1/9. In the long run...longitudinal studies of psychopathology in children. Committee on child psychology. Group for the Advancement of psychiatry. Report no.143.longitudinal studies are difficult to do well. Too short a study and the results may be meaningless. Too long a study and the subjects cannot be found, money runs out, and research methods become seriously out of date. Despite these problems, there have been some longitudinal studies done that have greatly advanced our understanding of the nature and the treatment of psychopathology in childhood. Without these studies, much less would be known about the clinical course of important disorders, the effects of treatments, and the various risk and protective factors. None of these studies has been perfect. Some longitudinal studies did not focus on quite the right questions, some produced contradictory results, and others produced results that were hard to interpret. What have we learned from the longitudinal studies reviewed in this Group for the Advancement of psychiatry (GAP) report? Many of the things that we have learned have been surprising--even counterintuitive. Pre- and perinatal insults need not necessarily lead to serious consequences in later life. Premature infants, if raised in nondeprived settings, are not likely to be mentally retarded or learning disabled. Today, of course, premature infants who would not have been kept alive 15 years ago are surviving. Will this advancement led to an untoward outcome? We do not know. New longitudinal studies need to be done. Certain serious illnesses emerging later in childhood may be associated with a greater risk of psychopathology. This risk is true at least for those with asthma. Psychological factors, such as psychological stress, also may lead to exacerbation of asthmatic attacks. Whether other illnesses are associated with a greater risk of psychopathology simply has not been studied adequately. infant temperamental characteristics can be classified and measured; however, they appear to predict little in terms of later personality development or psychopathology. Although temperamental characteristics measured in infancy correlate poorly with temperamental characteristics measured at age 4 or 5 years, they appear to be much more stable and correlate well with temperament at ages 8 and 12 years. Some of these temperamental characteristics, particularly aggressivity and negativity at age 5 years, are serious risk factors for behavioral psychopathology at age 8 or 12 years and, possibly, at later ages as well. Aggressive, negative 5-year-olds do not necessarily grow out of their problems. Their problems are important and should be dealt with when children are age 5 years. What does appear important is early language development. Delays in language development or developmental language disorders are risk factors for later psychopathology. Fifty percent of school-age children with language problems requiring speech and language therapy also have diagnosable psychopathology. Hyperactivity and short attention span are characteristics that tend to persist in later childhood--even adulthood. Alone, each of these characteristics may not be a risk factor for later psychopathology, but, when accompanied by oppositional behavior and conduct disorder, the outcome is detrimental to the child. Many studies have shown that children with these comorbid problems are at risk later for becoming delinquent and antisocial. conduct disorder in childhood, which is much more common in males, is a risk factor for later delinquency, even if it is not associated with hyperactivity. Again, early intervention is important. The effects of comorbidity in this instance can be serious. comorbidity in general, especially as children grow older, leads to adverse social, academic, and psychopathological outcomes. depression does occur in children, in all of its adult manifestations. Mania, in contrast, is rarely seen in children younger than age 12 years. depression in school-age children can be severe or mild. In contrast to adolescents with depression, depressed children are most likely to have somatic compla- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/9. twin study of central auditory processing disorder.We compared auditory, cognitive, and language test results in a pair of dizygotic twins, one of whom showed symptoms of central auditory processing disorder (CAPD). Results highlight the importance of testing binaural function. In particular, electrophysiologic measures of dichotic listening effectively demonstrated the auditory-specific nature of this child's listening problems. The importance of a thorough and comprehensive evaluation of children suspected of CAPD is stressed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/9. Neurodevelopmental pattern of succinic semialdehyde dehydrogenase deficiency (gamma-hydroxybutyric aciduria).Succinic semialdehyde dehydrogenase (SSADH deficiency) (MIM 271980) is a defect in gamma-aminobutyric acid catabolism, resulting in the accumulation of gamma-hydroxybutyric acid (GHB) and causing neurological and cognitive disorders of varying severity. The non-specific nature and the difficulties in detection of urinary GHB explain why this disorder is largely underdiagnosed. Of 350 patients identified worldwide, to date only six adults with SSADH deficiency have been reported in the literature. Here we describe two additional cases in brothers up to ages 26 and 28 years. This retrospective report sheds light on the clinical features of SSADH deficiency in relation to the physiopathological involvement of GHB, and tries to identify the specific neurodevelopmental pattern of this learning disability.* Features of this are: early impaired psychomotor development with hypotonia and disturbances in motor coordination; impaired development of language, mainly due to poor auditory perception; and seizures and psychotic features in late adolescence or adulthood. Moreover, narcolepsy-like symptoms could be a consistent feature of the disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/9. Molecular characterisation of patients with subtelomeric 22q abnormalities using chromosome specific array-based comparative genomic hybridisation.The 22q13 deletion syndrome is associated with global developmental delay, absent or delayed speech, and generalised hypotonia. In this study, the size and nature of 22q13 deletions (n=9) were studied in detail by high-resolution chromosome specific array-based comparative genomic hybridisation (array CGH). The deletion sizes varied considerably between the different patients, that is, the largest deletion spanning 8.4 Mb with the breakpoint mapping to 22q13.2 and the smallest deletion spanning 3.3 Mb with the breakpoint mapping to 22q13.31. In one case, a unique subtelomeric 3.9 Mb deletion associated with a 2.0 Mb duplication of 22q13 was observed, adding to a growing number of similar cases identified for other chromosome ends. Remarkably, this patient had signs suggestive of retinitis pigmentosa, which has never been reported before in the 22q13 deletion syndrome. The identification of two pairs of recurrent proximal breakpoints on 22q13 suggests that these specific regions may be prone to recombination, due to yet unknown genome architectural features. In addition to the copy number changes on 22q13, a duplication of approximately 330 kb on 22q11.1 was observed and shown to be a genetic large-scale copy number variation without clinical consequences. The current study failed to reveal relationships between the clinical features and the deletion sizes. Global developmental delay and absent or severely delayed speech were observed in all patients, whereas hypotonia was present in 89% of the cases (8/9). This study underscores the utility of array CGH for characterising the size and nature of subtelomeric deletions, such as monosomy 22q13, and underlines the considerable variability in deletion size in the 22q13 deletion syndrome regardless of the clinical phenotype.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
5/9. The language of dissociation.Three case studies of inner-city elementary school children illustrate the connection between speech-language disorders and dissociative disorders in children who have known or suspected trauma histories. The role of speech language pathologists in identifying and responding to dissociative symptoms in children is explored. Lack of adequate training concerning the impact of trauma and scarce literature on the communication profiles of dissociative children contributes and greatly impacts the diagnosis, referral, and treatment of these children. The case studies demonstrate how unusual speech and language symptoms and awareness of dissociative features may aid in identifying trauma-related problems and instituting effective treatment. Grounding techniques and specific language interventions can assist children in acquiring the vocabulary needed for communicating both their daily experiences and traumatic histories. The nature of the relationship between dissociation and communication disorders is explored, and the importance of future research, interdisciplinary collaboration, and trauma training in the speech-language curriculum is emphasized.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/9. Acquired epileptic aphasia. Diagnostic aspects of progressive language loss in preschool children.A case of acquired epileptic aphasia presenting as language delay and hyperactivity is presented and clinical aspects reviewed. Acquired epileptic aphasia is a diffuse neurologic process of unknown etiology that presents in preschool children. Although progressive language loss and seizures are the major manifestations, deficits in attention and socialization may be noted. The electroencephalogram is usually diagnostic. While almost all (80%) children develop seizures, approximately half present as language delay without seizures. Recognition of the progressive and global nature of the language dysfunction may permit earlier diagnosis and intervention.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/9. Returning meaning to semantic relations: some clinical applications.Two approaches for analyzing the meanings reflected in children's early word combinations are illustrated for possible application in the clinical setting. The first approach is interpretive in nature and involves detailed analysis of spontaneous speech. The second approach uses individualized probes that are designed to isolate the semantic factors involved in children's word combination patterns. Although both approaches have limitations, it is argued that each provides significantly more clinical information than assigning a child's utterances to predetermined semantic relation categories.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/9. Hereditary dysfunction of the brain stem auditory pathways as the major cause of speech retardation.Grossly abnormal auditory brain stem responses (ABR) and abnormally high stapedius reflex thresholds were found in 2 pairs of siblings, not akin. Pure tone audiometry showed moderate to moderately severe hearing impairment in all 4 subjects, but neither the ABR findings nor the stapedius reflex thresholds were compatible with pure cochlear lesions. In all the cases the benefit from using hearing aids was conspicuously poor, and the development of oral language markedly retarded, one pair of siblings being essentially incapable of oral--aural communication. A cousin to one of the pairs of siblings showed similar but less pronounced symptoms and signs. intelligence was judged to be normal in all 5 individuals and neurologic examination did not reveal CNS abnormalities besides the hearing impairment. We assume dysfunction of the brain stem auditory pathways to be the main cause of the speech retardation in the 2 pairs of siblings and the abnormality to be hereditary in nature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/9. Some controversies about type and nature of aphasic symptomatology in Landau-Kleffner's syndrome: a case study.The syndrome of acquired aphasia and convulsive disorders in childhood has received ample discussion since the initial description by Landau and Kleffner in 1957. Despite numerous investigations considering the linguistic and epileptic phenomena of the syndrome, the symptomatology still remains of a puzzling nature. Two aspects of the syndrome, the acquired nature of the aphasia and the type of linguistic symptomatology, have not been discussed thoroughly. In our case not only a history of developmental language pathology was present but language symptomatology also revealed a predominantly motor aphasia during a language deterioration phase. A careful review of the literature as presented here yields data in agreement with our findings. First we found further evidence in the literature that Landau-Kleffner's syndrome is not exclusively a primarily receptive language disturbance. Secondly, evidence in favour of a developmental type of Landau-Kleffner's syndrome was found in 25 additional cases. In this group of children with developmental language disturbances at least two distinct types seem to emerge. A group in which the already existing developmental language disturbances did not fluctuate during the course of the illness and a group in which a considerable deterioration of the developmentally disturbed language functions took place. The different patterns of language evolution not only seem to suggest the possibility of an acquired language pathology within a context of developmental language disturbances but also a congenital onset of the syndrome of Landau-Kleffner as the probable cause of developmental language pathology.- - - - - - - - - - ranking = 6keywords = nature (Clic here for more details about this article) |