International Congress Series 1282 (2005) 578 584

www.ics-elsevier.com

Brain damage related vision loss

Lea Hyvarinen*
Developmental Neuropsychology, University of Helsinki, Helsinki, Finland

Abstract. Twenty percent of visual impairment in children is caused by brain damage. Children
with brain damage related visual impairment (CVI) should be assessed in infancy and during
their preschool and school age for detection of deviations from normal development of cognitive
visual functions at the times these functions should normally emerge. During the last 20 years,
treatment of ophthalmologic diseases has advanced greatly. A similar development has occurred
in the knowledge of functioning of visually impaired children and assessment of impaired vision.
However, early intervention and assessment for special education for children with CVI have not
been taught by the leading teaching centres and thus paediatric vision (re)habilitation is lacking
behind other areas of ophthalmology and optometry. The groups of children with motor
problems, auditory processing problems, intellectual deficiencies of varying severity, who are at
high risk of having CVI, are well-known but routine assessment of vision of these children is not
a part of vision screening. With some further education of all paediatric services, a change in
attitudes toward CVI and minor changes in routine vision services this poorly served fifth of
visually impaired children could get adequate treatment, early intervention and rehabilitation.
D 2005 Published by Elsevier B.V.
Keywords: Paediatric visual impairment; CVI; Cerebral visual impairment; Brain damage related visual
impairment

1. Introduction
Brain damage related vision loss causes problematic situations in day care and schools
and is often a confusing burden in the life of the families because of poor assessment of
these childrens very individual conditions. In some countries brain damage related vision
loss, cerebral visual impairment (CVI), is not accepted as a drealT visual impairment;

* Current address: Apollonkatu 6 A 4, FI-00100 Helsinki, Finland.

E-mail address: lea.hyvarinen@lea-test.fi.
0531-5131/ D 2005 Published by Elsevier B.V.
doi:10.1016/j.ics.2005.05.065

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Fig. 1. The visual pathways from the eyes to the brain cortex. The retinocalcarine pathway from the eyes via the
lateral geniculate nucleus (LGN) to the primary visual cortex (V1) contains several types of nerve fibres, of which
the parvocellular (P) and the magnocellular (M) pathways are best known. The tectal pathway transfers visual
information via superior colliculus and pulvinar to the cortex bypassing form analysis in the occipital lobe. From
the primary visual cortex visual information flows upward as the dorsal stream (DS) and toward the temporal lobe
as the ventral stream (VS).

services and special education for the visually impaired children are denied and the
children with CVI are placed in groups of children with intellectual disabilities or
behavioural problems.
In this invited speech I will discuss the nature and the assessment of CVI and our
possibilities to understand children with CVI in their different world.

Table 1
List of clinical measurements on image quality
Image quality:
Fixation:
Following movements (smooth pursuit):
Saccades:
Strabismus:
Nystagmus:
Head position:
Refraction:
Accommodation:
Visual acuity at near:
Single symbols: binok ___ m/___M=____ RE _____________ LE _____________
Screening near test binok ____________ RE _____________ LE _______________
Standard near test binok ____________ RE _____________ LE _______________
50% crowding test binok ____________ RE _____________ LE _______________
25% crowding test binok ____________ RE _____________ LE _______________
Visual acuity at distance:
Single symbols at __ meter binok _________ RE __________ LE _______________
Line test at __meter binok ____________ RE _____________ LE _______________
Contrast sensitivity:
Colour vision:
Motion perception:
Visual field:
Visual adaptation:
Summary on image quality:

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2. Anterior and posterior visual impairment

Some concepts are central for understanding functions in the visual system. The
anterior part of the visual system, the eyes and the optic pathway anterior to the lateral
geniculate nucleus (LGN) (Fig. 1), conveys the image of the surrounding world to the
brain. The image can have normal structure if the eyes and the optic pathways are normal.
However, quite often there are changes in the eyes and/or the pathways that blur and
distort the image. Therefore we assess the quality of image (Table 1) before we assess the
cognitive visual functions (Table 2) that are the result of processing of the information in
the posterior part of the visual system, the optic radiation, the primary visual cortex and
the associative visual cortices.
Visual pathways from the eyes to the primary visual cortex are usually described as an
avenue for visual information to enter the brain. It is rarely stated that in the posterior part
of the pathways there are 10 times more nerve fibres transferring information from the
visual cortices toward the LGN than there are fibres passing information to the primary
visual cortex. This backwards flow of visual information is an effective filter of the
incoming visual information. Much if not most of the incoming information is inhibited
from moving further and only information that is necessary for the ongoing processes is

Table 2
List of cognitive visual functions assessed in children with CVI
Cognitive visual functions:
Recognition and reading
Concrete objects:
Landmarks:
Faces:
Facial expressions, body language:
Pictures of concrete objects:
Geometric forms:
Letters:
Numbers:
Words:
Crowding effect:
Reading speed:
Scanning lines of text:
Efficiency of reading:
Copying, motor planning and execution:
Perception of pictures
Length of lines:
Orientation of lines:
Details of pictures:
Figure-ground:
Visual closure:
Noticing errors:
Noticing missing details:
Comparison with pictures in memory:
dReadingT series of pictures:
Visual problems in copying pictures:

Perception of space
Depth perception:
Perception of near space:
Perception of far space:
Simultanagnosia:
Perception of textures and surface qualities:
Orientation in space:
Memorising routes:
Vision in traffic situations and in playgrounds:
Eyehand coordination
Grasping and throwing objects:
Drawing, free hand:
Copying, from near/from blackboard:

Integration problems
Vision not used when listening or exploring:
Vision not used when moving:
Balance:
Compensatory strategies
Auditory information:
Tactile, kinaesthetic and haptic information:
Memory:

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allowed entrance. Effective inhibitory function is one of the characteristic features of the
visual system and the most difficult function to assess.
We do not know whether there is lack of inhibition in some profoundly low functioning
children who do not open their eyes except when the luminance level is low and there are
only a few objects or light sources around them, but it could explain their behaviours. We
learned about the importance of loss of inhibitory functions in the 1960s and 1970s when a
few persons were operated after very long periods of blindness. Although good image
quality was present, these persons avoided visual information because it was chaotic,
entering the brain like huge waves. These case histories opened our eyes for understanding
the stress of living in a different world where visual information may have good image
quality but its processing is disturbed.
3. Parallel visual pathways
At each level of the long visual pathways there are functions parallel to each other. In
the retina the sensory cells, cone and rod cells, may function in harmony, one of them
may be missing or the normal rodcone interaction may be disturbed. In the optic nerve
the pathway has several different types of nerve fibres, of which the magnocellular and
the parvocellular pathway are the best known (Fig. 1). The main pathway from the eye
to the cortex is the retinocalcarine pathway to the primary cortex; the tectal pathway is
the parallel pathway to cortex bypassing form processing in the primary visual cortex
and in the specific nets of the ventral stream. From the primary visual cortex, visual
information flows upwards as the dorsal stream and downwards as the ventral stream. In
the cortex visual information is processed in numerous specialised groups of cells that
are so well interconnected that we see a stable, clear image although different parts of
the image have travelled through paths of varying length so they must reach the
destination at slightly different times.
If there is damage to the nerve fibres connecting the numerous specialised processes or
there is loss of function in any of the specialised cell groups, the resulting visual
perceptions are likely to differ from norm. The images that we perceive are more a
composition of our brains than exact pictures of the physical world.
4. Infants and children at risk
Visual impairment in children is mostly congenital or occurs early in life. Therefore it is
mandatory that all infants at risk are brought to paediatric neurologists and ophthalmologists who can help the families, therapists and teachers in the transdisciplinary
assessment that often takes years before all deviating functions have been assessed. The
risk groups are well-known: infants with abnormal eye contact or poorly developing early
interaction, infants with motor problems, with hearing impairment or intellectual
disabilities.
5. International recommendations
The International Classification of Functioning, Disabilities and Health (2001) and the
document Management of Low Vision in Children (WHO/PBL/93.27) should function as

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our guidelines in the evaluation of needs for services and special education. In the latter
document, the assessment of school children is discussed in detail. It differs from the
previous international classification, the ICD-10, by not listing only individual visual
functions, visual acuity and visual field, but activities and the effect of visual impairment
on activities and participation. The chosen activities, communication/interaction,
orientation and moving, activities of daily life (ADL) and sustained near vision tasks,
like reading and writing, are important in all cultures.
6. Case history as the primary information
In the transdisciplinary assessment, carefully chosen questions map which visual
milestones have been reached. Eye contact between the infant and the parents is noticed at
the age of 6 weeks or earlier. If it has not started at that age, two more weeks are waited
hoping for the function to appear. If it does not develop, referral to an ophthalmologist
should be considered without delay and early intervention started to support the
development of interaction.
Delay in development of eyehand coordination, watching and exploring the hands and
later pointing and grasping interesting objects may be a sign of problems in dorsal stream
functions. Bringing hands to the midline, making the infant aware of his hands and
bringing them to the mouth, often helps the infant to become aware of his hands and to use
them in exploration of objects.
At the age of 1011 months, normally developing infants respond to their family
members differently from strangers when these approach the infant without talking. If an
infant does not respond to family members as long as they are silent but does respond to
their voices, either the image is of poor quality or the child has poor or no face recognition.
Face recognition is crucial in the interaction of toddlers and thus a toddler who does not
recognize facial features or expressions is lost in a group of young children. If loss of face
recognition is not diagnosed, the child is in danger of being diagnosed as having bautistic
behavioursQ.
When the childs communication develops, new observations can be made about
cognitive visual functions. Transdisciplinary assessment should be repeated regularly and
every deviation from normal reported to the childs neurologist.
7. Oculomotor functions
In the clinical assessment, oculomotor functions are evaluated as the first functions
observing the alignment of the eyes, smooth pursuit movements, and convergence. Quality
of fixation (stable/unstable/fleeing) and saccades (regular/irregular/absent) are less often
recorded. Functions of the intraocular muscles that are important in focusing of the image,
accommodation, should be routinely examined.
8. Image quality
Images that we see are composed of three different simultaneous perceptions: forms,
colours and movement.
We process forms, colours and motion in the primary visual cortex as separate
functions, after which the processing goes on in the higher visual cortices where the

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percept of the objects evolves. When we assess the quality of images, we collect all the
information that is available from a thorough clinical examination (Table 1).
If this table is copied, it is easy to fill in the observations related to each function.
There are clinical tests for all other functions but motion perception. For infants there
is the Pepi Game on my homepage (http://www.lea-test.fi). It allows observations on
how the infant fixates the central, flickering target, whether there are quick saccades to
the picture of the dog when it appears in one of the corners and on the quality of the
following movements when the picture moves from one corner of the screen
diagonally to the other corner. There is no line around the picture of the dog so the
infant must perceive the group of coherently moving dots to follow the movement of
the picture.
Since we do not have several tests for motion perception we need to observe the
behaviours of children and discuss with them how they see moving objects, swings,
cars, fast moving balls, as soon as the child has that communication level. Children and
adult persons with poor motor perception seem to walk faster than their peers. Some of
them have explained the rushing movements that this gives them better balance. This
could be interpreted so that by walking fast they increase the angular speed of the
relative movement of objects close to them so much that these disappear and the objects
at distance are seen better. They bump into large objects, which they have seen at
distance, but which disappear when they come close to the object.
9. Cognitive visual function
The list of cognitive visual functions in Table 2 is not a complete list but a list of
functions that many teachers have found important for planning of individual educational
plans (IEPs).
10. Integration problems
Normal brain functions combine information from all senses, integrate them to a
holistic experience of objects with visual, auditory, tactile, kinaesthetic, haptic, taste and
smell qualities. If use of sensory information has not been normal, integration may not
have developed but the child uses information from the different modalities in
sequences: When a child explores with hands the gaze is turned away; when he
concentrates in listening, visual world steps in background, etc. In children who learn to
move late, use of vision and motor functions may not be simultaneously possible: the
child looks ahead, walks, stops, looks, walks. To a young child this is normal so we
need to ask the child about possible changes in the structure of the surrounding world
when they learn to move. bEverything disappearsQ is rarely heard as a spontaneous
observation before the age of five years.
Balance combines information from the peripheral visual field, the inner ear and the
proprioseptive information from the muscles and joints. Impaired vision with poor
quality of image or limited visual field and poor visual and proprioseptive awareness of
body parts and their positions is a common problem in children with cerebral palsy.
Neurodevelopmental therapy approach to moving and balance is therefore an important
part of early intervention of many children with brain damage related vision loss.

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11. Compensatory strategies

The goal of early intervention and special education is to help the infant/child to
function at an optimal level using all the functions that the child has and to develop
compensatory strategies to perform in activities, for which a childs visual functions are of
poor quality, poorly processed or inhibited by other pathologic functions.
Auditory and visual information are our distance senses. When vision is impaired, the
role of hearing becomes more important than it is in the life of normally sighted children.
Evaluation of hearing for communication and orientation in space is at least as important
as is assessment of visual functioning. Children with circulatory failures, trauma or
infection before or soon after birth are an important group of children with processing
problems in vision and hearing. If a child has processing problems of both visual and
auditory information, the child has dual sensory impairment and is entitled to special
services for deafblind people. Normal audiogram may be measured and yet the child may
have processing difficulties and may therefore benefit of an FM system.
Exploration with hands and mouth is a typical activity of infants to learn to know
concrete forms and structure of surfaces of objects detected and explored by sight. If an
infant has poor function of hands and arms, this important activity should be supported
everyday so that the visual world would get a normal concrete foundation. It is quite
common to meet teenagers who have never put their fingers in their mouth. Hyper- and
hyposensitivity of hands, head and mouth are common other problems in exploration of
objects and environment.
12. Conclusions
Visual functioning in infants and children with different types of vision loss shows a
great variation that makes assessment of vision difficult and time-consuming. Ophthalmologists, optometrists and paediatric neurologists play a small but important role in the
basic evaluation of the conditions. The most important observations and assessments are a
part of early intervention and special education. Therefore the classification of brain
damage related vision impairment should be more in the hands of rehabilitation specialists
and special educators who have opportunities of observing the child in different activities.
They need consultation with the medical and neuropsychological specialists to interpret
their observations and to further vary the activities with specific questions in mind. This
tedious, careful collection of data goes on through infancy and childhood as a
transdisciplinary approach of medical and educational specialists and the families
sometimes in frustratingly difficult situations, but also with rewarding experiences of
understanding one more piece of the puzzle.