Symptom substitution in Tourette disorder.
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Biomedical subjects
Publications and source records attributed to L Burd.
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In a series of prevalence and follow-up studies on North Dakota's 59 children with pervasive developmental disorders, the patient characteristics of hyperlexia, Tourette disorder (TD), and the absence of seizures were found to be associated with improved outcome or higher IQ. We entered these and 17 other characteristics into a regression model using forward, step-wise inclusion to identify the smallest set of predictor variables which were significantly associated with the dependent variables of IQ, and receptive and expressive language. Of the 20 predictor variables used in the regression analysis, the same four variables met inclusion criteria for each of the dependent variables. These predictor variables were: hyperlexia, a known aetiology, TD and age. The relevance of these findings is discussed.
Partial trisomy 6p with duplications ranging from 6p21 to 6p25-pter is emerging as an established syndrome. We report a case of duplication of 6p (6p23-pter) and deletion of 2q37-qter. Features characteristic of 6p partial trisomy present in the patient are low birthweight, and mental and developmental retardation. Major facial features include prominent forehead, flat occiput, multiple ocular abnormalities, low-set ears, prominent nasal bridge, long philtrum and small pointed mouth. Repeated examinations of the patient from birth to the age of over 5 years revealed that he has infantile autism. Since autistic children are generally not associated with chromosome anomalies, in view of the present case, it is suggested that karyotypic analysis be considered for such children. Where possible, extended study for autism in 6p trisomic children may also be desirable.
Two male children meeting criteria for Childhood Onset Pervasive Developmental Disorder (COPDD) are described. The current DSM-III category of COPDD may have value in separating these children from others with PDD. The authors suggest that these two children, and other children described in the literature as having dementia infantalis and/or disintegrative psychosis, have a distintegrative disorder resulting in muteness, profound mental retardation and severe autistic symptomatology. The term "pervasive disintegrative disorder" may be appropriate for such children and specific diagnostic criteria are suggested. The disorder appears to be extremely rare, with a prevalence estimate of 0.11 per 10,000.
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Rett syndrome is a mental retardation syndrome that occurs only in females and consists of normal pre-, peri-, and neonatal growth and development. It is followed by rapid neurobehavioral deterioration in late infancy or early childhood, a developmental arrest, plateauing, and then either a course of retarded development or continued deterioration. The period of rapid neuro-behavioral deterioration manifests as a partial autistic syndrome, with loss of production and comprehension of language, hyperactivity, hyperventilation, hand-wringing, and ataxic gait. Current diagnostic criteria are discussed and suggestions for further research are presented.
The breastfeeding rates for 50 children with pervasive developmental disorder (PDD) from North Dakota's roster of PDD patients were compared with the national average and with the rates for a control group matched for age, sex, and IQ. In addition, the breastfeeding rates for the normal siblings of the PDD and control groups were compared with the national average to help determine whether the lower breastfeeding rate among PDD patients was a function of parenting practices. The breastfeeding rates for the PDD and control groups were not significantly different from each other, but both were significantly lower than the national average. The breastfeeding rate for the normal siblings of PDD children was almost identical to the national average, but the rate for the siblings of the matched control group was significantly lower than the national average. These results are discussed in terms of hypotheses regarding the early parent-child interactions and characteristics in the families of PDD children.
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The authors report on three patients in North Dakota with an apparent onset of Gilles de la Tourette's syndrome before 1 year of age. Infantile onset may occur in 4.1% of the child patients with Tourette's disorder in that state. It is suggested that the diagnostic criteria for Tourette's disorder be revised to include patients who develop the illness before they are 1 year old.