Title: Visual, Visuospatial, and RightBrain Disorders
1Visual, Visuospatial, and Right-Brain Disorders
2- Perception refers to the ability to sense a
stimulus regardless of whether the stimulus is
recognized. Lesions of the eye or the optic
nerve produce ipsilateral blindness (partial or
total) lesions behind the optic chiasm result in
homonymous visual field defects pathological
changes in the inferior calcarine cortex produce
achromatopsia (central color blindness) medial
occipitotemporal lesions of the right hemisphere
are associated with prosopagnosia (defective
recognition of familiar faces) and environmental
agnosia (impaired recognition of familiar
places) and bilateral lesions of the medial
occipitotemporal cortex or inferior longitudinal
fasciculi cause visual object agnosia.
3Monocular Blindness
4- Ocular disorders including diseases of the lens
(cataracts), retina, or macula produce total or
partial blindness of the involved eye. Likewise,
diseases of the optic nerve (such as glaucoma
affecting the nerve head, ischemic optic
neuritis, and optic neuritis) produce unilateral
blindness ipsilateral to the lesion. Pupillary
responses are usually compromised in diseases of
the eye and optic nerve because there is
diminished light conduction, and this sign may
help distinguish blindness as a manifestation of
ocular or neurological disease from feigned
blindness or blindness as a manifestation of a
conversion reaction.
5- In addition, patients with conversion disorders
or who are malingering have intact optokinetic
nystagmus (nystagmus that is normally induced by
passing a striped cloth in front of the eyes of a
sighted person).
6- Long-standing bilateral blindness may result in
pendular nystagmus with spontaneous to-and-fro
movements of the eyes. Visual hallucinations
(release hallucinations) are not uncommon in
patients with ocular disorders and may be either
formed or unformed. Phosphenes (sudden flashes
of light) often accompany retinal or optic nerve
disease, and some patients may experience
synesthesia characterized by phosphenes occurring
in response to loud sounds or noises.
7Homonymous Hemianopsia
- Lesions of the optic tract, optic chiasm,
geniculate nucleus of the thalamus, or the
geniculocalcarine radiations produce homonymous
visual-field defects, defective vision or
blindess in the right or left halves of the
visual fields of both eyes. Temporal lobe
lesions are associated with asymmetric
(incongruent) field defects occipital lesions
cause symmetric (congruent) defects.
8- Lesions involving the entire radiation produce
homonymous hemianopsias (usually with sparing of
the central few degrees of vision), whereas
lesions affecting only some of the fiber tracts
produce quadrantanopsias or other incomplete
field defects. Pupillary responses are intact in
patients with homonymous visual-field defects,
and visual acuity is normal.
9- Hallucinations are common in the period
immediately after an injury to the
geniculocalcarine radiations (e.g. in the first
few days after a stroke) and may be unformed
(lights), semiformed (tire track or herringbone
patterns), or formed (complex scenes, animals, or
people). The hallucinations are commonly
confined to the area of the visual-field defect.
10Achromatopsia
- Achromatopsia refers to central color blindness.
The patient loses the ability to distinguish
color in the contralateral visual field. The
associated lesions involve the cortex inferior to
the calcarine fissure of the medial occipital
lobe. There is often an associated partial
hemianopsia. The color blindness is difficult to
demonstrate except when it is bilateral. Color
blindness is assessed with color-naming tests,
tests requiring sorting of colors by shades, and
Ishihara pseudoisochromatic plates.
11Simultanagnosia
- Agnosias are recognition deficits rather than
perceptual disturbances. Simultanagnosia is a
misnamed exception. In this disorder, when the
patients are shown two objects simultaneously,
they fail to perceive one of them. For example,
when shown a figure consisting of a circle and a
cross, they see the circle or the cross but not
both when shown a number drawn by juxtaposing
many small numbers (for example the number 2
drawn by using many little number 3s), they see
the number 2 or the number 3s but not both.
12- Simultanagnosia is often observed as part of
Balints syndrome. The latter consists of optic
ataxia (the inability to touch objects accurately
using visual guidance), sticky fixation or
occular apraxia (difficulty volitionally
redirecting gaze), and simultanagnosia.
13Prosopagnosia
- Prosopagnosia refers to the inability to
recognize familiar faces. Visual perception is
intact the patient sees normally and can
describe the unrecognized face in detail.
However, familiar faces, such as the faces of
family members and famous people, cannot be
recognized, and the patient cannot deduce the
individuals identity without the aid of
nonvisual clues. In some cases, specific facial
features (moustache, glasses of a specific type)
are used to facilitate recognition, but the
patient is easily fooled if someone with similar
characteristics appears.
14Environmental Agnosia
- Environmental agnosia is a clinical syndrome
characterized by the inability to recognize
familiar places. The patient sees normally and
is able to describe the surroundings but has no
sense of familiarity. Patients commonly adopt
verbal strategies to compensate for the deficit
for example, they may find their homes by using
the street signs and house numbers.
Environmental agnosia and prosopagnosia
frequently coexist.
15Visual Object Agnosia
- Two principal types of visual agnosia have been
identified apperceptive agnosia and associative
agnosia. The two identified syndromes reflect
disruption of different stages of processing of
complex visual information, and variations of the
syndromes are common. Apperceptive visual
agnosia is characterized by intact visual acuity
but an inability to recognize objects.
16- Patients can distinguish shades of light
intensity, identify colors, determine line
orientation, exhibit depth perception, see
movement, and distinguish thin from thick lines.
They can negotiate their surroundings but cannot
recognize, describe, or match perceived objects.
They cannot draw objects they see and cannot
point to objects named by the examiner. The
agnosia involves all visual stimuli including
faces, the environment, and objects.
Apperceptive agnosia may occur as a phase of
recovery from cortical blindness after anoxia,
carbon monoxide poisoning, or bilateral posterior
hemispheric strokes.
17Apperceptive visual agnosia is a failure in
higher level perception, also known as
visual-form agnosia. ?The person has normal
visual acuity, but cannot recognize objects based
on their shape. The person cannot recognize
objects, line drawings of objects, nor copy
drawings of objects. They do not know what they
are seeing. A person here could describe a rose
in intimate detail but not know it was a rose
until smelling it. ?Prosopagnosia is a form of
apperceptive visual agnosia in which the person
cannot recognize a face visually, but can do when
hearing their voice. Here, some individuals do
not recognize their own face.
18- Associative visual agnosia features intact
elementary perception and preserved ability to
describe, draw, and match visual stimuli. For
example, when shown an object (shoe, toothbrush,
etc.), patients can draw the object and match the
object with an identical one from a group of
objects, but they cannot recognize the object or
describe its use.
19?Associative visual agnosia refers to a
disconnection between perceptions and verbal
systems. The person cannot verbally label or name
what they see but they can label or identify it
nonverbally by gesture or behavior which
correctly discriminates the object. A person
here could not say an object was a car or not but
they could make gestures of using a steering
wheel to indicate they can correctly discriminate
the object but it cannot be verbally labeled. A
person here could copy a drawing of an object but
not name it but they could not produce a drawing
of an object if merely asked. A drawing of a car
could be reproduced but the person could not do
so in response to Draw a car.
20Color Agnosia
- Color agnosia (also called color anomia) is
present in some patients who have alexia
(inability to read) without agraphia (inability
to write). The patients do not have
abnormalities of color naming and can name the
colors appropriate to specific settings (a banana
is yellow, a fire engine is red) they also can
sort and match colors. They do not have color
blindness. They cannot point to named colors or
name colors pointed to by the examiner.
21Disturbances of Visuospatial Attention
22Unilateral Neglect
- Unilateral (or hemispatial) neglect refers to a
lack of attention to events and actions in
one-half of space. Neglect may involve all
sensory modalities visual, auditory, and
somatosensory as well as motor acts (motor
neglect in-intention) and motivation.
Unilateral visual neglect is the most commonly
observed form of hemispatial neglect. In its
most subtle form, the patient ignores the
stimulus presented in the neglected hemifield.
23- This type of neglect can be demonstrated by
holding one hand in equivalent portions of each
visual field while facing the patient, quickly
moving the fingers of each hand, and asking the
patient to point to the hand whose fingers moved
(the technique of double simultaneous
stimulation). The patient will perceive movement
only in the non-neglected visual field (this test
requires that the patient not have a field
defect).
24- In its more obvious form, unilateral visual
neglect may be evidenced by failure to copy
one-half of model objects, read one-half of
words, dress one-half of the body, or shave
one-half of the face. Tests for visual neglect
include line bisection (the patient crosses the
line in the middle of the nonneglected portion)
and the line-crossing test, in which the patient
is given a sheet of paper with randomly
distributed lines and is asked to cross each line
in the middle only those lines in the
nonneglected field will be crossed.
25- In severe forms of neglect, all types of stimuli
in the affected hemiuniverse are neglected.
Somesthetic neglect is demonstrated by touching
the patient simultaneously on both sides of the
body and asking the patient where he or she was
touched the patient perceives only the touch on
the nonneglected side of the body.
26- Auditory neglect is assessed by standing behind
the patient, snapping the fingers on either or
both sides of the head, and asking the patient to
point toward the side where a stimulus was heard.
The patient points only toward the nonneglected
side during double simultaneous auditory
stimulation.
27- Motor neglect syndromes involve action-intention
disorders affecting the half of space
contralateral to frontal lobe or basal ganglia
lesions. The patient may appear to have a
hemiparesis because the limb with
action-intention neglect is unused, but when
strength and coordination are tested, they are
found to be normal.
28Anosognosia and Anosognosic Syndromes
- Anosognosia is the clinical syndrome
characterized by denial of hemiparesis. It
classically occurs in patients with right
parietal lesions and left unilateral neglect who
deny their left hemiparesis. Variants of
anosognosia include somatoparaphrenia (denial of
ownership of the paralyzed limbs), anosodiaphoria
(minimization of and unconcern about the weakness
without complete denial), misoplegia (hatred of
the paralyzed or weak limb), personification
(naming the limb and giving it an identity), and
supernumerary limbs (reduplication of limbs on
the neglected side).
29Visual Anosognosia
30- Also called Antons syndrome, visual agnosia
(from the Greek, a negation nosos
disease gnosis knowledge) is the denial
of blindness in patients who have irrefutably
lost their vision. Whether they walk into the
walls under their own stream or are bluntly
confronted with their disability by you or
others, their response is indifference, am
implausible explanation (e.g., I lost my
glasses), or both.
31- Antons syndrome is distinct from phantom vision,
in which enucleated patients or those with
severed optic nerves claim to perceive light and
shape. The existence of visual subception
suggests two dissociable levels of seeing,
instrumental behavioral responses to optical
stimuli on the one hand, and subjective visual
awareness on the other. These patients do not
readily volunteer their experiences of seeing
things. In fact, they feel ashamed at
experiencing something that intellectually they
know to be not real.
32- For example, after fifty-year-old patient ER had
her left eye enucleated because of a melanoma,
she began to see geometric figures in that eye.
The shapes were external, close to the face, and
consisted of metabolic blobs, spirals, and
parallel lines. Her explanation was, This is
involuntary. My brain is doing it. (This claim
is most plausible. We have long known that
migraineurs who have no eyes nonetheless have
visual symptoms during their aura.
33- The inverse of Antons syndrome has also been
reported namely, the denial of visual
perception in a man with remarkably preserved
visual skills in his upper right quadrant. When
confronted with his success in correctly
identifying colors, objects, faces, facial
emotions, and words, he denied any awareness of
visual perception. His explanation for his
accurate performance was, I feel it.
34Visual Illusions and Hallucinations
35- Visual illusions and hallucinations may be either
positive or negative symptoms and can be seen in
drug reactions or withdrawal, delirium, epilepsy,
and mass lesions. They may be elementary or
formed. Epileptic discharges in Brodmann areas
18 and 19 (visual association cortex) (lateral
occipital) are said to cause twinkling or
pulsating lights. Striate lesions produce
elementary visual sensations of dark shapes,
phosphenes, and flashes that may be stationary or
moving, colored or achromatic. Red is perceived
most often, followed by blue, green, and yellow.
These visions may appear straight ahead or in the
visual field opposite the legion.
36- Elementary flashes of light, zigzags, or other
geometric shapes are called photisms, and are
distinct from phosphenes, the flashing lights one
sees on firmly rubbing the eyes. Phosphenes are
caused by mechanical deformation of the retina
and are an example of an entopic perception
(literally within the eye). These latter need
to be distinguished from perceptions of cerebral
origin as the eye and the occiput are poles apart.
37- Other entopic phenomena are the seeing of ones
own retinal blood vessels, vitreous floaters,
afterimages, or the muscae volitantes. Entopic
perceptions appear to move with the eyes, whereas
perceptions of cerebral origin are independent of
eye movement. Afterimages are produced by
fatigued retinal photoreceptors and are
complimentarily colored images of cerebral
origin are often chromatic. Retinal vessels and
floaters look like cobwebs or blobs, are fixed in
appearance, and can be viewed or ignored at will.
The muscae volitantes are the actual corpuscles
coursing through vessels near the macula. You
should normally be able to see your own,
particularly against a bright sky or a field of
snow.
38- They travel in lines or arcs, then disappear.
Awareness of your own normal retinal circulation
is called Scheerers phenomenon. Traction of the
vitreous or retina causes arch-shaped, achromatic
phosphenes called Moores lightning streaks.
Macular edema or hemorrhage will produce
distortions such as heat waves, and glaucoma
causes halos and rainbows around objects.
Certain maculopathies can also cause
metamorphopsia.
39- Release hallucinations are perceptions, in any
modality, that occur in a deafferented field.
Visual and auditory ones are most common. - Visual release hallucinations can wander out into
the normal field. They are experienced in
extrapersonal space, and patients almost always
appreciate their unreal nature. Elementary
hallucinations tend to occur with occipital
lesions, whereas formed and more complex ones
emanate from the temporal lobe. Hallucinations
due to temporal lobe lesions tend to fill both
visual fields, in contrast to those engendered by
occipital lesions, which usually inhabit only the
contralateral field.
40- Elementary visual hallucinations have many
causes, and the ground is strewn with confounds.
In addition to entopic and occipital lobe causes,
hallucinations can also result from lesions of
the anterior optic tract. These are special,
however, in that they are not spontaneous but
rather are induced by another sense, most often
sound. This is an example of an acquired
synesthesia. Sounds that induced photisms in
these patients included clanking of the radiator,
crackling of the walls as they cooled at night,
the whoosh of a furnace ignition, a dogs bark,
and slamming doors.
41- The photisms ranged from simple flashes of white
light to colored forms that looked like a flame,
amoebas, oscillating flower petals, a spray of
bright dots, or kaleidoscopic effects. All
lasted for just an instant. The monocular
visual-evoked response from the scotomatous eyes
showed conduction delays and reduced amplitudes.
It is curious that photisms arising in one eye
are perceived to be caused by sounds reaching
only the ipsilateral ear. This is, of course,
contrary to our conventional understanding as
acoustic localization depends on differences in
the sound reaching both ears.
42Metamorphopsia and Allied Experiences
43- Many bizarre visual experiences are subsumed
under the term metamorphosia, the essential
features of which are (1) deformation of shape,
(2) change in size, (3) the illusion of movement,
or (4) all three. Its locus is imprecise, being
vaguely in the occipitotemporo-parietal territory.
44- In metamorphopsia, objects may appear to advance
or recede relative to the viewer, their vertical
or horizontal orientation may suddenly skew, or
objects may break apart as if they were painted
on glass, the parts sliding over one another as
in Cubism. One object may transform into
another, sometimes with the two shapes
alternating rhythmically back and forth. Visual
field defects are frequently found in all these
subjective visual experiences. Specific types of
metamorphopsiae have earned their individual
terminology.
45- In micropsia and macropsia, objects seem too
small or too big, respectively. - Umkehrtsehen is German for inverted vision,
meaning that things look as if you are standing
on your head. Verkehrtsehen denotes the reversal
of right and left. These aberrations usually
appear and resolve suddenly, and the experience
is transitory in all recorded cases.
46- Palinopia (also known as paliopsia, paliopia, and
palinopsia) is visual preservation. For example,
a patient saw his wife leave the hospital room,
and then he saw her leave again a few moments
later. Aside from static cerebral lesions,
antiserotonergics such as LSD and mescaline can
produce it. LSD users speak of trails,
positive images that remain immediately behind an
object as it moves across their visual field.
47- Polyopia signifies multiple images of the same
object. For example, a patient looked at a
single rose and, on turning away to look at the
blank wall, saw multiple roses. Insect vision,
or entomopia (from the Greek entomon meaning
insect), connotes rows and columns of multiple
images numbering in the hundreds, as might be
experienced by looking through compound eyes.
Polyopia is a form of cerebral diplopia.
48- Cerebral diplopia (double vision) may be vertical
or concentric. Strict terminology would limit it
to only two images (the above terms of polyopia
and entomopia taking care of larger iterations),
yet triple impressions are especially common.
Migraine is probably the most common cause of
cerebral diplopia.
49- Monochromatopsia refers to illusory coloration,
such as erythropsia (red) or xanthopsia (yellow).
Vitreous hemorrhage causes the former and
digitalis intoxication the latter more often than
do cerebral lesions.
50- The term achromatopsia, indicating no color
seeing, is somewhat misleading because patients
do perceive some color. The chroma that they see
is pale and desaturated, however, as in a
television whose color is turned down. Patients
also describe a spatial gap between the
normally saturated field and the achromatic one.
The responsible lesion is in unimodal visual
association cortex.
51Auditory Illusions and Hallucinations
52- The term paracusia refers to an alteration of
volume, timbre, or some other distortion of
sound. This may be both unpleasant and
persistent. Unlike tinnitus and other auditory
perceptions caused by end-organ disease,
paracusiae are cerebral in origin. This is
analogous to the distinction between entopic and
cerebrally based visual hallucinations. Auditory
hallucinations may be elementary or complex,
ranging from humming and buzzing to music,
voices, and radio programs.
53- Their cause is not limited to the temporal lobe,
however. Ipsilateral musical hallucinations, for
example, also result from lesions in the pontine
tegmentum. That such brainstem hallucinations
usually occur in the context of hearing loss
suggests that they may be an instance of release
hallucinations.
54Olfactory and Gustatory Hallucinations
55- Olfactory hallucinations are customarily
associated with mass lesions or epileptic
discharges in the inferior and medial segments of
the temporal lobe, especially the hippocampal
convolution or uncus hence the name uncinate
fits when referring to the disagreeable smell
that sometimes constitutes the aura of partial
seizures. It is externally projected and
experienced as coming from some nearby but
unknown source. The smell is impossible to
identify other than being described as foul,
rancid, or vile. (This quality of
indescribableness is characteristic of experience
associated with temporal structures.
56- Gustatory hallucinations also arise from the
temporal lobe. Intense and sudden hunger can be
a symptom of temporal lobe epilepsy (TLE), and,
paradoxically, also of lobectomy. A boy was
reported to have experienced two episodes of
right anterior temporal intracerebral hemorrhage,
each preceded by exclamations of intense hunger.
57Synesthesia
58- The term synesthesia derives from the Greek syn
(meaning union) and aisthesis (sensation),
and refers to an involuntary joining of one or
more senses. That is, perception in one sense is
accompanied by a parallel perception in another
sense. Synesthesias medical and psychological
history reach back 300 years. - Idiopathic synesthesia is not a disorder per se,
but a perceptual curiosity found in roughly 1 in
25,000 individuals. Women outnumber men by at
least two to one, and left-handedness or mixed
dominance is more common than expected.
59- Through permutations of the five senses yield
twenty possible pair-wise combinations, some
synesthetic combinations are much more common
than others. The yoking of sight with sound is
by far most frequent, touch and taste less so,
and smell is least often involved. In colored
hearing synesthesia (chromesthesia), words,
voices, environmental sounds, or music will
trigger the perception of an involuntary photism
that is perceived in extrapersonal space. In a
case that I called geometric taste (i.e.,
taste-touch synesthesia) the taste of mint caused
subject MW to palpate a cold, smooth, curved
shaped in front of him.
60- We often emphasize the yoking of just two senses
when speaking of idiopathic synesthesia, though
polymodal synesthesia occurs as well.
Individuals mention that a third or fourth sense
sometimes participates, but not as often as the
main two that are joined. It is important to
distinguish that synesthetic percepts are neither
metaphoric nor pictorial. They are concrete,
generic, and unelaborated. By generic, it is
meant that synesthetes see blobs, lines, spirals,
and lattice shapes palpate smooth or rough
textures agreeable or disagreeable taste
flavors, such as salty, sweet, or metallic.
61- They do not see pastures and temples, taste
chicken soup, or feel a sponge or some other
specified object. Sensations never go beyond
this elementary, unembroidered level to become
specific exemplars. - Acquired synesthesia is classically seen in TLE,
head trauma, and mass lesions involving the
medial temporal lobe. Synesthesia may also be
induced by sensory deprivation, antiserotonergic
hallucinogens such as LSD and peyote, or direct
electrical stimulation of subcortical limbic
structures.
62Other Subjective Experiences Dependent on the
Temporal Lobe
63- Extremely unusual subjective perceptions have
been recorded in cases of TLE and in patients
with other known temporal lobe pathology. - Time dilation or time contraction occurs with
lesions of either temporal lobe. Changes in the
perception of time are certainly cognitive and,
because they emerge following temporal-lesions
one would expect to find them associated with
other interesting neurological conditions.
64- Déjà vu and deja vecu mean already seen and
already experienced (the latter literally means
already lived), and refer to ones sense that
something novel is extremely familiar or has been
experienced exactly so at some past time. Jamais
vu and jamais vecu refer to the opposite sense
that a familiar setting feels nonetheless alien
and strange.
65- The feeling of presence is not an uncommon
experience, yet its occurrence rarely is
volunteered for fear of negative judgment.
Sensing a presence can occur in pathological
states as well as be part of normal experience
in the latter case, it is most often experienced
late at night or at times of great creativity.
66- Other odd personality characteristics that are
said to be typical of those who suffer from
temporal lobe seizures are a heightened interest
in religion and cosmic matters, the keeping of
diaries or voluminous writings, a sense of
portentousness or the attribution of heightened
significance to otherwise mundane events, and a
viscous or sticky personality.
67Cortical Sensory Disturbances
68- Where we once were content to call the single
post-central gyrus the somatosensory cortex, we
now know that five somatosensory cortical areas
exist in humans, each with its distinct
somatotopic representation, cytoarchitecture, and
projections.
69- Knowing what relative roles the various
somesthetic cortices play in human sensation is
another matter. At present, we can say that
dorsomedial lesions produce severe anesthesia and
apraxia that eventually improve considerably.
Ventrolateral lesions, in contrast, produce
tactile agnosia that remains detectable years
after brain injury.
70- Pain, though less well understood than other
sensory systems, also has multiple cortical
representations. Painful heat, for example,
activates contralateral anterior cingulum, S1,
and S2, a pattern distinct from the predominant
S1 activation caused by vibrotactile stimulation.
Because the forebrain usually is thought to
regulate emotion, the specific cingulate
representation of pain is unexpected.
71- Categorization of sensory loss on clinical
grounds is far less confusing. Patients with
loss of one or more elementary sensations (touch,
pain, temperature, vibration) in a
face-arm-leg-trunk pattern have inferior-anterior
parietal lesions involving the parietal operculum
and insula. Theirs is a pseudothalamic sensory
syndrome in that it mimics a lesion of lemniscal
or spinothalamic projections. Other individuals
who lose the ability to discriminate sensation
(stereognosis, graphesthesia, proprioception)
have superior-posterior parietal lesions. Theirs
is called a cortical sensory syndrome.
72- A somatic hallucination that bears a superficial
resemblance to release hallucinations is
alloesthesia, a condition in which a noxious
sensory stimulus given on one side of the body
(where a sensory deficit exists) is perceived at
the corresponding locus on the opposite side.
This aberration occurs with lesions in the
putamen and spinal cord, represents an elementary
disturbance of sensory pathways, and is not to be
mistaken for a higher cortical dysfunction.
Segmental sensation or pain can also be referred
to a different and quite distant dermatome via
spinal mechanisms.
73Asomatognosia
74- Asomatognosia is a disturbance of
awareness/knowledge about ones own body and
bodily conditions, the perception of ones own
physical self, the relations of its parts to the
whole, and its orientation and extension into
extrapersonal space. One form would be asymbolia
for pain in which normal reactions to painful
stimuli are not evidenced or autotopagnosia, the
inability to localize, name, or orient correctly
different body parts
75Asomatognosia/Autotopagnosia
- Fails to point to correct body part upon command
- Fails to correctly imitate body positions,
postures or movements - Fails to correctly name body parts