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A diagnostic profile of gerstmann'ssyndrome: a case studyH. Naudé a & E. Pretorius ba Department of Educational Psychology, University of Pretoria,Pretoria, 0001, South Africa;b PO Box 2034, Department of Anatomy, University of Pretoria,Pretoria, 0001, South AfricaVersion of record first published: 03 Jun 2010.

To cite this article: H. Naudé & E. Pretorius (2003): A diagnostic profile of gerstmann's syndrome: acase study, Early Child Development and Care, 173:5, 519-526

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

Early Child Development and Care, 2003, Vol. 173(5), pp. 519–526

*Corresponding author: Tel: + 27 12 319 2533; Fax: + 27 12 319 2240, Email: rpretori@medic.up.ac.za

A Diagnostic Profile of Gerstmann’sSyndrome: A Case StudyH. NAUDEa and E. PRETORIUSb*

aDepartment of Educational Psychology, University of Pretoria, Pretoria, 0001, South Africa; bPO Box2034, Department of Anatomy, University of Pretoria, Pretoria, 0001, South Africa

(Received 19 April 2003)

The authors present a diagnostic profile of Gerstmann’s syndrome, the subject being a girl, aged 10 years 3months, right-handed, and at the time of this study in her fourth school year (Grade 4). The subject’s verbaland non-verbal IQ scores and additional assessment data were analyzed and summative responses given. TheSenior South African Individual Scale – Revised was administered to determine the intellectual functioningof the subject. A qualitative analysis of the results revealed average intellectual functioning on both the verbaland full scales. The non-verbal scale revealed low average intellectual functioning. The difference betweenthe verbal scale IQ and the non-verbal scale IQ was statistically significant at the 5% level of reliability. Basedon the scatter analysis it is evident that the subject experienced difficulties with regards to numericalreasoning, handwriting, spatial disorientation and difficulty in mental manipulation of images, as associatedwith Gerstmann’s syndrome.

Keywords: Gerstmann’s syndrome; Finger agnosia; Dysgraphia; Dyscalculia; Right–left disorientation

INTRODUCTION

Gerstmann’s syndrome was first described in 1930 by Gerstmann (1930, 1957), and is adisorder of cognition, characterized by the tetrad of finger agnosia, dysgraphia, dyscalculia,and right–left disorientation (Naude and Pretorius, 2002). This syndrome can either be adevelopmental syndrome (infrequently described in children with learning disability)(PeBenito, 1987; PeBenito, Fisch, and Fisch, 1988) or an acquired syndrome. AcquiredGerstmann’s syndrome has been ascribed to lesions to the left frontal posterior area of thebrain (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 angulargyrus, which extended into the supramarginal gyrus and minimally into the superior parietallobule, left angular gyrus, situated at the confluence of the temporal, parietal and occipitallobes (Foumier del Castillo et al., 2000), and second occipital convolution (Mayer, Martory,Pegna, Landis, Delavelle, and Annoni (1999).

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As mentioned by Naude and Pretorius (2002), Gerstmann’s syndrome manifests as limbor 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; Struband Geschwind, 1974). Suresh and Sebastian (2000) mentioned that early identificationand intervention is very important. Naude and Pretorius (2002) proposed an instructionaldelivery framework for children with Gerstmann’s syndrome but, based on collegialinquiry, the need for a diagnostic profile of Gerstmann’s syndrome became evident. Theauthors therefore present a case study of a child diagnosed by a neurologist as acquiredGerstmann’s syndrome.

MATERIALS AND METHODS

A case study was conducted where the verbal and non-verbal IQ scores and additionalassessment data concerning a child with Gerstmann’s syndrome (age 10 years 3 months)were analyzed and summative responses given. The parents of the child gave informedconsent.

Background

The subject is a girl, aged 10 years 3 months, right-handed, and at the time of the study inher fourth school year (Grade 4). The parents requested placement in a school for neurallydisabled children, because since entering school she has never mastered numerical reasoningto such a degree that warrants retention.

The pregnancy was complicated by placenta previa and, due to pre-partum bleeding, themother had been hospitalized since the 32nd week of gestation. The baby was born in the36th week of pregnancy by means of a caesarian. The birth weight of 2.57 kgs was slightlybelow normal range and the baby developed a serious degree of jaundice. The subjectreached her developmental milestones somewhat later than normally expected, with theexception of language development. The physical milestones were generally below expectednorms. At the age of 3 years she experienced a single epileptic seizure and, following thisincident, she was treated with Tegretol for 6 months.

Exemption from compulsory school attendance was granted and the subject commencedGrade One the following year. According to her teacher she suffered from attention deficitdisorder, and a general practitioner prescribed daily treatment with Ritalin (10 mg). Becauseof reported chronic fatigue and poor muscle tone, the subject received occupational therapyfor the following 2 years. Her muscle tone was found to be lowered, which affected themaintenance of an upright posture at a desk. Crossing the midline of the body and bilateralmotor co-ordination presented as problems, with the subject making postural adjustments toavoid crossing the midline. Visual memory and visual spatial relations were lowered and thesubject experienced difficulty when copying diagrams and figures. Perseveration anddifficulty drawing intersecting lines were noted. Pencil grip improved with therapy, butneeded correction at times as the subject tended to resort to an immature pencil grip and poorcontrol. She was referred for neurological assessment.

Neurological Report

Still in her first school year the subject’s 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 tooriginate from abnormalities in locations of the brainstem portion of the brain, althoughthe seizures can also originate from localized areas on the cortical surface. The subjectwas consequently treated with 400 mg Epilum daily. The physical examination showed herscull to be 50.8 cm in circumference, which equalled the 20th percentile. She weighed22 kgs.

In addition to pharmacological treatment, the subject was placed in an aid class (full-timeremedial class) during her second school year. Despite remedial intervention, her very poornumerical skills persisted. During her fourth grade the teacher suggested that she might belearning disabled, since she seemed to be successful at reading but had some difficulty withwriting and struggled particularly with perceptual-motor tasks and numerical reasoning. AnEEG recording was repeated and found to be normal. Medication was terminated. Eventuallyshe was referred for psychological assessment.

Measuring Instruments used in this Study

The Senior South African Individual Scale – Revised (SSAIS-R) (Van Eeden, 1992a) wasadministered to determine the intellectual functioning of the subject. The SSAIS-R has beenstandardized for South African children between the ages of 7 years 0 months and 16 years11 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 IntelligenceScale for Children – Revised (WISC-R) (Wechsler, 1974) and various other authors’descriptions (Claassen, 1990; Groth-Mamat, 1984; Kaufman and Kaufman, 1977; Madge andVan der Westhuizen, 1982; Sattler, 1982; Van Eeden, 1988; Wechsler, 1958). The SSAIS-Ris used to obtain a differential image of certain cognitive abilities. First the level of generalintelligence is determined, and second relative strengths and weaknesses in certain importantfacets of intelligence are evaluated to obtain diagnostic and prognostic information. The twoprimary mental abilities measured by the SSAIS-R are a verbal and a non-verbal factor interms of Thurstone’s model. These factors are related to different mental processes anddifferent test contents (Wechsler, 1958, 1974). The total score for the subtests of theintelligence scale (the Full Scale or Composite Scale) represents the broader, underlyingfactor of general intelligence (g). The battery, consisting of a number of relativelyindependent subtests, makes calculating a general intelligence score as well as an analysis ofthe performance on the different subtests possible. The subtests can be divided into twocategories on the basis of the item content. The verbal subtests are Vocabulary,Comprehension, Similarities, Number Problems, Story Memory, and Memory for Digits (thelatter 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 theverbal, non-verbal and full (composite) scales. Performance is indicated in terms of standardscores or normalized scaled scores. “If a point scale is used, the same intelligence test can beapplied throughout to all age groups and the meaning of a particular IQ score remainsconstant from one age group to the next. An individual’s scores on tests or a group of tests,for example the scores obtained on verbal and non-verbal tests, can also be compared withone another” (Van Eeden, 1992a).

Additional standardized and diagnostic assessment media, namely the Bender Visual-Motor Gestalt Test, the Quick Neurological Screening Test (1989), the Basic NumberScreening Test (Form B) (Gillham and Hesse, 1976), the Informal Diagnostic MathematicsTest 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 slowwork tempo. She tended to ignore her mistakes, and to gloss over items with which she haddifficulty. Syntax, grammar, comprehension, articulation, and other aspects of her speech andlanguage were essentially normal. She revealed an inability to reason numerically and herhandwriting was of very poor quality, resulting in slow classroom performance. Theassessment data revealed acalculia, dysgraphia, left–right spatial disorientation, and a milddegree of finger agnosia.

Intellectual Functioning

The SSAIS-R was administered and the results are as follows: verbal scale, 100; non-verbalscale, 87 (performance scale); and full scale, 93.

A qualitative analysis of the results revealed average intellectual functioning on both theverbal 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 statisticallysignificant at the 5% level of reliability (Van Eeden, 1992b). Her performance IQ (indicativeof non-language development) was in the mid-80s range. This score was indicative of herslow early milestone development and general poor classroom performance. The SSAIS-Rresults, which revealed thirteen-point superiority of verbal over performance IQ, suggestsuperiority of receptive language (subtests Vocabulary, Comprehension, Similarities, StoryMemory) over constructional praxis scores (subtests Pattern Completion, Block Designs,Form Board), supporting consistent superiority of left-hemisphere functioning. Thesefindings 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 indicatedby 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 wasfound to be significantly lower than her average performance on the verbal and full scales.A poor performance on subtest Number Problems suggests impaired numerical reasoningwith poor underlying logical reasoning and abstract thought. The subject thus revealed poorability to solve number problems and poor understanding of basic mathematicalcomputations; namely, addition, subtraction, multiplication and division. Despite adequatelanguage development, she revealed impaired ability to extract mathematical concepts froma verbal background (story sums), especially due to poor conceptualization of mathematical“words” and left–right temporal–spatial disorientation. During assessment she functioned atconcrete 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, specificbacklogs 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 wassignificantly lower than her verbal functioning at the 1% and 5% level of reliability (VanEeden, 1992a), with the exception of the subtest Missing Parts. Her performance on thesubtest Missing Parts suggested average ability to distinguish between essential and non-essential visual information. Jointly with her relatively intact verbal abilities, intact visualperception might thus be seen as one of her strengths. To the contrary, her performance onthe subtest Pattern Completion revealed poor spatial orientation at two-dimensional levelsand 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. Thesubject therefore revealed poor non-verbal concept formation, including perceptualorganization, spatial visualization and orientation, and abstract conceptualization, especiallyat three-dimensional levels. The results also suggested poor visual–motor integration andcoordination. The subject’s poor performance on the subtest Form Board suggested poorability to see the underlying relations between objects, as well as impaired visual–motorcoordination, where the motor activity is directed by visual perception and sensory-motorfeedback (Van Eeden, 1992a). The latter could also be the residual effect of mild fingeragnosia, as revealed by results of the Quick Neurological Screening Test (1989).

These results, in addition to the supporting hypothesis of functional superiority of the lefthemisphere, suggest that, while both anterior and posterior portions of the right hemisphereare depressed when compared with the left hemisphere, there may be slightly less impairmenton posterior portions of the right hemisphere than on anterior portions. Specifically, thepurely receptive functions (i.e., posterior functions of the right hemisphere, reflected inmeasures of the relatively motor-free Missing Parts [WISC-R = Picture Completion]) showslightly less comparative weakness than those hemisphere functions with an executorycomponent (e.g., Pattern Completion, Block Designs, Form Board). This indicates slightlyless perceptual than visual–motor–integrative impairment (Hartlage and Reynolds, 1981).Both her classroom and assessment behavioral data are compatible with depressed righthemisphere functioning. The subject was probably erroneously diagnosed with attentiondeficit disorder due to her inattentiveness and impulsiveness, and was therefore treated withRitalin. According to Hartlage and Reynolds (1981), carelessness, messy written work,impulsiveness, and perceptual–motor problems are fairly common behaviors in normalclassroom students with marginal chronic deficiencies of right cerebral hemispheredevelopment. Furthermore, poor monitoring of their own behavior is a very commonbehavioral trait reported in children with less efficient right-hemisphere functioning,independent of level of general intellectual ability (as suggested by the full scale of theSSAIS-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) thesubject performed well below her developmental age and current grade. With regards toaddition and subtraction she functioned at grade two levels (8 years age equivalent). Thesubject was unable to do multiplication and division. According to the Standardized DiagnosticReading Test (Grade 4) (Nieuwoudt, 1992) the subject’s reading skills equaled her scholasticlevel (i.e., Grade 4), although according to her chronological age her reading skills wereslightly lowered at 9 years 5 months. According to the results of the Bender Visual-MotorGestalt Test the subject functioned at 6 years age equivalent. Difficulties with the Benderdesigns 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 angleexecution, rotation, faulty integration and closure. Children who perform poorly with theBender Visual–Motor Gestalt Test often have difficulty with mathematics and with the correctplacing of numbers in mathematics. According to the results of the Quick NeurologicalScreening 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 slowwork tempo, as associated with Gerstmann’s syndrome.

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DISCUSSION

Based on the preceding results it is evident that the subject experienced difficulties withregards to numerical reasoning, handwriting, spatial disorientation and difficulty in mentalmanipulation of images, as associated with Gerstmann’s syndrome (Mayer et al., 1999). Asmentioned previously, the subject was born prematurely, and neonatally suffered from highlevels of unconjugated bilirubin, which affects the basal ganglia and possibly also thehippocampus and the central parts of the cerebellum (Johnston and Hoon, 2000). Lesions tothese structures might account for the subject’s chronic fatigue and attention deficits. Apartfrom the major role that the parieto-temporo-occipital dysfunction plays in visual–motorintegration functioning on subtest Bock Designs, it is postulated that poor cerebellum–cortexintegration and damage to the Angular Gyrus be considered etiological factors in the subject’sacalculia, dysgraphia, and left–right spatial disorientation. Also considering the fact that notall neurons were fully developed at the time of the subject’s premature birth, myelination wasstill actively taking place during the perinatal phase. It is thus postulated that areas of thebrain 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 isbecause the myelin sheath that should provide protection is to a great extent still absent inpremature babies (Kapp, 1990).

Since these vulnerable association areas were exposed to possible damage due to highlevels of unconjugated bilirubin, it might account for the subject’s variety of learningdifficulties including Gerstmann’s syndrome. These association areas are functionallyresponsible for body awareness, spatial localization, abstract and complex intellectualfunctions of mathematical reasoning, and logical propositions that have their basis invisuospatial thinking (Obrzut, 1981). The latter could possibly be tied to her apparent lack ofmathematical vocabulary such as inside, bigger, more, less, counting backwards, countingon, as well as her inability to understand concepts such as borrowing and carrying over. Itis thus essential that her conceptualization of mathematical vocabulary be expanded andstrengthened by activities that stimulate visuospatial thinking. According to Hynd and Obrzut(1981) left-sided dysfunction of the parieto-temporo-occipital lobe leads to defects inprogramming or ordering of movements necessary for constructional activity, associated withthe subject’s poor performance on the subtests Block Designs and Form Board, as well ashandwriting skills. “Writing deficits can occur with lesions limited to either the temporal orparietal lobe, but severity increases when there is damage to the parietal and any one of theother three lobes” (Obrzut, 1981). Right-sided defects in these association areas lead tovisuo-spatial constructional difficulty, associated with poor performance on the subtestsPattern Completion and Block Designs. The subject revealed poor performance at both two-dimensional and three-dimensional levels. Hynd and Obrzut (1981) postulated that puzzleconstruction in two-dimensional and three-dimensional space might be affected by bothright-hemisphere and left-hemisphere dysfunction of these association areas. It is thushypothesized that the subject revealed lesions of both the right and left association areas, assuggested by the EEG that was conducted during her first year of formal schooling. Becauseof lesions to the left parieto-temporo-occipital area, it is essential that symbol-processing andleft–right directional sense be enhanced and strengthened during intervention. Right-sidedlesions generally show up on written calculations and an inability to manipulate numbers inspatial relationships (Hynd and Obrzut, 1981).

In addition to subcortical suppression, the medial area of the prefrontal lobe also mediatesshort-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 fourbasic 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 solvenumber problems is closely linked with error detection, which is mediated by the anteriorcingulate girus (as imbedded in the prefrontal lobe) (Restak, 2000, p. 70). Lesions to theanterior 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 thatacquired Gerstmann’s syndrome is particularly associated with depressed right-hemispherefunctioning. The purely receptive functions (i.e., posterior functions of the right hemisphere,reflected in measures of the relatively motor-free Missing Parts [WISC-R = PictureCompletion]) show slightly less comparative weakness than those hemisphere functions withan executory component (e.g., Pattern Completion, Block Designs, Form Board). Thisindicates slightly less perceptual than visual–motor–integrative impairment. AcquiredGerstmann’s syndrome manifests itself as dysgraphia, dyscalculia, and spatial disorientation,associated with impaired parieto-temporal-occipital and prefrontal lobe functioning.Acquired Gerstmann’s syndrome may be accompanied by abnormal EEG activity and, inaddition to impaired anterior cingulate gyrus functioning, it may result in poor selectiveattention, and impaired error detection and problem-solving. The latter is indicative of poorproblem-solving and constructional praxis, particularly associated with spatial disorientationand an inability to regulate one’s own learning behavior.

Because this syndrome is characterized by the possible involvement of so many differentbrain areas, it is essential that a thorough neurological investigation be embarked upon,accompanied by a psychological assessment. The authors do not suggest that the diagnosticprofile of the subject in this study will fit all learners with Gerstmann’s syndrome, butfindings might be used as guidelines to identify such learners.

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