1. The Spasmodic Upper-body Squeeze: a Chalacteristic Behavior in Smith-Magenis Syndrome
- Author
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Scott Ci, Finucane Bm, Haas-Givler B, Konar D, and Kurtz Mb
- Subjects
Adult ,Male ,Spasm ,medicine.medical_specialty ,Adolescent ,Retinoic acid induced 1 ,Hand functions ,media_common.quotation_subject ,Audiology ,Behavioral traits ,Muscular Diseases ,Developmental Neuroscience ,Intellectual Disability ,medicine ,Humans ,Child ,media_common ,Upper body ,Diagnostic marker ,Syndrome ,Middle Aged ,Hand ,Smith–Magenis syndrome ,medicine.disease ,Pediatrics, Perinatology and Child Health ,Arm ,Happiness ,Female ,Neurology (clinical) ,Facial grimacing ,Chromosome Deletion ,Psychology ,Self-Injurious Behavior ,Chromosomes, Human, Pair 17 - Abstract
The authors have observed a hand- and arm-squeezing behavior that seems to be highly characteristic of Smith-Magenis syndrome (SMS). This behavior serves as a useful diagnostic marker for SMS, a disorder in which the physical phenotype is often subtle. The squeezing behavior appears to be part of a complex upper-body tic which is exacerbated by happiness, excitement or overstimulation. The tic most often manifests as a 'self-hug', and is frequently associated with facial grimacing. Fleeting arm- and hand-squeezing movements may be repeated hundreds of times over the course of a day, but they do not significantly interfere with other hand functions. The excitable self-hugging in people with SMS may be one of the more benign and appealing aspects of their behavioral phenotype.
- Published
- 2008