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Early Child Development and Care

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A diagnostic profile of gerstmann's syndrome: a case study

H. Naud & E. Pretorius
a a b

Department of Educational Psychology, University of Pretoria, Pretoria, 0001, South Africa;

b

PO Box 2034, Department of Anatomy, University of Pretoria, Pretoria, 0001, South Africa Version of record first published: 03 Jun 2010.

To cite this article: H. Naud & E. Pretorius (2003): A diagnostic profile of gerstmann's syndrome: a case study, Early Child Development and Care, 173:5, 519-526 To link to this article: http://dx.doi.org/10.1080/0300443032000086935

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Early Child Development and Care, 2003, Vol. 173(5), pp. 519526

A Diagnostic Profile of Gerstmanns Syndrome: A Case Study

a and E. PRETORIUSb* H. NAUDE
Department of Educational Psychology, University of Pretoria, Pretoria, 0001, South Africa; bPO Box 2034, Department of Anatomy, University of Pretoria, Pretoria, 0001, South Africa (Received 19 April 2003)
a

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The authors present a diagnostic profile of Gerstmanns syndrome, the subject being a girl, aged 10 years 3 months, right-handed, and at the time of this study in her fourth school year (Grade 4). The subjects verbal and non-verbal IQ scores and additional assessment data were analyzed and summative responses given. The Senior South African Individual Scale Revised was administered to determine the intellectual functioning of the subject. A qualitative analysis of the results revealed average intellectual functioning on both the verbal and full scales. The non-verbal scale revealed low average intellectual functioning. The difference between the verbal scale IQ and the non-verbal scale IQ was statistically significant at the 5% level of reliability. Based on the scatter analysis it is evident that the subject experienced difficulties with regards to numerical reasoning, handwriting, spatial disorientation and difficulty in mental manipulation of images, as associated with Gerstmanns syndrome. Keywords: Gerstmanns syndrome; Finger agnosia; Dysgraphia; Dyscalculia; Rightleft disorientation

INTRODUCTION Gerstmanns syndrome was first described in 1930 by Gerstmann (1930, 1957), and is a disorder of cognition, characterized by the tetrad of finger agnosia, dysgraphia, dyscalculia, and rightleft disorientation (Naud e and Pretorius, 2002). This syndrome can either be a developmental syndrome (infrequently described in children with learning disability) (PeBenito, 1987; PeBenito, Fisch, and Fisch, 1988) or an acquired syndrome. Acquired Gerstmanns syndrome has been ascribed to lesions to the left frontal posterior area of the brain (Brusa, Rossi, and Tartarini, 1960), the left parietal, temporal and occipital lobe (Fournier del Castillo, Garcia-Penas, Gutierrez-Solana, and Rulz-Falco Rojas, 2000; Heimburger, Demyer, and Reitan, 1964; Suresh and Sebastian, 2000), the left thalamic area (Santos, Cope, Keller, and DeLong, 1991), the right parietal area in left-handed patients (Dehaene and Cohen, 1997; Dozono, Hachisuka, Ohnishi and Ogata 1997; Moore, Saver, Johnston, and Romero, 1991), the left parietal area in right-handed patients (Mazzoni, Pardossi, Cantini, Giorgetti, and Arena, 1990), focal cortical lesion of the superior angular gyrus, which extended into the supramarginal gyrus and minimally into the superior parietal lobule, left angular gyrus, situated at the confluence of the temporal, parietal and occipital lobes (Foumier del Castillo et al., 2000), and second occipital convolution (Mayer, Martory, Pegna, Landis, Delavelle, and Annoni (1999).
*Corresponding author: Tel: + 27 12 319 2533; Fax: + 27 12 319 2240, Email: rpretori@medic.up.ac.za

ISSN 0300-4430 print; ISSN 1476-8275 online 2003 Taylor & Francis Ltd DOI: 10.1080/0300443032000086935

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As mentioned by Naude and Pretorius (2002), Gerstmanns syndrome manifests as limb or constructional apraxia and visual or sensory right-sided deficits (Dehaene and Cohen, 1997; Dozono et al., 1997; Kinsbourne and Warrington, 1962; Mazzoni et al., 1990; Strub and Geschwind, 1974). Suresh and Sebastian (2000) mentioned that early identification and intervention is very important. Naude and Pretorius (2002) proposed an instructional delivery framework for children with Gerstmanns syndrome but, based on collegial inquiry, the need for a diagnostic profile of Gerstmanns syndrome became evident. The authors therefore present a case study of a child diagnosed by a neurologist as acquired Gerstmanns syndrome.

MATERIALS AND METHODS

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A case study was conducted where the verbal and non-verbal IQ scores and additional assessment data concerning a child with Gerstmanns syndrome (age 10 years 3 months) were analyzed and summative responses given. The parents of the child gave informed consent. Background The subject is a girl, aged 10 years 3 months, right-handed, and at the time of the study in her fourth school year (Grade 4). The parents requested placement in a school for neurally disabled children, because since entering school she has never mastered numerical reasoning to such a degree that warrants retention. The pregnancy was complicated by placenta previa and, due to pre-partum bleeding, the mother had been hospitalized since the 32nd week of gestation. The baby was born in the 36th week of pregnancy by means of a caesarian. The birth weight of 2.57 kgs was slightly below normal range and the baby developed a serious degree of jaundice. The subject reached her developmental milestones somewhat later than normally expected, with the exception of language development. The physical milestones were generally below expected norms. At the age of 3 years she experienced a single epileptic seizure and, following this incident, she was treated with Tegretol for 6 months. Exemption from compulsory school attendance was granted and the subject commenced Grade One the following year. According to her teacher she suffered from attention deficit disorder, and a general practitioner prescribed daily treatment with Ritalin (10 mg). Because of reported chronic fatigue and poor muscle tone, the subject received occupational therapy for the following 2 years. Her muscle tone was found to be lowered, which affected the maintenance of an upright posture at a desk. Crossing the midline of the body and bilateral motor co-ordination presented as problems, with the subject making postural adjustments to avoid crossing the midline. Visual memory and visual spatial relations were lowered and the subject experienced difficulty when copying diagrams and figures. Perseveration and difficulty drawing intersecting lines were noted. Pencil grip improved with therapy, but needed correction at times as the subject tended to resort to an immature pencil grip and poor control. She was referred for neurological assessment. Neurological Report Still in her first school year the subjects EEG record revealed a petit mal seizure type, where the EEG pattern is characterized by a spike and dome pattern with a frequency

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of about three repetitions per second. The petit mal seizure type is usually considered to originate from abnormalities in locations of the brainstem portion of the brain, although the seizures can also originate from localized areas on the cortical surface. The subject was consequently treated with 400 mg Epilum daily. The physical examination showed her scull to be 50.8 cm in circumference, which equalled the 20th percentile. She weighed 22 kgs. In addition to pharmacological treatment, the subject was placed in an aid class (full-time remedial class) during her second school year. Despite remedial intervention, her very poor numerical skills persisted. During her fourth grade the teacher suggested that she might be learning disabled, since she seemed to be successful at reading but had some difficulty with writing and struggled particularly with perceptual-motor tasks and numerical reasoning. An EEG recording was repeated and found to be normal. Medication was terminated. Eventually she was referred for psychological assessment.

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Measuring Instruments used in this Study The Senior South African Individual Scale Revised (SSAIS-R) (Van Eeden, 1992a) was administered to determine the intellectual functioning of the subject. The SSAIS-R has been standardized for South African children between the ages of 7 years 0 months and 16 years 11 months with Afrikaans or English as their mother tongue. The SSAIS-R is a point scale (deviation IQ scale) and is based on research data derived from the Wechsler Intelligence Scale for Children Revised (WISC-R) (Wechsler, 1974) and various other authors descriptions (Claassen, 1990; Groth-Mamat, 1984; Kaufman and Kaufman, 1977; Madge and Van der Westhuizen, 1982; Sattler, 1982; Van Eeden, 1988; Wechsler, 1958). The SSAIS-R is used to obtain a differential image of certain cognitive abilities. First the level of general intelligence is determined, and second relative strengths and weaknesses in certain important facets of intelligence are evaluated to obtain diagnostic and prognostic information. The two primary mental abilities measured by the SSAIS-R are a verbal and a non-verbal factor in terms of Thurstones model. These factors are related to different mental processes and different test contents (Wechsler, 1958, 1974). The total score for the subtests of the intelligence scale (the Full Scale or Composite Scale) represents the broader, underlying factor of general intelligence (g). The battery, consisting of a number of relatively independent subtests, makes calculating a general intelligence score as well as an analysis of the performance on the different subtests possible. The subtests can be divided into two categories on the basis of the item content. The verbal subtests are Vocabulary, Comprehension, Similarities, Number Problems, Story Memory, and Memory for Digits (the latter being optional). The non-verbal subtests are: Pattern Completion, Block Designs, Missing Parts, Form Board, and Coding (the latter being optional). The standard deviation method is used to calculate norms for each subtest and for the verbal, non-verbal and full (composite) scales. Performance is indicated in terms of standard scores or normalized scaled scores. If a point scale is used, the same intelligence test can be applied throughout to all age groups and the meaning of a particular IQ score remains constant from one age group to the next. An individuals scores on tests or a group of tests, for example the scores obtained on verbal and non-verbal tests, can also be compared with one another (Van Eeden, 1992a). Additional standardized and diagnostic assessment media, namely the Bender VisualMotor Gestalt Test, the Quick Neurological Screening Test (1989), the Basic Number Screening Test (Form B) (Gillham and Hesse, 1976), the Informal Diagnostic Mathematics Test Battery (Naude and Van Wyk, 1996) and the Standardized Diagnostic Reading Test (Grade 4) (Nieuwoudt, 1992) were also administered.

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RESULTS Clinical Observation During the psychological assessment the subject revealed poor attention and a very slow work tempo. She tended to ignore her mistakes, and to gloss over items with which she had difficulty. Syntax, grammar, comprehension, articulation, and other aspects of her speech and language were essentially normal. She revealed an inability to reason numerically and her handwriting was of very poor quality, resulting in slow classroom performance. The assessment data revealed acalculia, dysgraphia, left right spatial disorientation, and a mild degree of finger agnosia. Intellectual Functioning The SSAIS-R was administered and the results are as follows: verbal scale, 100; non-verbal scale, 87 (performance scale ); and full scale, 93. A qualitative analysis of the results revealed average intellectual functioning on both the verbal and full scales. The non-verbal scale revealed low average intellectual functioning. The difference between the verbal scale IQ and the non-verbal scale IQ was statistically significant at the 5% level of reliability (Van Eeden, 1992b). Her performance IQ (indicative of non-language development) was in the mid-80s range. This score was indicative of her slow early milestone development and general poor classroom performance. The SSAIS-R results, which revealed thirteen-point superiority of verbal over performance IQ, suggest superiority of receptive language (subtests Vocabulary, Comprehension, Similarities, Story Memory) over constructional praxis scores (subtests Pattern Completion, Block Designs, Form Board), supporting consistent superiority of left-hemisphere functioning. These findings are compatible with a chronic functional inferiority of the right hemisphere, specifically in the region of the parieto-temporo-occipital lobes and frontal lobes, as indicated by analysis of the subtest scatter. Statistically significant subtest scatter was found on the verbal and full scales at the 1% and 5% levels of reliability. With regards to subtest Number Problems, her performance was found to be significantly lower than her average performance on the verbal and full scales. A poor performance on subtest Number Problems suggests impaired numerical reasoning with poor underlying logical reasoning and abstract thought. The subject thus revealed poor ability to solve number problems and poor understanding of basic mathematical computations; namely, addition, subtraction, multiplication and division. Despite adequate language development, she revealed impaired ability to extract mathematical concepts from a verbal background (story sums), especially due to poor conceptualization of mathematical words and left right temporal spatial disorientation. During assessment she functioned at concrete and semi-concrete levels of reasoning, and constantly needed concrete objects (i.e., counters and fingers) to assist her. Because of her overall low performance on the non-verbal (performance) scale, specific backlogs did not show up as subtest scatter on the non-verbal scale, but on the full scale (composite scale). On the full scale her performance on all non-verbal subtests was significantly lower than her verbal functioning at the 1% and 5% level of reliability (Van Eeden, 1992a), with the exception of the subtest Missing Parts. Her performance on the subtest Missing Parts suggested average ability to distinguish between essential and nonessential visual information. Jointly with her relatively intact verbal abilities, intact visual perception might thus be seen as one of her strengths. To the contrary, her performance on the subtest Pattern Completion revealed poor spatial orientation at two-dimensional levels and poor visual organizational functioning. Her performance on the subtest Block Designs

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revealed impaired ability to solve problems in spatial relations using logical reasoning. The subject therefore revealed poor non-verbal concept formation, including perceptual organization, spatial visualization and orientation, and abstract conceptualization, especially at three-dimensional levels. The results also suggested poor visual motor integration and coordination. The subjects poor performance on the subtest Form Board suggested poor ability to see the underlying relations between objects, as well as impaired visual motor coordination, where the motor activity is directed by visual perception and sensory-motor feedback (Van Eeden, 1992a). The latter could also be the residual effect of mild finger agnosia, as revealed by results of the Quick Neurological Screening Test (1989). These results, in addition to the supporting hypothesis of functional superiority of the left hemisphere, suggest that, while both anterior and posterior portions of the right hemisphere are depressed when compared with the left hemisphere, there may be slightly less impairment on posterior portions of the right hemisphere than on anterior portions. Specifically, the purely receptive functions (i.e., posterior functions of the right hemisphere, reflected in measures of the relatively motor-free Missing Parts [WISC-R = Picture Completion]) show slightly less comparative weakness than those hemisphere functions with an executory component (e.g., Pattern Completion, Block Designs, Form Board). This indicates slightly less perceptual than visual motor integrative impairment (Hartlage and Reynolds, 1981). Both her classroom and assessment behavioral data are compatible with depressed right hemisphere functioning. The subject was probably erroneously diagnosed with attention deficit disorder due to her inattentiveness and impulsiveness, and was therefore treated with Ritalin. According to Hartlage and Reynolds (1981), carelessness, messy written work, impulsiveness, and perceptual motor problems are fairly common behaviors in normal classroom students with marginal chronic deficiencies of right cerebral hemisphere development. Furthermore, poor monitoring of their own behavior is a very common behavioral trait reported in children with less efficient right-hemisphere functioning, independent of level of general intellectual ability (as suggested by the full scale of the SSAIS-R). Scholastic Functioning According to the results of the Basic Number Screening Test (Form B) (Gillham and Hesse, 1976) and the Informal Diagnostic Mathematics Test Battery (Naude and Van Wyk, 1996) the subject performed well below her developmental age and current grade. With regards to addition and subtraction she functioned at grade two levels (8 years age equivalent). The subject was unable to do multiplication and division. According to the Standardized Diagnostic Reading Test (Grade 4) (Nieuwoudt, 1992) the subjects reading skills equaled her scholastic level (i.e., Grade 4), although according to her chronological age her reading skills were slightly lowered at 9 years 5 months. According to the results of the Bender Visual-Motor Gestalt Test the subject functioned at 6 years age equivalent. Difficulties with the Bender designs are likely to appear in subjects with parietal lobe lesions (Garron and Cheifetz, 1965; Obrzut, 1981). The subject experienced difficulties concerning distortion of shape, faulty angle execution, rotation, faulty integration and closure. Children who perform poorly with the Bender Visual Motor Gestalt Test often have difficulty with mathematics and with the correct placing of numbers in mathematics. According to the results of the Quick Neurological Screening Test (1989) the subject did not meet the expected standards with regards to subtest 1 (hand skill), subtest 2 (figure recognition and production), subtest 3 (palm form recognition), subtest 6 (finger to nose), and subtest 8 (double simultaneous stimulation of hand and cheek). Especially, subtests 3 and 6 may point to mild finger agnosia, resulting in dysgraphia and slow work tempo, as associated with Gerstmanns syndrome.

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DISCUSSION Based on the preceding results it is evident that the subject experienced difficulties with regards to numerical reasoning, handwriting, spatial disorientation and difficulty in mental manipulation of images, as associated with Gerstmanns syndrome (Mayer et al., 1999). As mentioned previously, the subject was born prematurely, and neonatally suffered from high levels of unconjugated bilirubin, which affects the basal ganglia and possibly also the hippocampus and the central parts of the cerebellum (Johnston and Hoon, 2000). Lesions to these structures might account for the subjects chronic fatigue and attention deficits. Apart from the major role that the parieto-temporo-occipital dysfunction plays in visual motor integration functioning on subtest Bock Designs, it is postulated that poor cerebellum cortex integration and damage to the Angular Gyrus be considered etiological factors in the subjects acalculia, dysgraphia, and left right spatial disorientation. Also considering the fact that not all neurons were fully developed at the time of the subjects premature birth, myelination was still actively taking place during the perinatal phase. It is thus postulated that areas of the brain that myelinate last (those areas in the Angular Gyrus and the area where the temporal, parietal, and occipital lobes meet) were extensively exposed to possible damage. This is because the myelin sheath that should provide protection is to a great extent still absent in premature babies (Kapp, 1990). Since these vulnerable association areas were exposed to possible damage due to high levels of unconjugated bilirubin, it might account for the subjects variety of learning difficulties including Gerstmanns syndrome. These association areas are functionally responsible for body awareness, spatial localization, abstract and complex intellectual functions of mathematical reasoning, and logical propositions that have their basis in visuospatial thinking (Obrzut, 1981). The latter could possibly be tied to her apparent lack of mathematical vocabulary such as inside, bigger, more, less, counting backwards, counting on, as well as her inability to understand concepts such as borrowing and carrying over. It is thus essential that her conceptualization of mathematical vocabulary be expanded and strengthened by activities that stimulate visuospatial thinking. According to Hynd and Obrzut (1981) left-sided dysfunction of the parieto-temporo-occipital lobe leads to defects in programming or ordering of movements necessary for constructional activity, associated with the subjects poor performance on the subtests Block Designs and Form Board, as well as handwriting skills. Writing deficits can occur with lesions limited to either the temporal or parietal lobe, but severity increases when there is damage to the parietal and any one of the other three lobes (Obrzut, 1981). Right-sided defects in these association areas lead to visuo-spatial constructional difficulty, associated with poor performance on the subtests Pattern Completion and Block Designs. The subject revealed poor performance at both twodimensional and three-dimensional levels. Hynd and Obrzut (1981) postulated that puzzle construction in two-dimensional and three-dimensional space might be affected by both right-hemisphere and left-hemisphere dysfunction of these association areas. It is thus hypothesized that the subject revealed lesions of both the right and left association areas, as suggested by the EEG that was conducted during her first year of formal schooling. Because of lesions to the left parieto-temporo-occipital area, it is essential that symbol-processing and left right directional sense be enhanced and strengthened during intervention. Right-sided lesions generally show up on written calculations and an inability to manipulate numbers in spatial relationships (Hynd and Obrzut, 1981). In addition to subcortical suppression, the medial area of the prefrontal lobe also mediates short-term memory (Brenner, 2000, p. 165; Newport and Nemeroff, 2000, p. 216; Restak, 2000, p. 130). Impaired short-term memory particularly results in problems with the four basic operations because the leaner does not have the rote knowledge or has difficulty with

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memorizing specific algorithms, tables, and place values. Furthermore, the ability to solve number problems is closely linked with error detection, which is mediated by the anterior cingulate girus (as imbedded in the prefrontal lobe) (Restak, 2000, p. 70). Lesions to the anterior cingulate gyrus usually result in an impaired ability to recognize and solve problems, as well as impaired attention.

CONCLUSION In addition to research findings, and based on the preceding information, it seems that acquired Gerstmanns syndrome is particularly associated with depressed right-hemisphere functioning. The purely receptive functions (i.e., posterior functions of the right hemisphere, reflected in measures of the relatively motor-free Missing Parts [WISC-R = Picture Completion]) show slightly less comparative weakness than those hemisphere functions with an executory component (e.g., Pattern Completion, Block Designs, Form Board). This indicates slightly less perceptual than visual motor integrative impairment. Acquired Gerstmanns syndrome manifests itself as dysgraphia, dyscalculia, and spatial disorientation, associated with impaired parieto-temporal-occipital and prefrontal lobe functioning. Acquired Gerstmanns syndrome may be accompanied by abnormal EEG activity and, in addition to impaired anterior cingulate gyrus functioning, it may result in poor selective attention, and impaired error detection and problem-solving. The latter is indicative of poor problem-solving and constructional praxis, particularly associated with spatial disorientation and an inability to regulate ones own learning behavior. Because this syndrome is characterized by the possible involvement of so many different brain areas, it is essential that a thorough neurological investigation be embarked upon, accompanied by a psychological assessment. The authors do not suggest that the diagnostic profile of the subject in this study will fit all learners with Gerstmanns syndrome, but findings might be used as guidelines to identify such learners. References
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