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~~uropsychologlo.Vol. 28.No. 3.pp.309-312. 1990. Prmtedm GreatBntam.

NOTE TOPOGRAPHIC

DISORIENTATION-A

CASE REPORT

i;.BOTTINI,*~~S. CAPPA,~ G. GEMINIANI: and R. STERZI~

‘Istituto di Clinica Brescia, Italy; fIstituto

Neurologica Neurologico

dell’Universita di Milano, Italy; Wlinica Neurologica “C. Besta”, Milano, Italy and §Divisione Neurologica, CB Granda, Milano, Italy

dell’Universit8 di Ospedale Niguarda

(Received 22 December 1988: accepted 4 September 1989) Abstract-Topographical disorientation can be dissociated in two levels, agnosic and amnesic. A case of topographical disorientation due to a glioma of the splenium of corpus callosum illustrates the dissociation between the topographical memory impairment and the normal performances on the perceptual topographic tests.

INTRODUCTION TOPOGRAPHIC disorientation is a clinical condition in which a patient cannot remember or follow well-known itineraries, or learn new ones [S, 73. Usually this condition is found in the clinical context of a non-specific cognitive impairment, such as an acute confusional state, or in association with other neuropsychological disorders, as in dementia. Unilateral neglect may also cause a particular kind of spatial disorientation, characterized by failure to attend to the stimuli coming from the hemifield contralateral to the side of the cerebral lesion. Although rare, selective topographic amnesia and topographic agnosia are two specific disorders of spatial orientation, which occur following localized cerebral lesions. A case of topographic amnesia, due to a glioma of the splenium of corpus callosum, may illustrate the differences between these last two aspects of topographic disorientation.

CASE REPORT V.B. was a 72-year-old, right-handed, retired man, formerly a clergyman, with 11 years of education. He was referred to our department because 2 weeks before, while driving his car, he suddenly realized he was unable to recognize the streets along his way home. He got lost and had to call on some passer-by to help. Since then the patient has been severely disoriented in space. On admission to the hospital he was awake and co-operating. He correctly reported the date of the day, though he mistook the year. Bedside language evaluation was normal. Neurological examination revealed left homonymous hemianopia, slight weakness and extinction of double simultaneous stimulation in the left side of the body. The CT scan showed a bilateral median and right paramedian hypodense lesion centered on the splenium of the corpus callosum at basal examination, which became hyperdense after contrast medium infusion. ASPECT failed to reveal any asymmetry of cortical perfusion. A diagnosis of corpus callosum glioblastoma was made on the basis of the CT scan findings. The patient’s clinical condition worsened during the following days and he died 1 month after admission.

NEUROPSYCHOLOGICAL

EVALUATION

At the time of the first examination the patient was fully co-operating and oriented to day, month and year. Though he was unable to recall his age, he remembered his date of birth. He made some mistakes on simple mental

/[Correspondence to be addressed to: Dr Gabriella Bottini, Padiglione Ponti, Via F. Sforza n. 35, 20100 Milano, Italy. 309

Istituto

di Clinica Neurologica,

Milan0

University,

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calculations. He correctly answered questions concerning the cause of admission to the hopital. could adequate]) recognize family members and the attending doctors, and was able to give extensive autobiographic information. His general behaviour was not indicative of dementia, and his affect was fully appropriate. During the following weeks the patient’s condition deteriorated steadily, so that 15 days after admission it was impossible to submit him to a thorough examination. We report the neuropsychological evaluation performed during the first days. when the patient was fully co-operating. Test results are summarized in Table 1.

Table 1. Test scores Corsi’s Span Corsi’s Supraspan Digit Span Paired Associates Learning Short Story Recall Token test Raven’s PM Benton’s Line Orientation Face Recognition Visual Form Discrimination *Abnormal

criterion

4 not reached after 20 trials* 6 6* 3* 30,‘36 18:36 17; 36: 38.‘54 24;‘32

results.

On language examination the patient had fluent speech with normal oral comprehension. nammg and repetition. The Token test score was normal (30136). No motor apraxia was evident on detailed testing. Copying and spontaneous drawing were characterized by a general preservation of the global configuration of figures with omission. displacement and distortion of details. No signs of unilateral neglect were evident in specific test. as line bisection, cancellation tasks and other visual and tactile exploration tests. There was no left-right confusion when the patient was asked to use or to point to a part of his body or to an object in external space. On BENTO& Judgement of Line Orientation test [3] he obtained a score slightly below normal. He had no evidence of visual and tactile agnosia. colour agnosia, or finger agnosia. He was not impaired in the Visual Form Judgement test nor on the Facial Recongnition test [3]. No callosal disconnection symptoms were evident on detailed testing in the tactile modality. He obtained a score of 18136 in Raven’s PM 47, in the lower normal range for his age and education [2]. Verbal memory evaluation was indicative of normal short-term memory and defective learning and recall, with a severeley impaired performance on two standardized tests similar to the Logical Memory and Paired Associates Learning of the Wechsler Memory Scale [S]. In spatial memory tests, the patient had a normal Corsi’s spatial span whereas he failed to learn a 2-supraspan string after 20 trials. He also was unable to describe the map of his apartment or the well-known routes he used everyday, for instance to reach the department store or the church. When he tried to recall them, he did so with confabulating answers and by hanging on to verbal tag. such as the name of some well known street. He was unable to describe the Piazza Duomo of Milan, the city where he lived: his description was extremely vague, characterized by very general statements (“it is rectangular”: “there is a monument in the middle”) and by an apparent lack of recall of specific buildings, Including the Duomo itself. On the other hand, he promptly recognized the Piazza among different photographic choices (other squares in Milan). In the hospital. the patient could not find the way back to his room from the toilet or from the examination office and he was unable to recognize his bed among the eight in his room. He was unable to point the way to his room from the examination office. A severe constructional deficit was evident when the patient tried to draw maps. such as the map of Piazza Duomo, of the hospital department or of his home. The patient described correctly the furniture of the room where he was. but when he had to recall it with eyes closed he failed. He was unable to indicate the correct position ofsome important Itahan towns on a blank map. The patlent was submitted to a ALEXANDER and MONEY’S maze [I]. In this test the patient must indicate the correct direction (“left” or “right”) at every change of direction on a way with 32 turning points. He made only four mistakes. A test similar to the SEMMES’Map [12] was also administered. In this test 3 rows of 3 black discs (10 cm dia.) were placed on the floor of the room at a distance of 110 cm from each other. The subject was given a piece of paper reproducmg the position of the discs among which a zigzag way was drawn. The patient had to follow the itinerary without turning the map. He performed well as long as he could go forward. When the map required a turning greater than 180‘ the patient lost his ability to follow the itinerary. To avoid the contrast between the real situation (the discs on the floor) and the map, the patient was put on a wheelchair and passively moved forwards and backwards at his order without any rotation. In this condition his performance greatly improved.

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To test his ability to cope with the Mental Rotation task. an experiment was devised using abstract figures similar to the itinerary of the previous test as stimuli. The patient was required to judge if two presented stimuli were the same or mirror-reversed. The probe was presented under three different degrees of rotation (0”. 90. and 180’). When the probe and the stimuli were in the same position (0’ condition) the patient correctly identified the figures, but in the two other conditions he constantly failed.

DISCUSSION Topographical disorientation, as other neuropsychological disturbances, can be dissociated in two levels. topographical agnosia and topographical amnesia. The first, agnosic,level is manifested as the inability to recognize visual information which is relevant for topographic orientation, as specific buildings or other environmental “landmarks” (shops, street signs, landscape elements): recognition of the general category is usually preserved. In other cases, belonging to the same (agnosic) level, the main problem is a defective appreciation of spatial relationships between the correctly identified environmental stimuli. The second, amnesic level can be defined as the inability to recognize or to recall (verbally or by drawing a map) a previously known itinerary and to learn new ones. We suggest that also at this “memory” level it is often possible to identify two different aspects of impairment. Some patients are particularly defective in remembering and learning spatial relationships while in other cases the problem appears to involve the memory for specific landmarks. A review of the literature and our case provide evidence for these dissociations, even if different testing procedures often make the comparison between case reports difficult. The main features of the cases described by PALLIS [lo] can be ascribed to a topographical agnosia with a failure to identify the landmarks. The patient complained of an inability to recognize places whereas his spatial memory was spared and he was able to recall the position of the streets and the location ofshops. He could conjure up images of some squares and streets and draw or describe a map of the itinerary to his home, but when he had to find his way in a real situation he got lost, as his ability to recognize specific landmarks was impaired. He could tell at a glance the differences between a council house and a detached villa, or between a country lane and a main route, but was unable to recognize a specific building. Associated with this disturbance he presented achromatopsia and prosopagnosia. The association between topographical agnosia for landmarks with prosopagnosia is further supported by some of the cases reported by LANDIS [9]. An example of the other aspect of topographic agnosia is the case described by HBCAEN [7]. In a testing situation the patient was able to identify an itinerary drawn on a map only if the spatial relationship were clearly identified by different “artificial” landmarks (e.g. line drawings of common objects). He failed when he had to rely only on the spatial relationship between identical landmarks. The two levels of topographic amnesia could be exemplified by the cases of WHITHELEY and WARRINGTON [13] and by those of DE RENZI and FAGLIONI [6] and of SCOTTI [ll]. WHITHELEY and WARRINGTON [13] described a case of pure topographical amnesia for landmarks after a traumatic cerebral lesion. The patient had a selective deficit of memory for previously unknown buildings. Conversely he was able to match the same stimuli in perceptual spatial tasks. DE RENZI and FAGLIONI[6] observed a patient with a memory deficit for positions and a similar case was described by SCOTTI[II]. These two patients were unable to recall the arrangement of the furniture of the examination room after closing their eyes or to point to the newspaper column they had been reading if the paper was removed for a few seconds. Within this framework, the main aspects of the clinical picture of our patient could be ascribed to a topographical amnesia. This view is supported by his overall preserved or mildly impaired performances on visuo-perceptual tests involving recognition of objects, faces, buildings and spatial relationship, as in the ALEXANDERand MONEY’S[l] maze. On the contrary, he was unable to describe the map of his apartment or well known routes of his town. Moreover he could not learn new simple itineraries. That his memory for spatial relationships was severely impaired is further supported by his failure to learn 2 supraspan string on Corsi’s Spatial test. Our data do not allow to eliminate the possibility of an impairment of the memory for landmarks as the recognition memory of unfamiliar building was not tested because of the deterioration of the patient’s condition. The disturbance of our patient could hardly be ascribed to confusion or metal deterioration given his preserved performance in most neuropsychological tests. Our patient had a constructional apraxia, characterized in copying and spontaneous drawing tasks by preservation of the global configuration of the figures with omission, displacement and distortion ofdetails. He had also a mild mental rotation impairment, but normal performance on visual form discrimination tests; judgement of line orientation was only mildly impaired. This pattern of results argues against an interpretation of the topographical disorientation in terms of a general visuo-spatial disturbance. A review of the anatomical correlates of topographical disorientation [S] indicates a greater incidence of this symptom after right posterior cerebral lesions, the deficits being more severe when the hippocampus and the parahippocampal structures are involved. Neoplastic lesions are not will suited for clinical-anatomic correlation, especially in the case of rapidily progressive tumours as in the present case. The CT scan diagnosis was of a

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glioblastoma of the splenium of the corpus callosum. extending to the paramcdlan areas of the right temporal lobe. The presence of a left hemianopia is consistent with extension of the iehlun of the lesion to the posterior region of the right hemisphere; on the other hand the absence of disconnection symptoms points to a limited callosal involvement. The associated verbal memory disturbance, less severe than the spatial amnesia. could also be ascribed to extension to the controlateral hippocampus.

REFERENCES 1. ALEXANDER. D. and MONEY, J. Turner’s syndrome and Gerstmann’s syndrome: neuropsychological comparisons. Neuropsychologia 4, 265-273, 1966. 2. BASSO, A., CAPITANI. E. and LAIACONA, M. Raven’s coloured progressive matrices: normative values on 305 adult normal controls. Functional Neurology 2, 189-194, 1987. Contribution co .~eeurops!cllological 3. BENTON, A. L.. HAMSHER, K. DES., VARNEY, N. R. and SPREEN, 0. Assessment. a CLinical Manual. Oxford University Press, New York. 1983. 4. BYRNE, R. W. Geographical knowledge and orientation. In Normality and Pathology in Cognirioe Funcrion. A. ELLIS (Editor), pp. 239-264. Academic Press, New York, 1980. 5. DE RENZI, E. Disorders of Space Exploration and Cognition. Academic Press, New York, 1983. spaziale da lesione cerebrate. Sisrema Nerroso 14,409-436. 6. DE RENZI, E. and FAGLIONI, P. 11disorientamento 1962. memory without learning deficits. Cortex 16, 7. H~CAEN, H., TZORTZIS, C. and RONDOT, P. Loss of topographical 525-542, 1980. 8. NOVELLI, C., PAPAGNO, C., CAPITANI, E., LAIACONA, M., CAPPA, S. F. and VALLAR. G. Tre test clinici di memmoria verbale a lunge termine: taratura su soggetti normali. Archicio di Neurologia Psicologia Psichiatria 41, 278-296, 1986. familiarity 9. LANDIS, T., CUMMINGS, J. L., BENSON, F. D. and PRAATHER PALMER. E. Loss of topographic environmental agnosia. Archs Neural. 43, 132-136, 1986. of faces and places with agnosia for colors. Report of a case due to cerebral 10. PALLIS C. A. Impaired identification embolism. J. Nemo/. Neurosurg. Psychiatr. 18, 218-224, 1955. descrizione di un case. Sistema Ncrroso 20, 352-361. 1968. 11. SCOTTI, G. La perdita della memoria topografica: in personal and 12. SEMMES,J., WEINSTEIN, S., GHENT. L. and T~UBER, H. L. Correlates of impaired orientation extrapersonal space. Brain 86, 742-772, 1963. memory: a single case study. 13. WITHELEY. A. M. and WARRINGTON, E. K. Selective impairment of topographical J. Neural. Neurosurg. Psychiatr. 41, 575-578, 1978.

Topographic disorientation--a case report.

Topographical disorientation can be dissociated in two levels, agnosic and amnesic. A case of topographical disorientation due to a glioma of the sple...
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