Neurocase (2005) 11, 463–474

Copyright © Taylor & Francis LLC

ISSN: 1355-4795 print

DOI: 10.1080/13554790500423602

NNCS

Lack of orientation due to a congenital brain malformation:

A case study

Congenital brain damage and orientation

GIUSEPPE IARIA1,2, CHIARA INCOCCIA2, LAURA PICCARDI1,2, DANIELE NICO1,2, UMBERTO SABATINI2 and CECILIA

GUARIGLIA1,2

1Dipartimento di Psicologia, Universitá di Roma “La Sapienza”2I.R.C.C.S. Fondazione Santa Lucia, Roma, Italy

Topographical disorientation is usually described in patients who have lost the ability to orient themselves as a consequence of acquired

focal brain damage. Here, we describe the case of a 20-year-old woman with a congenital brain malformation who has never been able to

orient herself within the environment. We addressed in detail her ability to orient and navigate within the environment by administering a

number of tasks in both ecological and experimental surroundings. The results indicate a complete inability to use any kind of strategy

useful for orientation.

Introduction

Topographical disorientation is generally defined as the

impaired ability to orient within the environment. In the earli-

est reported case, Jackson (1876) describes a woman with a

glioma, involving the entire right occipito-temporal region,

who got lost while attempting to show to a visiting relative a

familiar park. In this situation, the patient was unable to find

the entrance of the park even through it was within her visual

field. Afterwards, she was persistently unable to recognize

and find the way around her neighbourhood, despite the pre-

served ability to verbally report the correct routes between

familiar destinations. Following this first neuropsychological

report, many more topographical disorientation cases were

described, revealing a great deal of heterogeneity in the way

the disorder becomes apparent. For instance, patients were

described as being unable to recognize very familiar land-

marks with distinctive architectural features (for example, a

church; Paterson and Zangwill, 1945), or learn the layout of

novel surroundings while they were still able to move and

orient in familiar environments (Meyer, 1900). In some

cases, patients showed a selective deficit in describing famil-

iar places despite a spared ability to orient within them

(Badal, 1888), or they correctly described places without

being able to use that information for orientation (Wilbrand,

1892). Since these pioneering studies, the variety of topo-

graphical disorders remains evident across the many cases

reported in the literature (for a review see Farrell, 1996;

Barrash, 1998). This suggests that the term “topographical

disorientation” is rather generic since topographical disorders

can be manifested in several ways and result from different

cognitive impairments.

In 1982, De Renzi (1982) suggested the first taxonomy of

topographical disorientation, dissociating three different sub–

types depending on the cognitive and perceptual processes

that can be independently compromised following a brain

lesion. First, patients can show topographical disorientation

as a consequence of defective exploration. In such cases,

brain lesions impair the ability to correctly scan the environ-

ment and patients are unable to shift attention towards differ-

ent visual targets. Second, patients can show topographical

disorientation following a deficit in localizing objects and

correctly perceiving specific features such as depth and

shape, which affects the ability to generate and manipulate

visual images. Finally, patients can show disorientation

because of memory problems; indeed different memory sys-

tems can be involved depending on the strategy subjects use

to move within the environment and the different kind of

stimuli they use for orientation. In fact, one can orient and

move within the environment basing on verbal (name of

streets), visual (landmarks), spatial (relative positions of

landmarks), semantic (facts), and episodic information. Alto-

gether, the variety of skills that may be involved in orienta-

tion suggests the presence of different cognitive processes

and, as a consequence, that topographical disorientation can

occur for many different reasons, not just those confined to

the spatial domain.

The neuroanatomical correlates of topographical disori-

entation are also unclear. In fact, selective deficits in

Received 20 April 2005; accepted 15 September 2005

We would like to thank MGC for her long-standing collaboration

with testing, and Professor Luigi Pizzamiglio, Kate Watkins and

Ysbrand Van der Werf for helpful comments on a previous version

of this manuscript. This study was supported by MIUR (Cofin

2005), Ateneo and Ministero della Salute (RC 2004) and European

Community FPS-Strep-Wayfinding-N. 012959.

Address correspondence to Giuseppe Iaria, Ph.D, Dipartimento di

Psicologia 39, Via Dei Marsi, 78 CAP 00185, Roma, Italy.

E-mail: giuseppe.iaria@uniromal.it

464 Iaria et al.

topographical functioning, such as place recognition or

way-finding, have been reported in more than 200 cases

following focal lesions due to stroke, penetrating missile

wounds and surgical resection for treatment of epilepsy or

tumor. This suggests different regions located almost any-

where in the brain can be responsible for topographical

disorientation (see Barrash, 1998). In a more recent

review; however, Aguirre and D’Esposito (1999) suggest a

new detailed taxonomy of topographical disorientation

based on specific brain lesions. The authors identified four

different categories of selective navigational disorders.

Patients affected by egocentric disorientation, following

lesions in the posterior parietal cortex, are unable to use

egocentric co-ordinates to localize environmental land-

marks; although these patients are able to recognize a

landmark, they cannot encode its position relative to them-

selves (see patient GW; Stark et al., 1996). Patients with

heading disorientation, arising from lesions of the retros-

plenial cortex (posterior cingulate gyrus), recognise land-

marks available in the environment but are unable to

recover directional information from them (see Case 2,

Takahashi et al., 1997). Landmark agnosia, due to lesions

in the medical occipital-temporal cortex (including fusi-

form, lingual and parahippocampal gyri), is a more selec-

tive damage in which patients are unable to recognize

salient environmental landmarks (see Case AH, Pallis,

1955). Finally, lesions to the parahippocampal cortex

result in anterograde disorientation, defined as the

impaired ability to learn pathways in novel environments

(see Case 1, Habib and Sirigu, 1987).

Following different models, both the De Renzi (1982) and

the Aguirre and D’Esposito (1999) taxonomies suggest that

human topographical orientation is a very complex multi-

component ability relying on the integrity of different cogni-

tive skills. For this reason, different cognitive impairments

can result from different brain lesions, giving rise to the vari-

ety of specific topographical disorders. Furthermore, we do

not have a clear understanding of the functional development

and interactions of the different cognitive skills subserving

orientation. This may be due to the fact that topographical

disorientation is described only in patients who have lost spe-

cific orientation abilities as a consequence of acquired focal

brain damage in the context of previously developed naviga-

tional skills.

In the present study, we describe a case that differs from

the ones previously described in the literature. We report the

first neuropsychological study of a patient who has never

been able to orient herself within the environment as a result

of a congenital brain malformation involving the retro-Rolandic

regions of both hemispheres. This case provides us with the

opportunity to investigate topographical orientation from a

developmental perspective. We believe that the patient’s

impairment confined to the spatial-orientation domain may

further elucidate the functional development of the cognitive

abilities necessary to develop the entire cognitive system

devoted to orientation.

Case report

At the time of testing, MGC was a 20 year-old right-handed

(Questionnaire by Salmaso and Longoni, 1985) woman who

underwent a ventricular-peritoneal valve derivation when she

was 21 days old, due to congenital hydrocephalus. At the age

of six months, suffering from meningitis, the patient under-

went brain surgery to monitor the shunt draining system.

After the surgery, she presented with unilateral partial motor

seizures that were pharmacologically treated with barbitu-

rates for three years. During the course of her life, MGC

underwent several brain surgeries to monitor the shunt, with

the last surgery in July 2001, at the age of 17 years.

Motor development was within the normal range: MGC

obtained trunk control at the age of six months and self-

perambulation before the age of 2 years. Language develop-

ment was normal and she achieved normal levels of skill:

MGC attended schools normally, successfully completing

high school.

Despite her normal cognitive development, MGC reports to

have never been able to orient herself within the environment.

Apart from moving correctly in the six-room apartment where

she has lived since birth, MGC never leaves her home by her-

self, since she loses her way on every occasion. For instance, at

her local grocery store she becomes lost each time her mother

disappears from direct sight. During her entire life, she has

learned only two short routes after five years of training with

her father. Starting from the main entrance of her school, MGC

is able to walk straight ahead until the end of the main street

where she is able to turn right in order to reach her father’s

shop 200 meters away, or turn left to reach her grandmother’s

home at about the same distance.

In 2003 the patient was referred to the Centre of Neuropsy-

chology at Fondazione Santa Lucia, Rome for the evaluation

of her topographical disorder. We submitted MGC to a

neuro-radiological examination, a complete neuropsycholog-

ical assessment, and an evaluation of the various cognitive

abilities involved in topographical orientation. We obtained

informed consent in a manner approved by the local ethics

committee.

Neuroradiological examination

Magnetic Resonance Imaging (MRI) was performed on a

Siemens Magneton Vision scanner operating at 1.5 T and

equipped with 25 mT/m gradients. A circularly polarized

head coil with a diameter of 270 mm was used both for RF

transmission and for reception of the MR signal. The pro-

tocol included axial and coronal T2-weighted fast spin-

echo (FSE) sequences (TR = 3800 ms, TE = 22/90 ms),

axial (see figure 1) and sagittal T1-weighted spin-echo

(SE) sequences (TR = 600, TE = 14) and coronal fluid-

attenuated inversion recovery (FLAIR) sequences (TR =

9000, TE = 119, inversion time = 2470) covering the

whole brain. Twenty-one 5 mm-thick sections with no gap,

Congenital brain damage and orientation 465

Fig. 1. Axial scans of MGC’s brain showing the malformation present at the time of testing.

466 Iaria et al.

a 23- to 24-cm field of view (FOV), and 256 ! 256 matrix

were obtained. Axial T1-weighted 3D images (magnetiza-

tion prepared rapid gradient echo sequence, MPRAGE)

were also acquired. The axial and the coronal sections

ran respectively parallel and perpendicular to a line that

connects the anterior and posterior commissure (AC-

PC line).

MRI scans show an association of malformations of the

posterior fossa and supra-tentorial abnormalities. The

malformations of the posterior fossa include a vermian-

cerebellar hypoplasia with associated large retro-cerebellar

cerebro-spinal fluid collection and elevation of the tento-

rium and torcular, as well as a dysmorphia of the fourth

ventricle. The supra-tentorial malformations include a

hypoplasia of the rostrum, posterior part of the corpus and

the splenium of the corpus callosum; a thickening of the

white and gray matter of the right mesial temporal and

occipital lobes; a cortical dysplasia (polymicrogyria) of

the left mesial temporo-parietal-occipital lobes. In the

temporal lobe, a craniotomic foramen is present for place-

ment of ventriculoperitoneal shunt; the ventricular cathe-

ter enters into the right trigone and terminates in the left

trigone. Both the posterior horns of the lateral ventricles

appear dysmorphic, with reduced size and irregular con-

tours of the walls. The loss of deep white matter, sur-

rounding the posterior horns, may be the result of

periventricular leukomalacia. Taken together, the MRI

study suggests complex anomalies of the cerebellum, of

the cerebral commissural system and the structural organi-

zation of the cortex in both the temporal and occipital

lobes (see figure 1).

Neuropsychological assessment

During evaluation the patient was alert and fully cooperating.

She underwent a series of standard neuropsychological tests

assessing general intelligence, attention, memory and visu-

ospatial abilities (see Table 1).

General cognitive level was tested by means of the WAIS-

R (Orsini and Laicardi, 1997): MGC obtained a Verbal IQ of

94 and a Performance IQ of 78 (total IQ = 86).

She was fluent and had normal verbal comprehension. No

apraxia (ideo-motor, ideative or constructional) was

observed. As reported in Table 1, MGC did not show any

sign of unilateral neglect, visual imagery defect, nor face,

colour or object agnosia. The only performances below the

cut-off scores were recorded on the Benton Visual Retention

Test (Benton et al., 1974), the Judgement of Line Orientation

Test (Benton et al., 1978), the Cube Analysis-subtest of the

VOSP battery (Warrington and James, 1991) and the delayed

recall of the Rey-Osterrieth Complex Figure (Osterrieth,

1944).

With regard to memory abilities, MGC performed nor-

mally on both short- and long-term verbal memory tests; her

visuo-spatial span was within the normal range, whereas she

showed an impairment in the Corsi Supraspan Block Test

(Spinnler and Tognoni, 1987). Finally, Goldman’s perimetry

showed no visual field defect.

Navigational skills assessment

To assess MGC’s navigational skills, we developed a battery

of tests to be administered both in experimental and ecologi-

cal environments.

The battery includes three different categories of tasks.

The first category consists of tests assessing specific cogni-

tive abilities relevant for navigation: mental rotation

(Mental Rotation Test), mental representation of familiar

environments (Map Drawing), body displacements accord-

ing to a paper map (Road Map Test) and processing of ves-

tibular information in a task of path estimation (Distance

Replication). In the second category, specific navigational

processes are tested in experimental environments: the abil-

ity to use idiothetic information in order to reach a target

location (Place Learning Test in Experimental Environ-

ment) and the ability to translate a schematic representation

(a map) into locomotion (Semmes Test). In the third set

of tasks, we assessed navigational skills in ecological envi-

ronments by testing the ability to learn a pathway using

different strategies (Route-Based Way Finding, Landmark-

Based Way Finding), to use a map (Map-Based Way

Finding), and to recognise salient elements available in the

environment and useful for orientation (Landmark Identifi-

cation). The whole battery for navigational skills is reported

in Table 2.

Hereafter, we report detailed descriptions of each task and

the results the patient obtained. Four females matched for age

(mean, 20.75; SD, 0.96) and years of education (mean, 13.25;

SD, 0.5) volunteered as controls.

Mental rotation test

We tested mental rotation ability by asking MGC to identify

a target stimulus (a pattern of simple connected dots) among

four alternatives in which the target is depicted with a rota-

tion of 45°, 90°, 135° or 180° respectively (Grossi, 1991).

MGC correctly identified 6 out of 10 stimuli, failing when-

ever stimuli rotation exceeded 90°. The mean score for the

controls was 9.07 (SD = 1.72).

Map drawing

MGC was asked to draw a sketch map of her home from

memory. Although the patient correctly reported the number

of rooms in the apartment, she produced a greatly distorted

map, showing errors of both scaling and spatial relationships

among elements (see Figure 2).

Congenital brain damage and orientation 467

Table 1. Neuropsychological assessment. The table shows the patient’s score on the neuropsychological assessment evaluating general

intelligence, attention, memory, orientation, visual imagery and visuo-spatial perception abilities. WAIS-R, Wechsler Adult Intelligence

Scale, Intelligent quotient, (IQ) impaired performance (*), performance not impaired (+)

Test Adjusted score Cut-off

General Intelligence

Wechsler adult intelligence scale-R

(Orsini and Laicardi, 1997)

Verbal IQ 94

Performance IQ 78

Full scale IQ 86

Memory

Verbal memory

Rey’s 15 word learning task (Carlesimo et al., 1996)

Immediate recall 29.9 28.53

15 min delayed recall 5.2 4.69

Digit Span (Orsini et al., 1987)

Forward 6 3.75

Backward 4

Short Story Immediate recall (Novelli et al., 1986) 11.5 10

Spatial memory

Corsi Block Test (Spinler and Tognoni, 1987) 3.25 3.5*

Corsi Block Test Supra-Span

(Spinler and Tognoni, 1987)

0.92 5.5*

Rey’s figure A (delayed recall) (Osterrieth, 1944) refused *

Visual memory

Benton Visual retention test (Benton et al., 1974) 6/10

Attention

Visual search (Spinler and Tognoni, 1987) 52/60

Visual-perceptual abilities

Visual Object and Space Perception battery

(Warrington et al., 1991)

Object perception

Screening test 20/20 15/20

Incomplete letters 18/20 17/20

Silhouettes 17/30 16/30

Object decision 19/20 15/20

Progressive silhouettes 10/20 14/20

Space perception

Dot counting 10/10 8/10

Position discrimination 18/20 18/20

Number location 8/10 7/10

Cube analysis 4/10 6/10

Judgement of Line orientation 14/30 19/30*

Street’s Completion Test (Spinler and Tognoni, 1987) 8/14 2/14

Unusual Prospective (Pizzamiglio et al., 1989) 11/16 8.5/16

Rey’s figure A (copy) (Osterrieth, 1944) 33/36 29/36

Neglect battery (Pizzamiglio et al., 1990)

Line cancellation +

Letter cancellation +

Wundt-Jastrow Area Illusion Test +

Reading test +

Line bisection test +

Imagery abilities

O’clock test (Grossi et al., 1989) 27/32

Shape recognition (Grossi, 1991) 9/10 8.45/10

468 Iaria et al.

Table 2. Navigational skills assessment. Summary of the navigational skills battery performed by MGC. Tasks are grouped according to

the relative cognitive abilities. See text for details. Impaired performance (")

Cognitive Skill Task MGC’s SCORE

Mental Rotation Grossi et al. 1989

Identification of a target stimulus out of four

alternatives in which the target is differently rotated.

"

Mental representation Map Drawing

To make a schematic draw of a familiar environment.

"

Road Map Test (Money et al. 1965)

To imagine moving by using a paper map.

"

Distance Replication (Pizzamiglio et al. 2003f)

To evaluate and replicate distances by using idiothetic information.

"

Place Learning test in experimental environment (Guariglia et al. 2005)

To find a hidden location by using idiothctic and

geometric environmental information.

"

Semmes Test (Semmes et al. 1955)

Translate visual information into locomotion.

"

Way Finding

in ecological environment

Map-Based

To follow a pathway and reach a target location by using a city-map.

"

Route-Based

To replicate a previously travelled pathway.

"

Landmark-Based

To replicate a previously travelled pathway learned

with a landmark-based acquisition.

"

Landmark IdentificationLandmark Identification

To recognise landmarks and views of places previously visited.+

Fig. 2. Figure 2a shows the geometric representation of MGC’s apartment. Figure 2b reports the drawing that MGC made from memory.

Congenital brain damage and orientation 469

Road map test

The Road Map Test (Money et al., 1965) consists of an A4-sized

paper map on which a pathway is traced. Subjects are required

to imagine themselves moving along the pathway, reporting

verbally whether they would make a left or a right turn at each

change of direction. MGC correctly judged 14 out of 32 turns

of the path (score of matched controls: average 22, SD 2.4).

Distance Replication

The processing of vestibular and somatosensory information

subserves the ability to evaluate distances moved and pathway

lengths. To assess whether MGC can correctly process this kind

of information, we administered a linear distance replication

task by using the same apparatus and procedure described by

Pizzamiglio and co-authors (2003). The apparatus consists of a

robot allowing both the programming of passive whole-body

displacements and the subject’s execution of displacements by

operating a pressure-sensitive spring button. During this loco-

motion task, the subject was required to use the pressure-

sensitive spring button in order to replicate actively the previ-

ous passive displacement. Both passive displacements and

active replications were performed along the same vector, either

moving forward or laterally leftward or rightward. Testing was

performed in an empty room without visual cues. We adminis-

tered a total of 30 trials: six 3-meter trials for each direction

(leftward, forward or rightward) and six catch trials of either

two or four meters (data from catch trials were not recorded).

In her attempts to actively reproduce the displacement dis-

tances, MGC’s mean distance travelled was 1.51 m leftwards

(SD: 0.40), 2.45 m forwards (SD: 0.24), and 1.75 m rightwards

(SD: 0.92). The patient’s responses in all directions were sig-

nificantly (less than control mean-2SD) shorter than the con-

trols’ performances (leftwards: mean 2.83, SD 0.07; forwards:

mean 3.02 m, SD 0.15; rightwards: mean 2.97 m, SD 0.33).

Place learning test in experimental environment

This test, derived from the Morris’ Water Maze, has been

devised for assessing the ability to find and memorize a target

point in an environment when visual information (i.e. land-

marks) are absent (no-landmark condition: NL) or are avail-

able (landmark condition: L) (Guariglia et al., 2005). The test

was performed in a 5 ! 6 meters experimental room; in order

to eliminate all visual cues except those included for experi-

mental purposes, the walls were completely covered by grey

curtains, covering both door and window, and the floor was

painted in the same homogeneous grey hue. In both condi-

tions (Landmarks/No-Landmarks), subjects are required to

find a target location and to memorize its position. The target

location consists of the receptive field (15 cm diameter circu-

lar spot) of a hidden infrared sensor, triggering a tone when-

ever the subjects’ head enters the receptive field. Blindfolded

subjects are introduced in the room while seated on an

automatic wheelchair and placed in the centre of the room.

After removing the blindfold, subjects move in full vision by

using a joystick to find the hidden location indicated by the

acoustic signal (searching condition, unique trial). Afterwards,

they are asked to find the same hidden location by following

the shortest pathway (immediate recall condition, five trials).

Thirty minutes later, subjects perform a single recall trial

(delayed recall condition).

Before each trial subjects are disoriented and placed in the

room centre while blindfolded; all trials are performed in full

vision.

The landmark-condition differs from the no-landmark con-

dition because of two objects present in the room (a lamp and

a hat stand) that could be used as reference points for reach-

ing the target location. In the present study the two conditions

(L/NL) were administered in two sessions, on different days

with two different target locations. For each task (searching,

immediate and delayed recall condition) and landmark condi-

tions (absent/present), we analysed the time that MGC and

control subjects spent to reach the target location.

In the sesion without landmarks, MGC spent 80 sec. in

“searching”, and average of 131.8 sec. (SD: 101.1) in “imme-

diate recall” and 28 sec. in “delayed recall” condition. Con-

trol subjects spent on average 46.3 sec. (SD: 31.3) in the

searching condition, 48 sec. (SD: 27.7) in the immediate

recall condition, and 26.3 sec. (SD: 4.3) in the delayed recall

condition. In the session with landmarks, MGC spent 428

sec. in “searching”, an average of 224 sec. (SD: 105.6) in

“immediate recall” and 68 sec. in “delayed recall” condition;

control subjects, instead, spent 24 sec. (SD: 13) in searching

condition, 25.8 sec. (SD: 9.9) in immediate recall condition,

and 17.3 sec. (SD: 6.2) in delayed recall condition. As evi-

dent by comparing MGC’s performance with controls, her

time spent reaching the target location in all experimental

sessions (with the exception of the delayed recall condition

without landmarks) was greater than the mean plus at least

one standard deviation. In addition, while controls took

advantage of the presence of landmarks while performing the

task, MGC’s performance worsened. In fact, a paired t-test

comparison across the control group shows that they spent

less time to reach the target location when landmarks were

available (Landmarks vs. No-landmarks: t(3) = 2.401, p < 0.05);

whereas, for the patient, a paired t-test comparison was made

across her four attempts at immediate recall revealing that

she took longer when landmarks were present within the

environment than when they were not (Landmarks vs. No-

landmarks: t(4) = 2.701, p < 0.05).

Semmes test

This classic test (Semmes et al, 1955) assesses the subject’s

ability to use a map for real navigation. The subject is given a

schematic drawing reproducing a 3 ! 3 point grid located on

the floor (3 ! 3 m), with a red-coloured pathway that the

subject has to follow without rotating the map. The test

470 Iaria et al.

includes five maps of five different paths of increasing

lengths. MGC was completely unable to reproduce any of the

pathways included in the test, showing a severe inability to

translate allocentric into egocentric coordinates.

Map-based way finding

This test was performed in the centre of Rome, where the patient

had never been before. She was given a city map on which a

starting position and a final location were indicated. MGC was

asked to choose on the map the shortest pathway and follow it in

order to reach the final location from the starting position (about

200 meters long). Performing the task, the patient got lost as

soon as a turn on the map indicated a change of direction.

Although the experimenter indicated the correct direction when-

ever she was lost, the patient kept failing at each turn. After

approximately two hours of repeated attempts, MGC got frus-

trated and nervous and the experimenter led her to the target

location. Figure 3 shows the city map and MGC’s performance.

Route-based way finding

In this way-finding test the subject is required to follow a pre-

viously travelled path. The experimenter led MGC along the

route (about 200 meters long) to the final location without

giving her any information. Then, she was blindfolded and

guided back to the starting point via a shortcut. Immediately

after, MGC was asked to reach the target location by herself,

following the same pathway travelled together with the

experimenter. The task was carried out in two different places

and in different sessions: beside the hospital (route A) and

downtown (route B). The whole procedure was repeated

three times for each route. MGC never succeeded in reaching

her destination: she felt disoriented at the beginning of each

route and made random right/left turns.

Way finding landmark-based

Since MGC proved completely unable to reproduce even

the simplest route, we devised an easier version of the pre-

vious test. The task was performed downtown along a new

route (route C) similar to route B, using the same procedure.

In this case, however, whenever a crossing point was

reached, the experimenter indicated a specific landmark

(selected among the many landmarks available on the

street), useful for orientation. MCG had to carefully exam-

ine and verbally describe each landmark before moving far-

ther. When asked to replicate the route, MGC promptly

Fig. 3. Map-based way finding. Centre of Rome. The city map shows MGC’s performance from the starting position A (centre of the

figure) to reach the target location B (lower part of the figure).

Congenital brain damage and orientation 471

recognised the landmark relevant for each turning, but was

unable to derive directional information from any of them.

As a consequence, she was not able to correctly follow the

route and got lost at each turn.

Landmark’s identification

In this task, we tested the patient’s ability to recognise land-

marks and views previously seen while performing Routes A,

B and C. The test included 36 pictures of sculptures, foun-

tains, churches, buildings, shops, etc., mixed with 24 distrac-

tors, which were pictures of architectural features, similar to

the targets with respect to function, shape and age, but from a

different area of Rome, to which the patient had never been.

A computer randomly administered the sequence of 60 pic-

tures and for each trial MGC was asked to answer “YES” or

“NO” at the question “Have you ever seen this item?”. The

patient correctly classified 42 pictures out of 60: she identi-

fied 81% of the familiar items and correctly rejected 54% of

the unfamiliar ones.

Discussion

The patient we describe differs from the cases of topographi-

cal disorientation previously reported in the literature. Topo-

graphical disorientation, in fact, is usually described in

patients with an acquired focal brain damage who selectively

(or not) lost orientation abilities (for a historical review see

Barrash, 1998). Here, we reported the case of a patient who

suffered from a congenital brain malformation and has never

been able to orient herself within the environment. This case

provides us with the opportunity, therefore, to investigate

topographical disorientation by focussing on the functional

development of the cognitive abilities considered to be funda-

mental for orientation.

In a recent study, Lehnung and co-authors (2003) provided

evidence that, during the first year of life, orientation is

linked to egocentric information, i.e. children mark locations

in relation to their own body; afterwards, the ability to refer

and use environmental landmarks develops; and finally, after

the age of 7–8 years, children start to use the spatial relation-

ships between landmarks independently from their own body

position. The ability to represent the relative locations of

landmarks is necessary for creating a mental representation of

the environment (cognitive maps): the ability to create and

use those cognitive maps is not fully achieved before the age

of 10 years. Interestingly, Lehnung and co-authors (2003)

also showed that in case of traumatic brain injury (TBI),

interfering with the development of these navigational skills,

children remain impaired in using cognitive maps many years

after the accident. For instance, performing cognitive map-

ping tasks four years post-trauma, children who suffered a

TBI at an early age (under ten years) are more severely

impaired than children who suffered a TBI later in childhood.

These results suggest that orientation and navigational skills

in children follow a sequential development and that a brain

injury during the early stages of cognitive development may

affect the ability to orient and navigate within the environ-

ment. In accordance with Hermer and Spelke (1994), a fur-

ther suggestion may be drawn from the present study, namely

the possibility that the development of navigational processes

could be modularly and hierarchically organised. That is,

more primitive processes should be completely developed

before the development of a successive process begins. The

failure to develop early modules (i.e., egocentric/idiothetic-

based processes) may prevent the development of more

complex processes, such as that based on environmental

landmarks.

To assess the entire navigation system including the sev-

eral cognitive skills and strategies subserving orientation, we

developed a navigational skill battery including several table-

top tests and different orientation tasks in both ecological and

experimental environments. The battery included tests evalu-

ating the ability to recognise familiar landmarks and general

environmental views (Landmark/Place Recognition), mental

rotation skill (Grossi, 1991), the ability to mentally represent

familiar environments such as the patient’s home (Map

Drawing), body displacements on a paper-map (Road Map

Test; Money, 1965), and real distances travelled along a route

(Distance Replication; Pizzamiglio, 2003). These different

cognitive abilities subserve several strategies useful for ori-

enting within the environment (Redish, 1999; Berthoz, 2001).

For example, the ability to mentally rotate visual information

allows the recognition of environmental landmarks from dif-

ferent points of view, and mental representation skills are

necessary to orient in familiar surroundings and reach a target

place moving from different locations. The battery included

also tests assessing the ability to use specific cognitive strate-

gies for orientation in both experimental and ecological

environments. In experimental environments, we also investi-

gated the processing of vestibular, somatosensory, proprio-

ceptive and environmental geometric information in the

absence of any relevant visual elements (Place learning task

in experimental environment; Guariglia, 2005), and the abil-

ity to transform pathways reported on a paper-map into real

locomotion independently of the environmental context and

visual information (Map-Based experimental environment;

Semmes, 1955). Finally, the battery included tasks assessing

the ability to orient and navigate within real surroundings.

Since it has been shown that humans spontaneously adopt

different strategies for orientation (Iaria, 2003), we first eval-

uated the spontaneous use of cognitive strategies in replicat-

ing a previously followed pathway (Route-based task). Then,

we assessed the ability to use environmental landmarks for

orientation (Landmark-based strategy), and the ability to find

a target location by using a real city-map (Map based strat-

egy). The administering of this battery, together with a

complete neuropsychological evaluation, provided a detailed

assessment of the entire cognitive system devoted to

orientation.

472 Iaria et al.

The patient’s neuropsychological assessment revealed spe-

cific cognitive deficits confined to the spatial domain (see

Table 1). MGC was impaired in performing a space percep-

tion (Cube analysis; Warrington and James, 1991) and a spa-

tial supra-span memory task (Corsi Block Test Supra-Span;

Spinnler and Tognoni, 1987); in addition, she was unable to

judge the orientation of lines (Benton et al., 1978) and

impaired in two visual recall memory tasks (Complex figure

of Rey A-delayed recall, Osterrieth, 1944; Benton Visual

retention test, Benton et al., 1974). On the navigational skills

battery, the patient failed all the tests, showing an impaired

ability to perform any task involving orientation and body

displacements within the environment (see Table 2). On the

one hand, the cognitive deficits revealed by the neuropsycho-

logical assessment could partially explain the patient’s topo-

graphical disorder: visual recall and spatial supra-span

memory, in fact, surely contribute to the correct use of some

cognitive strategy useful for orientation (Redish, 1999). On

the other hand, those cognitive impairments do not justify the

MGC’s severe topographical disorder since patients with

similar cognitive deficits do not usually show such a severe

topographical disorientation. We believe that the foetal per-

turbation severely altering this patient’s brain morphology

resulted in a specific functional development that affected the

entire cognitive system devoted to orientation. MGC, in fact,

suffered from a brain malformation and a congenital hydro-

cephalus during a very early stage of cognitive development

(21 days after birth). The navigational battery revealed that

she is impaired in mental rotation and representation and

unable to use any cognitive strategy useful for orientation,

even the ones available to children at a very early stage of

cognitive development. Performing a place learning task in

an experimental environment, in fact, we found that MGC

was impaired in memorizing a hidden target location based

on geometric and idiothetic (vestibular, somatosensory, prop-

rioceptive) information in the absence of visual elements, and

even when landmarks were available in the room. In addition,

compared to her performance based on geometric and idio-

thetic information, when the landmarks were available in the

room she needed twice the time to find the hidden target loca-

tion. In contrast, control subjects needed half the time when

landmarks were present in the environment compared to their

performances based on idiothetic and geometric information

only. Hermer and Spelke (1994) showed that young children

(18 months old) are able to use geometric and idiothetic

information to reach a target location when no other informa-

tion is available; whereas, when salient elements are avail-

able in the environment, 5-7-years-old children tend to orient

themselves by using those landmarks (Hermer-Vazquez

et al., 2001). In accordance with Hermer-Vazquez’s finding

(2001), our results show that control subjects automatically

shift to the landmark-based navigational strategy when land-

marks are provided, with a significant decrease in the time

required to find the hidden target location; in contrast, the

presence of landmarks within the environment had no helpful

effect on MGC, in fact they slowed her performance further.

This suggests that MGC, like controls, may attempt to adopt

a landmark-based strategy but this attempt fails because of

her orientation deficit. Similarly, when moving in an ecologi-

cal environment, she proved able to perceive and localise

salient landmarks, but she failed in referring to them for the

purpose of orientation. In addition to these impairments in

using cognitive strategy for orientation, the patient was also

unable to use right/left directional information to move even

in an oversimplified context (Semmes test). To sum up,

MGC’s impairments in performing any task involving spatial

orientation confirmed a severe topographical orientation dis-

order, which suggests the arrested development of the entire

functional system devoted to navigation.

One might ask; however, why, among various cognitive

functions only the normal development of navigational skills

has been impeded. We hypothesize that, at first, the organi-

zation of MGC’s brain favored more basic cognitive func-

tions. In fact, in spite of the abnormalities involving the

posterior parts of her brain, MGC did not develop any visual

field defect or agnosic disorders, confirming normal func-

tional development of the primary and associative visual cor-

tex. For this reason, we maintain that those cognitive

functions developing at a later stage have suffered from the

priority bestowed on primary and earlier cognitive functions.

Besides the ability to localize objects referring to our body’s

position, cognitive skills in the spatial domain (as in mental

rotation and representation) might develop only when those

abilities are essential for individuals; namely, when individu-

als need to move, orient and find their way within the envi-

ronment. The use of mental representation is available at a

very late stage of cognitive development (10 years) (Lehnung

et al., 2003). The lack of these specific cognitive functions is

in accordance with the patient’s brain malformation. The

neuroradiological examination showed cortical alterations of

both parietal and temporal lobes, which are well documented

to be involved in learning and execution of those cognitive

functions in the spatial domain subserving navigation and ori-

entation (Aguirre and D’Esposito, 1999). Finally, it is note-

worthy that since the patient’s clinical history started very

early in her life, MGC never had the opportunity to practice

spatial competence in the way healthy children do. This prob-

ably contributed to her failure to develop any alternative

strategy useful for orientation.

Human orientation is usually considered as a functionally

fragmented cognitive ability. In fact, the very few studies

aimed at developing a taxonomy of topographical disorien-

tation describe (anatomically and functionally) distinct cate-

gories of the disorder depending on the precise nature of the

brain lesions (De Renzi, 1982; Aguirre and D’Esposito,

1999). That is, distinct brain structures are differently

involved in human orientation and damage to any one of

them induces a specific navigational impairment. According

to these data, brain-damaged patients with a specific cogni-

tive impairment are able to adopt alternative strategies for

orientation (Bohbot et al., 2004); e.g. a patient affected by

landmark agnosia, could easily navigate by using the strategy

Congenital brain damage and orientation 473

of counting the turns or reading signs along a route. This

suggests that the human navigation system includes several

sub-components that independently contribute to the ability

to orient within the environment: since the different abili-

ties required to navigate and orient follow a different pat-

tern of development (Lehnung et al., 2003; Hermer and

Spelke, 1994) it is likely that only when all of them are

available the functional navigation system reaches its final

configuration.

From a neuropsychological perspective, in the case of

acquired focal brain damage, adult patients lose specific

cognitive abilities (such as landmark recognition, mental

rotation, etc.), which prevent them from using specific

navigational strategies while others remain available. On

the other hand, the case we report here shows that a

congenital brain malformation interferes with the dev-

elopment of the entire functional system devoted to orien-

tation, preventing even the possibility to shift among

different cognitive strategies. Hence, MGC’s case sug-

gests that, although relying on different cognitive abilities,

the orientation skill should be considered as an indepen-

dent cognitive system that may be available, or not,

depending on the functional development of specific

cognitive skills.

In summary, previous models and data on children with

traumatic brain injury suggest that the functional system

devoted to navigation requires several developmental stages

to reach its final configuration (Lehnung et al., 2003). This

final configuration is available only at a later stage of cogni-

tive development, and allows humans to use different strate-

gies to correctly orient and navigate within the

environment. When a brain accident occurs, adult patients

may be able to use alternative strategies in order to orient

themselves within the environment (Bohbot et al., 2004). In

the case of traumatic brain injury, children are still able to

recover later those orientation skills that have been inter-

rupted by the accident (Lehnung et al., 2003). The case we

report here suggests that when a foetal perturbation is

present, the brain malformation could interfere with a nor-

mal pattern of cognitive development. The abnormal brain

development, in fact, may favour primary basic functions

such as vision at the cost of more complex ones, i.e. the

ability to orient within the environment. In this case, the

impairment in navigation can be pervasive and prevent also

the cognitive functions essential for developing any alterna-

tive strategy. This conclusion is in accordance with work

(Stiles et al., 2005) showing that in the case of early brain

injury developmental adaptation (or plasticity) depends on

the specific cognitive domain. Children with pre- or perina-

tal brain lesions, in fact, are affected more severely in the

spatial domain than the linguistic one, suggesting that the

neural mechanisms involved in spatial processing are prob-

ably specified early in development and may be compro-

mised at the expense of reorganisation for language

development. The case we described in this study is consis-

tent with this evidence.

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