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Self-injurious behaviour and deliberate self-harm
Published in Tim Riding, Caron Swann, Bob Swann, Colin Dale, The Handbook of Forensic Learning Disabilities, 2021
Smith–Magenis syndrome is a rare condition that affects approximately 1 in 50 000 live births. It is associated with moderate learning disability and characteristic physical features. Some affected individuals show maladaptive behaviours in the form of hyperactivity, sleep disorders and autistic features. People with Smith–Magenis syndrome often show a degree of insensitivity to pain and may exhibit self-injurious behaviours, including head banging, hand biting, pulling out fingernails and toenails (onychotillomania) and insertion of foreign objects into various body orifices (poly-embolokilamania).
Smith-Magenis Syndrome—A Developmental Disorder with Circadian Dysfunction
Published in Merlin G. Butler, F. John Meaney, Genetics of Developmental Disabilities, 2019
Ann C.M. Smith, Wallace C. Duncan
Smith–Magenis syndrome is associated with a distinct craniofacial appearance as well as other physical, developmental, and behavioral features. Some features may be subtle or nonspecific while others are more characteristic, especially at different developmental stages (Table 1). The core constellation of features that is present within the syndrome is discussed below.
Functional communication training plus extinction is effective in reducing problem behavior maintained by environmental rearrangement1
Published in Evidence-Based Communication Assessment and Intervention, 2018
Russell Lang, Patricio Erhard, Toya Harmon, Katherine Ledbetter-Cho, Alyson Lee
Participants: One 12-year-old female with autism spectrum disorder (ASD) and Smith-Magenis syndrome participated. The Repetitive Behavior Scale-Revised (Bodfish, Symons, Parker, & Lewis, 2000) indicated the presence of stereotypic behavior with the highest number of items being endorsed on the Stereotypic Behavior Subscale and the Ritualistic/Sameness Subscale producing scores of 12 (7 of 9 items endorsed) and 11 (10 of 12 items), respectively. She used gestures and one-word to three-word utterances to communicate.
Clinical guidance on pharmacotherapy for the treatment of attention-deficit hyperactivity disorder (ADHD) for people with intellectual disability
Published in Expert Opinion on Pharmacotherapy, 2020
Jonjo Miller, Bhathika Perera, Rohit Shankar
Genetic disorders are common among people with ID. Certain genetic syndromes such as Downs’ syndrome, William’s syndrome, and Smith Magenis syndrome are strongly associated with ADHD. While the evidence is limited especially for specific disorders, it is likely that treating ADHD with MPH will show a positive response. Importantly the dosing needs to be slow and go low.
Patterns of comorbidity and psychopharmacology in adults with intellectual disability and attention deficit hyperactivity disorder: an UK national cross-sectional audit
Published in Expert Opinion on Pharmacotherapy, 2021
Bhathika Perera, John Chen, Laura Korb, Aditya Borakati, Ken Courtenay, William Henley, Sam Tromans, Rohit Shankar
Genetic disorder was found in 52 people (12%). More than 20 different genetic syndromes were represented in the group. The three most common were Down syndrome (trisomy 21), Smith-Magenis syndrome (chromosome 17p deletion), and Di George syndrome (chromosome 22q11.2 deletion).