Food-Related Obsessive Speech and Compulsive Behavior (OS/CB) scores did not differ significantly between parent and teacher reports. Food-Related Behavior Problem scores were higher in parent versus teacher reports when the mother had less than a college education (difference of 13.6 points, 95% Confidence Interval (CI) 5.1 to 22). Parents assigned higher Food-Related OS/CB scores than teachers (difference of 5.7 points, 95% CI 2.4 to 9.0). Although teachers reported fewer Food-Related OS/CB, they scored overall OS/CB higher for interfering with daily activities compared with parents (difference of 0.9 points, 95% CI 0.4 to 1.4). Understanding how behaviors manifest in home and school settings, and how they vary with socio-demographic and patient characteristics can help inform strategies to reduce behavior problems and improve outcomes.

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Section: Discussionmentioning

confidence: 99%

Food and Non-Food-Related Behavior across Settings in Children with Prader–Willi Syndrome

Gantz¹,

Andrews²,

Wheeler³

2020

Genes

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“…This may have been due to a sampling bias as we had more female than male PWS subjects. However, there is some evidence that rates of behavioral abnormalities in PWS are similar between genders, which may explain the difference in expected ASD gender distribution in PWS compared to the DS or ASD alone group (Gito et al, ; Sinnema et al, ). One study showed similar scores on the Developmental Behavior Checklists for adults between genders in PWS for total and subscale scores but did note higher scores for females on questions regarding irritability and becoming upset over small changes in routine or environment (Jauregi, Laurier, Copet, Tauber, & Thuilleaux, ).…”

Section: Discussionmentioning

confidence: 99%

Comparison of Aberrant Behavior Checklist profiles across Prader–Willi syndrome, Down syndrome, and autism spectrum disorder

Salehi

Herzig

Capone

et al. 2018

American J of Med Genetics Pt A

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Prader-Willi syndrome (PWS, OMIM # 176270) and Down syndrome (DS, OMIM #190685) are neurodevelopmental genetic disorders with higher rates of autism spectrum disorder (ASD). The Aberrant Behavior Checklist (ABC) is a caregiver rating scale that assesses maladaptive behaviors.Overlapping symptoms exist between PWS, DS, and ASD, including maladaptive behaviors. We aimed to evaluate ABC profiles between PWS, DS, and ASD alone (without known genetic syndrome). In addition, we hypothesized PWS and DS with a comorbid ASD positive screen or diagnosis would have similar ABC profiles to ASD alone. ABC data from the following cohorts were analyzed: PWS (Seattle Children's Hospital, n = 28, mean age = 12.8 AE 4.9 years; University of Florida, n = 35, mean age = 9.3 AE 7.1 years), DS (Johns Hopkins, n = 406, mean age = 8.1 AE 2.4 years), and ASD (University of Florida, n = 102, mean age = 10.8 AE 3.5 years). ASD alone had significantly higher ABC scores. Subgroups of PWS and DS with a comorbid ASD positive screen or diagnosis had similarities in scores with the ASD only group, with subscale patterns unique to each syndrome. The ABC indicated worse maladaptive behaviors in children with ASD, including those with genetic syndromes. Although more studies are needed to evaluate the utility and the accuracy of the ABC as a tool to screen for ASD in special populations, it may be a useful adjunct in screening those children with PWS or DS who need more in depth ASD evaluation. K E Y W O R D S aberrant behavior checklist, autism, autism spectrum disorder, Down syndrome, maladaptive behavior, Prader-Willi syndrome

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“…In stark contrast to male Cyfip1 J/mice with the maternal deletion, female Cyfip1 J/mice ( Fig.3F,G) with the paternal Cyfip1 J/deletion showed a markedly enhanced, initial consumption of PF that obfuscated detection of escalation over time (Fig.4H,I). In considering sex differences in PWS, male PWS patients with 15q11-q13 deletions showed greater food-related preoccupation with food, impaired satiety, and other food-related negative behaviors in the absence of differences in OC or other non-food-related behaviors 70 . It is unclear whether sex differences in food-related problems differ depending on Type I versus Type II PWS deletions.…”

Section: Effect Of Cyfip1 Deletion On Pf Intake Depends On Genetic Bamentioning

confidence: 99%

Cyfip1 haploinsufficiency increases compulsive-like behavior and modulates palatable food intake: Implications for Prader-Willi Syndrome

Babbs

Ruan

Kelliher

et al. 2018

Preprint

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Binge eating (BE) is a heritable trait associated with eating disorders and involves consumption of a large quantity of food in a short time. We identified cytoplasmic FMRP-interacting protein 2 (Cyfip2) as a major genetic factor underlying BE and concomitant compulsive-like behaviors in mice. CYFIP2 is a gene homolog of CYFIP1 -one of four paternally deleted genes in patients with the more severe Type I Prader-Willi Syndrome (PWS). PWS is a neurodevelopmental genetic disorder where 70% of cases involve paternal deletion of 15q11-q13. PWS is defined phenotypically by the emergence of hyperphagia and obesity during childhood as well as cognitive deficits and obsessive-compulsive behaviors. We tested the hypothesis that Cyfip1 haploinsufficiency would enhance premorbid compulsive-like behavior and palatable food (PF) consumption in a parent-of-origin-selective manner.Additionally, because our initial studies involved mice on a C57BL/6N background that possess the BEassociated missense mutation in Cyfip2, we tested mice on a mixed background where mice were homozygous for the C57BL/6J (B6J) allele at the Cyfip2 locus. Cyfip1 haploinsufficiency increased compulsive-like behavior on both backgrounds and PF consumption was greater with paternal inheritance. Gene expression in the hypothalamus revealed a paternal effect of Cyfip1 deletion on transcription of Cyfip1 but not Cyfip2 or Magel2. To summarize, the selective increased compulsive-like behavior and PF consumption in paternally deleted Cyfip1 +/-mice could translate to enhancing hyperphagia in a subset of individuals with neurodevelopmental disorders involving reduced expression or haploinsufficiency of CYFIP1, including PWS but also Fragile X Syndrome and 15q11.2 Microdeletion Syndrome.

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Gender Differences in the Behavioral Symptom Severity of Prader-Willi Syndrome

Cited by 14 publications

References 55 publications

Food and Non-Food-Related Behavior across Settings in Children with Prader–Willi Syndrome

Food and Non-Food-Related Behavior across Settings in Children with Prader–Willi Syndrome

Comparison of Aberrant Behavior Checklist profiles across Prader–Willi syndrome, Down syndrome, and autism spectrum disorder

Cyfip1 haploinsufficiency increases compulsive-like behavior and modulates palatable food intake: Implications for Prader-Willi Syndrome

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