Comparing Rates of Psychiatric and Behavior Disorders in Adolescents and Young Adults with Severe Intellectual Disability with and without Autism

Cerebral creatine deficiency disorders that result in very low levels of creatine in the brain, can cause in intellectual disability, seizures, expressive speech disorder and behavior disorders if not treated in early childhood. CCDDs comprise disorders of creatine synthesis (arginine:glycine [AGAT; MIM 602360]; guanidinoacetate methyltransferase deficiency [GAMT; MIM 601240]) and of creatine transport (SLC6A8 deficiency [SLC6A8; MIM 300036]). Inborn errors of creatine synthesis-but not, as yet, of transport-can be treated by creatine substitution and are thus treatable causes of intellectual disability.

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Association of Autism Onset, Epilepsy, and Behavior in a Community of Adults with Autism and Severe Intellectual Disability

Brain Sciences ◽

10.3390/brainsci10080486 ◽

2020 ◽

Vol 10 (8) ◽

pp. 486

Author(s):

Stefano Damiani ◽

Pietro Leali ◽

Guido Nosari ◽

Monica Caviglia ◽

Mariangela V. Puci ◽

...

Keyword(s):

Intellectual Disability ◽

Autism Spectrum ◽

P Value ◽

Adults With Autism ◽

Behavioral Profiles ◽

Severe Intellectual Disability ◽

Spectrum Disorders ◽

Farm Community ◽

Clinical Conditions ◽

And Behavior

Autism spectrum disorders (ASDs) are hard to characterize due to their clinical heterogeneity. Whether epilepsy and other highly prevalent comorbidities may be related to specific subphenotypes such as regressive ASD (i.e., the onset of symptoms after a period of apparently typical development) is controversial and yet to be determined. Such discrepancies may be related to the fact that age, level of cognitive functioning, and environmental variables are often not taken into account. We considered a sample of 20 subjects (i) between 20 and 55 years of age, (ii) with severe/profound intellectual disability, (iii) living in the same rural context of a farm community. As a primary aim, we tested for the association between epilepsy and regressive ASD. Secondly, we explored differences in behavioral and pharmacological profiles related to the presence of each of these conditions, as worse behavioral profiles have been separately associated with both epilepsy and regressive ASD in previous studies. An initial trend was observed for associations between the presence of epilepsy and regressive ASD (odds ratio: 5.33; 95% CI: 0.62–45.41, p-value: 0.086). Secondly, subjects with either regressive ASD or epilepsy showed worse behavioral profiles (despite the higher pharmacotherapy they received). These preliminary results, which need to be further confirmed, suggest the presence of specific associations of different clinical conditions in subjects with rarely investigated phenotypes.

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A de novo 10p11.23-p12.1 deletion recapitulates the phenotype observed inWACmutations and strengthens the role ofWACin intellectual disability and behavior disorders

American Journal of Medical Genetics Part A ◽

10.1002/ajmg.a.37686 ◽

2016 ◽

Vol 170 (7) ◽

pp. 1912-1917 ◽

Cited By ~ 2

Author(s):

Fatma Abdelhedi ◽

Laila El Khattabi ◽

Nouha Essid ◽

Geraldine Viot ◽

Dominique Letessier ◽

...

Keyword(s):

Intellectual Disability ◽

Behavior Disorders ◽

De Novo ◽

And Behavior

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Transition Satisfaction and Family Well Being Among Parents of Young Adults With Severe Intellectual Disability

Intellectual and Developmental Disabilities ◽

10.1352/2009.47:31-43 ◽

2009 ◽

Vol 47 (1) ◽

pp. 31-43 ◽

Cited By ~ 51

Author(s):

Cameron L. Neece ◽

Bonnie R. Kraemer ◽

Jan Blacher

Keyword(s):

Young Adults ◽

Intellectual Disability ◽

Family System ◽

Transition Period ◽

Life Stage ◽

Sudden Change ◽

Well Being ◽

Future Research ◽

Environmental Characteristics ◽

Severe Intellectual Disability

Abstract The transition from high school to adulthood is a critical life stage that entails many changes, especially for youth with severe intellectual disability. The transition period may be especially stressful for the families of these young adults, who often experience a sudden change, or decrease, in services. However, little research has examined what constitutes a successful transition for the families of these individuals. The present study examined parent perspectives of transition for 128 young adults with severe intellectual disability, specifically, parent satisfaction with transition. Results suggested that transition satisfaction is related to young adult, family, and environmental characteristics, with environmental characteristics being the strongest predictors of transition satisfaction. Furthermore, transition satisfaction is related to multiple measures of family well being, indicating the tremendous need for considering the broader family system when planning for a young adult's transition. Implications and directions for future research are discussed.

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Ethical Challenges Related to Participation of Adolescents and Young Adults with Intellectual Disability in Research

Ethics and Research with Children ◽

10.1093/med-psych/9780190647254.003.0002 ◽

2018 ◽

pp. 7-21

Author(s):

Pamela G. Nathanson ◽

Theodore E. Schall ◽

Chris Feudtner

Keyword(s):

Young Adults ◽

Intellectual Disability ◽

Research Participant ◽

Adolescents And Young Adults ◽

Institutional Review Boards ◽

Ethical Challenges ◽

Individual Level ◽

Institutional Review ◽

Study Teams ◽

Illustrative Case Study

This chapter lays out the ethical challenges and proactive strategies involved in balancing the rights of adolescents and young adults (AYAs) with intellectual disability (ID) to participate in clinical research with the obligation to protect a potentially vulnerable population from research harms. The chapter presents an illustrative case study involving a young adult research participant with Down syndrome whose capacity and legal authority to consent to participate in a study was questioned after an exchange with a staff member aroused suspicion about potential misconduct. Techniques to enhance participation, especially in the consent or assent processes, of AYAs with ID in research are outlined. Both individual-level and structural-level protections to ensure ongoing voluntary participation, relevant for both study teams and institutional review boards, are recommended.

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Both high and low testosterone levels may play a role in suicidal behavior in adolescent, young, middle-age, and older men: a hypothesis

International Journal of Adolescent Medicine and Health ◽

10.1515/ijamh-2016-0032 ◽

2016 ◽

Vol 30 (2) ◽

Cited By ~ 1

Author(s):

Leo Sher

Keyword(s):

Young Adults ◽

Suicidal Behavior ◽

Middle Age ◽

Older Men ◽

Adolescents And Young Adults ◽

Middle Aged ◽

Testosterone Levels ◽

Low Testosterone ◽

And Behavior ◽

The Relationship

Abstract Many studies suggest that testosterone plays a role in the regulation of mood and behavior. Most but not all investigations of the relationship between testosterone and suicidality found relations between testosterone and suicidal behavior. The balance of evidence is in favor of the view that testosterone is involved in the pathophysiology of suicidality. The author has previously proposed that suicidal behavior in adolescents and young adults is associated with high testosterone levels, whereas suicidality in older men is associated with decreased testosterone levels. However, both high and low testosterone levels can be observed in adolescent, young, middle-aged and older men. In this article, the author proposes that both high and low testosterone levels may play a role in suicidal behavior in men of any age.

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