Neuropsychologia 41 (2003) 1387–1395

Agnosia for scenes in topographagnosia
Mario F. Mendez∗ , Monique M. Cherrier
Departments of Neurology and Psychiatry & Biobehavioral Sciences, University of California at Los Angeles, Los Angeles, CA 90073, USA
Received 24 January 2002; received in revised form 7 January 2003; accepted 7 January 2003

Abstract
Topographagnosia is most commonly attributed to an agnosia for landmarks. In order to define the nature of this agnosia, we studied a patient with isolated topographic disorientation (TD) after a stroke in the right medial occipitotemporal region. The patient got lost in familiar
environments but could readily read and draw maps, describe familiar routes, and provide correct directions. He had normal perceptual test
performance and met criteria for topographagnosia rather than for other forms of topographic disorientation. Two ecologically valid route
tests assessed the nature of his agnosia. On a familiar route, he could recognize major landmarks. He could not, however, recognize route
configurations made up of combinations of visual features each lacking individual distinctiveness. On a test of route learning, he learned
landmarks that differed in minor details and could use them to orient himself along a route. He had difficulty, however, recognizing and learning scenes lacking salient landmarks. This agnosia for scenes was worse for semantically-related environments, but improved with semantic
knowledge such as street names. In addition, the patient lacked overt prosopagnosia but tended toward semantic errors in the recognition of
famous faces. Together these findings suggest that this patient’s inability to recognize a route resulted from an inability of intact perceptual
units for scenes, composed of specific visual configurations of individually indefinite features, from accessing stored representations.
© 2003 Elsevier Science Ltd. All rights reserved.
Keywords: Topographic disorientation; Environmental disorientation; Spatial agnosia; Visual agnosia; Prosopagnosia; Route(s)

1. Introduction
Topographic disorientation (TD) is the inability to find
one’s way in a familiar environments and to learn new
routes. The concept of TD dates to Hughlings Jackson
(1876) who described environmental difficulty in a patient
with a glioma of the right temporal lobe (Jackson, 1932).
Since then, clinicians have observed TD in posterior circulation strokes, dementia, and a number of other conditions.
Studies examining neuroanatomic correlates of TD have
implicated medial occipitotemporal regions, hippocampus,
posterior parahippocampal gyrus, and parietal cortex, with
right greater than left hemisphere involvement (Barrash,
Damasio, Adolphs, & Tranel, 2000; Maguire et al., 1998;
McCarthy, Evans, & Hodges, 1996).
The underlying cognitive mechanisms for TD are not entirely clear. TD is dissociable from other cognitive deficits
and occurs without other explanatory perceptual or memory
problems. Landmark recognition and spatial map construc∗ Corresponding author. Present address: Neurobehavior Unit (116AF),
West Los Angeles Veterans Affairs Medical Center, 11301 Wilshire Blvd.,
Los Angeles, CA 90073, USA. Tel.: +1-310-478-3711x42696;
fax: +1-310-268-4181.
E-mail address: mmendez@ucla.edu (M.F. Mendez).

tion are two important cognitive operations that assist navigation through familiar surroundings (Paterson & Zangwill,
1945). Accordingly, TD may be largely due to failure to
recognize unique landmarks (topographagnosia) or to failure to retrieve spatial maps of an area (topographic amnesia) (De Renzi, Faglioni, & Villa, 1977; Hécaen, Tzortzis, &
Rondot, 1980; Maguire, Burke, Phillips, & Staunton, 1996;
McCarthy et al., 1996). Questions arise, however, about the
role and extent of perceptual or memory changes. Both topographagnosia and topographic amnesia may have at least
subtle perceptual changes and both usually involve spatial
memory. For example, most patients with TD have both retrograde and anterograde memory difficulty for routes; only
mild cases are restricted to impairments in navigating new
routes (Habib & Sirigu, 1987).
This study examined a rare patient who lost the ability to
find his way in familiar environments, but retained the ability to recognize landmarks. In the absence of agnosia for
major landmarks, TD for familiar routes could result from
visuospatial difficulties in integrating spatial relationships
or an inability to access or retrieve adequate mental maps.
Based on this patient’s clinical presentation and history, we
hypothesized that problems in recognizing complex visual
scenes would prove to be the source of his TD. The study
used ecologically valid route tests to determine the cognitive

0028-3932/03/$ – see front matter © 2003 Elsevier Science Ltd. All rights reserved.
doi:10.1016/S0028-3932(03)00041-1

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difficulties associated with navigating a familiar route and
learning a new route. These tests identified whether problems were more in the recognition of salient landmarks or
of non-descript scenes. Further visuoperceptual and visual
memory tasks characterized whether the patient’s TD was
associated with perceptual problems, such difficulty with visual integration, or with memory problems, such as difficulty
with visuospatial learning.

2. Case report
GN, a 76-year-old, right-handed man, developed TD after a stroke. Initially, he complained of the acute onset of
headache, dizziness and imbalance, and scintillations at the
periphery of his left visual field. GN considered this his
usual migraine headache but became alarmed when he experienced a “strobe light” effect while watching people move.
As these acute symptoms resolved, he became aware of environmental disorientation. GN had difficulty finding his way
in familiar surroundings such as the hospital and his neighborhood. In addition, he had difficulty seeing things in the
upper left quadrant of his visual field.
His medical history was significant for recurrent migraine
headaches since age 25, hypertension, coronary artery disease with prior bypass surgery, and a history of prostatic
carcinoma removed by radical prostatectomy. His usual
headaches began with an aura of warmth around his head,
followed by throbbing pressure behind both eyes, nausea,
phonophobia, and photophobia. His headaches responded
well to caffeine and analgesics. His medications included
amitriptyline (10 mg qhs), citalopram (20 mg), ranitidine
(150 mg b.i.d.), verapamil (240 mg qd), and vitamin E and
folic acid. GN had 16 years of education, worked in electronics, and had a negative history for familial diseases.
He underwent extensive mental status testing. GN scored
a 29/30 on the Mini-Mental State Examination (Folstein,
Folstein, & McHugh, 1975), missing one attentional item.
His digit span was seven forward, and he performed normally on sustained attention tasks (e.g. the “A” vigilance
test and months in reverse). Language examination revealed
normal fluency, auditory comprehension, and repetition. He
scored 14/15 on the abbreviated Mini-Boston Naming Test
(Welsh et al., 1994) and generated a word-list of 18 “F”
words in a minute. Reading and writing were intact. He was
mildly impaired on an auditory verbal learning task, recalling 5/10 items spontaneously at 15 min and recognizing 8/10
without false positives. His remote memory was intact for
current events and major historical incidents. On visuospatial tasks, he had normal constructions for three drawings,
including a three-dimensional figure. On 5 min delayed recall, he failed to spontaneously recall the three test drawings
but could recognize them from among 12 choices. There
was no evidence of ideomotor apraxia, acalculia, digit agnosia, or right-left confusion. Frontal systems tasks such as
alternating programs were normal.

His neurological examination was remarkable only for
a visual field deficit in his upper left quadrant. The rest
of his cranial nerves were intact. Coordination testing was
normal and motor examination did not reveal abnormalities of power or tone. His reflexes were +2 and symmetrical without pathological reflexes, and his toes were
downgoing. The sensory examination was intact to all
modalities.
Magnetic resonance imaging (MRI) of the brain showed
a large region of gyriform FLAIR and T2 weighted hyperintensity, and T1-weighted hypodensity, consistent with an infarction involving the right posterior–inferior temporal and
occipital lobes, predominantly in the lingulus (see Fig. 1).
MRI angiographic images of the great vessels of the neck
showed normal appearing cerebral vasculature.
GN was diagnosed with a right posterior cerebral artery
stroke and residual topographagnoic disorientation. The
stroke was suspected to be migraneous in etiology, however,
he was started on antiplatelet therapy (clopidrogel 75 mg).
In addition to TD, the only other findings on his examination were mild memory retrieval difficulty and a persistent
left quadrantanopia.
Further evaluation was undertaken at 2 months post-stroke.
He was aware of his field cut, but denied other visual difficulties, such as problems with detecting depth, motion,
color, or faces. He could not, however, find his way in his
neighborhood, in previously traveled parts of the city, or
around the hospital. Yet, he could give verbal directions of
familiar routes, describe how to get out to the parking lot
from the hospital, and instruct how to travel to his house.
GN proudly described himself as having a “Thomas Street
Guide” readily available in his mind. GN described his difficulty as “familiar routes now look unfamiliar” and denied
that the difficulty was in detecting or describing landmarks.
Familiar houses and streets, however, appeared unfamiliar
until he recognized a major landmark.
GN’s TD was improved by verbal cues and was particularly impaired in certain settings. Knowing the street names
helped him get a mental picture and get oriented. In contrast, he had special problems with corridors, public bathrooms, and theaters. At one point, he could not get out of
a public bathroom because he could not find the exit. He
stated that “things look so similar in bathrooms; they look all
white”. Movie theaters, including their lobbies, all “looked
the same”. In the hospital and clinic, minor variations resulted in a loss of the sense of familiarity. For example, he
relied on a yellow floor barrier for identification of a specific hospital corridor and became totally disoriented when
it was moved.
GN underwent tests of basic visual functions. His visual
acuity was 20/20 with glasses, but he remained with a left
upper quadrantanopia on visual field testing. He had no
difficulty naming colors of even minor differences in hue.
GN performed normally on the Randot stereopsis plates, a
motion parallax box, and an assessment of monocular depth
cues (perspective, superimposition, relative size) (Mendez,

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3. Methods
In order to further characterize his visuoperceptual and
visuospatial abilities, GN underwent a series of complex
visual tests followed by two specific route tests.
3.1. The Benton Visual Form Discrimination Test
(Benton, Hamsher, Varney, & Spreen, 1983)
GN was asked to discriminate complex visual figures.
Each one of 16 test items was composed of a set of three
complex figures (two major and one peripheral) which must
be matched with 1 of 4 items in a multiple choice paradigm.
There were three types of potential errors: (1) displacement
or rotation of the peripheral figure, (2) rotation of one of the
major figures, and (3) distortion of one of the major figures.
3.2. The Gollin Incomplete Figures (modified-5 series)
(Gollin, 1960) and the Southern California Figure-Ground
Visual Perception Test (modified-8 series) (Ayers, 1966)
GN was asked to identify a series of incomplete Gollin
drawings. The original set consisted of 20 picture sets of
five line drawings of familiar objects in increasing degree
of completeness. GN was administered five representative
sets of Gollin figures: the cat, table, elephant, sailboat,
and telephone. On the figure-ground test, he was asked to
identify the three overlapping figures in each of first eight
plates. Although these overlapping figures were developed
for use with children, they have been a good screen for
figure-ground discrimination in impaired adults.
3.3. The Hooper Visual Organization Test
(Hooper, 1983)
In order to test complex visual integration, GN was shown
30 black and white line drawings that have been divided into
component pieces and rotated or moved on the page. GN
had to visually integrate the component parts and name the
object.
Fig. 1. Magnetic resonance images: (a) FLAIR image, horizontal cut,
shows the stroke in medial occipitotemporal region prominently involving
the lingual gyrus; (b) T1-weighted image, saggital cut, shows the extent
of the stroke in the right hemisphere.

Meadows, & Cherrier, 1996). Screening tests did not
demonstrate object agnosia, drawing agnosia, simultanagnosia, oculomotor apraxia, or optic ataxia. GN did not have
difficulty with dressing or in localizing stimuli in peripersonal space. He performed normally on a double simultaneous stimulation task and was able to draw an accurate floor
plan of his house. In the clinic, he could not find his way
out of the clinic area, into the correct corridor, and to the
elevator.

3.4. The Rey–Osterreith complex Figure Copy and
Memory (Meyers & Meyers, 1995)
In order to test visuospatial constructional ability and visual memory, GN was presented a complex figure to copy
and was subsequently asked to repeat the test after a 30 min
delay.
3.5. Complex Picture Scanning (Mendez, Cherrier, &
Cymerman, 1997)
This test assessed the processing of details of a complex
scene. This unstructured visual search task consisted of the
presentation of a picture of a toy store with multiple visual

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items. GN was instructed to “name as many observed objects
as possible within 5 min”. Positive recognition was accepted
if he named, visually described, or indicated usage of the
objects.
3.6. Mesulam Cancellation Task (visual search)
(Mesulam, 1985)
This test assessed visual search and consisted of an untimed, revised version of the Mesulam letter cancellation
test. A 21.6 cm × 28 cm page with 100 different figures distributed randomly across the page was aligned along his midline. GN was instructed to circle a specific figure (a “sun”
with a diagonal band) every time he saw the figure. There
were 24 targets, 12 targets in each hemispace. The score was
determined by the number of targets correctly canceled.
3.7. The Benton Judgment of Line Orientation Test
(Benton et al., 1983)
This test assessed visuospatial orientation and consisted
of 30 items preceded by 5 practice items. The test items
were cards with a pair of partial lines, with each partial line
corresponding to the orientation of one of the lines appearing in the stimulus card. GN was asked to determine which
two lines on the grid below were in the same position as the
stimulus lines. The lines were 3.8 cm long and arranged at
different angles from center, corresponding to 18 in. differences.
3.8. The Card Rotation Test (Ekstrom, French,
Harman, & Dermen, 1976)
This test evaluated the ability to mentally rotate and visualize geometric shapes. Each item of the test consisted of
a drawing of a card cut into an irregular shape. To its right
were six drawings of the same card, sometimes merely rotated and sometimes turned over to its other side. GN indicated whether or not the card had been turned over.
3.9. Money’s Standardized Road Map Test of Direction
Sense (Money, 1976)
This test assessed sense of direction and orientation using
a two-dimensional map. GN was shown an 8.5 in. × 11 in.
black and white outline map of a neighborhood layout. The
layout consisted of rectangular blocks evenly spaced with
triangular blocks at various places. The examiner traced a
practice route, and GN was asked to imagine himself walking the route traced with a felt-tip pen. He was asked to say
which way he would go (right or left) at each turn. After
GN responded correctly to the practice items, a longer route
was drawn, and he was again asked to determine which way
he would turn at each corner.

3.10. The Benton Face Recognition Test (Benton et al.,
1983) and a Famous Faces Recognition Task
On the Benton test, GN was asked to match front-view
photographs with six photographs presented either in
front-view, three-quarters view, or in different lighting.
He was also shown 24 photographs of famous people for
naming or for identification. The famous people were politicians, entertainers, and other persons well known to most
residents of Southern California.
3.11. The Corsi Block-Tapping Task
(Milner, 1971)
This task involved nine 1.5 in. cubes fastened in random
order on a black board. Each time the examiner tapped the
blocks in a prearranged sequence, GN attempted to copy his
tapping pattern. By adding one tap to each succeeding successful sequence the examiner ascertained GN’s immediate
visual recall.
3.12. Modified Visuospatial Learning Test
(Malec et al., 1992)
This test measured visuospatial learning. It was modified to substitute black buttons for specific designs. GN was
shown a 5 × 7 black and white grid. On the grid were placed
seven black buttons in a particular pattern. GN was allowed
to examine the pattern for 10 s. The buttons were removed,
and GN was asked to put the buttons back on the grid in
their proper position. At this point, three additional identical
buttons were included as distractor buttons for a total of 10
buttons. GN was instructed to remember the correct number of buttons as well as their placement. This process was
repeated for a total of five trials. After a 30 min delay, GN
was asked to recall the pattern from memory.
3.13. Familiar Route: Home to Hospital (20 km)
GN was tested on recall of the non-freeway route from his
home in the West Los Angeles area to the hospital. This was
a highly familiar route for him, but he could not longer drive
it without getting lost. GN was provided with a schematic
map of the area, devoid of any landmarks or words. GN was
instructed to trace his route and to write the street names on
the map. Twenty intersections or key roads were identified
as key for his route. Subsequently, he was given pictures of
landmarks and critical scenes along this route (see Figs. 2
and 3). Ten well-known landmarks and 10 critical scenes
were presented each along with three distractors. These
scenes were devoid of major, salient landmarks but were critical junctures required for deriving information necessary
for successful navigation of the route. The distractors for
landmarks and scenes consisted of one semantically-related
(same street or neighborhood) item, a perceptually similar
item, and a third totally unrelated item. If he recognized the

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Fig. 2. Key landmark along route. Major chapel scene along route to hospital (picture was shown in color to the patient).

landmarks or scenes, he was then asked to indicate the direction he would take along the route. Missed landmarks
or scenes were cued by providing the street names. Finally,
two photographs of missed scenes varying in angle of photographic orientation, were presented, along with an equal
number of distractors, for matching.
3.14. West LA Route Learning Test (Cherrier,
Mendez, & Perryman, 2001)
This test was previously developed for testing route learning in patients with Alzheimer’s disease and was slightly
modified for use here. During a learning trial, the subject

and examiner walked a predetermined hospital route, and
the examiner pointed out specific landmarks, often differing
only in minor detail, and scenes without salient landmarks.
The examiner instructed GN to remember the walking route
as well as the indicated landmarks and scenes. After the
learning trial, a test trial was administered in which GN was
instructed to retrace the exact route and to point out the
designated landmarks and scenes. The following items were
measured during the test trial: walking recall of landmarks
and scenes, necessity for route cues and redirection, route
map recognition from one of four black and white maps,
and picture recognition of landmarks and scenes. In this last
section, GN was shown color photographs of landmarks and

Fig. 3. Key scene along route. One of the major entrances to the hospital; turn-off to the right (picture was shown in color to the patient).

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scenes that were located on the route. GN was asked to indicate the correct choice among distractors.

4. Results
The results of the complex visual tests are summarized
in Table 1. He performed quite well on perceptual tests including tests of complex figure discrimination, incomplete
figure recognition and figure-ground analysis, visual integration, picture search and scanning, shape and angle visualization and rotation, and face discrimination. He did not
demonstrate any spatial orientation problems, including in
egocentric spatial orientation on the Mooney Map Test. The
patient did manifest difficulty in spatial memory, evident on
all visuospatial memory tests.
Although he readily identified most of the famous faces,
his pattern of errors suggested semantic confusion. For example, John F. Kennedy was reported as Robert Kennedy,
despite very distinctive profiles. Henry Kissinger was called
Albert Einstein; although, he later correctly identified the
picture of Einstein. Robert Mitchum, the actor, was reported
as John Wayne. (He also misidentifying Sophia Loren as
Jacqueline Kennedy and did not recognize former L.A.

Table 1
Visual Perception Tests
Test

Basic Perception Tests
Benton Visual Form Discrimination Test
Gollin Figures (modified-5 series)
S. Calif Figure-Ground (modified-8 series)
Hooper Visual Organization
Rey–Osterreith Complex Figure Copy
Visuospatial Tests
Complex Picture Scanning
Cancellation Task (visual search)
Benton Judgment of Line Orientation
(form H)
Card Rotation Test
Money Road Map Test
Facial Processing Tests
Benton Facial Recognition
Famous Faces Recognition
Recognition/description of person
from name
Visual Memory Tests
Corsi Block span 4; unable to reach
2-supraspan criterion after 20 trials
Rey–Osterreith Complex Figure
30 min recall
Modified Visuospatial Learning Test
(trials 1–5)
Delayed 30 min recall
wnl: within normal limits.

GN’s age
Adjusted
score

Significance

29
22
18
16
32

wnl
wnl
wnl
wnl
wnl

19
24
25

wnl
wnl
wnl

120
32

wnl
wnl

43
19
24

wnl
wnl
wnl

Abnormal
4

Low score

16

Abnormal

1

Abnormal

mayor Tom Bradley.) He recognized all of the missed faces
when given the correct names.
On the familiar route test, GN was able to draw maps of
his neighborhood, area of Los Angeles in which he lived,
and other regional maps. When given a schematic map without street names or markings, he quickly recognized it as his
neighborhood and put in the streets. GN could quickly recognize pictures of the major landmarks in his neighborhood
and area, and he could describe what direction to take from
the landmarks. Most pictures of scenes in his neighborhood
were not recognized. Street signs and names facilitated route
recognition. Finally, he could visually match scenes varying
in angle of photographic orientation.
On the route learning test, GN was able to adequately
learn delineated landmarks along a new route. Some of these
landmarks had only minor distinguishing features from each
other. He also knew which direction to go in relation to
the landmarks. When he arrived at a spot where there was
a break in the learned landmarks, he quickly got lost and
needed route cues to stay on course. He did not recognize
scenes without salient landmarks either on the walking test
or on subsequent picture recognition.

5. Discussion
This patient had a relatively isolated topographagnosic
disorientation. He could not find his way in familiar surroundings, but he could describe and draw maps of familiar
routes. When he was out on the actual routes, however, he
quickly got lost. Unlike many case reports of topographagnosia, he could identify common buildings and landmarks
and knew what direction to turn from them in order to find
his way. The GN’s problem appeared to be in deriving information from scenes and visual configurations of his environment.
There have been several different theories for TD
(Barrash, 1998; Benton, Levin, & Van Allen, 1974; Pallis,
1955). Many authors supported the division into topographagnosia and topographic amnesia (Bottini, Cappa,
Geminiani, & Sterzi, 1990; De Renzi et al., 1977; Hécaen
et al., 1980; Landis, Cummings, Benson, & Palmer, 1986;
Paterson & Zangwill, 1945; Welsh et al., 1994). Levine,
Warach, and Farah (1985) suggested that these differences involved identification of salient landmarks used in
route-learning, and the representation of spatial positions
in mental maps, respectively (Levine et al., 1985). Grüsser
and Landis (1991) further divided TD into four types. Type
A was due to the formation of an incorrect percept (Grüsser
& Landis, 1991) and Type B (apperceptive topographagnosia) to an inability to integrate perception in parallel
across the visual field (Riddoch & Humphreys, 1987). Type
C (associative topographagnosia) patients could not match
a normal percept with its inner representation, and Type
D (topographic amnesia) patients could not access inner
representations of mental maps. More recently, Aguirre

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and D’Esposito proposed a somewhat different set of TD
subtypes (Aguirre & D’Esposito, 1999). They postulated
the existence of an egocentric disorientation from problems
coding body-centered spatial relations, and a heading disorientation from problems deriving a sense of direction,
possibly related to difficulty coding allocentric spatial relations between objects. They also described a landmark
agnosia and an anterograde topographic amnesia. Together,
these classifications suggested that TD for familiar routes
could result from visuospatial difficulties in integrating spatial relationships, an inability to access or retrieve adequate
mental maps, or a continuum of agnosia for minor and major landmarks. Our patient was assessed for these putative
mechanisms for TD.
This patient did not have evidence of visuospatial disturbances for TD. In this type of TD, the right superior parietal
lobule is most often involved, but this area was spared in GN
(Assal, 1969; Hécaen, Penfield, Bertrand, & Malmo, 1956;
Newcombe & Ratcliff, 1990; Paterson & Zangwill, 1945).
Furthermore, with damage to the egocentric coordinate system, other spatial difficulties have usually occurred, such as
hemispatial neglect or Balint’s syndrome. In heading disorientation, which may follow right posterior cingulate injury,
salient landmarks have been devoid of orientation (location)
value, but our patient knew which direction to proceed from
landmarks (Brain, 1941; Whitty & Newcombe, 1973).
In addition to coding single objects in space, multiple visual objects must be integrated into a meaningful scene. The
failure of integration in a spatially parallel fashion may result
in reversion to a feature-by-feature identification strategy of
details of landmarks (Paterson & Zangwill, 1945; Whitty &
Newcombe, 1973). GN’s ability to quickly describe complex scenes, learn landmarks with minor distinguishing features, and match scenes at different angles mitigates against
this “apperceptive” or integrative agnosia.
Our patient’s memory difficulty also does not explain his
TD. Topographic amnesia could include difficulty in retrieving stored knowledge of spatial maps, in learning new maps,
or in temporarily coding spatial relations in working memory (spatial sketchpad) (Hanley, Young, & Pearson, 1991).
Unlike GN’s performance, if there is retrieval difficulty,
verbal descriptions of routes are impaired. Several investigators have proposed a deficit specific to forming new topographical memories (Assal, 1969; Barrash & Tranel, 1996;
Epstein, DeYoe, Press, Rosen, & Kanwisher, 2001; Whiteley
& Warrington, 1978). On the route learning test, however,
GN demonstrates sufficient ability to learn new, designated
landmarks. In addition, he could represent and use spatial
information in working memory to access or use spatial
maps and visualize a route.
This patient’s lesion suggests associative topographagnosia similar to landmark agnosia. Similar to his lesion, the
most frequent cause for landmark agnosia is a stroke involving the territory of the right posterior cerebral artery
with infarction of the medial occipitotemporal cortex (Habib
& Sirigu, 1987; Hécaen et al., 1980; Landis et al., 1986;

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Whitty & Newcombe, 1973). GN’s lesion includes the “lingual landmark area”, a region straddling the right lingual sulcus specialized for the perception of salient environmental
features such as buildings (Aguirre, Zarahn, & D’Esposito,
1998). The route tests revealed that GN’s “landmark” agnosia was specifically for scenes. Visual scenes are a correlation of minor as well as major environmental features with
each other (Polk & Farah, 1995), and damage to the lingual
area may impair the identification of complex scenes as well
as usual landmarks (Aguirre & D’Esposito, 1999; Barrash
et al., 2000; Incisa della Rochetta, Cipolotti, & Warrington,
1996). There may be a continuum of damage to a single environmental representation area depending on the saliency
of landmarks (Barrash, 1998).
Alternatively, these patients may be impaired in the
arrangement of visual stimuli into coherent scenes. Like
GN, those with landmark agnosia may become disoriented
within buildings, suggesting that they are no longer able to
distinguish the configurations of stimuli necessary to differentiate one place from another (Incisa della Rochetta et al.,
1996). Although GN could tell the general direction to take
from a recognized landmark, he quickly got lost if not actually viewing the landmark. Moreover, recent data shows
that parieto-occipital junctions and parahippocampal gyri
respond selectively to overall scenes, regardless of individual details (Epstein & Kanwisher, 1998; Nakamura et al.,
2000). These findings suggest a specific deficit in interpreting the configuration of visual scenes that are important for
navigation.
The roles of his PPA lesion and the hippocampal complex
may support the concept of a deficit at the level of spatial
configuration or geometry. The PPA is critical for combining the geometry and spatial cues of scenes into a unified
topographical representation for encoding or consolidating
into memory (Epstein et al., 2001; Ploner et al., 2000; Smith
& Milner, 1981). The right PPA may be particularly necessary for spatial learning and encoding scenes that must
be recognized “holistically” solely on the basis of spatial
structure (Aguirre & D’Esposito, 1999; Ploner et al., 2000;
Smith & Milner, 1981). The hippocampal complex plays a
further role in the processing of scenes by the retention and
retrieval of details about topographical and environmental
features (Rosenbaum et al., 2000) (Table 2).
Further clues to the nature of his topographagnosia come
from prosopagnosia (Landis et al., 1986; Maguire et al.,
1996; McCarthy et al., 1996), a related category specific
deficit involving the perception of familiar faces (Clarke,
Lindemann, Maeder, Borruat, & Assal, 1997; De Renzi &
di Pellegrino, 1998; Dixon, Bub, & Arguin, 1998;
Kanwisher, McDermott, & Chun, 1997; Sams, Hietanen,
Hari, Ilmoniemi, & Lounasmaa, 1997). Both cognitive disorders may correlate with disturbance of a “recognition
network” in the anterior temporal cortex (Gorno-Tempini
& Price, 2001; Mesulam, 1998; Tyrrell, Warrington,
Frackowiak, & Rossor, 1990). Right medial temporal
lobectomies may impair scene recognition, as well as face

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Table 2
Topographic Orientation Tests
GN’s score

Correct (%)

Familiar route: home to hospital (12 miles)
Schematic, unmarked map: indicate turns on route (n = 20)
Pictures of major landmarks (three distractors each) (n = 10)
Compass orientation from major route landmarks
Pictures of scenes without major landmarks (three distractors each) (n = 10)
Semantic errors (two correct when given street name)
Perceptual errors
Unrelated errors

20
10
9
1
5
2
2

100
100
90
10

Choice of direction from identified landmarks or scenes (n = 11)
Misidentified scenes: match photos at different angles (nine distractors)

11
9

100
100

GN’s score

Normals

9
2
Failed (>5 cues)
Failed to choose correct route
10
1

9.83 (0.41)a
9.3 (0.82)
1.17 (1.17)
All six choose correct route
10
9.83 (0.41)

West LA Route Learning Test (Cherrier et al., 2001) (n = 6)
Walking recall of 10 landmarks (spontaneous recall)
Walking recall of 10 scenes (spontaneous recall)
Number of necessary route cues
Route map recognition (real map with three distractors)
Landmark picture recognition among distractors
Scene picture recognition among distractors
a Standard deviations in parentheses.

recognition (Spiers et al., 2001). In prosopagnosia, as in
GN, face recognition may be worse for semantically-related
people, indicating difficulty accessing similar stored representations (Clarke et al., 1997; Dixon et al., 1998; Nyffeler,
Leeman, & Schnider, 2001). Moreover, his topographagnosia was worse for semantically-related scenes (theaters,
bathrooms, public buildings). In contrast, prosopagnosic
patients may access intact images of famous faces when
presented with their names or other semantic information
(De Renzi & di Pellegrino, 1998). Similarly, in GN, verbal
tags, street names, and other semantic information facilitated route orientation.
In conclusion, the patient’s topographagnosia corresponded with problems recognizing critical scenes rather
than salient landmarks. There was a loss of sense of familiarity for familiar scenes, and he seemed to fail to recognize
the interrelationships of minor elements that contribute to
a specific scene. In addition, his appreciation of scene configurations improved with semantic cueing and suggested
problems in semantic access. The mechanism for topographagnosia proposed in this case study can lead to further investigation and delineation of agnosia for scenes.
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