Developmental Gerstmann Syndrome Research Articles

Mirror Visual Feedback for Right–Left Discrimination in a Patient With Developmental Gerstmann Syndrome

Mirror Visual Feedback for Right–Left Discrimination in a Patient With Developmental Gerstmann Syndrome

A case of developmental Gerstmann's syndrome

A case of developmental Gerstmann's syndrome

SÍNDROME DE GERSTMANN DEL DESARROLLO Y TRASTORNOS EN LA ADQUISICIÓN DE LAS MATEMÁTICAS

La tétrada caracterizada por una dificultad en el conocimiento práxico de los dedos, indistinción derecha/izquierda, discalculia y trastornos de la escritura (síndrome de Gerstmann) ha sido descrita en el desarrollo, probablemente vinculada a una perturbación madurativa del gyrus angular de manera bilateral o hemisférica derecha. En este trabajo se presentan tres casos con los tres elementos obligados de la tétrada y en un solo caso una perturbación vinculada a los niveles grafémico y ortográfico de la escritura. Dentro de la heterogeneidad de los casos, la perturbación en el dominio de las matemáticas se configura como una anaritmetia, caracterizada en base a un trastorno nuclear en la representación semántica del número. Se realizan las consideraciones teóricas acerca de la relación entre el conocimiento somestésico digital y la capacidad de representarse un espacio regularmente asimétrico, con la adquisición de las facultades matemáticas.

Does finger training increase young children's numerical performance?

Butterworth (1999) suggested that fingers are important in representing numerosities. Furthermore, scores on a finger gnosis test are a better predictor of numerical performance up to 3 years later than intellectual measures (Marinthe et al., 2001; Noël, 2005). We hypothesised that training in finger differentiation would increase finger gnosis and might also improve numerical performance. Accordingly, 47 first-grade children were selected and divided into 3 groups: children with poor finger gnosis who followed the finger-differentiation training programme (G1), a control-intervention who were trained in story comprehension (G2), and a group with high finger gnosis scores who just continued with normal school lessons (G3). The finger training consisted of 2 weekly sessions of half an hour each, for 8 weeks. Before the training period, children in G3 performed better in finger gnosis and enumeration than children in the two other groups. After the training period this pattern remained for the children in G2 and G3, but the children in G1 were significantly better than those in G2 at finger gnosis, representation of numerosities with fingers, and quantification tasks; they also tended to be better at the processing of Arabic digits. These results indicate that improving finger gnosis in young children is possible and that it can provide a useful support to learning mathematics. Such an approach could be particularly appropriate for children with a developmental Gerstmann syndrome. Theoretically, these results are important because they suggest a functional link between finger gnosis and number skills.

What ever happened to developmental Gerstmann's syndrome? Links to other pediatric, genetic, and neurodevelopmental syndromes.

Developmental Gerstmann's syndrome is a neurodevelopmental disorder infrequently described in the literature. The limited literature might result from controversy surrounding developmental Gerstmann's syndrome as a "true syndrome." Developmental Gerstmann's syndrome requires a tetrad of symptoms: left-right confusion, finger agnosia, dyscalculia, and dysgraphia, with constructional dyspraxia often included as a fifth symptom. The etiology of developmental Gerstmann's syndrome is unknown, but several hypotheses have been proposed, and none have been conclusively confirmed. Based on the paucity of recent research on developmental Gerstmann's syndrome, individuals who meet the criteria for the disorder could be given other diagnoses. A clustering of neuropsychologic features across other seemingly related disorders suggests that the conceptualization of the tetrad of symptoms traditionally associated with developmental Gerstmann's syndrome more appropriately reflects soft signs that are commonly associated with a number of other neurodevelopmental disorders. Thus, although developmental Gerstmann's syndrome is of historical interest to neurodevelopmental specialists, there appears to be no basis for considering this disorder as a unique syndrome.

Developmental cognitive neuropsychology of number processing and calculation: varieties of developmental dyscalculia

This article provides a brief overview about the current state of cognitive developmental neuropsychology of developmental dyscalculia (DD) as well as results from a Zurich study that investigates different subtypes of DD according to various aspects of numerical abilities that are impaired or preserved. The differential effects of impairments of one particular numerical area on the development of other numerical abilities are highlighted in the case of a 17 year old boy with severe DD and Developmental Gerstmann Syndrome. A comprehensive model of developmental dynamics of number processing and calculation abilities will be proposed in the last section with respect to the development of intelligence theory.

Developmental Gerstmann syndrome: case report and review of the literature

The tetrad of finger agnosia, dyscalculia, dysgraphia, and right-left confusion constitutes the Gerstmann syndrome (GS). A case of developmental Gerstmann syndrome (DGS) that occurred in a normal, highly intelligent child with exceptional reading skills is reported, together with a review of the literature. DGS occurs in both brain-damaged and seemingly normal children. Multiple neurological and behavioral manifestations coexisting with the Gerstmann elements suggest brain injury, whereas the occurrence of the Gerstmann tetrad (plus constructional apraxia) in an otherwise normal and intelligent child implies what is herein referred to as constitutional. The scarcity of reported cases indicates the rarity of the syndrome in children. Routine testing for the Gerstmann elements in learning-disabled children may uncover unrecognized cases.

Developmental Gerstmann's syndrome

Developmental Gerstmann's syndrome

Developmental Gerstmann's syndrome

Developmental Gerstmann's syndrome

Developmental Dyscalculia and Medical Assessment

Developmental dyscalculia (DC) is a primary cognitive disorder of childhood manifested by disturbance of arithmetic ability. As an isolated learning disability (LD), it is usually treated by remedial education and not referred for further medical evaluation. We examined a group of 7 third-grade children with DC attending a mainstream school who had not progressed academically in spite of specific special education intervention. We were able to identify in all 7 children neurological conditions that had direct bearing on the children's cognitive disabilities and remedial programs. One child had petit mal seizures, another developmental Gerstmann syndrome, a third had dyslexia for numbers, and 4 children had attention deficit disorders without hyperactivity. Based on this experience, we suggest that the indications for medical or neurological assessment be broadened to include children who are not improving academically in spite of appropriate professional intervention.

Right hemisphere 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.

Neuropsychological dysfunction among affected heterozygous fragile X females

Fragile X (or Martin-Bell) syndrome is an X-linked disorder that often produces mental retardation in males, but usually affects heterozygous females to a lesser degree. Here we report the results of a brief neuropsychological examination of 20 heterozygous fra(x) girls and women and two control groups of 20 individuals each. One control group was composed of fra(x)-negative mothers (obligate carriers) and sisters of male probands with fra(x) syndrome, whereas the other was composed of 14 head-injured and six learning disabled women and girls. In addition to general intellectual impairment, several specific cognitive deficits were consistently found in individuals with the Martin-Bell syndrome, suggesting focal neuropsychological dysfunction. Significant differences were noted between fra(x) individuals and controls on most cognitive and neuropsychological measures studied. Over one-third of the fra(x) individuals demonstrated neuropsychological symptoms characteristic of the full developmental Gerstmann syndrome, whereas another third had three or four of the five signs of possible parietal lobe dysfunction. In our sample, there was an association between improved performance and increasing age. Differences among heterozygous individuals in number of focal symptoms may reflect some variability in the penetrance of the fra(x) gene, as well as in the functional organization of the brain.

Developmental Gerstmann Syndrome

The tetrad of finger agnosia, dyscalculia, dysgraphia, and right-left confusion constitutes the Gerstmann syndrome (GS). A case of developmental Gerstmann syndrome (DGS) that occurred in a normal, highly intelligent child with exceptional reading skills is reported, together with a review of the literature. DGS occurs in both brain-damaged and seemingly normal children. Multiple neurological and behavioral manifestations coexisting with the Gerstmann elements suggest brain injury, whereas the occurrence of the Gerstmann tetrad (plus constructional apraxia) in an otherwise normal and intelligent child implies what is herein referred to as "constitutional." The scarcity of reported cases indicates the rarity of the syndrome in children. Routine testing for the Gerstmann elements in learning-disabled children may uncover unrecognized cases.

Developmental Gerstmann syndrome without aphasia in Fragile X syndrome

Considerable controversy surrounds Gerstmann's syndrome, with some authors questioning its localizing significance, and others doubting its existence as a distinct entity. This literature is reviewed, and a case is presented of a young boy with Fragile X syndrome who showed all five symptoms of the developmental Gerstmann syndrome. He was one of a group of individuals found to be cytogenetically positive for Fragile X syndrome, who were observed to possess three or more of Gerstmann's symptons in the complete absence of aphasic disorder.

Dyscalculia and Elements of the Developmental Gerstmann Syndrome in School Children

Fourteen (14) dyscalculic school children were drawn from a larger population of learning-disabled children. The subjects were divided into two groups, those with normal-or-better reading ability and those with dyslexia equal in degree to their dyscalculia. Both groups showed a variety of behavioral deficits in addition to those comprising Gerstmann's syndrome and were notably poor in auditory and visual discrimination and motor coordination. Good readers showed severely-impaired ability to make right-left discriminations, while the poor readers were average in this ability. Poor readers showed marked impairment of word fluency and hand writing, while good readers were average in this regard. The dyscalculia and reading deficits reported here appear unrelated to central-language impairment. Subjects with all four elements of the developmental Gerstmann's syndrome did not constitute an homogeneous behavioral group and were found among samples of both good and poor readers. The pattern of behavioral deficits shown by these subjects suggests cerebral impairment rather than slow maturation as a probable etiology. While the DGS is not useful as a behavioral description, its value as a possible localizing neurological sign cannot yet be ruled out.

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.

Rotation tendency and cerebral dysfunction in children.

Rotational errors in tests such as Bender's Visual-motor Gestalt Test or Benton's Visual Retention Test have been accepted in individual diagnostics of children as an indication of an organic disturbance. A series of experiments were designed to test the hypothesis that the tendency to rotate in perceptual tests would be characteristic of children with a suspected or confirmed brain injury. The tendency to rotate figures was measured with different types of perceptual tests. Besides Benton's test and Bender's Gestalt test, newly constructed tests were employed to measure the rotational tendency quantitatively and more objectively. The tasks were made more complicated by presenting geometrical figures tachistoscopically or by requiring intersensory communication for solving the test task. Experimental groups referred for an EEG examination on the suspicion of an organic disturbance in the majority of experiments did not display a greater tendency to rotate figures than control groups; nor did a pathological EEG attend an increased rotational tendency. Younger children committed more rotational errors than older children. Children exhibiting a high rotational tendency, compared with the remaining children, more often had concentration difficulties according to the teachers' ratings; however, hyperactive behavior or motor difficulties were not more frequent. Nor did they have a greater tendency, based on the teachers' rating, to transpose, reverse, or to rotate letters or digits. No relationship between the tendency to rotate and IQ, measured with the WISC, was noted. Individuals with right-left discrimination difficulties committed rotational errors more often on Bender's test. The hypothesis that individuals with right-left discrimination difficulties would also exhibit finger agnosia, dyslexia, and dysgraphia at a high frequency (Developmental Gerstmann's Syndrome) was not confirmed.