Movement Disorder in Down's syndrome: A possible marker of the severity of mental handicap

Haw, Camilla; Barnes, Thomas R. E.; Clark, Kate M.; Crichton, Paul; Kohen, Dora

doi:10.1002/mds.870110408

Cited by 25 publications

(26 citation statements)

References 14 publications

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“…It is possible that this behavior is a form of stereotypy and requires further study. Children with DS also have been shown to display stereotypic motor behaviors (Haw et al, 1996).…”

Section: Discussionmentioning

confidence: 99%

Harm Avoidance, Anxiety, and Response to Novelty in the Adolescent S-100β Transgenic Mouse: Role of Serotonin and Relevance to Down Syndrome

Bell

Shokrian

Potenzieri

et al. 2003

Neuropsychopharmacol

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S-100b is an astroglial-derived protein, which plays a role in brain development and maintenance, and is known to play a specific role in the regulation of growth of the serotonergic neuronal system. In humans, the gene for S-100b is found on chromosome 21, within the region that is considered important for the phenotype of Down syndrome (DS). Thus, we have been studying a model of DS, the S-100b transgenic mouse. In the current study, we have examined anxiety and responses to novelty in adolescent (60-90 days) animals, at a time when we have shown the animals to be relatively lacking in serotonin innervation, compared to their CD-1 nontransgenic controls. In a test for approach/avoidance, the light/dark test, the S-100b transgenic mice animals showed no differences from control CD-1 mice. However, in the hole-board test for exploratory behavior, the S-100b animals were found to be less responsive to the inhibiting effects of the serotonin receptor 5-HT1A agonist, buspirone. Three tests were used to measure response to novelty. In the open field, the S100b animals showed greater activity longer than the control animals, and in the Y-maze test, the S-100b animals spent more time in the novel arm. In a test for novelty-induced gnawing, the S-100b animals were also more active than control animals. All of these suggest that the S-100b transgenic mice are slower to habituate to novelty than control animals. Finally, we tested the animals in a new procedure that we are proposing as a test for harm avoidance. In this apparatus, the S-100b animals showed more approaches to a novel and potentially harmful object than the control mice did. These results are discussed in reference to the known lack of serotonin in the animals, and to the behavioral phenotype of DS.

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“…It is possible that this behavior is a form of stereotypy and requires further study. Children with DS also have been shown to display stereotypic motor behaviors (Haw et al, 1996).…”

Section: Discussionmentioning

confidence: 99%

Harm Avoidance, Anxiety, and Response to Novelty in the Adolescent S-100β Transgenic Mouse: Role of Serotonin and Relevance to Down Syndrome

Bell

Shokrian

Potenzieri

et al. 2003

Neuropsychopharmacol

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“…In addition, problems such as orofacial dyskinesias (abnormal and involuntary movements of the jaw and face), bruxism (persistent tooth grinding), and tongue protrusion are common in the DS population and are viewed as attempts to compensate for craniofacial abnormalities in order to effectuate as efficient eating, drinking and swallowing as possible [11]. These problems can become even more apparent and exaggerated when the person with DS reaches early adulthood [12].…”

Section: Eating Drinking and Swallowing In Downõs Syndromementioning

confidence: 98%

The Impact of Aging on Eating, Drinking, and Swallowing Function in People with Down’s Syndrome

Lazenby¹

2007

Dysphagia

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Many people with Down's syndrome (DS) experience eating, drinking, and swallowing (EDS) difficulties, which can potentially lead to life-threatening conditions such as malnutrition, dehydration, and aspiration pneumonia. As the life expectancy of people with DS continues to improve, there is an increasing need to examine how the aging process may further affect these conditions. Published research studies have yet to address this issue; therefore, this article draws on the literature in three associated areas in order to consider the dysphagic problems that might develop in aging people with DS. The areas examined are EDS development in children and adolescents with DS, EDS changes associated with aging, and EDS changes associated with dementia of the Alzheimer's type (DAT) because this condition is prevalent in older adults with DS. This article concludes that unlike in the general population, the aging process is likely to cause dysphagic difficulties in people with DS as they get older. Therefore, it is suggested that longitudinal studies are needed to examine the specific aspects of EDS function that may be affected by aging and concomitant conditions in DS.

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“…In about 1/3 of our subjects with DS þ ASD, there is a history given of lost cognitive-language-social skills without co-occurring clinical seizures. Dyskinesia and stereotypic movements are common in persons with DS [Haw et al, 1996]. Children diagnosed with DS þ SMD may at first glance be regarded as ''autistic-like'' because of their frequently intense repetitive behaviors or stereotypic movements, however they retain functional socialcommunication and reciprocity, thus excluding them from a label of ASD.…”

Section: Autistic Spectrum and Stereotypy Movement Disordersmentioning

confidence: 99%

Neurobehavioral disorders in children, adolescents, and young adults with Down syndrome

Capone

Goyal

Ares

et al. 2006

American J of Med Genetics Pt C

148

125

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The term dual-diagnosis refers to a person with mental retardation and a psychiatric disorder. Most children with Down syndrome (DS) do not have a psychiatric or neurobehavioral disorder. Current prevalence estimates of neurobehavioral and psychiatric co-morbidity in children with DS range from 18% to 38%. We have found it useful to distinguish conditions with a pre-pubertal onset from those presenting in the post-pubertal period, as these are biologically distinct periods each with a unique vulnerability to specific psychiatric disorders. Due to the increased recognition that psychiatric symptoms may co-occur with mental retardation, and are not inextricably linked to cognitive impairment, these conditions are considered treatable, in part, under a medical model. Improvement in physiologic regulation, emotional stability, and neurocognitive processing is one of the most elusive but fundamental goals of pharmacologic intervention in these disorders.

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Movement Disorder in Down's syndrome: A possible marker of the severity of mental handicap

Cited by 25 publications

References 14 publications

Harm Avoidance, Anxiety, and Response to Novelty in the Adolescent S-100β Transgenic Mouse: Role of Serotonin and Relevance to Down Syndrome

Harm Avoidance, Anxiety, and Response to Novelty in the Adolescent S-100β Transgenic Mouse: Role of Serotonin and Relevance to Down Syndrome

The Impact of Aging on Eating, Drinking, and Swallowing Function in People with Down’s Syndrome

Neurobehavioral disorders in children, adolescents, and young adults with Down syndrome

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