Cortical visual impairment
Encyclopedia
Cortical visual impairment (CVI) is a form of visual impairment
Visual impairment
Visual impairment is vision loss to such a degree as to qualify as an additional support need through a significant limitation of visual capability resulting from either disease, trauma, or congenital or degenerative conditions that cannot be corrected by conventional means, such as refractive...

 that is caused by a brain problem rather than an eye problem. (The latter is sometimes termed "ocular visual impairment" when discussed in contrast to cortical visual impairment.) Some people have both CVI and a form of ocular visual impairment.

CVI is also sometimes known as cortical blindness, although most people with CVI are not totally blind. The term neurological visual impairment (NVI) covers both CVI and total cortical blindness. Delayed visual maturation, another form of NVI, is similar to CVI, except the child's visual difficulties resolve in a few months. Though the vision of a person with CVI may change, it rarely if ever becomes totally normal.

The major causes of CVI are as follows: asphyxia, hypoxia (a lack of sufficient oxygen in the body’s blood cells), or ischemia (not enough blood supply to the brain), all of which may occur during the birth process; developmental brain defects; head injury; hydrocephalus (when the cerebrospinal fluid does not circulate properly around the brain, and collects in the head, putting pressure on the brain); a stroke
Stroke
A stroke, previously known medically as a cerebrovascular accident , is the rapidly developing loss of brain function due to disturbance in the blood supply to the brain. This can be due to ischemia caused by blockage , or a hemorrhage...

 involving the occipital lobe
Occipital lobe
The occipital lobe is the visual processing center of the mammalian brain containing most of the anatomical region of the visual cortex. The primary visual cortex is Brodmann area 17, commonly called V1...

; and infections of the central nervous system, such as meningitis and encephalitis.

Symptoms

Symptoms of CVI usually include several (but not necessarily all) of the following:
  • The person with CVI exhibits variable vision. Visual ability can change from one day to the next but it can also fluctuate from minute to minute, especially when the person is tired. When undertaking critical activities, people with CVI should be prepared for their vision to fluctuate, by taking precautions such as always carrying a white cane
    White cane
    A white cane is used by many people who are blind or visually impaired, both as a mobility tool and as a courtesy to others. Not all modern white canes are designed to fulfill the same primary function, however: There are at least five varieties of this tool, each serving a slightly different...

     even if they don't always use it to the full, or always having very large print available, just in case it's needed. (For example, consider the consequences of losing vision while giving a public speech). Managing fatigue
    Fatigue (physical)
    Fatigue is a state of awareness describing a range of afflictions, usually associated with physical and/or mental weakness, though varying from a general state of lethargy to a specific work-induced burning sensation within one's muscles...

     can reduce fluctuations but does not eliminate them.

  • One eye may perform significantly worse than the other, and depth perception
    Depth perception
    Depth perception is the visual ability to perceive the world in three dimensions and the distance of an object. Depth sensation is the ability to move accurately, or to respond consistently, based on the distances of objects in an environment....

     can be very limited (although not necessarily zero).

  • The field of view
    Field of view
    The field of view is the extent of the observable world that is seen at any given moment....

     may be severely limited. The best vision might be in the centre (like tunnel vision
    Tunnel vision
    Tunnel vision is the loss of peripheral vision with retention of central vision, resulting in a constricted circular tunnel-like field of vision.- Medical / biological causes :Tunnel vision can be caused by:...

    ) but more often it is at some other point, and it is difficult to tell what the person is really looking at. Note that if the person also has a common ocular visual impairment such as nystagmus then this can also affect which part(s) of the visual field are best. (Sometimes there exists a certain gaze direction which minimises the nystagmus, called a "null point.")

  • Even though the field of view may be very narrow indeed, it is often possible for the person to detect and track movement. Movement is handled by the 'V5' part of the visual cortex
    Visual cortex
    The visual cortex of the brain is the part of the cerebral cortex responsible for processing visual information. It is located in the occipital lobe, in the back of the brain....

    , which may have escaped the damage. Sometimes a moving object can be seen better than a stationary one; at other times the person can sense movement but cannot identify what is moving. (This can be annoying if the movement is prolonged, and to escape the annoyance the person may have to either gaze right at the movement or else obscure it.) Sometimes it is possible for a person with CVI to see things while moving their gaze around that they didn't detect when stationary. However, movement that is too fast can be hard to track; some people find that fast-moving objects "disappear." Materials with reflective properties, which can simulate movement, may be easier for a person with CVI to see. However, too many reflections can be confusing (see cognitive overload
    Mental space
    The Mental space is a theoretic construct proposed by Gilles Fauconnier and Armen Khederlarian corresponding to possible worlds in Philosophy. The main difference between a mental space and a possible world is that a mental space does not contain a faithful representation of reality, but an...

    ).

  • Some objects may be easier to see than others. For example, the person may have difficulty recognising faces or facial expressions but have fewer problems with written materials. This is presumably due to the different way that the brain processes different things.

  • Colour and contrast are important. The brain's colour processing is distributed in such a way that it is more difficult to damage, so people with CVI usually retain full perception of colour. This can be used to advantage by colour-coding objects that might be hard to identify otherwise. Sometimes yellow and red objects are easier to see, as long as this does not result in poor contrast between the object and the background.

  • People with CVI strongly prefer a simplified view. When dealing with text, for example, the person might prefer to see only a small amount of it at once. People with CVI frequently hold text close to their eyes, both to make the text appear larger and to minimise the amount they must look at. This also ensures that important things such as letters are not completely hidden behind any scotoma
    Scotoma
    A scotoma is an area of partial alteration in one's field of vision consisting of a partially diminished or entirely degenerated visual acuity which is surrounded by a field of normal - or relatively well-preserved - vision.Every normal mammalian eye has a scotoma in its field of vision, usually...

    s (small defects in parts of the functioning visual field), and reduces the chances of getting lost in the text. However, the simplification of the view should not be done in such a way that it requires too rapid a movement to navigate around a large document, since too much motion can cause other problems (see above).

  • In viewing an array of objects, people with CVI can more easily see them if they only have to look at one or two at a time. People with CVI also see familiar objects more easily than new ones. Placing objects against a plain background also makes them easier for the person with CVI to see.

  • For the same reason (simplified view), the person may also dislike crowded rooms and other situations where their functioning is dependent on making sense of a lot of visual 'clutter'.

  • Visual processing can take a lot of effort. Often the person has to make a conscious choice about how to divide mental effort between making sense of visual data and performing other tasks. For some people, maintaining eye contact
    Eye contact
    Eye contact is a meeting of the eyes between two individuals.In human beings, eye contact is a form of nonverbal communication and is thought to have a large influence on social behavior. Coined in the early to mid-1960s, the term has come in the West to often define the act as a meaningful and...

     is difficult, which can create problems in Western culture (for example, bonding can be difficult for some parents who have an infant with CVI, and lack of contact in an older child can cause others to regard him or her with suspicion).

  • It can also be difficult for some people with CVI to look at an object and reach for it at the same time. Looking and reaching are sometimes accomplished as two separate acts: look, then look away and reach.

  • People with CVI can sometimes benefit from a form of blindsight
    Blindsight
    Blindsight is a phenomenon in which people who are perceptually blind in a certain area of their visual field demonstrate some response to visual stimuli...

    , which manifests itself as a kind of awareness of one's surroundings that cannot consciously be explained (for example, the person correctly guesses what they should do in order to avoid an obstacle but does not actually see that obstacle). However, this cannot be relied on to work all the time. In contrast, some people with CVI exhibit spatial difficulties and may have trouble moving about in their environment.

  • Approximately one third of people with CVI have some photophobia
    Photophobia
    Photophobia is a symptom of abnormal intolerance to visual perception of light. As a medical symptom photophobia is not a morbid fear or phobia, but an experience of discomfort or pain to the eyes due to light exposure or by presence of actual physical photosensitivity of the eyes, though the term...

    . It can take longer than usual to adjust to large changes in light level, and flash photography can be painful. On the other hand, CVI can also in some cases cause a desire to gaze compulsively at light sources, including such things as candle
    Candle
    A candle is a solid block or cylinder of wax with an embedded wick, which is lit to provide light, and sometimes heat.Today, most candles are made from paraffin. Candles can also be made from beeswax, soy, other plant waxes, and tallow...

     flames and fluorescent overhead lights. The use of good task lighting
    Task lighting
    Often task lighting refers to office lighting, where the task light is used to increase illuminance on the reading area. However, the illuminance level is not the only factor governing visibility. Contrast is also important, and a poorly positioned light source may cause contrast reduction,...

     (especially low-temperature lamps which can be placed at very close range) is often beneficial.

  • Although people (with or without CVI) generally assume that they see things as they really are, in reality the brain may be doing a certain amount of guessing and "filling in", which is why people sometimes think they see things that turn out on closer inspection not to be what they seemed. This can occur more frequently when a person has CVI. Hence, a person with CVI can look at an optical illusion or abstract picture and perceive something that is significantly different from what a person without CVI will perceive.


The presence of CVI does not necessarily mean that the person's brain is damaged in any other way, but it can often be accompanied by other neurological problems, the most common being epilepsy
Epilepsy
Epilepsy is a common chronic neurological disorder characterized by seizures. These seizures are transient signs and/or symptoms of abnormal, excessive or hypersynchronous neuronal activity in the brain.About 50 million people worldwide have epilepsy, and nearly two out of every three new cases...

.

Diagnosis

Diagnosing CVI is difficult. A diagnosis is usually made when visual performance is poor but it is not possible to explain this from an eye examination. Before CVI was widely known among professionals, some would conclude that the patient was faking their problems or had for some reason engaged in self-deception
Self-deception
Self-deception is a process of denying or rationalizing away the relevance, significance, or importance of opposing evidence and logical argument...

. However, there are now testing techniques that do not depend on the patient's words and actions, such as fMRI scanning, or the use of electrodes to detect responses to stimuli in both the retina and the brain. These can be used to verify that the problem is indeed due to a malfunction of the visual cortex and/or the posterior visual pathway.
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