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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 has been identified worldwide among diverse ethnic groups. The prevalence is estimated to be 1/25,000 births (4), a likely underestimate since the syndrome remains vastly under diagnosed. Since initial description, more than 100 individuals with SMS have been described in the literature (23), and approximately 600 are registered with the international parent support group, parents and researchers interested in Smith-Magenis syndrome (PRISMS) (www.smithmagenis.org). Among individuals with mental retardation, the frequency of SMS (del 17p11.2) is estimated at approximately 1/600 (24–26).
Lesch–Nyhan disease and variants
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop
The differential diagnosis of self-injury of this disease includes the De Lange syndrome and dysautonomia. Self-injurious behavior has also been observed in as many as 58 percent of boys with fragile X syndrome [16]. The behavior consisted predominately of biting fingers or the dorsum of the hand or hitting the head. Information on severity, for example loss of tissue or amputations, was not provided. In patients with Prader-Willi syndrome [17, 18], the behavior consisted of picking the skin over an extensive body area. In Smith-Magenis syndrome, self-injurious behavior was described [19] as near universal. Hand biting was the most common behavior in these patients too, but they also manifested some unusual behaviors, including: picking at fingers or toenails until they bled, onychotillomania, and even complete removal of the nail, and polyembolokoilamania (the inserting of foreign objects into orifices of the body). It is thought that in this disorder there may be peripheral neuropathy. The behavioral phenotype of Smith-Magenis syndrome also includes extreme disturbance of sleep and a “self-hugging” stereotype. The differential diagnosis does not really include sensory neuropathy and indifference to pain. Those patients tend to look like pugilists and the injuries are accidental. Lesch–Nyhan patients do not have sensory abnormalities; they scream in pain when they bite themselves and cry in terror of its anticipation. As patients become older, they learn to become aggressive with speech. Four-letter Anglo-Saxon expressions are common. Males appear to have a considerable interest in the opposite sex. This sometimes leads to inappropriate groping; more often it leads to frustration.
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).