Finger gnosia: a predictor of numerical abilities in children?

This paper aimed to test the specificity of predicting power of finger gnosia on later numerical abilities in school-age children and to contribute to the understanding of this effect. Forty-one children were tested in the beginning of Grade 1 on finger gnosia, left-right orientation (another sign of the Gerstmann "syndrome"), and global development. Fifteen months later, numerical and reading abilities were assessed. Analyses of the results indicated that, contrary to the general measures of cognitive development, performance in the finger gnosia test was a good predictor of numerical skills 1 year later but not of reading skills, which proves the specificity of that predictor. The same conclusion was also true for the left-right orientation. However, finger gnosia could equally predict performance in numerical tasks that do or do not rely heavily on finger representation or on magnitude representation. Results are discussed in terms of the localizationist and the functional hypotheses.     

Reflections on the Gerstmann syndrome.

There has been much debate about whether or not the Gerstmann aggregate is a syndrome in any meaningful sense. Some investigators have marshalled evidence suggesting that the syndrome is an artifact of selective observation and that its components are merely particular expressions of aphasic disorder or mental impairment. Other investigators have attacked the design and logic of these negative studies and have contended that the syndrome is an index of focal brain disease. In this paper, aspects of the early history of the syndrome that are relevant to the question are reviewed, the nature of the components of the syndrome is discussed from a cognitive standpoint, and the findings and logic of recent major studies dealing with the question are analyzed. On this basis, the neuropsychological significance of the syndrome is assessed and some persisting questions are considered.     

Disorders of oculomotor scanning and graphic orientation in developmental Gerstmann syndrome

A patient with developmental Gerstmann syndrome who experiences great difficulty in performing tasks requiring visuoconstructive and reading abilities is described. Neuropsychological assessment revealed normal range verbal abilities but severe deficits in directional orientation, finger differentiation, calculational ability, copying, and reading ability. Achievement in reading was measured at the sixth-grade level despite the fact that this patient is a third-year college student. Using infrared photoelectric sensors, we monitored the horizontal saccades of this patient while reading from a standardized reading test. Fixation durations were not abnormal. However, instances of reverse-staircase movement were exhibited. While reading from an inverted test, she showed a normal eye-movement pattern (although in the reverse direction since the task now called for right-to-left saccades). The processing of spatial information in this patient is severely disturbed and this may account for the apparently abnormal oculomotor scanning, an “irrepressible tendency” to move her eyes in a right-to-left direction.     

Agraphia selective for written spelling : An experimental case study

A 56-year-old right-handed man suffered left posterior parieto-temporal ischemia leading to mild aphasia, Gerstmann syndrome, and a novel variant of agraphia. This variant compromised his spelling by writing and manual sorting of letters more than his oral spelling. The dissociation was experimentally documented. It principally involved the intrusion of extraneous letters, independent of input modality. It did not generalized to numbers or an arbitrary code. Postoperatively the disability disappeared. It was concluded that the programs which translate letter choice into visual terms for purposes of written (as distinct from oral) spelling either originate or are transmitted in a distinct cerebral location. This location, which may be the left posterior parasagittal parietal area, can be selectively impaired by a focal lesion.     

Finger gnosia: a predictor of numerical abilities in children?

This paper aimed to test the specificity of predicting power of finger gnosia on later numerical abilities in school-age children and to contribute to the understanding of this effect. Forty-one children were tested in the beginning of Grade 1 on finger gnosia, left–right orientation (another sign of the Gerstmann “syndrome”), and global development. Fifteen months later, numerical and reading abilities were assessed. Analyses of the results indicated that, contrary to the general measures of cognitive development, performance in the finger gnosia test was a good predictor of numerical skills 1 year later but not of reading skills, which proves the specificity of that predictor. The same conclusion was also true for the left–right orientation. However, finger gnosia could equally predict performance in numerical tasks that do or do not rely heavily on finger representation or on magnitude representation. Results are discussed in terms of the localizationist and the functional hypotheses.     

Right hemispheric dysfunction in nonverbal learning disabilities: social, academic, and adaptive functioning in adults and children

This review addresses recent research on social and nonverbal learning disabilities. Involvement of right hemispheric dysfunction in these disabilities has been hypothesized, as studies with adults have suggested that documented right hemisphere damage may lead to deficits in social skills, prosody, spatial orientation, problem solving, and recognition of nonverbal cues. Studies of children purported to evidence nonverbal learning disabilities are reviewed and compared with the results from studies of adults with right hemisphere damage. Specific subtypes of nonverbal learning disabilities are reviewed, including the nonverbal perceptual-organization-output subtype, Asperger's Syndrome, Developmental Gerstmann Syndrome, left hemisyndrome, right hemisphere syndrome, and right parietal lobe syndrome. Finally, implications and future research needs are addressed. The need for a diagnostic nosology and improved and validated intervention techniques is stressed as is early identification of these types of specific nonverbal learning disabilities.     

Acalculia and Dyscalculia

Even though it is generally recognized that calculation ability represents a most important type of cognition, there is a significant paucity in the study of acalculia. In this paper the historical evolution of calculation abilities in humankind and the appearance of numerical concepts in child development are reviewed. Developmental calculation disturbances (developmental dyscalculia) are analyzed. It is proposed that calculation ability represents a multifactor skill, including verbal, spatial, memory, body knowledge, and executive function abilities. A general distinction between primary and secondary acalculias is presented, and different types of acquired calculation disturbances are analyzed. The association between acalculia and aphasia, apraxia and dementia is further considered, and special mention to the so-called Gerstmann syndrome is made. A model for the neuropsychological assessment of numerical abilities is proposed, and some general guidelines for the rehabilitation of calculation disturbances are presented.     

Deep dysphasia: An analog of deep dyslexia in the auditory modality

A right-handed patient, with two left hemisphere lesions, a small one in the prefrontal lobe and a larger one in the temporal, presents an unusual syndrome: a massive deficit for oral language (expression and comprehension) contrasting with a fairly good preservation of written language (expression and comprehension). The processing of isolated words and sentences has been extensively tested with repetition and dictation tasks. The patient performs rather well with nouns, verbs, and adjectives, poorly with adverbs and function words, and completely fails with nonsense words. A remarkable feature of his repetition is the frequency of semantic paraphasias. Thus, this patient exhibits a behavior rather similar to deep dyslexia, hence the possible label "deep dysphasia." The paper presents a "preunderstanding" hypothesis to account for such behaviors.     

Williams syndrome: a genetic deletion disorder presenting clues to the biology of sociability and clinical challenges of hypersociability

Williams syndrome is a neurodevelopmental disorder that results from the deletion of approximately 25-30 genes spanning about 1.5 megabases in the q11.23 region of chromosome 7. Patients with this syndrome present with a combination of a distinctive elfin-like facial appearance; growth retardation; mild mental retardation; an inconsistent cognitive profile that includes visuospatial impairments with good facial discrimination and relatively preserved expressive language skills; and cardiovascular abnormalities. In addition, a striking behavioral feature of the syndrome is the high sociability and empathy that these patients show for others. The study of patients with "partial" deletions of the chromosome band 7q11.23, mutated genes in this region and knockout mice with deletions of specific genes in the homologous G1-G2 region of mouse chromosome 5 are clarifying some genotype/phenotype relationships. Furthermore, genes located in this region that are prominently expressed have been implicated in brain development and function. The neuropsychological profile of patients with Williams syndrome is heterogeneous, highlights important dissociations between cognitive functions and suggests that the behavioral dimensions of sociability, empathy, engageability, and talkativeness may be independent of, or not easily explained by, the cognitive deficits. Williams syndrome has enormous heuristic value because its pathological feature of heightened "sociability" can be a "deficit" symptom of major complex neuropsychiatric disorders, such as schizophrenia and autism. Data consistent with a core inability of patients with Williams syndrome to inhibit social approach suggest that this disorder may afford an opportunity to study the biological basis of the "drive" toward socialization. From a research perspective, the syndrome lends itself to neurobiological studies of sociability as a dimension that varies independently of cognition (or at least many separable cognitive processes). Importantly, from a clinical perspective, the syndrome challenges us to administer strategic psychosocial interventions that take advantage of the opportunities that "pathological" sociability provide, while avoiding its threats. An illustrative example of an effective strategically planned psychosocial intervention for a patient with Williams syndrome is briefly presented.     

Multiple object tracking in people with Williams syndrome and in normally developing children

Multiple object tracking is hypothesized to utilize visual indexes, which may provide rapid, parallel access to a limited number of visual objects, thereby supporting a variety of spatial tasks. We examined whether faulty indexing might play a role in the severe visuospatial deficits found in Williams syndrome. We asked observers to track from one to four targets in a display of eight identical objects. Objects remained stationary (static condition) or moved randomly and independently (moving condition) for 6 s, after which observers pointed to the objects they thought were targets. People with Williams syndrome were impaired in the moving condition, but not the static condition, compared with mental-age-matched control participants. Normal children who were younger than the mental-age-matched control children did not show the same profile as individuals with Williams syndrome, which suggests that the difference between the tasks in Williams syndrome did not reflect simple developmental immaturity. Error analysis revealed that all groups had "slippery" indexes, falsely identifying target neighbors, and further suggested that people with Williams syndrome deploy fewer indexes than do people without this disorder.     

Surface dyslexia: A regression effect?

Although the disorder of acquired surface dyslexia is considered as a pathological syndrome in the psycholinguistic and neuropsychological literature, it may also be conceived of as part of a general regression in functioning consequent upon brain damage. Data on some of the identified features of surface dyslexia were obtained from normal children learning to read and adult subjects, in order to examine the hypothesis that the symptoms are similar to the behavior of unskilled readers, suggesting regression as a more parsimonious explanation for the disorder. Attention is also drawn to problems in quantifying its major feature--the "regularity" effect.     

Hierarchical planning disability in the drawings and constructions of a special group of severely aphasic children

A small group of severely receptive aphasic children who appear to be suffering from a rare aphasic syndrome that has been referred to in the literature as "acquired aphasia with convulsive disorder in children" was tested on drawing and construction tasks which tap hierarchical planning abilities. Although they could draw and construct a complex figure when using a serial method, they could not do so when required to use hierarchical planning in building up the model in terms of its subunits. Their scores reflecting hierarchical ability were significantly lower than those of age-matched profoundly deaf and normal children. The nature of this disability is discussed and its effects noted on tasks in other modalities. It is argued that neither an auditory deficit theory nor a short-term memory deficit theory is adequate to account for the observed disabilities. Individual differences in the group were noted. It was also found that a smaller percentage of developmentally expressive aphasic children evidenced the hierarchical planning disability. It is concluded that a primary hierarchical planning disability in some aphasic children can account for some aspects of their linguistic disabilities and for disabilities on other nonlanguage and nonauditory tasks.     

Attitudes of Mothers of Children with Down Syndrome Towards Noninvasive Prenatal Testing

Noninvasive prenatal testing (NIPT) allows for highly sensitive detection of Down syndrome early in pregnancy with no risk of miscarriage, therefore potentially increasing the number of pregnancies identified with Down syndrome. This study assesses how mothers of children with Down syndrome perceive NIPT, especially the impact they think it will have on their families and other families with children who have Down syndrome. Seventy-three self-reported mothers of children with Down syndrome responded to an anonymous online survey emailed to, and posted on, message boards of various Down syndrome support groups and networks. Data analysis included chi-square tests and thematic analysis. Fifty-nine percent of respondents indicated they would use NIPT in the future; respondents who had not used prenatal testing in the past were significantly less likely to report interest in using NIPT in the future than those who had prenatal testing previously (p?MOST important factor. They also highlight the need to provide a diagnosis of Down syndrome in a balanced and objective manner.     

Parents attributions of blame for the birth of a child with down syndrome: A pilot study

Abstract

Attributing blame to others for negative events is consistently associated with poor adjustment. We report here a study assessing whether failure to detect a problem on screening, in this case Down syndrome not detected during prenatal screening, is associated with blame and poor adjustment in parents. Twenty eight mothers and 23 fathers of children with Down syndrome were interviewed. Although no parent blamed themselves or their partner, eight fathers and five mothers blamed health professionals or the health care system in general for not preventing the birth of their children with Down syndrome prenatally. None of the parents who declined testing were blaming. Six of the 11 parents who received a negative test and seven of the 34 not offered a test blamed others. Both mothers and fathers who blamed others reported significantly higher parenting stress. Mothers, but not fathers, who blamed others were significantly more angry and depressed than those who did not.     

Depressive syndromes within the scope of schizophrenic diseases

The results of a standardized examination of 135 schizophrenics shows that the "depressive factor" is less pronounced during differential neuroleptic therapy (Clozapin, Haloperidol) and in the control group of schizophrenics receiving no drug treatment. This suggests that the depressive syndrome among schizophrenics is not manifested as a result of pharmaceutical action. The author proposes that neuroleptic therapy tailored to the patient can ameliorate the depressive syndrome observed in schizophrenics. Both psychoreactive and disease specific factors may play a role in the pathogenesis of depression among patients with this disease.     

"Who should survive?: one of the choices on our conscience": mental retardation and the history of contemporary bioethics

The film "Who Should Survive?: One of the Choices on Our Conscience" contains a dramatization of the death of an infant with Down syndrome as the result of the parents' decision not to have a congenital intestinal obstruction surgically corrected. The dramatization was based on two similar cases at The Johns Hopkins Hospital and was financed by the Joseph P. Kennedy, Jr., Foundation. When "Who Should Survive?" was exhibited in 1971, the public reaction was generally critical of the parents' decision and the physicians' inaction. Although technological developments in medicine were a necessary condition for the production of this film and its unanticipated reception, they were not a sufficient condition. The proximate cause was a changed understanding of the capabilities of individuals with Down syndrome. Part of the impetus for this change was data showing the adverse effects of institutionalization on normal children.     

Situation-Behavior Profiles as a Locus of Consistency in Personality

Traditional approaches have long considered situations as "noise" or "error" that obscures the consistency of personality and its invariance. Therefore, it has been customary to average the individual's behavior on any given dimension (e.g., conscientiousness) across different situations. Contradicting this assumption and practice, recent studies have demonstrated that by incorporating the situation into the search for consistency, a new locus of stability is found. Namely, people are characterized not only by stable individual differences in their overall levels of behavior, but also by distinctive and stable patterns of situation-behavior relations (e.g., she does X when A but Y when B ). These if . . . then . . . profiles constitute behavioral "signatures" that provide potential windows into the individual's underlying dynamics. Processing models that can account for such signatures provide a new route for studying personality types in terms of their shared dynamics and characteristic defining profiles.     

Can Inattention/Overactivity Be an Institutional Deprivation Syndrome?

Elevated rates of attention deficit and overactivity have been noted previously in samples of institution-reared children. This study examined the hypothesis that inattention/overactivity(I/O) might constitute a specific deprivation syndrome. One hundred and sixty five children adopted at varying ages (e.g., 0–42 months of age) into the UK following severe early deprivation were compared with 52 within-UK adoptees who did not suffer deprivation. The children were rated by teachers and parents on levels of I/O, conduct difficulties, and emotional difficulties using the Revised Rutter Scales. Data were collected at age 6 for the entire sample and at age 4 for the UK adoptees and for the subsample of Romanian children who entered the UK before the age of 2 years. Mean level analyses suggested a significant effect of duration of deprivation on I/O, but not on conduct or emotional difficulties. The effects of duration of deprivation were specific to I/O and were not accounted for by low birth weight, malnutrition, or cognitive impairment. Levels of I/O correlated with attachment disturbances. Furthermore, the effects of duration of deprivation on I/O did not attenuate over time. We conclude that I/O may well constitute an institutional deprivation syndrome, but that the type of attention deficit and overactivity exhibited by these children may present a different clinical picture from that of ordinary varieties of attention deficit disorder or hyperkinetic syndrome.     

Comparisons of second-generation holocaust survivors, immigrants, and nonimmigrants on measures of mental health

To determine whether a "children-of-holocaust-survivors syndrome" could more parsimoniously be explained as an "immigration effect," we performed a comparative study on a sample of 25 children of holocaust survivors, 25 children of immigrants, and 25 children of American-born parents. Subjects were matched on age and educational level and were assessed with four measures of mental health. Contrary to the findings of some researchers whose data supported such a syndrome, our data indicate no significant differences between children of holocaust survivors and the children of other immigrants. These data are suggestive of an immigration effect that is common to the children of immigrants and not limited to the children of holocaust survivors, rather than a survivors syndrome. The children of American-born parents showed greater alienation, less religiosity, and a tendency toward feelings of less guilt than the other two groups. Our data underscore the need to control immigrant status in multiple samples and studies before definitive conclusions can be drawn in this area.