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Hepatorenal tyrosinemia/fumarylacetoacetate hydrolase deficiency
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
Neurologic crises of pain and paresthesia are a result of peripheral neuropathy [42–45]. These may occur in as many as 42 percent of patients. Crises may be mistaken for porphyria [43]. There may be extensor hypotonus or the patient may have hypertonia. Systemic, autonomic signs include hypertension, tachycardia, and ileus. Pain usually begins in the legs. The patient may position the head and trunk in extreme hyperextension and may be thought to have opisthotonus or meningismus [29]. Muscular weakness may progress to paralysis requiring artificial ventilation [42]. Self-injurious behavior has been observed. Some patients have had seizures [43], some of them associated with hyponatremia [8]. Death may occur during a neurologic crisis [44, 45]. During most crises, consciousness is normal. These crises are not associated with hepatic relapse. Most crises subside in 1–7 days and resolve slowly, but there may be residual weakness. Intelligence is usually normal.
Prader–Willi Syndrome: An Example of Genomic Imprinting
Published in Merlin G. Butler, F. John Meaney, Genetics of Developmental Disabilities, 2019
Subjects with PWS are known to have behavioral problems including obsessive compulsivity and self-injurious behavior. Self-injurious behavior in persons with intellectual impairment, autism, and related developmental disabilities ranges from 5% to 60%, depending on the methods used and populations studied (70). Serious health problems can occur relating to this behavior. Investigators have previously reported that self-injurious behavior is a prevalent behavioral problem for 69% of adolescents with PWS (71) and in 81% of adults (72). A study reported by Symons et al. (73) characterized self-injurious behavior in 62 PWS subjects via a questionnaire survey and found skin picking to be the most common form of self injury. Prader–Willi syndrome individuals with the 15q11–q13 deletion injured at significantly more body sites than did individuals with maternal disomy.
The use of applied behavior analysis in traumatic brain injury rehabilitation
Published in Mark J. Ashley, David A. Hovda, Traumatic Brain Injury, 2017
Craig S. Persel, Chris H. Persel
An antecedent event is followed by the occurrence of a behavior. If the behavior has been chosen for modification, to either increase or decrease, it is referred to as the target behavior. People with TBI can exhibit a wide variety of behaviors that require intervention. A target behavior must be observable and immediately recordable. The target behavior must also be very clearly defined in terms of observable actions.196 This is known as an operational definition. Two therapists, for instance, can have very different ideas about what constitutes a behavior. For example, take the behavior of physical aggression. Does it include spitting or threatening? What about self-injurious behavior? Should throwing or breaking objects be included? Clear and concise definitions of target behaviors are critical to identifying the behaviors and to implementing programs consistently.
Assessing Non-Suicidal Self-Injury in the Laboratory
Published in Archives of Suicide Research, 2018
Brooke A. Ammerman, Mitchell E. Berman, Michael S. McCloskey
The search targeted published papers between July 1984 and November 2016. An electronic search on the following databases was carried out: PsycINFO, PsycARTICLES, ERIC, CINAHL, and MEDLINE. The following search terms were included: (a) self-injury or self-injurious behavior or non-suicidal self-injury or self-harm or deliberate self-harm or self-mutilation or self-aggression, and (b) experimental or laboratory or behavioral or real-time. Studies had to be in English and meet the following three inclusion criteria: (1) included a task that was an analogue of intentional self-injury (e.g., directly assessing an NSSI-proxy behavior). Examples of tasks that did not meet the proxy for self-injury criterion included EMA/daily diary designs, behavioral measures of impulsivity, and assessments of only pain tolerance; (2) the study was conducted in a controlled laboratory setting (e.g., no case studies); and (3) the sample was not specific to those with intellectual disabilities, autism spectrum disorder, or traumatic brain injury.
Use of the Columbia-Suicide Severity Rating Scale (C-SSRS) to Classify Suicidal Behaviors
Published in Archives of Suicide Research, 2018
Alejandro Interian, Megan Chesin, Anna Kline, Rachael Miller, Lauren St. Hill, Miriam Latorre, Anton Shcherbakov, Arlene King, Barbara Stanley
Another potential gap pertains to the ability to classify self-harm behaviors, where the intent of the behavior remains undetermined after reviewing several considerations. Other classification systems (i.e., SDVCS, C-CASA) include a category for “undetermined” suicidal intent, but such a rating is not available in many versions of the C-SSRS (Crosby, Ortega, & Melanson, 2011; Posner et al., 2014). The option to rate self-injurious behavior with undetermined or “unknown” intent is available in one version of the C-SSRS for pediatric/cognitively impaired populations (Posner et al., 2010). Instances of self-injurious behavior with undetermined intent are likely to be of clinical concern, valuable for empirical study, and to be encountered in clinical practice. Therefore, another recommendation would be for more versions of the C-SSRS to include a classification for self-injurious behaviors where suicidal intent remained undetermined. There is a cautionary note to this recommendation, however. While difficulties in assessing intent can be expected to occur, the determination of intent is critical to our understanding of the self-injurious behavior (Mundt et al., 2013; Silverman et al., 2007). Thus, care should be taken to utilize a classification of “undetermined” intent only after careful efforts have been made by the rater to query and consider other intents (i.e., suicidal, non-suicidal).
A psychometric analysis of the Ottawa self-injury inventory-f
Published in Journal of American College Health, 2018
Joshua Travis Brown, Fred Volk, Gabrielle L. Gearhart
College campus counseling centers are challenged to deal with a wide range of mental health issues. The development of tools that facilitate the improved efficiency of psychological intake processes is essential for colleges and universities given that they simultaneously possess limited resources but must also increase accessibility in order to adequately meet the mental health needs of students. The purpose of this study was to evaluate the psychometric properties and validate the use of a measure of self-injurious behavior in a clinical setting for the diagnosis of NSSI according to the DSM-5. Self-injurious behavior is an increasing issue and is especially prevalent within the adolescent and young adult populations. We see concerning trends within this study, as 36.4% of the 1,064 students who visited the student counseling center indicated a history of self-injurious behavior and 11% indicated having self-injured or thought about self-injuring in the past 30 days. This suggests the importance of awareness regarding NSSI for counselors of young adults and adolescents. Thus, a validated and reduced measure of self-injurious behavior is necessary for the evaluation of NSSI among a set of mental health issues presently being addressed by university mental health professionals.