Down syndrome is the most common genetic cause of mental disabilities.
Although overall there is an increased incidence of psychiatric disorders in people with mental retardation, some psychiatric illnesses are more common among people with Down syndrome than in the general population.
In addition to the fact that prevalence is greater, there is also a difference in the type of disorder that occurs in people with Down syndrome. Alzheimer-type dementia, obsessive compulsive disorders and depression are more common among people with Down's syndrome.
Rates of psychiatric disorders in Down syndrome also change with age, which may be related to the significant weight of medical and mental illnesses as well as social and developmental changes that evolve with age.
Dementia
The first reported relationship between Down syndrome and dementia in Alzheimer's disease was made by Fraser and Mitchell in 1876. Commenting on people with Down syndrome, they wrote: "In some cases, death was attributed to nothing more than general disintegration - a sort of precipitous gerontology." Since then, strong clinical, epidemiological, neuropathological and genetic correlations have been established.
The high risk of dementia in Alzheimer's disease in adults with Down's syndrome is primarily due to the triplication and over-expression of the gene for the amyloid precursor protein (APP) found on chromosome 21. The data increasingly show that the presence of one or more copies of APOE E4, as well as in the general population, increases the risk of dementia in Alzheimer's disease in the Down syndrome population). The presence of the APOE E2 allele probably increases longevity and reduces the risk.
Also, a recent review by Schupf et al (2017) reports that decreased levels of estrogen, for example as observed in early menopause, and variation of genes for estrogen receptor activity, are associated with the early onset of dementia in Down syndrome.
Symptoms of dementia in Alzheimer's disease in adults with Down syndrome may be difficult to detect due to underlying mental deficiency.
Lai and Williams in 1989 describe three phases of dementia in Alzheimer's disease:
In the initial phase, memory impairment, temporary disorientation and reduced speech ability were evident in people with a higher level of Down syndrome functionality. For people with Down's syndrome with a more severe learning disability, the first signs of dementia were apathy, carelessness, and reduced social interactions.
In the second phase, there are losses of self-handling skills such as dressing, toilet and the use of utensils, and gait disturbances occur which slows down.
In the final phase, patients are locked in bed with incontinence of sphincters and in 20% of cases parkinsonism and in some cases seizures.
Treating dementia in Down syndrome as soon as diagnosis is completed, includes four main areas of management:
- Treatment of the underlying disease with anticholinesterase inhibitors (AChE) (Donepezil, Rivastigmine, Galantamine) has now been established as drugs that can slow down the rate of deterioration of dementia in Alzheimer's disease in both the general population and the Down syndrome population.
- Tackling the clinical symptoms of dementia such as insomnia, aggression, irritability, low mood, psychotic features and seizures.
- Psychological intervention: there are growing indications that psychological / behavioral intervention may benefit adults with dementia in the general population. Psychological intervention through various techniques can maintain current skills.
- Support for carers: Training and support for carers can help to preserve the autonomy of these people.
Depression
The presentation of depression in adults with Down syndrome may differ significantly from that of the general population.
The cognitive traits (e.g. memory impairment, loss of concentration, suicidal ideation) may not be so obvious. Organic features (psychomotor retardation, disturbed sleep, loss of appetite, weight loss, fatigue) may be more prominent. The decline in adaptive skills (e.g. washing, dressing, etc.), anhedonia, anxiety and stress, are associated with depression. The treatment of emotional disorders in Down's syndrome is both pharmacologically and psychotherapeutically. In some cases electroconvulsive therapy is effective in depression.
Obsessive Compulsive disorder
Obsessive compulsive disorder (OCD) is characterized by the presence of disturbing thoughts, images or impulses that lead to compulsions (physical or mental) to help reduce anxiety associated with obsession.
In adults with Down syndrome, obsessions can occur that relate to ritual touching, cleaning, changing light switches, opening and closing the door, and so on.
The distinction of obsessive-compulsive disorders from the stereotypes observed in people with mental retardation may be difficult. The treatment of obsessive compulsive disorder is pharmaceutical. Other psychiatric disorders such as somatization, drug abuse, and personality disorders also occur in people with Down syndrome at a different frequency, and their treatment is both pharmaceutical and psychological.
All Programs offered by “Veresies” Clinic are approved and supervised by the Cyprus National Addictions Authority (CNAA).
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