Epidemiology and comorbidity. The North Dakota prevalence studies of Tourette syndrome and other developmental disorders.
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Biomedical subjects
Publications and source records attributed to J Kerbeshian.
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A 40-year-old man with childhood-onset Tourette syndrome (TS) developed Huntington disease (HD). We believe this to be the first reported case of childhood-onset TS with adult onset HD. Discovery of other cases with both disorders may provide clues to the pathophysiology of both conditions.
Service providers for children in the state of North Dakota were surveyed to determine the prevalence rate of Rett syndrome (RS). Five patients with definite RS were identified in a population of 203,801 children (98,932 girls) 0-18 years of age yielding a prevalence rate of RS of 1 in 40,760 in North Dakota children. For girls the rate of RS is 1 in 19,786. Similar surveys of other states using comparable modes of ascertainment will be important as points of comparison for prevalence rates of RS.
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To determine the prevalence of psychoactive medication usage among persons with mental retardation residing in community settings in the state of North Dakota, we sent a questionnaire to all group homes serving persons with developmental disabilities. The return rate was 97%. Psychoactive medications were used by 37% of residents represented. Results were discussed in relation to previous studies that have primarily involved institutionalized populations.
Institutionalized adults with mental retardation (N = 297) were surveyed to determine the prevalence of symptoms of Rett syndrome. No symptom of the syndrome occurred more frequently in males than females. When symptoms were analyzed in clusters (e.g., severe mental retardation, no prenatal complications, walked before 15 months, and wide-based gait), no single cluster of symptoms appeared to differentiate males from females. However, as individuals, only females were found to meet the necessary criteria for a diagnosis of Rett syndrome. Symptoms were seen with equal frequency in this population and no one or two symptoms differentiated patients with and without Rett syndrome.
A prevalence study of Prader-Willi syndrome (PWS) has been conducted in North Dakota. All pediatricians, neurologists, child psychiatrists, psychologists, and clinical geneticists were surveyed. The state's comprehensive evaluation center, the state hospital, the state institution for the mentally retarded, and group homes for the developmentally disabled, including one for persons with PWS, were also contacted. Seventeen patients were identified, 8 males, 8 females, and one patient whose sex was not specified. This suggests a prevalence rate of 1 per 16,062 in North Dakota.
A case report of a 33-year-old woman with atypical bipolar disorder, autistic disorder, profound mental retardation and a chromosomal anomaly is presented. This patient is behaviourally similar to a patient reported by Akuffo et al. (1986), who apparently did not have a chromosomal investigation. The chromosomal anomaly and similarities between the two patients are discussed.
It has been questioned whether Asperger's syndrome (AS) is in fact a specific (high functioning) subgroup of autism, rather than a distinct entity. Thirteen AS patients were compared with 13 autistic patients and 13 developmentally disordered controls. While there was symptom overlap between AS and autism, patients could be separated into one or other group. However, current criteria are based on symptoms, and it is argued that studies of genetics and treatment response are needed to elucidate the relationship between these developmental disorders.
A 14-year-old girl developed a movement disorder after a streptococcal infection. In the acute phase of the illness she exhibited simple and complex motor tics and chorea, but all abnormal movements ceased over the following eight months, without recurrence. This case raises questions about the relationship between tics, chorea and auto-immune reactivity.
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Children with developmental regression and emerging symptoms of autism have been given a variety of classifications. The authors compare two boys with Heller dementia with six girls with Rett syndrome. They all differed from children with classic autism in that they had normal prenatal and perinatal periods, followed by marked developmental regression, after which they acquired few or no skills. The boys differed from the girls in terms of estimated prevalence, age at onset, stereotypic breathing patterns, midline hand stereotypies, hand and gait apraxia and speech development. It is suggested that these children should be distinguished from those with classic autism, and should be classified as 'pervasive disintegrative disorder, Heller type' and 'pervasive disintegrative disorder, Rett type'.
Three boys with an early history of attention deficit disorder with hyperactivity developed Tourette disorder. At 13, 12 and eight years of age, respectively, each met DSM-III criteria for a manic episode or bipolar disorder. Each of the boys had a family history of affective or affective spectrum disorder. Lithium carbonate in a range of 0.8 to 1.2 meq/L markedly improved their bipolar symptomatology with Tourette symptoms improving in two patients. Further study is suggested to determine the significance of these findings.
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