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Agnosias

Article  in  Wiley interdisciplinary reviews. Cognitive science · March 2010

DOI: 10.1002/wcs.42

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 Overview

Agnosias
Marlene Behrmann∗ and Mayu Nishimura

The neuropsychological disorder, known as visual agnosia, refers to the impairment

in deriving the meaning of a visually presented stimulus, in spite of the affected
individual having intact sensory and low-level vision, and normal language
and semantic function. This type of disorder is intriguing both clinically and
scientifically, and vision scientists have studied visual agnosia as a means of
shedding light on how the normal visual system functions. Considerable progress
has been made in this domain, in parallel with detailed behavioral and neural
investigations of the visual system of neurologically intact individuals and of
nonhuman primates. Here, we focus specifically on the neuropsychological studies
and provide a broad overview of the wide range of impairments that fall under
the label ‘visual agnosia’, including those acquired following brain damage in
premorbidly normal individuals, those that appear to have been present since
birth, and those whose onset is late in life and is associated with neurodegeneration.
We also outline the different subtypes of visual agnosia, including those that affect
primarily the recognition of faces, words, or objects, and we lay out some of the
key questions currently being addressed by researchers in this domain.  2010 John
Wiley & Sons, Ltd. WIREs Cogn Sci 2010 1 203–213

V isual agnosia refers to a diverse class of

neuropsychological disorders in which the
affected individual is impaired at deriving the meaning
of some or all categories of visual stimuli. The term
‘agnosia’, which comes from the Greek, means ‘not
knowing’ or ‘without knowledge’, and aptly captures
the dramatic failures of recognition which characterize
the behavior of individuals with visual agnosia. For
example, some agnosic individuals fail to recognize
the faces of close family members, others fail to recog-
nize common everyday objects such as a lamp or a salt
shaker, and yet others have difficulty recognizing com-
mon written words, even when the words are printed
in the largest and clearest of fonts. Importantly, these
visuoperceptual failures occur despite the agnosic indi-
vidual exhibiting normal or near-normal elementary
visual functions such as acuity, brightness discrimina-
tion, motion perception, and color vision, along with
normal or near-normal semantic and memory func-
tioning. These individuals also have intact alertness,
intelligence, and language, thus setting aside ques-
tions about whether visual agnosia arises simply from
reduced elementary visual function or from compro-
mised intellectual ability. Similar interesting failures
∗ Correspondence to: behrmann@cmu.edu
Department of Psychology, Carnegie Mellon University, Pittsburgh,
PA 15213-3890, USA
DOI: 10.1002/wcs.42
of recognition can be observed in other modalities
such as audition (auditory agnosia) and touch (tactile
agnosia) but, because visual agnosia is perhaps the
best studied of all the modality-specific agnosias, we
focus on this particular subset of neuropsychological
disorders.1–3
VISUAL AGNOSIA: OVERVIEW
The key characteristic of visual agnosia is the patient’s
failure to access the meaning of perceptual infor-
mation presented to the visual modality, in spite
of normal sensory vision, language, and intelligence.
Importantly, too, individuals with agnosia demon-
strate normal recognition of objects through modal-
ities other than vision (touch, audition, and verbal
description of its function); that the patients are able
to recognize inputs in other sensory domains further
attests to the fact that the impairment does not arise
from a difficulty in retrieving names or in accessing
the necessary semantic/knowledge information.
Visual agnosia can be general, affecting the
recognition of all types of visual stimuli or it can
be more specific: for example, there are agnosias that
appear to be relatively selective, affecting only the
recognition of objects and leaving the recognition
of other visual stimuli largely intact. Among these
selective disorders are the agnosia for faces, termed

Vo lu me 1, March /April 2010  2010 Jo h n Wiley & So n s, L td. 203

 Overview
‘prosopagnosia’, the agnosia for words, termed
‘agnosic alexia’ or ‘pure alexia’, the agnosia for body
parts, the agnosia for colors, and the agnosia for
environmental scenes, including landmarks. A large
nomenclature exists to define and distinguish between
the different forms of visual agnosia and these different
types of agnosia are listed in Table 1, and described
briefly in the second half of this review (see also Ref 2).
Note that visual agnosia of any form is rather rare
and, consequently, variants of this disorder are even
rarer.
Independent of the particular form of agnosia,
researchers who examine these disorders as a
means of elucidating the fundamental operational
characteristics of the normal visual system typically
address a key set of questions. Among these are
whether these apparently different forms of agnosia
are indeed domain-specific or whether the presenting
symptom is simply the most pronounced of a host
of recognition failures across multiple domains—that
is, whether an individual who is impaired at
word recognition, for example, truly evinces the
impairment only for orthographic input or whether,
with careful examination and refined testing, other
related perceptual deficits can also be uncovered.
The answer to this particular question is central to
theoretical accounts of category-specificity and the
way in which information is processed and organized
at a psychological and neural level. If it is indeed the
case that, for example, the representation of words
is mediated by a circumscribed, dedicated process for
recognizing words and no other visual stimuli, a lesion
to this dedicated process will naturally result in alexic
agnosia. If, however, the underlying psychological
and neural substrate supporting word recognition
(to continue with the example) is not limited to
words, and patients show a concurrent impairment
for numbers and other alphanumeric symbols (or even
for common everyday objects), then a lesion to that
area of ventral visual cortex might result in an agnosia
that cuts across categories rather than being tightly
restricted to one domain. Other possible forms of
organization and predictions are also possible.
Another key, related question concerns the pro-
file and distribution of the differing forms of agnosia.
There have been several reported cases of fairly ‘pure’
forms of these agnosias but there are also many
reported cases in whom more than one form of
agnosia coexist, and certain types of agnosia appear
to co-occur more frequently than others.4 The obvi-
ous question, then, is why do some forms co-occur
more frequently than others and furthermore, which,
if any, agnosias occur in the complete absence of
any other neuropsychological deficit? Understanding
204  2010 Jo h n Wiley & So n s, L td.
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the co-occurrences and dissociations has important
implications for understanding how visual representa-
tions are organized. An additional intriguing question
concerns the psychological mechanisms that underlie
agnosia. For example, some have suggested that a
problem in discriminating curvature might give rise to
prosopagnosia5 whereas others have suggested that it
is the failure to carry out holistic or configural pro-
cessing that is to be blamed.6 Another, closely related
question concerns the brain mechanism or neural sub-
strate that is affected and whether the different forms
of agnosia and their co-occurrences or dissociations
are systematically predicted by the location of the
lesion. Questions concerning the potential for recov-
ery from such impairments, and procedures by which
such recovery can be enhanced, are also of great inter-
est to researchers and clinicians alike.7 Below, we
provide a general overview of visual agnosia and the
range of etiologies that give rise to the impairments,
and, thereafter, we describe some of the variants in
greater details.
The standard profile of visual agnosia is that
it arises following a lesion sustained by an adult
who possessed normal premorbid perceptual abilities.
Thus, etiologies such as stroke or tumor or other
forms of brain damage such as anoxia (e.g., as
a result of carbon monoxide poisoning) in adults
have, to date, constituted the majority of reported
instances of agnosia. There are, however, several
case reports of children who have become agnosic
following a brain lesion sustained early in life,
a disorder usually referred to as ‘developmental
agnosia’.8,9 Such cases are even more rare than the
adult form of the disorder. More recently, there has
been growing recognition of two other etiologies for
visual agnosia, both of which may turn out to be
more common than the acquired forms described
above. One etiology is the deterioration of perceptual
skills in individuals with progressive posterior cortical
atrophy, which can occur in the absence of dementia.
This visually selective degenerative condition is rather
different from more conventional neurodegeneration,
as in Alzheimer’s disease, and this disorder is being
increasingly detected and identified as a separate
entity, with several reports of such individuals now
in the literature.10 Finally, in the last decade or so,
there have been a growing number of reports of
individuals who demonstrate a lifelong impairment in
recognition that occurs in the absence of any obvious
concomitant—that is, where there is no obvious
alteration in cortical structure evident on conventional
MRI scan. This last form of agnosia has been labeled
‘congenital’ to distinguish it from the disorder in which
children have an acquired, obviously discernable
Vo lu me 1, March /April 2010
 WIREs Cognitive Science
lesion, as mentioned above. There can also be different
forms of congenital agnosia, including congenital
prosopagnosia11 and congenital color agnosia,12 a
selective neuropsychological condition in which color
perception per se is intact, while the identification and
naming of color is disrupted. Developmental dyslexia
or the failure to acquire word recognition normally
(or perhaps just certain subtypes) may also be a form
of neurodevelopmental agnosia. Note that there may
be corresponding atypicalities that are present from
birth in other modalities, such as congenital amusia in
the auditory domain.13
VISUAL AGNOSIA: SUBTYPES
The term ‘visual agnosia’ was originally coined by
Freud to refer to object recognition problems observed
in some individuals. Lissauer, however, was the first
scientist to provide a detailed account of a patient with
visual agnosia and, in so doing, went on to identify
two separate stages through which visual information
passes for successful recognition. The first or
apperceptive stage constitutes ‘the stage of conscious
awareness of a sensory impression’ whereas the
second or associative stage involves the simultaneous
activation of a number of concepts related to the
object (i.e., the activation of associated memories).14
Based on this two-stage theory, an impairment to the
first stage would result in ‘apperceptive mindblindness
(seelenblindheit)’ in which the impaired individual is
unable to construct a good perceptual representation
from the visual input. Damage to the second stage
would result in ‘associative mindblindness’, in which
the affected individual can derive a reasonably (if not
entirely) precise description of the input but cannot
use the well-specified perceptual representation to
access stored knowledge of the object’s functions and
associations.
Apperceptive agnosia is thought to arise from a
breakdown at relatively early stages of visual process-
ing at which the elementary features of the stimulus
are processed and at which a coherent structural
description is achieved. Consequently, this individual
is unable to identify, copy, or even match a drawing
based on the impoverished visual representation
derived from the image. Individuals like this are
obviously very substantially impaired in representing
incoming visual information. Consistent with this,
the lesions associated with apperceptive agnosia are
generally to the occipital lobe and are large and often
diffuse in nature (see Table 1 for further description).
In such cases, knowledge of the shape, form, and/or
other stored knowledge of the physical attributes of
an object cannot be accessed, with the result that the
Vo lu me 1, March /April 2010  2010 Jo h n Wiley & So n s, L td.
Agnosias
stimulus is not successfully identified. The failure to
recognize the object occurs under a whole range of
conditions and independently of whether the stimulus
is presented as a real three-dimensional object, a
black-and-white line drawing, or as a photograph.
Conversely, Lissauer suggested that perception
itself was normal in cases of associative agnosia and
that the disorder arises downstream of perception.
The hallmark of associative agnosia is that the
individual is able to copy and match the object (or
a drawing/rendition of it) well despite the inability
to identify it. This disorder is often described as
‘perception stripped of meaning’, a phrase coined
by Teuber.15 Whether the derived percept is truly
normal in associative agnosia (and simply cannot be
used to access meaning) or whether the representation
is still not sufficiently rich and precise in these cases,
and hence is the source of the recognition failure,
is a topic of much contention. The lesions that
give rise to the higher-order associative forms of
agnosia are usually more circumscribed and affect
cortical structures that are somewhat more anterior,
including the fusiform gyrus, temporal gyrus, or even
the anterior portions of the temporal lobe (see below
for further specification, and Ref 16 for descriptions of
different profiles associated with different lesion sites).
The classical dichotomy of apperceptive–asso-
ciative agnosia, while useful in delimiting cases at
one end or the other of the agnosia spectrum, has
mostly fallen out of favor given that it captures the
extreme cases but not the variants falling in between
the extremes. This simple dichotomy has been sup-
planted by a more fine-grained continuum with a more
precise characterization of the multiple variants of
agnosia.17 Indeed, one reason that the visual agnosias
are so intriguing as a class of clinical phenomena is
that they have important implications for current the-
ories of high-level vision, and this is consistent with the
increased understanding of the complexity of visual
cognition. Thus, amending the classical dichotomy
is compatible with the growing evidence that visual
object recognition comprises a number of distinct
steps (and multiple brain regions) in transforming the
retinal input into representations that reflect invariant
properties of objects in the real world.
The more refined characterization of agnosia
is obviously still in progress but a number of key
points along this more refined continuum have been
demarcated. It is also the case that, relative to
Lissauer’s distinction, the extremes of the continuum
have been expanded and elaborated (where one
endpoint is extreme difficulty deriving the percept,
as in apperceptive agnosia, and the other endpoint
is intact perception but failure to access meaning,
205
 Overview wires.wiley.com/cogsci

TABLE 1 Classification of Types of Visual Agnosia, their Underlying Neuropathology, and Clinical Manifestation
Apperceptive agnosia
Visual form agnosia
Integrative agnosia
Associative agnosia
Impaired access to structural
knowledge
Impaired access to/within
semantic knowledge
Perceptual categorization
deficit
Prosopagnosia
Agnosia for words
Agnosia for scenes
Developmental agnosia
Posterior cortical agnosia
Neuropathology
Stroke, anoxia, carbon monoxide poisoning
affecting occipital, parietal, or posterior
temporal regions bilaterally
Extensive extrastriate damage bilaterally or
just to the right hemisphere
Both types: usually bilateral infarction of the
posterior cerebral arteries but unilateral
temporo-occpital damage may suffice
Not yet definitively specified
Acquired form: bilateral infero-mesial visual
association cortices (lingual and fusiform
gyri) and subjacent white matter
Left occipitotemporal cortex
Bilateral or right posterior artery infarction
involving the fusiform and lingual gyri,
extending to the parahippocampal gyrus
No obvious neural concomitant on
conventional CT or MRI scanning
Focal right temporal and/or occipital lobe
atrophy
Clinical Manifestation
Unable to copy, match, or identify visual stimuli
Able to copy and match stimuli, may even be
able to provide verbal description of aspects of
input but still fails to recognize object
Fails to access stored representation of object
structural description
Fails to access or activate stored semantic
knowledge of objects either just from vision or
from all modalities
Impaired recognition of objects from unusual
views or under unusual lighting conditions
Failure to recognize faces
‘Pure’ alexia—fails to read words normally
despite normal language and sensory visual
function
Failure to recognize landmarks or known scenes
Difficulty in acquiring mastery over word or face
recognition (developmental dyslexia or
developmental/congenital prosopagnosia)
Progressive decline in complex visual functions
and recognition

as in associative agnosia). The nomenclature below
proceeds from the most severe perceptual impairment
to the most intact perception.
Apperceptive Agnosia
Patients with apperceptive agnosia are unable to
construct a stimulus-specific structural description.
Two predominant forms exist, as detailed below.17
Visual Form Agnosia
Visual form agnosia, a term introduced by Benson
and Greenberg,18 and synonymous with severe form
perception, refers to the class of individuals who
complain of blurred or unclear vision but, on formal
examination, their acuity is more than adequate to
recognize objects. In addition, color, brightness, and
movement discrimination are typically preserved,
while the ability to perceive figural properties such
as size, orientation, and shape is typically lost. These
individuals cannot recognize, copy, match, or dis-
criminate simple visual stimuli, and cannot recognize
even simple shapes such as triangles or circles. Some
individuals may also have achromatopsia,19 in which
case they cannot match stimuli by color either. These
patients usually do better with real images than with
line drawings—for example, one such patient recog-
nized only 17% of black-and-white line drawings but
when the same images were presented in color they
were recognized with 26% accuracy.20 Performance
also improves when motion accompanies the input,
presumably because this provides additional cues
to assist in shape perception. Tracing the contours
also aids recognition, because the traced information
can be represented internally through the hand
movements, perhaps by visual imagery processes,
which provides a compensatory approach to support
recognition. The lesions in visual form agnosia are
typically diffuse and posterior. The prominence of

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 WIREs Cognitive Science
white matter lesions suggests that disconnection, often
of very local intralaminar connections, rather than
neuronal loss, may cause the visual deficit. Stroke,
anoxia, carbon monoxide poisoning are common
causes of the disorder; demyelination, tumor, and
mercury poisoning are also causes but somewhat less
common.21,22 A particularly well-known patient who
fits the visual form agnosia description is DF, a patient
who was unable to determine whether a square and
rectangle, matched for total flux, were the same shape
or not.23,24 DF also was impaired at segmenting figure
from ground, deciding whether shapes were symmet-
rical or not and, not surprisingly, was profoundly
impaired at object recognition. DF has garnered
special scientific attention because, in spite of her
profound perceptual disorder, she is nevertheless able
to utilize shape information to correctly orient her
hand to match the orientation of a slot, and to scale
her grasp appropriately to objects of different sizes.
Her pattern of performance has generated a deep
debate about the independence of information used
for perception and action, and many intriguing studies
have been conducted with DF and with similar apper-
ceptive agnosic individuals25 to assess the integrity of
their reaching behavior in the absence of perceptual
precision provided by the ventral visual system.
Integrative Agnosia
While some consider individuals with ‘integrative
agnosia’ to be a less severe form of visual form agnosia,
others consider its distinguishing features sufficiently
different from visual form agnosia and prefer to rec-
ognize it as an independent entity. The key feature in
integrative agnosia is that the coding of single, simple
shapes may be relatively unimpaired, with the deficits
being revealed only under conditions that particularly
stress visual segmentation and grouping. Critically,
patients with integrative agnosia are able to process
the basic features or elements present in a display but
appear unable to group or integrate all aspects into
a meaningful whole.26,27 These patients operate in
a ‘piecemeal’ manner to identify objects, sometimes
oversegmenting the single input object into several
different objects (for example, identifying the handle
of a fork as one object and the tines as a second).
These individuals may perform normally on visual
matching and copying tasks (thus, are similar to the
associative agnosia subtype described below) but their
perception is compromised (and thus, they are similar
to the apperceptive agnosia subtype). Crucially, these
patients perform poorly on tasks that require the
binding of visual elements in a spatially parallel fash-
ion across a visual display containing multiple stimuli.
The failure to integrate the disparate elements occurs
Vo lu me 1, March /April 2010  2010 Jo h n Wiley & So n s, L td.
Agnosias
equally with displays of two- and three-dimensional
stimuli and with black-and-white and chromatic dis-
plays, although in some cases, the presence of depth,
color, and/or surface cues may be of some assistance
to the patients in segmenting the display.28,29 These
integration/segmentation problems are most clearly
demonstrated when there are multiple items present,
such that there is competition in assigning elements
between shapes, as in displays with overlapping
shapes or ones that require boundary assignment.
Interestingly, in some patients, the presence of local
information is so captivating that it reduces the effi-
ciency of visual recognition; thus, counterintuitively,
in contrast with normal perceivers, some patients with
integrative agnosia identified silhouettes better than
line drawings, whose internal details apparently led to
incorrect segmentation.30 Figure-ground segregation
and other forms of perceptual grouping, such as
deriving a unified whole based on Gestalt heuristics,
may be especially challenging for such individuals.
Also, the rapid and efficient access to the local
information may impede the patients’ ability to gain
access subsequently to the global information.31
Associative Agnosia
Further along the continuum of visual agnosia are the
associative types who show normal or near-normal
performance on tests of perception but are still
impaired at object recognition. In some cases,
recognition of all objects is abnormal whereas, in
others, recognition of visually simpler objects such
as a pen or watch may be better than recognition of
objects that are more complex visually (and perhaps
also less familiar) like mushroom, harmonica, or
harp. The most common cause of associative visual
agnosia is bilateral infarction of the posterior cerebral
arteries. Demyelination, hemorrhage, and tumor are
uncommon causes whereas bilateral lesions involving
the inferior temporo-occipital junction and subjacent
white matter are the most common anatomic lesions
associated with this form of agnosia.22 Note, however,
that this disorder can also occur with unilateral dam-
age to the left or right temporo-occipital region.32,33
As with apperceptive agnosia, the associative form
can be subdivided into two variants.17
Impairment in Structural Knowledge
One form of associative agnosia arises from the
difficulty in accessing the perceptual knowledge or
structural description of an object. These individuals
would likely also have difficulty accessing the
semantics associated with the observed object but not
vice versa. For example, one of the ways of assessing
207
 Overview
the integrity of access to the structural description
is to ask patients to decide whether objects are real
or not (when they are not real, they are ‘legitimate’,
in other words made of real parts of objects just
incorrectly combined such as the head of a horse on
the body of a dog). Given the sensitivity of these
individuals to the perceptual nature of the input, it is
not surprising that recognition is adversely affected by
increasing complexity of the object. Some of these
individuals evince a category-specific impairment,
being disproportionately poorer in recognition of
living things. A possible explanation for this is that
many living things have a similar perceptual structure,
and this creates increased competition for recognition
and naming. The competition results in longer reaction
times as well as higher error rates (and this pattern
may also be seen in normal individuals, albeit to a
much milder extent).
Some of these associative agnosic patients still
retain the long-term representation of the object (i.e.,
can provide a verbal description of the objects) but,
nevertheless, fail to recognize the stimulus presented.
It has been possible, for example, to demonstrate
reasonably good performance in visual imagery tasks,
demonstrating that stored visual knowledge may be
at least relatively preserved.34,35 Thus, some patients
may be able to visualize in their ‘mind’s eye’ a
particular object but, presented with the same object,
may fail to identify it from vision. Consistent with
this is the claim that neuronal templates that match
a visual stimulus with a visual memory are stored
primarily in ventral visual association cortex and the
failure to update or refresh these representations in
individuals with a long history of agnosia can affect
the integrity of these internal representations such that
they then begin to decay.36 Note that even patients
who have impaired semantic knowledge, may still be
able to make correct object decisions, indicating that
access to structural descriptions may be intact and
independent of the status of semantic knowledge,17
supporting the dissociation between structural and
semantic knowledge.
Impairment in Accessing Semantics
Semantic knowledge specifies information about
object function and interobject associations, and this
forms the basis of our full concept of an object. There
are some cases of associative agnosia in whom the
problem is closer to semantics37 and others in whom
the problem arises in the semantic system itself. In
both cases, patients may be unable to make decisions
about which two of three visually presented objects
from the same category belong together and which
one ‘odd’ as when presented with a hammer, a nail
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and a screw, or a pyramid, a palm tree and a pine
tree.38 In those cases, in whom the deficit is in the
semantic system, the problem may extend beyond their
knowledge of the visual or perceptual properties of
stimuli and affect knowledge of functional attributes
too (for example, the function of a hammer vs.
a screwdriver). There are also cases who evince
disproportionate impairment in some categories over
others, for example, performing more poorly when
recognizing living or animate objects than inanimate
objects and others in whom the disorder affects
mainly recognition of nonliving or inanimate objects.
Interesting theoretical exchanges about the type of
information (functional vs. perceptual) tapped for the
recognition of living versus nonliving objects,39 as
well as questions about differential lesion localization
in these two cases abound in the literature.40 Patients
with disorders within the semantic system will have
damaged long-term representations,41 and imagery
should not be possible in these cases.
Perceptual Categorization Deficit
Warrington and colleagues described a set of patients
who performed poorly at recognizing or matching
objects seen from unusual views or illuminated in ways
that produced confusing shadows.42 These individu-
als were neither apperceptive nor associative agnosics
but experienced difficulty in object recognition under
these particular conditions. The term ‘perceptual cat-
egorization deficits’ was coined to describe this deficit
in object shape constancy that impaired specifically
the processes that allow us to recognize the equiv-
alence of an object’s three-dimensional appearance
when viewed from different perspectives and under
different lighting conditions rather than a problem
in shape perception per se. The recognition deficit is
usually apparent only when an object is seen from an
unusual view that eliminates important features and
is often identified in patients who have no problems
with object recognition in their normal environments.
Impairment of perceptual categorization may there-
fore be considered an abnormality in the systems that
solve visual problems, perform mental rotation, and
support visual imagination—not in the visual systems
that mediate object recognition per se.
Prosopagnosia
Individuals with prosopagnosia are unable to recog-
nize individual faces although they are almost always
capable of detecting whether a face is present in the
input.43 The disorder may be so profound that the
affected individual may fail to recognize him/herself
from photographs and may fail to recognize family
Vo lu me 1, March /April 2010
 WIREs Cognitive Science
members and close friends too. In many instances,
these patients can describe the face they are looking at
in detail, including the age and gender of the person,
but are unable to say whose face it is. A prosopagnosic
patient’s self-report, provided by Pallis,44 is illustra-
tive:
I can see the eyes, nose and mouth quite clearly but
they just don’t add up. They all seem chalked in, like
on a blackboard. I have to tell by the clothes or voice
whether it is a man or woman, as the faces are all
neutral, a dirty gray color (he also had achromatopsia
or a cortical color vision problem). The hair may help
a lot, or if there is a moustache. . .All the men appear
unshaven. . .I cannot recognize people in photographs,
not even myself. At the club I saw someone strange
staring at me and asked the steward who it was.
You’ll laugh at me. I’d been looking at myself in the
mirror. . .I later went to London and visited several
cinemas and theaters. I couldn’t make head or tail of
the plots. I never knew who was who. . .I can shut my
eyes and can well remember what my wife looked like
or the kids.
The ability to extract information about emo-
tional expression is preserved in some cases but
impaired in others. Invariably, these patients rely
on non-face characteristics such as gait or voice to
recognize individuals in their daily lives. The deficit is
typically perceptual in nature, rather than arising from
a memory problem (although there are prosopamnesic
cases for whom memory failure for faces is the core
of the deficit45 ) or semantic deficit, nor from a fail-
ure to label the face (an anomia). In most cases, the
affected individual also performs poorly at discrimi-
nating between two faces, even novel faces and even
under conditions in which they are given unlimited
time in which to make the discrimination decision,
although the extent to which the deficit is more apper-
ceptive versus more associative may determine the
exact nature of the failures.46 Many studies suggest
that these patients with acquired prosopagnosia pro-
cess faces differently than controls, focusing more on
individual features than on the holistic or second-order
relations between the features, and on some accounts,
the ‘configural deficit’ that impairs perception of spa-
tial structure, affects not just faces but also other
non-facial patterns (for recent example, see Ref 6).
There are now several studies in the recent literature
showing that individuals with acquired prosopagnosia
are less sensitive to the information in the eye region
than their counterparts and perhaps focus unduly on
the mouth region (for a recent example, see Ref 47).
The idea is that these individuals have lost the ability
to represent multiple elements of an individual face as
a perceptual unit (holistic face perception) and so rely
Vo lu me 1, March /April 2010  2010 Jo h n Wiley & So n s, L td.
Agnosias
more on local features such as the mouth to identify
a given face, and they focus very precisely on local
features rather than seeing the whole of a face from
its diagnostic center.
The lesion that gives rise to prosopagnosia is usu-
ally secondary to temporo-occipital lesions, affecting
the fusiform and lingual gyri, and whereas a bilateral
lesion was thought to be necessary, there is growing
consensus that a unilateral right lesion to these regions
may be sufficient to give rise to this disorder.19
Agnosia for Words
Agnosia for words is also known as pure alexia, alexia
without agraphia (as in the original label assigned
by the French neurologist Jules Dejerine), perceptual
word-form agnosia, or pure word blindness. Although
this phenomenon is usually discussed in the context
of language impairments, it is an agnosic symptom,
as subjects who suffer from this deficit show a
language impairment limited to visually presented
stimuli (e.g., reading), but not to auditorily presented
stimuli.48 These patients may be able to spell words
out loud and to write well but fail to read their own
handwriting subsequently. This disorder has also been
called ventral simultanagnosia to indicate the failure
to process multiple letters simultaneously and this
failure manifests most dramatically in word reading
where individuals laboriously and sequentially process
one letter at a time (giving rise to the label ‘letter-by-
letter’ reading). Pure alexia is one of the more common
forms of agnosia and can be severely debilitating, with
some patients requiring 1.4 s to process each letter in
a string. Whether this form of agnosia is entirely
limited to word recognition or whether other classes
of visual objects are also affected, perhaps to a lesser
degree, remains controversial. In one recent study
with four such individuals, recognition of single letters
and digits in the central visual field was impaired in
all patients. In addition, visual apprehension span
was also reduced for both letters and digits in all
patients.49 A similar study has shown that seven
such patients are even impaired at deciding whether
two black-and-white checkerboards are the same or
different (with one cell of the matrix flipped) and
that this was especially true as the size of the matrix
increased.50 These recent findings, along with those
of other researchers, offer strong support for a deficit,
which is perceptual in nature and rather widespread
in impact, and affecting not just orthographic input
but other forms of fine-grained visual discrimination.
The lesion site in these patients is typically in the left
ventral occipitotemporal cortex, sometimes, but not
always, involving the splenium of the corpus callosum.
209
 Overview
Agnosia for Landmarks and Environment
An impairment in recognizing landmarks or scenes,
referred to as topographic agnosia, can co-occur
with agnosia, prosopagnosia, or it can also occur
in isolation. As with other forms of agnosia, these
patients perform well on a wide range of memory and
basic perception tasks. In these topographic cases,
known landmarks and buildings lose their familiarity
to the patients. Unsurprisingly, these patients get lost
and are unable to learn new routes. They show undue
reliance on street names and numbers of buildings to
identify the landmarks. Topographic agnosia can be
differentiated from other disorders affecting spatial
orientation in a large-scale environment. In one
recent study,51 the authors described a patient who
got lost in familiar environments but could readily
read and draw maps, describe familiar routes, and
provide correct directions. On a familiar route, this
particular patient could recognize major landmarks
but could not 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
but had difficulty in recognizing and learning scenes
lacking salient landmarks. The lesion giving rise to
topographic agnosia most commonly follows bilateral
or right posterior artery infarction and the lesions
(as in prosopagnosia) may implicate the fusiform and
lingual gyri, extending to the parahippocampal gyrus.
Neuroimaging studies provide converging evidence for
topographic-specific representations in these cortical
regions, such as in the so-called parahippocampal
place area.52
DEVELOPMENTAL FORMS
OF AGNOSIA
The taxonomy and illustration of the agnosias
described above refer to those instances in which
the object recognition failures occurred in adults who
were premorbidly normal. As mentioned at the outset
of this review, however, there is growing interest in
the type of agnosia that appears to be present from
childhood. These childhood agnosias may themselves
be subdivided into two subgroups: ‘developmental
agnosia’ in which individuals who are born normal
become agnosic following a lesion, such as stroke,
sustained during childhood (for example, see Refs 9
and 53), and ‘congenital agnosia’ in which the disorder
is congenital or, at a minimum, lifelong, in the absence
of any obvious neurological concomitants (of course
it is difficult to determine whether the disorder is truly
210  2010 Jo h n Wiley & So n s, L td.
wires.wiley.com/cogsci
in existence from birth but there seems no reason
to believe otherwise).54 Developmental dyslexia, or at
least one subtype thereof in which perceptual encoding
is affected, may also be a form of childhood agnosia,
and it is the case that while some individuals appear to
have an obvious underlying neural concomitant, this
is not the case for all individuals. Recently, there have
been a substantial number of reports of individuals
who are congenitally prosopagnosic.11 Some of these
individuals appear to be as impaired as cases in whom
a clear lesion is evident. For example, one of the
individuals who has participated in several of the
investigations in our lab said the following:
I grew up in a small town of about 7000 people
and most everyone knew everyone else. During High
School I worked in the local movie theater and was
quite good at selling the tickets, making change
by hand and doing the reports that went with it.
However, my Mom would say things like, ‘Mr. and
Mrs. X (vocal emphasis), our neighbors, or Mary
(vocal emphasis) from our church told me that you
are stuck up. They went to the movies and you sold
them a ticket; but, you didn’t say hello to them.’ I
never realized that they were people I knew or was
suppose to know. I’ve always been amazed at how
people recognized other people so easily.
Because of the availability of high resolution imaging,
and because these congenitally prosopagnosic indi-
viduals do not have any vascular limitations making
them ideal candidates for imaging, many neural stud-
ies have been conducted with such individuals. Among
the results obtained is that many, albeit not all, of
these individuals evince normal cortical activation,
in functional magnetic resonance imaging investiga-
tions, in the ‘fusiform face area’, the pre-eminent
occipitotemporal region of cortex associated with
face perception.55 They do, however, in contrast with
matched counterparts, fail to show activation in more
anterior regions perhaps associated with biographical
and individual identity representations,56 and, consis-
tent with this, the white matter tracts that project from
the more posterior to more anterior regions appear
to be compromised in the individuals, to an extent
commensurate with their face recognition difficulty.57
Finally, there have been several reports of
individuals who are congenitally agnosic for color.
These individuals are unable to recognize colors
despite intact color perception, semantic memory for
color information, and color naming.12
Much research remains to be done with
individuals with the congenital variants of agnosia to
characterize the disorders more fully and to uncover
the underlying neural mechanism that may give rise to
these disorders. It is also the case that there appears to
Vo lu me 1, March /April 2010
 WIREs Cognitive Science
be a familial hereditary aspect associated with most of
these neurodevelopmental disorders and this promises
a fruitful avenue for further genotyping studies in
agnosic individuals and their family members.
PROGRESSIVE POSTERIOR CORTICAL
ATROPHY
Just as there appear to be forms of agnosia that arise in
childhood so there are forms of agnosia that can occur
relatively selectively as a form of progressive cortical
deterioration associated with aging. Posterior cortical
atrophy refers to the progressive decline in complex
visual processing ability along with relative sparing
of other cognitive and perceptual functions.58,59 Later
on, memory and other cognitive dysfunctions may also
be involved. Progressive visual agnosia is associated
with occipitoparietal atrophy and hypometabolism
on single photon emission computed tomography
(SPECT) and on positron emission tomography (PET)
scans,60 especially in the right hemisphere, and can be
independent of Alzheimer’s disease.
Progressive prosopagnosia is a degenerative
agnosia, too, in which there is a more selective
progressive impairment in the recognition of faces.61
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