Specific Disorders of Vision

Disorders of the visual system, along the pathway from the eye to the occipital lobes depend on
where along the pathway a lesion occurs. Because the pathway is topographically oriented, visual
deficits give a good indication of lesion location (Zilmer & Spiers, 2001).

Diplopia
Diplopia, commonly known as double vision, is the simultaneous perception of two images of a
single object that may be displaced horizontally, vertically, or diagonally (i.e. both vertically and
horizontally) in relation to each other (Kernich, 2006).
It is usually the result of impaired function of the extraocular muscles where both eyes are still
functional but they cannot converge to target the desired object. If the two eyes are misaligned and
aim at different targets, two non-matching images will be sent to the viewer's brain. When the brain
accepts and uses two non-matching images at the same time, double vision results (Kernich, 2006).
Problems with extraocular muscles may be due to mechanical problems (such as the muscle-fibrosis
that occurs with Grave’s Disease), disorders of the neuromuscular junction, disorders of the cranial
nerves (III, IV, and VI) that stimulate the muscles, or ingestion of toxins. There are four muscle(s)
(SR,SO,IO,IRJ which must be extensively evaluated(Kernich, 2006).
Double vision is dangerous to survival, so, the brain naturally guards against its occurrence. In an
attempt to avoid double vision, the brain will eventually disregard one of the mismatching images.
That is, the brain will ignore one eye (called suppression) (Kernich, 2006).
Due to the brain's ability to suppress one eye, a person's double vision can appear to go away
without medical evaluation or treatment. Bear in mind that the causes of the double vision are very
likely still present and that loss of vision in one eye has probably occurred due to lack of treatment.
When vision in one eye is lost, the person has also lost normal depth perception and stereo vision.
However, the loss of vision could be temporary and treatable (Kernich, 2006).
If the double-vision remains when you cover an eye then you have a monocular diplopia. Monocular
double vision isn’t a neurologic problem at all and your exam just got easier! Monocular doubling is
often caused by a refractive problem in the front part of the eye (Kernich, 2006)..
Monocular diplopia may be of external, optical, neurological, neuromuscular, or psychogenic origin.
It may develop spontaneously or it may be induced by surgery or trauma. Because treatment is
usually directed toward the cause, determination of the etiology is important.
There aren’t any mechanisms of monocular doubling that occur at the retina or further back in the
neuro pathway. The most common cause of monocular diplopia is astigmatism, an abnormal
curvature of the corneal surface. New onset astigmatism could occur from corneal deformation from
an overlying lid lesion or after surgery with tight corneal stitches through the cornea. Other causes of
monocular diplopia include cataract irregularities, lens displacement, or primary problems with
corneal curvature. A form of double vision in which two objects are seen with the same eye, and
that is caused by an opacity in the visual axis (Kernich, 2006).

Ptosis
Ptosis is a drooping or falling of the upper or lower eyelid with the eye in primary gaze and may
cause a reduction in the field of vision when the eyelid either partially or completely obstructs the
pupil. The drooping may be worse after being awake longer, when the individual's muscles are tired
(Cluster, 2008).
There are many causes of ptosis including age related weakening of the muscle, congenital
weakness, trauma (injury to the oculomotor nerve), or sometimes neurologic disease. As we age, the
tendon that attaches the levator muscle, the major muscle that lifts the eyelid can stretch and cause
the eyelid to fall. This represents the most common cause of a droopy eyelid (Cluster, 2008).
 Monocular blindness
If the optic nerve is lesioned, vision functions only in the intact eye (monocular blindness) (Martin,
2006). If a lesion occurs anterior to the optic chiasm, effectively cutting off all visual input from one
eye, the effect is blindness in one eye. Both visual fields with full peripheral vision remain in the
other eye (Zilmer & Spiers, 2001).

Cortical Blindness
Cortical blindness refers to loss of vision following damage to the primary visual cortex. Damage to
area V1 causes cortical blindness, or hemianopia, in the opposite field (Zilmer & Spiers, 2001).
It results in the inability to distinguish forms and patterns while remaining responsive to light and
dark. It has been argued that visual discrimination takes place at the thalamic level while the cortex
is necessary for the conscious experience of visual stimuli. In fact, total blindness requires some
destruction of the thalamus and visual cortex and its afferent pathways (Martin, 2006).

Blindsight
Patients who have recognised sensory impairment such as cortical blindness but who present with a
more unusual problem: they appear able to make simple perceptual decisions about visual stimuli
despite claiming to be unaware of the stimuli and despite the absence of the part of the brain
responsible for higher visual functioning, the striate cortex. This is known as blindsight (Martin,
2006). Patients with blindsight are often not able to follow a moving object with their eyes. Some
cases have been reported of patients who could grasp moving objects or indicate the direction of
movement despite reporting being unable to see the objects (Martin, 2006). Blindsight patients can
discriminate some stimuli in these areas but report being unaware of the stimuli presented (Martin,
2006). Sanders et al. (1974) reported that damage to the striate cortex affected the ability to ‘see’
objects but not the ability to locate apparently unseen objects. Thus in blindsight, the patient acts
and feels blind yet shows a residual ability to localise stimuli (Gazzaniga, Ivry & Mangun, 2002). If
patients with total cortical blindness perceive movement of objects consciously, this is Riddoch’s
syndrome; unconsciously is blindsight.
Sometimes individuals develop blind spots or scotomas, which result from isolated lesions of the
primary visual cortex. Individuals are aware of what they see and are capable of responding to
stimuli because involuntary eye movements are made that cover these blind spots (nystagmus).
When the eyes move about the scotomas move about too. More information about the visual field
thus reaches the brain. However, if the patient remains still and an object is placed in front of the
scotoma that object cannot be seen (Martin, 2006).
Patient DB (Weiskrantz, 1986) had surgery to remove an AVM in his right occipital lobe. The removal
of the right striate cortex resulted in left visual field scotoma, specifically in the lower left quadrant
of his visual field. He was unaware of stimuli presented in his blind field of vision but he performed
well above chance on a number of simple perceptual tasks – whether a stick was horizontal; the
location of stimuli by pointing; presence or absence of stimuli and distinguishing certain shapes.
When stimuli were within 20 degrees of the fovea, DB’s eye movements were highly correlated with
the position of the stimuli.

Anton’s Syndrome
Anton’s syndrome describes the condition in which patients deny their blindness (visual
anosognosia) despite objective evidence of visual loss and often confabulate to support their stance.
It is a rare extension of cortical blindness (Maddula, Lutton & Keegan, 2009).
In denial of blindness – visual anosagnosia or Anton’s syndrome, patients do not acknowledge that
they are blind and behave as if sighted (Maddula, Lutton & Keegan, 2009).
Anterior visual tracts are often intact. In addition to injury to the occipital cortex, other cortical
areas, such as language areas, are affected. In the absence of input, functioning speech areas often
confabulate. Corticothalamic connections are thought to be disrupted here, as are sensory loops
(Martin, 2006). cerebrovascular accident is the most common cause of Anton’s syndrome. However,
any condition that causes cortical blindness may result in Anton’s syndrome (Maddula, Lutton &
Keegan, 2009).

Anopias
Homonymous Hemianopia
Anopsia refers to or visual difficulties.
Damage to the optic nerve can also result in blindness in the temporal crescent on the lesioned side.
Damage to the fibres crossing the optic chiasm results in bilateral hemianopia, which is the loss of
vision in the temporal parts of both visual fields (Martin, 2006).
If the optic nerve is cut posterior to the optic chiasm, one visual field in both eyes is destroyed –
homonymous hemianopia (Zilmer & Spiers, 2001). Quadrantic anopia occurs when there is partial
loss of vision in one visual field (Martin, 2006).
A homonymous hemianopia can result from a lesion to one optic tract (Martin, 2006). This term
refers to partial blindness on the same side (visual field) of each eye (Zilmer & Spiers, 2001) / there is
complete loss of vision in the contralateral visual field (Martin, 2006). This problem is also attributed
to unilateral damage to the right or left occipital lobes. This condition is often compounded by other
problems, such as muscle weaknesses of the eyes that prevent a synchronised movement of the
eyes across space (Zilmer & Spiers, 2001). Often right- or left- homonymous hemianopias occur as a
result of haemorrhage, tumour or trauma (Zilmer & Spiers, 2001).

Achromatopsia
When we speak of someone who is colour blind, this describes someone who has inherited a gene
that produces and abnormality in the photoreceptor system. Dischromats or people with only 2
photopigments can be classified as red-green colour blind if they are missing the photopigment
sensitive to medium or long wavelengths; blue yellow colour blind if they are missing the
photopigment sensitive to short wavelengths (Gazzaniga, Ivry & Mangun, 2002).
Anomalous trichcromats have all three photopigments but one has abnormal sensitivity (Gazzaniga,
Ivry & Mangun, 2002).
V4 is selective for the electromagnetic wavelengths of colour, some aspects of line orientation and
form. Damage to V4 results in achromatopsia, which is the complete loss of the ability to detect
colour (Zilmer & Spiers, 2001), in which the world is perceived in shades of grey (Martin, 2006).
Patients describe colours as dirty shades of gray. The shading reflects variations in brightness rather
than hue. Patients are better able to perceive brightness than hue. Depth and texture perception are
intact, allowing these patients to see and recognise objects (Gazzaniga, Ivry & Mangun, 2002).
Achromatopsia has been associated with lesions that encompass V4 and the area anterior to V4 but
lesions typically extend to neighbouring regions of the visual cortex. Colour sensitive neurons are
also orientation sensitive, so damage affects form perception. Thus, characterising V4 as a colour
area is too simplistic. This area is part of secondary visual areas devoted to shape perception. Colour
can provide important cues about an object’s shape (Gazzaniga, Ivry & Mangun, 2002).
A deficit of colour perception is less likely to occur if the lesion is in regions upstream to V4, such as
blobs of V1 or the thin stripes of V2. Severe colour discrimination may occur in lesions of the
parvocellular layers of the LGN (Schiller & Logothetis, 1990). These lesions also affect perception. V4
may be important in using colour information to define regions of visual space and partition objects
within that space (Gazzaniga, Ivry & Mangun, 2002).

Akinetopsia
Area V5 or the human equivalent of MT contains visual motion detector cells, specialised to respond
to the direction of movement. Columns of cells within the layers of the cortex are responsive to
different directions (Zilmer & Spiers, 2001). The importance of MT was confirmed in primate studies
in which motion perception deficits were induced by focal lesions to this area. Also, the contribution
of the M system was shown by the fact that more striking deficits were found when lesions were
targeted to the magnocellular layer of the LGN. These lesions did not cause deficits in hue
discrimination (Gazzaniga, Ivry & Mangun, 2002).
Lesions to V5 result in akinetopsia, which is the inability to identify objects in motion (Zilmer &
Spiers, 2001). The patient cannot see or understand the world in motion. Curiously, when objects
are still, they can be seen clearly; when they are moved they disappear. This occurs even when
motion is actually illusory (Martin, 2006).
M.P. had akinetopsia; perception was akin to viewing the world as snapshots. Rather than seeing
things move continuously, she would see objects appear in one position and then in another. Her
colour and form perception were intact. Her ability to perceive briefly-presented objects was
normal. But she was unable to judge direction and speed of moving objects. This was most apparent
for things moving at high speeds. CT scans revealed large bilateral lesions of the temporoparietal
cortices. On each side, the lesion included the posterior and lateral portion of the middle temporal
gyrus. These lesions roughly correspond to areas that participate in motion perception. The lesions
were lateral and superior to V4 (Gazzaniga, Ivry & Mangun, 2002).
Severe forms of akinetopsia only seem to result from bilateral lesions; similar patients have not been
identified for many years. Unilateral lesions, the motion perception deficits are more subtle (Plant et
al., 1993). Perhaps people can perceive motion as long as human MT is intact in at least one
hemisphere (Gazzaniga, Ivry & Mangun, 2002).
Damage to areas V3 – V5 can result in the general inability to perceive form. The ability to copy is
often preserved, but patients are unable to understand that the connection of lines corresponds to a
specific shape or object (Zilmer & Spiers, 2001).

Optic/Oculomotor Apraxias and Optic Ataxias

Lesions to the superior parietal cortex can lead to impairments in visually directed reaching
movements. This is called optic ataxia and is characterised by inaccurate movements and an erring
of movement in the direction ipsilateral to the side of the lesion. The duration of movement is longer
in these patients and velocity of movement is reduced. Commonly it is found that patients cannot
guide their hand to a slit in an object and are sometimes impaired at grasping and manipulating
objects (Martin, 2006). Patients are unable to use visual information to guide their action. They are
able to recognise objects. Their eye movements present a loss of spatial knowledge, saccades or
directed eye movements may be directed inappropriately and fail to bring the object within the
fovea (Gazzaniga, Ivry & Mangun, 2002).
Optic ataxia is associated with lesions of the parietal cortex.
ataxia - abnormality in performing smooth and coordinated move
apraxia - inability to form the appropriate (voluntary) move
optic ataxia - person is unable to gaze and search smoothly
oculomotor apraxia - person is unable to control eye movements
A 47-year-old man with a left temporo-occipital infarct in the area of the posterior cerebral artery is
presented. The neuropsychological examination did not reveal aphasia or gross mental deficits. The
patient presented with alexia without agraphia, color agnosia, but few visual perceptual deficits. The
patient exhibited a deficit in the evocation of gesture from the visual presentation of an object (optic
apraxia) and a difficulty in “conjuring up” visual images of objects (impaired visual imagery) and loss
of dreams (Peña-Casanova, Roig-Rovira, Bermudez, & Tolosa-Sarro, 1985).

Syndromes of the ‘What’ system

Metamorphopsia
Metamorphopsia encompasses a wide spectrum of visual perceptual distortions, such as alteration
of perceived object size (micropsia and macropsia) or, rarely, altered perception of faces
(prosopometamorphopsia; ffytche & Howard, 1999). Prosopometamorphopsia can involve the
whole face, but also only one side of face, usually after right hemisphere damage.
DG suddenly developed prosopometamorphopsia after a childbirth; she claimed that the left half of
well-known and unfamiliar faces looked distorted. Brain MR was normal, whereas SPECT showed
hypoperfusion of the left infero-lateral occipital cortex. No visual recognition defects for objects or
faces were present. In three matching tasks with half-faces, chimeric faces, or chimeric objects, the
patient was impaired only when she matched pairs of chimeric faces differing in their left half; the
same results were obtained after 1 year (Trojano, Conson, Salzano, Manzo, & Grossi, 2009).

For instance, Brust and Behrens (1977) observed a patient with a right posterior temporal damage
who had episodes of visual illusions during which the right half of faces seemed to melt, “like clocks
in a Dalì painting”. An example of stable unilateral face distortion (the right side of a face appearing
smaller than the left) has been reported by Ebata et al. (1991) in a patient with a small haematoma
in the right retrosplenial region. In such cases, visual distortion did not involve perception of objects
other than faces, and tasks assessing apperceptive or associative face processing did not show
relevant impairments.
Unilateral prosopometamorphopsia after a left hemisphere lesion has been described in patients
who however also showed metamorphopsia for objects or body parts (Imai, Nohira, Miyata, Okabe,
& Hamaguchi, 1995).
Patient M.Z. described by Nijboer et al. (2008) had a left temporo-occipital lesion and unilateral
contralesional metamorphopsia; however, in an object confrontation task, she reported that objects
presented on her right side appeared distorted as well. From the above overview, it should appear
that only right hemisphere lesions can determine selective unilateral prosopometamorphopsia,
consistent with the idea of a right specialization for face processing.
All these cases can be explained in terms of abnormal patterns of activity produced when later
processing in the ventral visual processing stream is driven by sub cortical afferents rather than its
usual inputs from early vision.

Agnosias
Agnosia is classically defined as a failure of recognition that cannot be attributed to elementary
sensory defects, mental deterioration, attentional disturbances, aphasic misnaming or unfamiliarity
with the sensorially presented stimuli. Visual agnosia is commonly studied. Patients are unable to
recognise an object by sight but will be able to name it if allowed to palpate it (Martin, 2006).

Agnosia, which is a term that was originally coined by Sigmund Freud is a literal absence of knowing
(gnosis) that can occur in any sensory domain. In the modality of vision, people with visual object
agnosia may fail to recognise objects, or in milder forms, may confuse objects that are observed
from different angles or in different lighting conditions. Patients are visually unaware of the totality
or gestalt of objects. Patients are able to see and identify form and colour but are unable to combine
these aspects into a higher sense of meaning. This is typical of how visual agnosia primarily involves
the processes necessary for object recognition or object meaning while leaving elementary visual
processes intact (Zilmer & Spiers, 2001).
Patient DF could not recognise orientation of a three dimensional object. This is indicative of severe
agnosia. Yet when DF was asked to insert a card into a slot, her performance indicated that she had
processed the orientation of the slot. This dissociation suggests that the ‘what’ and where systems
support different aspects of cognition. The ‘what’ system is essential for determining the identity of
an object. The ‘where’ system appears to be essential for more than determining the location of
different objects; it is also critical for guiding interactions between objects. DF’s performance
provides an example of how information accessible to action systems can be dissociated from
information accessible to consciousness (Gazzaniga, Ivry & Mangun, 2002).
People with visual agnosia are able to see; the disorder is less a pure sensory disorder than a higher
perceptual disorder of ‘knowing’ (Zilmer & Spiers, 2001).

Apperceptive agnosia
Apperceptive visual agnosia is due to damage to the right parietal or temporal lobes (Martin, 2006).
The essential difficulty in apperceptive visual agnosia is object recognition, or the inability to
combine the individual aspects of visual information such as line, shape, colour and form together to
form a whole percept. Their brains are not synthesising the entire picture. Patients with
apperceptive agnosia are often, at first glance, thought to be blind because they take no apparent
notice of people and objects in their vicinity. But sensory functions are clearly intact. Many people
are aware that they are able to see but have a problem in perceiving things correctly. However,
others are unaware of their condition. It is only through observation that one notices they avoid
objects (Zilmer & Spiers, 2001).
Some apperceptives may appear to disregard their problem until neuropsychological testing reveals
it for them. Specifically, visual recognition tasks in which an object must be identified from
fragments, is embedded or at an odd angle are notoriously difficult. These patients also have
difficulty copying objects. Because they only ‘see’ pieces, their drawings are likely to appear as a set
of unconnected fragments focussing on the details and not the entire gestalt of the object (Zilmer &
Spiers, 2001).
Patients may perform normally on shape discrimination tasks yet make many mistakes when asked
to recognise line drawings or photographs of objects. Patients are asked to make fine discriminations
between shapes of simple geometric figures, their performance is often only slightly worse than
control subjects. The problem then, is that perceptual categorisation is impaired – the patient’s
ability to achieve object constancy. Patients had difficulty naming objects from unusual orientations
and on a second task, patients could not recognise that pairs of photographs were depicting the
same object (warrington, ). Thus patients with right parietal lobe lesions are less able to recognise
objects, especially if salient features of objects must be inferred from limited perceptual input
(Gazzaniga, Ivry & Mangun, 2002).
The most common site of damage is the parietal-occipital area of the right hemisphere. Sudden
insults to the brain are the most common cause, often from carbon monoxide poisoning, mercury
intoxication, cardiac arrest or stroke. In these cases, apperceptive agnosia does not usually occur in
isolation without other visual-spatial impairment because these brain insults are likely to affect large
areas of the cortex. Some cases of apperceptive agnosia are caused by bilateral cortical atrophy
(Zilmer & Spiers, 2001).
If both hemispheres are involved, then the patient may show Balint’s syndrome, which includes
visual agnosia and other visual-spatial difficulties such as misreaching and left-sided neglect (Zilmer
& Spiers, 2001).

Balint’s syndrome
Patients with Balint’s syndrome (Balint, 1909 as cited in Martin, 2006) show bilateral lesions of the
inferior parietal cortex. It is especially severe when frontal lesions are present too. While rare, this
disorder is characterised by three principal symptoms:
Neglect of the left visual field and parts of the right with attentional gaze at 35-40 degrees to
the right;
Inattention to other objects when the object in the field of vision has been detected; and
Difficulty in reading under guidance or optic ataxia.
Apart from these visuo-spatial impairments, patients do not exhibit cognitive or visual deficits
(Martin, 2006).
 Associative agnosia
Associative visual agnosia is a failure of visual object recognition that cannot be attributed to
perceptual abilities and is due to damage in the left posterior areas of the brain (Martin, 2006), often
including the occipital region and extending to the posterior temporal cortex. Patients are rarely able
to perform normally on perceptual tasks but perceptual deficiencies are not proportional to their
recognition problems (Gazzaniga, Ivry & Mangun, 2002).
Lesions in the right hemisphere - they fail to recognise many objects, especially those depicted in an
unconventional manner, such as a closed umbrella. Patients with left-sided lesions can often
recognise objects in isolation but they cannot make the functional connection between the 2 visual
percepts. They lack the semantic representations needed to link the functional association
(Gazzaniga, Ivry & Mangun, 2002).
Associative visual agnostics have difficulty to varying degrees in assigning meaning to an object.
These patients are able to recognise differences in form between pictures of a pair of scissors and a
punch by matching the scissors to a like office object, but they have lost the link between the visual
percept and the semantic meaning. Neither apperceptive or associatives would be able to name the
object. Associatives are unable to demonstrate the use for scissors (Zilmer & Spiers, 2001).
Associative visual agnosia is differentiated behaviourally from apperceptive agnosia in that the
primary difficulty is loss of knowledge of the semantic meaning of objects. Conceptually the person
can recognise objects at a perceptual level by picking them out or copying them correctly but
perception breaks down at a higher level of meaning. Associative visual agnostics are able to copy
pictures of objects in great detail but are unable to name them. Many patients also show aspects of
apperceptive agnosia. For example, they copy objects inconsistently however the perceptual
mistakes of the Associative visual agnostic are likely to show problems of either recognition of the
whole or of an object’s details. The neural correlates of Associative visual agnosia are confusing. A
lateralised left hemisphere parieto-occipital lesion may be enough to lose meaning, aspects of vision,
although Associative visual agnosia may arise in the presence of a unilateral right occipital lesion.
Indeed, a number of structural areas may produce Associative visual agnosia. It is suggested that the
variety of sites that produce Associative visual agnosia may lead to heterogeneous perceptual
impairments. Because assigning meaning is such a high-level cortical process, different lesion sites
producing similar effects also speaks to the complexity of the perceptual-meaning system. It is
possible that the left hemisphere assigns meaning while the right hemisphere governs the global
aspects of perceptual integration (Zilmer & Spiers, 2001).
Many patients show both apperceptive and associative aspects to their visual agnosia. The
differentiation between the two agnosias is useful for descriptive and conceptual understanding but
does not correspond to strict anatomic correlates that can readily be differentiated or dissociated
(Zilmer & Spiers, 2001).

Prosopagnosia
The term prosopagnosia refers to the specific case of the inability to recognise people by their faces
that is not attributable to deterioration in intellect. However, the person can be recognised by other
means, such as gait or tone of voice (Zilmer & Spiers, 2001). Sometimes faces are described as
having a distorted, warped or flat appearance. Patients are able to identify a face as a face and
discriminate between faces. The problem lies at being able to identify individual faces (Martin,
2006). Some patients have difficulty recognising faces or familiar and unfamiliar people. They may
even not recognise themselves. Patients are often able to recognise common objects, read and
recognise line drawings. Thus, it is not related in a simple way to problems with recognising
nonfacial stimuli (Gazzaniga, Ivry & Mangun, 2002).
Dr P (Sacks, 1985) had severe prosopagnosia. He saw faces where there were no faces to see. He
patted the heads of water hydrants, assuming they were the heads of children.
Some patients might be sensitive to particular features of faces; some patients may be worse at
matching eyes than mouths. The accurate perception of facial perception may also be impaired but
this is complicated by the inability to identify emotional facial expression (Martin, 2006).
Some patients have difficulty recognising famous people or very familiar people like spouses. To
date, prosopagnosia has never occurred in a pure form e.g. without associated perceptual deficits
(Martin, 2006).
Prosopagnosia rarely occurs with single, well-circumscribed lesions. Prosopagnosia tends to be right
hemisphere based but it does occur with bilateral damage to white matter and cortex in the
occipito-temporal gyrus. However prosopagnosia has been found where there is no lesion in this
area. Lesions generally involve occipital and temporal cortices. Prosopagnosia is thought to be due
to right poster lesions, possibly of the parahippocampal gyrus, lingual/fusiform gyri and splenium
(Martin, 2006). Lesions along the ventral pathway often result in prosopagnosia (Gazzaniga, Ivry &
Mangun, 2002). Cells in two distinct regions of the temporal lobe are preferentially activated by
faces, one in the superior temporal sulcus and the other in the inferior temporal gyrus (Rolls, 1992)

Category-specific agnosia
Brain injured that produce agnosia do not completely destroy the connections to semantic
knowledge. Because damage is not total, it is reasonable that circumscribed lesions might destroy
tissue that is devoted to process similar types of information (Gazzaniga, Ivry & Mangun, 2002).
Some agnostic patients appear poorer at recognising some visual stimuli than others. Warrington
and Shallice’s (1984) patient JBR is unable to name living things or musical instruments but can name
non-living things and parts of the body. This dissociation was found whether the stimuli were verbal
or non-verbal. The authors argued that the distinction could be explained on the basis of the
perception of different features of these objects. Living things would be perceived in terms of their
individual attributes such as shape, colour and size; whereas the non-living things are perceived in
the function they carry out. Objects’ identity appears to be processed by different regions of the
brain depending on the meaning assigned to that object. Other theories are that inanimate objects,
such as a cup are a lot less detailed than animate objects, such as a fly (Martin, 2006). Manufactured
items are easier to identify because they activate additional forms of representation; we can activate
a sense of how it feels or the actions required to manipulate the object. Other theories are that
there are specialised systems sensitive to categorical distinctions (Gazzaniga, Ivry & Mangun, 2002).
It has been found that the entorhinal cortex responds selectively to the pictures of animals, the
anterior hippocampus to images of famous faces and the hippocampus in general to layouts, houses
and interiors (Kreiman et al., 2000).

Syndromes of the ‘Where’ system

Unilateral Spatial Neglect
Damage to the right parietal-occipital or inferior parietal area is the most common site of damage
for an odd type of inattention termed unilateral neglect. People with neglect lose conscious
awareness of an aspect of personal space despite adequately functioning sensory and motor
systems. This problem may first look like the result of a right hemisphere stroke or lesion affecting
motor and somatosensory strips on the contralateral side. The left limbs appear useless and hang
limply. However, these limbs can move and feel pain – they are simply ignored by the conscious
mind. This failure of awareness extends beyond the body to the entire left hemispace from the
patient’s perspective. People often collide with objects on their left side and leave out words on the
left side of the page when reading. Neglect can be thought of as a lack of conscious attention to the
left side or as if attention is being pulled to the right. When navigating, patients often tend to veer
right and end up travelling in circles. Many people with neglect think that the left sided part of their
body does not belong to them, failing to dress half of the body or put makeup on only the right side.
Neglect can look similar to visual problems but it is actually a problem at a much higher level of
integration (Zilmer & Spiers, 2001).
The classic picture of neglect is most likely to occur with lesions in the right inferior parietal lobe or
generally, the posterior regions of the cortex. Neglect cannot be solely attributed to sensory
processing deficits, implying that lesions in the somatosensory strip are not enough to produce
neglect. Neglect can also appear with other right hemisphere lesions and much less frequently, with
left hemisphere lesions. Other right hemisphere lesions reported to produce neglect include a
variety of subcortical structures, the majority implicating the thalamus. Left hemisphere lesions may
also include other than parietal cortical areas. It is reasonable to speculate that neglect results from
a disconnection in higher-order processing that involves the coordination of many second-order
systems, such as visual processing, attention, memory and possibly others. Mesulam’s (1985) neural
network model identifies three major functional areas that must interact for the body-space system
to work normally, each corresponding to a cortical site. The parietal lobes control perceptual
processing, the premotor and prefrontal cortices mediate exploratory – motor behaviour and the
cingulated gyrus directs motivation. In turn, subcortical structures probably coordinate the
orchestration of all 3 areas. The reticular formation directs arousal, the thalamus, especially the
pulvinar of the thalamus, is postulated to focus and guide attention between spatial locations (Zilmer
& Spiers, 2001).
Classic neglect produces abrupt alteration of consciousness most commonly occurring as a result of
right parietal stroke but can coincide with traumatic brain injury. The manifestation is typically
sudden and rarely seen in slow growing tumours or disease processes. Temporary and reversible
neglect may occur in conjunction with seizures, ECT and intracarotid sodium amytal testing (Wada
testing). Neglect may also stand out against a background of widespread right hemisphere damage.
In this instance there are accompanying motor, sensory or attentional problems. A variety of
neglect-type symptoms are associated with motor weakness. In these cases awareness of left-sided
stimuli may be less impaired but neglect type symptoms become evident with the inability to
perform or sustain motor acts on the side contralateral to the lesion (Zilmer & Spiers, 2001).

Disorders of Three Dimensional Space

Left-right disorientation
Left-right disorientation is an inability to distinguish right from left. A patient presented with
topographical disorientation following left retrosplenial haemorrhage. His directional information
about familiar places, encoded by previous navigation, was severely impaired, and he could not learn
the direction to new places in large-scale spaces beyond the range of visual surveillance. By contrast,
he had no difficulties with directional information encoded in a tabletop manner: he could locate
major cities or countries on a map, and he also could memorise the spatial relationship of objects in
a room. Six months after the ictus, when he had recovered from his directional disorientation, a
functional magnetic resonance imaging (fMRI) study of mental navigation demonstrated prominent
activation in the retrosplenial area along the right parieto-occipital sulcus and the circumference of
the injured area on the left side. The study, suggests that the ‘sense of direction’ in a large-scale
locomotor environment is subserved by the visual area along the parieto-occipital sulcus, and that
bilateral deterioration of this function causes directional disorientation (Ino, Doi, Hirose, Kimura, Ito,
& Fukuyama, 2007).

Depth Perception
Riddoch, (1917) described a patient to who the world appeared essentially flat. This depth
perception impairment existed despite the patient’s ability to perceive colour and shading
(Gazzaniga, Ivry & Mangun, 2002).
Patients who have lost visual acuity after cortical lesions generally have widespread problems in
visual perception. Thus, there are no unambiguous reports of selective deficits in form and depth
perception (Gazzaniga, Ivry & Mangun, 2002).
 Special Frames of Reference
In view-dependent theories, perception is assumed to depend on recognising an object from a
certain viewpoint. This theory posits that we have a cornucopia of specific representations in
memory; we simply match a stimulus to a stored representation. This would place heavy demands
on perceptual memory. Each object would require multiple representations in memory, each
associated with a different vantage point (Gazzaniga, Ivry & Mangun, 2002).
An alternate scheme is that recognition occurs in a view-invariant frame of reference (Marr, 1982).
Recognition does not happen by analysing stimulus information. Sensory input defines basic
properties; the objects properties are defined with respect to these properties. In this theory, a
critical property for recognition is establishing the major and minor axes inherent to the object. The
major axis of a bicycle runs along its length. The handlebars are minor axis. These properties will
hold across different vantage points.

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