Prognosis of depression in Down's syndrome.
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Biomedical subjects
Publications and source records attributed to R A Collacott.
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Widespread inquiry identified 378 adults with Down's syndrome resident in Leicestershire, England. The immediate carer of 351 of these (92.8%) was interviewed for the purpose of establishing a past history of seizures, including the age at which the seizures began. The immediate carer was also invited to provide information to enable the completion of an Adaptive Behaviour Scale (A.B.S.) rating. Individuals with a history of seizures were divided into two groups on the basis of whether or not seizures commenced prior to or after age 35 years. Two control groups of individuals with Down's syndrome, but without a history of seizures were selected. Adaptive Behaviour Scale scores for those in whom seizures commenced at a younger age were similar to those who had no recorded history of seizures. However, in those in whom seizures began in later life, scores on all domains of the A.B.S. were significantly reduced compared to both young epileptic patients and their controls. Adaptive Behaviour Scale scores for the older control group held an intermediate position, suggesting that late-onset epilepsy may be a late manifestation of a dementing process. A clinical diagnosis of dementia recorded in the case records was significantly associated with the presence of late-onset epilepsy. This is supportive of the hypothesis that late-onset epilepsy in individuals with Down's syndrome is associated with Alzheimer's disease.
A 38-year-old man with a moderate mental handicap and Asperger's syndrome is described. His transvestism and history of offending behaviour is interpreted in the light of this.
Though widely reported in the general population, pathological jealousy has not previously been reported in an individual with a mental handicap. We describe the case of a 39-year-old man with a borderline mental handicap and an abnormal premorbid personality who demonstrates the condition.
A case report of a male mentally handicapped patient who developed delusions of passion is described. The relationship of this case to de Clerambault's syndrome and its pathogenic features are described.
Four men with learning disabilities were considered to show transvestic fetishism, and a fifth to show transvestism. However, developmental retardation and personality problems may modify the concepts behind such categorisation.
Until recently, it was considered that Down's syndrome precluded a diagnosis of mania, or gave rise to an atypical presentation. There have been seven case reports of mania in people with Down's syndrome and all these cases are reviewed. The clinical features of mania are noted to be similar to those previously described in individuals with learning disabilities due to other causes. However, all reported cases are male and none has a family history of affective disorder. In two of the seven men reported, the illness followed a rapid cycling pattern. Hypothyroidism and monoamine biochemistry in people with Down's syndrome are discussed in the context of these atypical features.
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The total number of adults with Down's syndrome living in Leicestershire, ascertained by widespread enquiry, was found to be 378. Of these, 371 were matched with adults with mental handicap due to other pathologies, on the basis of age, sex, and type of residence. Those with Down's syndrome were found to have a different spectrum of mental disorders from those without the syndrome. In particular, Down's syndrome patients were more likely to have been diagnosed as having depression and dementia; the controls were more likely to have been diagnosed as suffering from conduct disorder, personality disorder, or schizophrenia/paranoid state. The same proportion of each group had been given a diagnosis of autism.
Through widespread enquiry, the population of adults with Down's syndrome (aged over 18 years) in Leicestershire was estimated at 376. For 315 of these (83.8%), the immediate carer was invited to complete the Adaptive Behavior Scale (ABS). Completed ABS assessments were obtained on 81.9% of the adult population with Down's syndrome in the county. Additional information concerning residential history was obtained. When the sample was divided into five cohorts on the basis of age when the ABS assessment took place, an exponential decline in ability was observed. Deterioration in most domains of the ABS achieved statistical significance in the cohort aged 50-59, and in all domains in those aged 60 and over. The deterioration in global skills in older cohorts was attributed to ageing (and thereby probably Alzheimer's disease). Institutional placement was associated with low scores in younger groups only.
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The present authors report the case of a 42-year-old female with Down's syndrome who developed a misidentification syndrome, erotomanic delusions towards her Bible instructor, and increasing grandiosity following the death of her father. The authors believe that her symptoms may be formulated in developmental and dynamic terms.
Patients with Down's syndrome are particularly vulnerable to the development of both hypothyroidism and Alzheimer's disease. Both hypothyroidism and Alzheimer's disease may be associated with elevated serum concentrations of thyroid stimulating hormone. In a group of institutionalized Down's syndrome patients with normal thyroid function, global scores of ability were higher than in a group of patients with elevated thyroid stimulating hormone levels in the presence of normal T3 and T4. The actual concentrations of thyroid stimulating hormone were shown to be significantly and inversely correlated with scores of global abilities. If these findings are reproducible, the authors believe that thyroid stimulating hormone estimation may provide confirmatory evidence of clinical dementia in this group of mentally handicapped individuals.
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A profoundly mentally handicapped patient is described with the karyotype 47,X, fra (X) (q27), + 21. Her phenotype demonstrates features of both Down's Syndrome and fragile-X syndrome. The authors believe only one other similar case has been described.
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A patient with Down's syndrome and asymptomatic atlanto-occipital instability is described. The relationship between atlanto-occipital instability, atlanto-axial instability and generalized ligamentous laxity, is discussed.