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Cerebral Visual Impairment <br />

AZ AER Workshop <br />

April 8, 2011


Objec4ves <br />

• Defini9ons <br />

• Brain anatomy 101 <br />

• Dorsal and ventral stream disorders <br />

• The CVI Range/Phases


Prevalence <br />

• Cor9cal visual impairment is the leading cause <br />

of visual impairment in children in developed <br />

countries (Hoyt, 2003).


Defini4ons


Cor4cal Visual Impairment <br />

• Historically used to primarily to describe poor <br />

visual acuity or visual fields due to damage of <br />

the brain. <br />

• Problem—cerebral cortex is rarely damaged <br />

on its own


Cogni4ve Visual Dysfunc4ons (CVD) <br />

• When primary visual deficit is not visual acuity <br />

loss, but visual percep9on and integra9on, <br />

some groups are calling it CVD <br />

• Many children have both CVI and CVD and <br />

the defini9ons for the two have not yet been <br />

clearly defined in the medical literature with <br />

some research groups using CVI as an <br />

umbrella term for CVI and CVD <br />

Good, W.V., Jan, J.E., Burden, S.K., Skoczenski, A., Candy, R. (2001). Recent advances in cor9cal visual impairment. Developmental <br />

Medicine & Child Neurology. 43, 56-­‐60.


Cerebral Visual Impairment <br />

• Impaired vision due to damage to the brain. <br />

– Visual input and primary processing <br />

– Higher visual processing (Cogni9ve visual <br />

impairment)


CVI vs. DELAYED VISUAL <br />

MATURATION <br />

• DVM is characterized by visual unresponsiveness in <br />

early infancy which subsequently improves to <br />

normal levels. <br />

• These children may retain subtle mo9on or aben9on <br />

deficits. <br />

• Hoyt, D.C, Jastrzebski, G., Marg, E. (1983). Delayed visual matura9on in infancy. Bri9sh Journal of <br />

Ophthalmology. 67, 127-­‐130. <br />

• Harris, C.M., Kriss, A., Shawkat, F., Taylor, D., Russell-­‐Eggib, I., (1996). Delayed visual matura9on in infants: <br />

A disorder of figure-­‐ground separa9on Brain Research Bulle9n. 40, 5/6, 365-­‐369.


Why Does it MaLer <br />

• Medical professionals <br />

• Educators <br />

• Rehabilita9on professionals <br />

• Parents and family members


Brain Anatomy 101


The Brain and Vision <br />

• Over 40 percent of the brain is devoted to <br />

vision <br />

• Damage to different areas produces different <br />

effects <br />

From Dubon, G.N. (2003). Cogni9ve vision: Its disorders & differen9al diagnosis in adults & children. Knowing <br />

where & what things are. Eye, 17, 289-­‐304


VISION INVOLVES MORE THAN<br />

THE EYES<br />

CEREBRUM<br />

4 LOBES WITH SPECIALIZED FUNCTIONS<br />

FROM SECRET LIFE OF THE BRAIN: PBS


LEFT HEMISPHERE <br />

FRONTAL LOBES:<br />

command center for decision making<br />

and executive tasks<br />

FROM SECRET LIFE OF THE BRAIN: PBS


LEFT HEMISPHERE<br />

OCCIPITAL LOBES<br />

process vision<br />

FROM SECRET LIFE OF THE BRAIN: PBS


LEFT HEMISPHERE<br />

PARIETAL LOBES<br />

analyze sensation<br />

FROM SECRET LIFE OF THE BRAIN: PBS


LEFT HEMISPHERE<br />

TEMPORAL LOBES<br />

hearing, understanding speech,<br />

integrated sense of self<br />

FROM SECRET LIFE OF THE BRAIN: PBS


Match that Lobe! <br />

• Occipital lobe <br />

• Parietal lobe <br />

• Temporal lobe <br />

• Frontal lobe <br />

• Discrimina9on and <br />

processing <br />

• Emo9ons and judgment <br />

• Vision <br />

• Hearing and speech


How Visual Informa4on is Processed <br />

• Reflect Visual Pathways: <br />

– SUBCONSCIOUS; PROTECTION <br />

• Higher level processing <br />

– Dorsal stream: WHERE PATHWAY <br />

– <strong>Ventral</strong> stream: WHAT PATHWAY


Reflex Visual Pathways <br />

Upper mid-­‐brain (superior colliculi) and pulvinar <br />

Rains, G.D.(2001).Principles of Human Neuropsychology. New York: McGraw-Hill


REFLEX VISUAL PATHWAYS <br />

• Ability to perceive and react to moving targets <br />

at a subconscious level <br />

• Some children s9ll react to moving targets <br />

even though there is profound vision loss due <br />

to cerebral damage <br />

• Can see an object brought in from the side <br />

(blindsight) and can navigate without bumping <br />

into objects (travel vision) <br />

• Develops at around 4-­‐5 years of age


HIGHER VISUAL FUNCTIONS <br />

What Happens to Informa4on that <br />

Reaches the Visual Cortex


Via Dorsal <strong>Stream</strong> information goes to the<br />

Posterior Parietal Lobes<br />

WHERE<br />

Dorsal<br />

<strong>Stream</strong><br />

<strong>Ventral</strong> <strong>Stream</strong><br />

WHAT<br />

From Dutton, G.N. (2003). Cognitive vision: Its disorders & differential diagnosis in adults & children. Knowing where &<br />

what things are. Eye, 17, 289-304.


POSTERIOR PARIETAL LOBES - WHERE<br />

Process whole visual scene and work with <br />

frontal lobes to give aLen4on to <br />

areas of interest and <br />

plan motor ac4on <br />

WHERE<br />

Dorsal<br />

<strong>Stream</strong><br />

<strong>Ventral</strong> <strong>Stream</strong><br />

WHAT<br />

From Dutton, G.N. (2003). Cognitive vision: Its disorders & differential diagnosis in adults & children. Knowing where &<br />

what things are. Eye, 17, 289-304.


Via <strong>Ventral</strong> <strong>Stream</strong> information goes to the<br />

Temporal Lobes<br />

WHERE<br />

Dorsal<br />

<strong>Stream</strong><br />

<strong>Ventral</strong> <strong>Stream</strong><br />

WHAT<br />

From Dutton, G.N. (2003). Cognitive vision: Its disorders & differential diagnosis in adults & children. Knowing where &<br />

what things are. Eye, 17, 289-304.


TEMPORAL LOBES - WHAT<br />

Facilitate recognition of what is being viewed<br />

WHERE<br />

Dorsal<br />

<strong>Stream</strong><br />

<strong>Ventral</strong> <strong>Stream</strong><br />

WHAT<br />

From Dutton, G.N. (2003). Cognitive vision: Its disorders & differential diagnosis in adults & children. Knowing where &<br />

what things are. Eye, 17, 289-304.


FRONTAL LOBES - Mediate choice<br />

MOTOR CORTEX -Receives input from the dorsal<br />

stream about where, how far, how to position body<br />

parts<br />

WHERE<br />

Dorsal<br />

<strong>Stream</strong><br />

<strong>Ventral</strong> <strong>Stream</strong><br />

WHAT<br />

From Dutton, G.N. (2003). Cognitive vision: Its disorders & differential diagnosis in adults & children. Knowing where &<br />

what things are. Eye, 17, 289-304.


Dorsal and <strong>Ventral</strong> <strong>Stream</strong>s are linked<br />

WHERE<br />

Dorsal<br />

<strong>Stream</strong><br />

<strong>Ventral</strong> <strong>Stream</strong><br />

WHAT<br />

From Dutton, G.N. (2003). Cognitive vision: Its disorders & differential diagnosis in adults & children. Knowing where &<br />

what things are. Eye, 17, 289-304.


PERIVENTRICULAR LEUKOMALACIA <br />

Next to ventricle <br />

loss of white maLer <br />

• Periventricular leukomalacia (PVL), the most <br />

common cause of visual impairment due to <br />

brain damage (Dubon & Jacobson, 2001), is <br />

extremely common in small premature infants <br />

(Volpe, 2003). <br />

• PVL is seen in increasing frequency in <br />

premature children due to improved survival <br />

rates of very premature infant


VENTRICLES <br />

Fluid-­‐filled cavi4es in the cerebral hemisphere,mid-­‐brain, and behind the <br />

brainstem <br />

Neuroscience for kids - Ventricleshttp://faculty.washington.edu/chudler/vent.html<br />

AboutKidsHealth- http://www.aboutkidshealth.ca/clinicalAreas.asppageContent=BT-nh1-02


Periventricular Leukomalacia <br />

Next to ventricle loss of white maLer <br />

• Cap4on: Picture 7. Cranial MRI, T1-­‐<br />

weighted axial image, in an 18-­‐<br />

month-­‐old with periventricular <br />

leukomalacia (PVL). The lateral <br />

ventricles are enlarged without <br />

hydrocephalus. The periventricular <br />

white maber is diminished. Courtesy <br />

Normal adult brain<br />

of Mabhew Omojola, MD. <br />

From emedicine http://www.emedicine.com/ped/topic1773.htm<br />

http://bissells.com/library/articles/pvl.htm


Dorsal <strong>Stream</strong> Disorders <br />

Dorsal <strong>Stream</strong> processes <br />

whole visual scene <br />

and works with frontal lobes to <br />

give aben9on to areas of <br />

interest and plan motor ac9on


Dorsal <strong>Stream</strong> Disorders <br />

• Incoming informa9on goes to: <br />

– Occipital cortex for processing <br />

– Dorsal stream sends the informa9on to posterior parietal <br />

visual cortex where whole scene is processed and a part of <br />

the scene is chosen <br />

– This informa9on goes to the frontal cortex which tells the <br />

head and eyes to look at the scene detail <br />

– The informa9on coordinates are also sent to the motor <br />

cortex which ini9ates movement to toward the scene <br />

detail and causes other motor adjustments to interact <br />

with the objects in the scene.


Dorsal <strong>Stream</strong> Disorders <br />

• Dorsal <strong>Stream</strong> processes whole visual scene <br />

and works with frontal lobes to give aben9on <br />

to areas of interest and plan motor ac9on <br />

• Damage can result in problems extrac9ng <br />

details from complex visual scenes <br />

– loca9ng objects on paberned background, <br />

– func9oning in busy environments, <br />

– reading small text


Dorsal <strong>Stream</strong> Disorders <br />

• Dorsal <strong>Stream</strong> processes whole visual scene <br />

and works with frontal lobes to give aben9on <br />

to areas of interest and plan motor ac9on <br />

• Damage can result in disordered visually <br />

guided movements (children with PVL) <br />

– Difficulty moving through space, differen9a9ng boundaries <br />

of floor surfaces, <br />

– Nego9a9ng curbs, stairs <br />

– Inaccurate reach and grasp <br />

– (Stereopsis may be intact)


<strong>Ventral</strong> <strong>Stream</strong> Disorders <br />

<strong>Ventral</strong> <strong>Stream</strong> facilitates <br />

recogni9on of WHAT is <br />

being viewed


<strong>Ventral</strong> <strong>Stream</strong> Disorders <br />

• <strong>Ventral</strong> <strong>Stream</strong> facilitates recogni9on of <br />

WHAT is being viewed <br />

• Incoming informa9on goes to: <br />

– Occipital cortex for processing <br />

– <strong>Ventral</strong> stream sends the informa9on to temporal <br />

lobes which deal with visual recogni9on, <br />

orienta9on, and visual memory <br />

– If there is a match of informa9on, an object is <br />

recognized


<strong>Ventral</strong> <strong>Stream</strong> Disorders <br />

• <strong>Ventral</strong> <strong>Stream</strong> facilitates recogni9on of <br />

WHAT is being viewed <br />

• Damage can result in impaired <br />

– Recogni9on of faces, facial expressions <br />

(commonly via right ventral stream) <br />

– Ranges from not recognizing familiar people to <br />

not recognizing these people seen out of context <br />

– Incorrect iden9fica9on of strangers


A child may be or may not be able to match these cards of <br />

close friends, and may not recognize the faces if he or she can <br />

match them (Hyvarinen, 2003).


<strong>Ventral</strong> <strong>Stream</strong> Disorders <br />

• <strong>Ventral</strong> <strong>Stream</strong> facilitates recogni9on of <br />

WHAT is being viewed <br />

– Impaired recogni9on of objects (shape and form) <br />

– Difficulty with route finding-­‐geqng lost <br />

– Difficulty with visual memory tasks such as <br />

copying and learning informa9on


CVI Characteris4cs <br />

Roman-­‐Lantzy


Characteris4cs-­‐ The Big 10… <br />

1. Strong color preference <br />

2. Need for movement to illicit visual response <br />

3. Visual latency <br />

4. Visual field preferences <br />

5. Difficul9es with visual complexity <br />

6. Light-­‐gazing and non-­‐purposeful gaze <br />

7. Difficulty with distance viewing <br />

8. Absent or atypical visual reflexes <br />

• Blink <br />

• Threat <br />

9. Difficulty with visual novelty <br />

10. Absence of visually guided reach


Which Array is more Complex


CVI Difficult to Iden4fy at Times <br />

• Visual acuity can be normal <br />

• Can have CVI without other manifesta9ons of <br />

brain damage <br />

• Such children are unaware of problem since <br />

they have not experienced anything else <br />

• Commonest associated clinical sign is a <br />

convergent or divergent eye turn <br />

• Difficult to iden9fy manifesta9ons of CVI in <br />

children who cannot communicate


Ranges <br />

• CVI Range 1-­‐2: Student func9ons with minimal <br />

visual response <br />

• CVI Range 3-­‐4: Student func9ons with more <br />

consistent visual response <br />

• CVI Range 5-­‐6: Student uses vision for <br />

func9onal tasks <br />

Roman-­‐Lantzy, C. (2007). Cor$cal visual impairment: An <br />

approach to assessment and interven$on. New York: AFB <br />

Press.


Ranges <br />

• CVI Range 7-­‐8: Student demonstrates visual <br />

curiosity <br />

• CVI Range 9-­‐10: Student spontaneously uses <br />

vision for most func9onal ac9vi9es


Phases for Program Planning and <br />

Interven4on <br />

• Phase I (Range 0-­‐3): visual behavior building <br />

• Phase II (Range 4-­‐7): Integra9ng vision with <br />

func9on <br />

• Phase III (Range 8-­‐10): Resolu9on of <br />

remaining CVI characteris9cs


Collabora4ve Assessment <br />

• Jacobson and Dubon (2000) call for a <br />

mul9disciplinary assessment <br />

• ophthalmologists, optometrists, pediatric <br />

neurologists, neuroradiologists, psychologists, <br />

teachers. To this list we can add occupa9onal and <br />

physical therapists. <br />

• thorough knowledge of visual func9on, motor <br />

func9on, intellectual profile, and <br />

• knowledge of abili9es and disabili9es in verbal and <br />

performance to iden9fy most appropriate <br />

interven9on strategies.


Assessment <br />

Hyvarinen stresses the need for assessment in the <br />

following areas <br />

– Oculomotor func9ons <br />

– Quality of the image <br />

– Use of visual informa9on, percep9on <br />

– Visual memory func9ons <br />

– Comprehension <br />

• Hyvarinen, L. (2003).Lea Hyvarinen's lectures at San Francisco State 15. November 2003. hbp://www.lea-­test.fi/


Structured History Taking <br />

• Seeking evidence of <br />

– Reduced acuity <br />

– Limita9ons in visual field <br />

– Dorsal and <strong>Ventral</strong> stream problems <br />

– Confirmed with neural imaging


Interven4ons for CVI <br />

Train to improve weak areas and <br />

provide compensatory methods that <br />

build on strong func$ons <br />

Hyvarinen (2003)<br />

Hyvarinen, L. (2003). Lea Hyvarinen's lectures at San Francisco State 15. November 2003. hbp://www.lea-­‐test.fi/


Strategies Developed by Children <br />

COMPENSATORY STRATEGIES CAN BE EXHAUSTING <br />

• Coding and recognizing by color <br />

• Wayfinding by remembering a sequence of <br />

landmarks <br />

• Finding things on communica9on charts by <br />

memorizing the loca9on <br />

• Using other senses


Reading Solu4ons <br />

• Read print at most efficient size above threshold <br />

• Be certain that children have any accommoda9on <br />

problems corrected <br />

• If small eye movements (saccades) are affected, <br />

enlarge text and learn by whole word approach <br />

• If ventral stream pathology, problems iden9fying <br />

lebers/words need alterna9ve reading modes


Reading Solu4ons <br />

• If dorsal stream pathology, problems with impaired <br />

simultaneous percep9on leads to problems with <br />

crowding. <br />

– Acuity may be reduced for crowded as opposed to single <br />

lebers. Lower linear acuity at near. If single symbol or <br />

gra9ng acuity alone is tested, visual acuity can be <br />

overes9mated for some children. <br />

– To assist, mask off surrounding print: use typoscope or <br />

present words singly on a computer screen. <br />

• In some cases of alexia, braille or other non-­‐visual <br />

reading media are recommended.


Recogni4on Solu4ons <br />

• Geqng Lost <br />

– Learning route by verbal cues related to <br />

landmarks <br />

– Using color coding in the home to label loca9ons <br />

things <br />

• Recognizing People/Objects <br />

– Use alterna9ve sensory channels -­‐ voice of sound <br />

of footsteps <br />

– Recognize by touch


Main References <br />

Dubon, G.N. (2003).Cogni9ve vision, it’s disorders and <br />

differen9al diagnosis in adults and children: Knowing where <br />

and what things are, Eye. 17, 289-­‐304. <br />

Dubon, G.N.& Jacobson, L.K. (2001). Cerebral visual impairment <br />

in children. Neonatology. 6, 477-­‐485. <br />

Hyvarinen, L. (2003). Lea Hyvarinen's lectures at San Francisco <br />

State 15. November 2003. hbp://www.lea-­‐test.fi/ <br />

Jacobson, L.K., & Dubon, G.N. (2000). Periventricular <br />

leukomalacia: An important cause of visual and ocular mo9lity <br />

dysfunc9on in children. Survey of Ophthalmology. 45(1), 1-­‐13.


REFERENCES <br />

• Blind Babies Founda9on. (1998). Pediatric Diagnosis Fact Sheet: Cor9cal Visual Impairment. San <br />

Francsico: author. <br />

• Dubon, G.N. (2003). Cogni9ve vision, its disorders, and differen9al diagnosis in adults and <br />

children: knowing where and what things are. Eye, 17, 289-­‐304. <br />

• Dubon, G.N., & Jacobson, L.K. (2001) Cerebral visual impairment in children. Seminars in <br />

Neonatology. 6, 47-­‐485 <br />

• Dubon, G.N., Saaed, S., Fahad, B., Fraser, R., McDaid, G., McDade, J., Mackintosh, A., Rane, T., <br />

Spowart, K. (2004). Associa9on of binocular lower visual field impairment, impaired <br />

simultaneous percep9on, disordered visually guided mo9on, and inaccurate saccades in <br />

children with cerebral visual dysfunc9on – a retrospec9ve observa9onal study. Eye, 18, 27-­‐34. <br />

• Ek, U., Fellenius, K., Jacobson, L. (2003). Reading acquisi9on, cogni9ve and visual development, <br />

and self-­‐esteem in four children with cerebral visual impairment. Journal of Visual Impairment <br />

& Blindness. 97 (12), 741-­‐754. <br />

• Good, W.V., Jan, J.E., Burden, S.K., Skoczenski, A., Candy, R. (2001). Recent advances in cor9cal <br />

visual impairment. Developmental Medicine & Child Neurology. 43, 56-­‐60. <br />

• Harris, C.M., Kriss, A., Shawkat, F., Taylor, D., Russell-­‐Eggib, I., (1996). Delayed visual matura9on <br />

in infants: A disorder of figure-­‐ground separa9on Brain Research Bulle9n. 40, 5/6, 365-­‐369. <br />

• Hoyt, C.S. (2003). Visual func$on in the brain-­‐damaged child. Eye 17, 369-­‐384 <br />

• Hoyt, D.C, Jastrzebski, G., Marg, E. (1983). Delayed visual matura9on in infancy. Bri9sh Journal <br />

of Ophthalmology. 67, 127-­‐130.


• Hyvarinen, L. (2003). Lea Hyvarinen's lectures at San Francisco State 15. November 2003. <br />

hbp://www.lea-­‐test.fi/ <br />

• Jacobson, L.K., Dubon, G.N. Periventricular Leukomalacia: An important cause of visual and <br />

ocular mo9lity dysfunc9on in children. (2000). Survey of Ophthalmology 45(1,) 1-­‐13. <br />

• Jacobson, L., Ek, U., Fernell, E., Flodmark, O., & Broberger, U. (1996). Visual impairment in <br />

preterm children with periventricular leukomalacia – Visual, cogni9ve, and neuropaediatric <br />

characteris9cs related to cerebral imaging. Developmental Medicine and Child Neurology. 38, <br />

724-­‐735. <br />

• Morse, M. T. (2004). Another view of cor9cal visual impairment: Issues related to facial <br />

recogni9on. (unpublished manuscript) <br />

• PBS (2001). Secret life of the brain. hbp://www.pbs.org/wnet/brain/ <br />

• Rains, G.D.(2001).Principles of Human Neuropsychology. New York: McGraw-­‐Hill <br />

• S9ers, P. deCock, P., & Vandenbussche, E., (1999) Separa9ng visual percep9on and non-­‐verbal <br />

intelligence in children with early brain injury. Brain and Development 21, 397-­‐406. <br />

• Ungerleider, L.G., & Haxby, J.V., (1994). “What” and “where” in the human brain. Current <br />

Opinion in Neurobiology, 4, 157-­‐165

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