In this section, the cerebral visual impairments described come under the term, Dorsal Stream Dysfunction. The affected part of the brain is called the posterior parietal lobes. The visual information sent from the eyes and produced in the occipital lobes travels along the dorsal stream to the posterior parietal lobes. This is explained in a little more detail in the Understand section conclusion.
All cerebral visual impairments are unique to each person. In the first section, we looked at impairments to clarity of vision, the ability to see contrast and the visual fields. In the most severe cases these cause blindness or partial blindness (or sight impairment and severe sight impairment). With dorsal stream dysfunction, the most severe cases are called Balint Syndrome.
Rezso Balint was a Hungarian neurologist and psychiatrist, and identified Balint Syndrome in the early 1900s. The three key areas are simultanagnosia, optic ataxia and apraxia of gaze.
There is no 'typical' person with Balint Syndrome, however all those affected share degrees of the following:
Balint Syndrome is not the same as, or part of blindness, they are quite separate, although of course some people have both. Here are some examples of different people who all have Balint Syndrome:
The experience of having reduced visual attention due to simultanagnostic vision varies considerably from person to person. It also varies considerably for the same affected person, depending on their environment. Mary can cope well in a familiar clutter free environment like her office, yet was recently left unable to function when lost in a busy airport.
Mary explains how her simultanagnostic vision left her "highly emotional and stressed".
My connecting flight home was cancelled due to fog and I had to transfer to a direct flight. This in itself was not a problem other than it upset me and made me anxious for a period of time that I wasn't going to get home. So then when I was told I had to go back to the check in area, uplift my bad and re check in for the new flight, the panic really started. I had arrived at the airport with a colleague and hadn't taken any notice of where we had walked, so had no clue how to get back to the check in area. Because I was already in that stressed, emotional state, I felt unable to navigate my way round a busy, crowded airport to get to where I needed to go.
Of course I was unable to find the check in area or my bag and by this stage was feeling extremely upset, useless, vulnerable and frustrated. Realising of course that sitting down and crying was not going to help much, I tried to calm down and use my techniques to get me though. But I was in too much of a distressed state for these to be effective, proving again that once I have gone into fight or flight mode, it is very difficult to come back from it. Fortunately, a kind lady from the airline rung me to tell me my flight had changed and when I told her what I was trying to do and that I had a vision impairment and couldn't find my way around the airport (something I never do, as I never want to admit that I am incapable of doing something), she told me to stay where I was and she would come and find me to take me to where I needed to be - phew.
The experience left me highly emotional and very stressed - frustratingly so. Now I am trying to work out what I could have done differently to make the situation better. Even if I had managed to stay calm, I am not sure I would have been able to find where I needed to go.
For more information on simultanagnostic vision please visit our Simultanagnosia Spectrum sections.
Optic ataxia effects how we perceive depth and distance, making our accuracy of reach less precise. This can be confusing and at times frightening. Things you can see and reach for may not be there, when you try to touch or grab them. Conversely, things you thought you were clear of, like doorways, or other people, you may knock into.
Please visit our Optic Ataxia section for a more detailed explanation with further examples.
When someone's eyes move in correct harmony together, but they can't look from one thing to another in the normal way, this is called apraxia of gaze. In her blog, Nicola McDowell describes how her eyes felt when she was trying to follow a football match "I would struggle as my eyes flitted from one player to another, never actually finding the person that had the ball. Then after only a short period of focusing on the mass of players on the field, I would find myself staring off into an area where no one was, without any idea when or why I had averted my gaze from the field altogether". Watching Nicola's eyes when this happens, they seem to look around at random, with no purpose.
When less severe, apraxia of gaze may mean that the person has to try much harder than others to stay focused on something, and may appear to lack attention for this reason. Their eyes may seem to 'flit' at times. With the more severe cases, the person has little, if any, conscious control over their eye movements and this can look like the eyes range from seemingly staring into space at nothing, to darting around in every direction.
Note, there are many other conditions that cause the eyes to move in an atypical way including to and fro movements of the eyes or nystagmus, and squint or strabismus, more information on these conditions is available here.
Some key factors to consider when understanding the spectrum of dorsal stream dysfunction to Balint Syndrome are:
A common view of Balint Syndrome and its range of features is that of a profoundly disabled blind person, however hopefully this brief introduction of the condition shows that it covers a wide range of people.
Many (but not all) people who have Balint Syndrome also have lower visual field impairments, this is because the two affected areas of the brain are close together.
For this reason, if someone has a lower visual field impairment, it may be a good idea to check for dorsal stream dysfunction or the more marked variant, Balint Syndrome.
Daniel is a boy who had a number of conditions as a young child, requiring a long stay in intensive care, extensive surgeries and ongoing medical interventions. As Daniel recovered, it was not mentioned that his brain may have been affected. Daniel was still a young child, and his visual responses were normal when tested. Once home the family tried to get back to normal, however something had changed in Daniel. When he learnt to walk he regularly tripped over things and bumped into things. He didn't learn to talk, and developed severe behaviours. He was often physically aggressive both directed at himself and others. He was so stressed he ground his teeth to the nerves, requiring multiple extractions, and became obsessed with small objects. Daniel was assessed and his behaviours were attributed to severe autism.
At school Daniel's wellbeing deteriorated and his behaviours became more extreme. There were many incidents, including a trip to an activity centre where Daniel was taken on a fast ride several times - his mother describes him as having a complete breakdown after this point.
Following extensive questions and reviews of behaviour, it emerged that Daniel has a lower visual field impairment, explaining why he regularly tripped over things, and liked climbing up things, but not down. Daniel also has optic ataxia, demonstrated by not only issues with guidance of reach, but being very severely affected by looming. Daniel also has simultanagnostic vision, and can only attend to a small amount of visual information, like the very small objects he is so obsessed with. This can also be observed when looking at Daniels eyes when out and about and his loathing of noisy busy cluttered places.
Daniel presents with the features of Balint Syndrome.
Daniel's mother is now aware of this and has taken many positive steps including using a tent at home; a safe place where Daniel can rest and let his brain re-charge. The single colour of the tent removes the pressure from his simultanagnostic vision (making his eyes flit from one thing to another) and he can relax. Daniel's mother also now uses a buggy to take him places, even though Daniel can walk. The chair provides a protective shield, and in it, Daniel lets her take him to places, safely so that he won't feel threatened.
Following many traumatising years, Daniel is slowly learning to trust the people around him, and engage with the world again. It will be a slow process, but with the understanding the family now have, Daniel is responding positively.
Daniel's mother writes:
With Daniel's acknowledgement of my deepening understanding of his visual world he is allowing me to take him out of the buggy and encourage him to walk holding my hand to unknown places. Before he would sit down after a few steps and refuse to move. Now we are walking hand in hand for around five minutes together calmly enjoying the countryside. Letting go of the autism label has meant we can provide a Daniel centred learning and nurturing environment which will hopefully over time promote wellbeing and confidence in Daniel's sense of self.
I used to use the 'hand over hand' principle incorrectly, following poor advice. I placed my hand on top of his and guided it for him. I have now been shown the correct method in which his hand explores what I am touching by moving it forward over mine, and it worked immediately. Daniel now opens doors after only a few attempts with hand over hand, and he is learning to navigate the computer with this method, before he would have had a CVI meltdown. He controls the remote control with hand over hand and he is so relaxed as opposed to being gripped with anxiety when I did it the wrong way. Daniel is home-schooled and participating in 1-1 climbing tutoring at the local climbing centre. He has also started 1-1 horse riding as group sessions are overwhelming for Daniel. The 1-1 allows him the opportunity to focus and enjoy the experience. Daniel also enjoys aquafit classes as it provides a meaningful stimulus in the form of movement, water and music with the local ladies! He is responding to my prolonging single words describing experiences as he has them, whereas before he was thought to be completely non-verbal. The understanding of simultanagnosia has allowed us to provide meaningful visual stimuli and he's showing interest where before he would dismiss and withdraw. The most stark change is in Daniel's relative calm and self-belief, as he now believes we are listening to his needs. We are communicating together on a level that we have never experienced before. Daniel believes he has more control over his life and is allowing himself the opportunity to explore the world around him at his pace.
Hand Over Hand (sometimes called Hand Under Hand)
The whole point of hand-over-hand is that the child (or affected adult) is in control. The child is encouraged to explore with his or her hand over the adult's, and should never be forced to move the hand anywhere. You are helping them to gain confidence and trust as they overcome their fear of reaching out. Imagine having inaccurate guidance of your hand movement, so that often in the past you reached out and hurt yourself. You would want a reliable safe guide over which you can reach out and explore. The alternative of having one's hand thrust forward into an unknown space is frightening.
Suzanne Little is a well published and highly experienced senior teacher of visually impaired children, with expertise in profoundly disabled children with CVI. Suzanne has shared both a case-study on Ali, and the approach she used to develop skills and increase potential.
Ali suffered brain damage at birth resulting in quadriplegic spastic cerebral palsy, the need for a brain shunt, epilepsy and cerebral visual impairment.
Ali experiences sensory overload and distress if placed in a noisy and cluttered environment. If Ali is given an experience of a small number of stimuli at once she can be involved in interaction with her environment and with her helper. She benefits from visual and auditory distraction kept to the minimal. Ali demonstrates all the features of Balint Syndrome.
Previous Educational Experience:
In Ali's first school she did not have an assessment for a sensory programme and was misunderstood and often excluded from the classroom experience, as it was reported that Ali upset the other children with her shouting. The result was often that Ali was placed in a corridor with a teaching assistant without any activity or individual learning programme.
The negative experience continued and Ali was removed from school plays because of the noise she created (in an environment that had clutter without any meaning for her). There was no routine or structure to enable Ali to engage in activities or interaction with others, she was considered unresponsive to any visual stimulus and within the general classroom setting.
New Alternative Educational Experience:
When Ali was seven she left her old school, and went to a residential school for children with profound disabilities. Ali required a multidisciplinary team assessment to provide an individualised, holistic and integrated therapy learning programme created around her physical, medical and learning capabilities. Ali's parents shared information which described Ali's responses of distress at any sensory overload; for example, when taken to a supermarket or when the family of four children were together after school or other family occasions. Ali would be calm if on her own or in a calm environment with 1:1 attention. In other noisy cluttered environments, she could become greatly agitated vocalising unhappily for long periods of time and consequently had difficulties in calming. She would frequently self-harm by banging her head with her fist, bite her hands or put her fingers down her throat.
Educational Assessment: Multidisciplinary Assessment Framework:
Detailed observations of Ali's communication, cognitive and sensory responses were taken for the first term and these contributed to the multidisciplinary assessment which is shared with other members of staff:
From these observations, an individualised sensory programme was written to provide Ali with learning activities based upon her responses.
Monitoring with observational assessment and recording of student outcomes is required to design and evaluate, and regular multidisciplinary meetings are a part of this process.
Careful consideration was given to planning the optimal environmental design and types of activities that would provide options to enhance learning and engagement within the curriculum planning.
A habilitation approach of teaching was used, which enables a child to make best use of their available functions and this was applied through a timetable of sensory activities to develop sensory, cognitive and communication awareness and attention.
Habilitation learning approach: planning that enables a person to learn, keep, or improve skills and functional abilities that have never developed, or have not developed in a typical manner
The multidisciplinary assessment and video evidence for evaluation is a part of planning and designing an individual learning programme for Ali. The need to observe and evaluate Ali's underlying abilities based upon:
The multidisciplinary assessment brings together the visual, physical, cognitive and social skills as a part of Ali's learning programme.
Considering the complexities of Ali's disabilities, the use of visual stimulus might have been overlooked in planning for learning and quality of life. Ali as a registered blind child and displaying limited visual responses of brief awareness of light and dark, might not have had interventions for visual stimulus planned for her daily routine. During the initial assessment of Ali's capabilities it was clear that sounds and music were a key intervention in planning development in learning, as well as using integrated therapy for movement and sensory integration. All of these areas of the assessment framework were things that built upon capabilities, which were part of the plan to develop interventions for progress with cognition, sensory and communication skills.
A tent was used as a means to cut out extraneous stimuli and remove clutter, creating a 'little room' space with one colour surrounding Ali.
The tent provided the key to open the door to the possibility of awakening visual awareness for Ali. Her response was an immediate trigger to cross the threshold of visual awareness, therefore this became a part of her one thing at a time visual programme.
The tent as part of the observation assessment for Ali has provided a novel method for visual assessment of visual awareness and attention. Prior to using the tent Ali had appeared to have a severe visual acuity impairment as she does not react to any size of object within close proximity and no response to visual field awareness, contrast or colour perception. She would only reach out to an object if it had a sound that interested her, or a texture that she could explore with her mouth. However, within the first experience of being positioned within a colour tent, Ali used her limited vision immediately to become visually aware, gazing around moving her eyes and head, at times fixing her gaze for a few seconds. The level of awareness developed into visual attention over time as well as greater interest and motivation to look at bright colours and light and colour tent spaces. Before the tent experience these responses did not exit, within the tent the visual awareness continued and this also had the effect of increasing calmness and aiding Ali's communication skills.
Tents as well as having a visual lock have the advantage of allowing the child to evolve gradually into the real world, something that the sensory room does not afford, as the lights are suddenly switched on and the session is over with sudden return to the cluttered world.
Tents also provide the opportunity to have a visual lock to what is vertical and what is horizontal, potentially allowing the balance system to integrate with the visual system. In this context, it could be worth exploring the idea of vertical corners, for those children who tilt to one side, and have limited body and head control. When in the 'real world' they may not be able to integrate the balance system with the visual system, but when in the tent world, this provides the opportunity to do so, if given vertical and horizontal meridians to view, and to integrate with the concept of vertical and horizontal that is unconsciously afforded by the balance system.
Time of observations, before and after effects inside and outside of the tent.
These developments over a three year period have improved opportunities for learning and for quality of life and these are a part of Ali's individual learning programme; providing her with time and environments which enable her to engage within her level of capabilities. She has increased her periods of visual awareness and attention and can express colour preferences with her vocalisations and facial expression and is motivated to gaze at light and colour movement in tent environments which is a new found awareness. The increase in periods of calmness through being aware visually of being in an environment that surrounds her appears to have given Ali a sense of a pure space without clutter, which opens the door to her mind. From this experience Ali has become calmer in general situations and more open to interaction with others through the use of sounds and music, which have also been introduced one at a time.
Combination of sensory stimuli:
During periods of health issues the tent environment has provided periods of focused attention and calmness, especially with the use of gentle light and colour movements which are reflected onto the tents from a projector.
Ali enjoys the Multisensory room now after her visual awakening within colour tents, as long as the room is without clutter of music and movement from one thing to another. During periods of ill health the Multisensory room with a projection of slow moving lights and colour have provided calming moments and relaxation from pain. Integrated therapy physical programmes in which Ali has had a change of position for floor positioning with the combination of the visual stimulus have proved beneficial from simply laying on a bed after hospital visits and during times of recovery.
Also, despite entering the tent at a 'ripe old age' Ali started to be able to look around and enjoy their environments when in the 'real world'. This suggests that a structured programme of evolution from visually and auditorily silent world into the cluttered world is needed. This was done intuitively using the light projection system. In addition to this it would be good to progressively introduce more items into the tent world, to train the ability to see pairs of items and make choices, followed by triads and more.
For both Daniel and Ali, their Balint Syndrome had not been identified. Daniel, at an early age, was assessed and his challenges and behaviours were attributed to severe autism. Ali had profound disabilities and her visual responses were so poor that there seemed little point trying to develop further skills.
With both cases, the unknown Balint Syndrome not only prevented valuable learning opportunities, because the optimal clutter free environment was not known, it also was the cause of considerable distress for both children.
Technically there is a challenge, because many people with Balint Syndrome are unable to understand even simple assessment questions, like 'can you look at that?' or 'where is ...?'. A diagnosis of Balint Syndrome is based on how the person answers questions and responds. If the person is unable to do this, as is the case with Ali and Daniel, even with overwhelming evidence, Balint Syndrome can only technically be a hypothesis, not a diagnosis. Based on this hypothesis however, with a supportive and appropriate developmental programme, Ali and Daniel's lives are being transformed.
Further information about Suzanne Little's work using tents can be found on the CVI Society and Scottish Sensory Centre websites.
Our professional advisors have noted that Balint syndrome is a truly hidden disability in children.
In adults the diagnosis is made by investigation of cooperative subjects. Something that is not attainable in affected children.
Affected children can never know they have it, and so cannot describe it.
Their lack of ability to encompass the whole scene with their vision is not at all obvious to the observer because it is masked by their overall behaviour.
So how can it be identified?
The route to finding out what the matter is involves:
thinking of the possibility in the first place
hypothesising that it may be present
taking appropriate action,then
finding out whether this proves effective.
It is efficacy of the harmless 'therapeutic trial' of decluttering and making things simple and singular that retrospectively lends weight to the diagnosis.
Update: 10 May 2017: Comment by Professional Advisors (above) and additional case-study (below).
Janet Harwood is an expert teacher of children with cerebral visual impairments and is the founder member of the CVI Society.
A Privileged Experience
A few years ago, I was privileged enough to be able to work for a year with Sammy, a young person who has profound and multiple learning difficulties and a diagnosis of cerebral visual impairment. Sammy has significant damage to the occipital lobes (the seeing part of the brain).
I understood from a colleague who knew Sammy well he had shown no visual response. Classroom observation indeed proved this to be the case. Sammy was at times quite ill and was awaiting spinal surgery. My colleague reported that activities which engaged Sammy generally involved sound.
I observed Sammy in a music therapy session and it was wonderful to see him so responsive, smiling and vocalising.
For children with severe cerebral visual impairment including Balint Syndrome, classrooms, even when 'calm' often have much to visually distract. For many children 'visual clutter' may inhibit visual responses.
Having discussed the use of colour tents with a fellow trustee (Suzanne Little) I decided to try using a rudimentary one, to see if it would enable Sammy to respond visually.
Before starting 'tent work' I contacted the school's visiting occupational therapist. I was keen to ensure that Sammy could be positioned as comfortably as possible, knowing that this could mean the difference between a response or no response, especially as Sammy was often unwell and in pain, two factors which were likely to prevent him from being able to attend.
The occupational therapist was very interested in what I intended to try and asked if we could carry out joint visits.
Our initial session was in a small sensory room, no equipment in use, just the only room available although there was noise overspill from an adjoining room on occasions which was not ideal. I had purchased a selection of fluorescent fabrics but did not yet have a tent frame. Having someone else to support and observe proved very helpful.
The first session began under ultra violet light, something I would not do now. It has been noted that in some children with profound CVI, engagement may continue further, but changing lighting may disrupt this. Dim lighting will often be more successful. Sammy was on his tummy on a padded stretcher, with his chin propped slightly on a small pillow.
I knelt on the floor, out of his view, the occupational therapist observing. I held a long piece of orange fabric about 6 inches from Sammy's face, being careful that it didn't touch him.
Almost instantly his eyes, which had previously showed no purposeful gaze, alerted and tracked across about a foot of the fabric.
After while I decided to try a piece of fluorescent lime green fabric.
Sammy looked intently and raised his head a fraction, with a questioning expression on his face, not one of displeasure but rather 'What's this'?
Spurred on I discussed what we had witnessed with Sammy's teacher who agreed to try and ensure that Sammy would have opportunities to repeat 'tenting'. As a visiting teacher, I was only able to visit monthly. Each time this was a joint visit with the occupational therapist. We moved to a room which wasn't completely dark but dimmed.
As the year, progressed Sammy continued to vocalise as we held the fabric, raising his head to look around, which was a great effort for him but which he did when surrounded by his green tent.
The most magical session took place when we were accompanied by a student occupational therapist. By chance Sammy was in his chair and we were unsure if he would respond.
The tent was by now being held further away, in this case it was held about a metre above his head and a couple of feet outwards at the sides. Sammy vocalised and reached up towards the top of the 'tent'. The occupational therapist was amazed as he was using his weaker arm. Sammy vocalised with pleasure.
Throughout all sessions, we did not speak, just watched Sammy enjoying the 'tent' experience.
I understand from a colleague that Sammy still enjoys his tent time.
Liaison with therapists to ensure that the child is comfortable is important, as in Sammy's case physical comfort is more likely to bring success.
It is so important that children with such complex needs as Sammy are given clutter free, quiet experiences. Visual potential may be there but we need to remove visual clutter and other sensory distractions to set it free.
Sammy is another example, like Ali, of a child with profound disabilities who were considered to have no visual responses because their Balint Syndrome was unrecognised. In both cases, the lack of visual responses was not due to lack of visual function, but rather due to the clutter in their environment making visual responses almost impossible due to their simultanagnostic vision.
The optimal environment of the tent proved to be to 'key' to open the door of visual awakening. With this knowledge and understanding both children have shown great potential, including Sammy reaching with his 'weak arm' for the first time.
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