Juvenile Huntington chorea with persistent sexual deviance. A case report

A case of juvenile hereditary chorea is reported on in which there was repeated sexual delinquency. Our point of view in respect of psychopathological and forensic aspects of the disorder is set out.     

Neurocognitive correlates of type 1 diabetes mellitus in childhood.

Type 1 Diabetes Mellitus (T1DM) is one of the most prevalent chronic health conditions in children under the age of 18 years. Complications of the disease include hypo- and hyperglycemia, which can have an impact on children's performance in assessment situations, in the clinic, and in school. Because there is no cure for this disease, there is a need to understand the cognitive deficits associated with some of its complications, as this knowledge will impact on the choice of treatment regimens as well as educational interventions. This paper provides a comprehensive review of the relevant literature on the neurocognitive outcome of T1DM. In particular, disease- and treatment-related variables that are associated with poor performance on cognitive domains will be reviewed. Specifically, age of onset, duration, pubertal effects, and presence of hypoglycemia or hyperglycemia will be examined. These findings are not without controversy, and limitations to conclusions will also be presented. Where relevant, recommendations for future research directions will be provided.     

Visual agnosia and prosopagnosia in childhood: a prospective case study.

Selective impairments in visual processing are well documented in adults but rarely reported in children. The few childhood cases reported are mostly retrospective accounts with little attention paid to developmental, assessment or management issues. We report a prospective case study of a boy with prosopagnosia and visual processing deficits of presumed developmental origin. At the age of 4 years, AL presented with a range of cognitive and visual recognition deficits. Subsequent assessments revealed an evolving pattern in visual recognition and dissociations between developing skills. At the age of 7 AL has impairments in early perceptual analysis, visual organisation and in complex visual processing. Although he can identify facial features and match faces he is unable to recognise familiar faces. His reading and spelling are developing normally. The nature of his deficits and his progress are discussed within a cognitive neuropsychological framework.     

An unusual case of self-inflicted death in childhood

The authors report the details of the case of a ten year old boy whose self-inflicted death by gunshot featured the involvement of a nine year old companion. The authors examine the material from the viewpoints of a possible case of suicide, of a certain case of violent death, and of a case with repercussions on the other child and the community.     

Functional psychosis in Huntington chorea

The paper considers a case of acute paranoid-hallucinatory psychosis that made its appearance in the fifth year after the manifestation of hereditary chorea. A brief account of the problems involved in psychoses when hereditary chorea is present is given. These psychoses are taken to be functional psychoses as according to Wieck.     

Oral and written language in a case of childhood phonemic deafness

Neuropsychologic and neurolinguistic studies performed on an 11-year-old Landau-Kleffner boy are reported. At age 3 his language began to deteriorate progressively until complete disappearance. At the same time, the patient developed epileptic seizures. When 6 years old, he was taught to match objects to the corresponding written word, and subsequently he was able to learn reading and writing to such extent that he could attend primary school. BAER and primary cortical auditory responses were normal, whereas late cortical components were grossly retarded and distorted. Phonemic discrimination, identification, and production were absent, and in contrast, lexical-semantic abilities tested through reading and writing were normal. In visual STM tasks, phonologic mediation was absent. The role of phonologic mediation in language acquisition is then hypothesized.     

Effects on an adult of incest in childhood: a case report

Recognition of the widespread incidence of overt incest in childhood has significantly increased in recent years. The described long-term effects range from severe psychopathology and behavioral disorder to mild and subtle manifestations. The interplay of the child's sexual developmental processes and intrapsychic fantasies with the overtly incestuous behaviors adds complexity to the understanding of longterm effects. In this case presentation successful analysis revealed previously repressed extensive father-daughter incest resulting in typically autoplastic neurotic symptomatology and character formation. Effects on ego development and function were traced, and an attempt was made to develop recognition of cues suggestive of past overt incest. The case suggests that many additional factors contribute to the final long-term effects of overt incest in childhood.     

Quantitative and qualitative analyses of clock drawings in Alzheimer's and Huntington's disease.

Although visuoconstructive impairment has been reported in both Alzheimer's (DAT) and Huntington's (HD) disease, there is little knowledge concerning how this cognitive deficit differs quantitatively and qualitatively in these two progressive dementias. To address this issue, the present study compared performances on the Clock Drawing Test (CDT: command and copy) of 25 DAT patients, 25 equally demented HD patients, and 25 elderly normal controls (NC). In the command condition, both patients groups were significantly impaired compared to the NC group. Although there was no significant difference between DAT and HD patients' total quantitative scores, a qualitative error analysis revealed a number of dissociations between the two patient groups. Graphic difficulties, very common in HD patients, were virtually absent in DAT patients; in contrast, conceptual errors were almost exclusively seen in DAT patients and were related to the severity of their dementia. Perseveration and "stimulus-bound" responses were also more frequent in DAT patients, and both groups made visuospatial errors. In the copy condition, the DAT, but not the HD, patients evidenced a marked improvement in performance. These results indicate that while both DAT and HD patients have significant visuoconstructive difficulties even in the early stages of their disorders, the specific cognitive processes underlying their quantitative impairments are quite different. It is possible that the DAT patients' conceptual errors are yet another indicator of the deterioration of their semantic knowledge.     

Communication patterns in pedodontics.

This investigation examined communication patterns between 16 randomly selected senior dental students and their child patients. Interactions were videotaped during a regular treatment appointment and analyzed according to four Practitioner Communication categories and four Child Cooperation categories. Communication categories included Directive Guidance Behavior, Permissive Behavior, Coercive Behavior, and Other Behavior. Child behavior categories included Cooperation, Resistance, Noncooperation, and Other. The probability that a given category of child's behavior would follow a given category of practitioner's behavior was calculated. Results indicated a .85 probability that Directive Guidance would be followed by Cooperation; a .67 probability that Permissiveness would lead to Noncooperation; and a .97 likelihood that Coerciveness would lead to either Noncooperation or Resistance. Practitioners' confidence was also considered; less confident operators account for 95% of the Coercive behaviors, 86% of Permissive behaviors and 87% of Uncooperative behaviors.     

Psychological correlates of the course of Sydenham's chorea

Published reports provide contradictory evidence concerning the cause, nature, and course of Sydenham's chorea: Physiological, psychological, and interactional hypotheses have been advanced. Reports supporting any of these views suffer from lack of detail, objective observations, and/or longitudinal behavioral sampling. This case was studied longitudinally with numerous psychological tests. They revealed acute encephalopathy and severe personality disturbance in the acute stage, followed by rapid and lasting clearing of both symptoms. Thus, this case provided evidence consistent with virtually all published reports and with each hypothesis about Sydenham's chorea, suggesting the need for more careful longitudinal studies and a conceptual framework which can more fully embrace all the data.This study was begun while the author was at the William S. Hall Psychiatric Institute in Columbia, South Carolina.The author gratefully acknowledges the comments of Professor Arthur L. Benton.     

Analyzing the role of culture in shaping American childhood: A twentieth-century case

Widespread beliefs about the nature and functions of children change over time, and affect actual treatment of children within and without the family. Significant changes in beliefs about children developed in the United States from the 1920s onward, with emphasis both on emotional value and on weakness or vulnerability. Increased consumption of expert advice played a key role in this shift, along with cultural translation of new issues concerning child labor and schooling. Key changes occurred in all industrial societies, but American culture generated some distinctive features that continue to show up in the anxieties many parents harbor about children and their effort to intervene on their children's behalf.