Cortical/Cerebral Visual Impairment What is it? · Lueck, A (2010) Cortical or Cerebral Visual...
Transcript of Cortical/Cerebral Visual Impairment What is it? · Lueck, A (2010) Cortical or Cerebral Visual...
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Cortical/Cerebral Visual Impairment What is it?
Perkins/MCB CVI Symposium May 28-20 2015
Barry S Kran, OD, FAAO Darick W Wright, MA, COMS, CLVT
Acknowledgement: D Luisa Mayer, PhD. MEd
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CVI Overview
Anatomy Terminology Case Example Current Issues
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Ocular Vision Impairment
Eyes, retina, optic nerves – Significant uncorrected refractive
error – Media opacities (cataracts) – Retinal lesions (colobomas) – Retinal degeneration/dystrophy – Optic nerve damage
Chiasm
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Ocular Vision Impairment
Additional examples: – Retina
• Retinopathy of prematurity* • Achromatopsia • Leber congenital amaurosis
– Albinism (macular hypoplasia and reduced ON fibers crossing at chiasm)
– Optic nerve hypoplasia*
*Brain related visual difficulties may co-occur
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Primary Visual Pathway
Ocular structures to Optic Chiasm
Optic chiasm to primary visual cortex
http://www.nature.com/nrn/journal/v6/n3/images/nrn1630-f4.jpg Accessed 11 July 2010
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The Cerebrum • Comprised of
– 4 lobes – Cerebellum
• Facilitates complex behaviors • Last portion to evolve in human brain
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Frontal Lobes
• Attention • Judgment • Personality • Emotion • Memory
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Parietal Lobe
• Sensory processing
• Sensation • Spatial
information
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Occipital Lobe
• Visual Processing • Visual Perception
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Temporal Lobe
• hearing, speech, memory “library”
• right side – facial recognition
• left side – shapes/object recognition, reading
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Cerebellum
• “small brain” • Posture • Balance • Coordination
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Brain Stem
• responsible for basic life functions: – Breathing – heart rate – body temperature
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Dorsal & Ventral “pathways”
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Ventral Stream
Occipital Lobe Temporal Lobe
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Ventral Stream – “What is it?” Recognition of objects
Occipital lobes – Receive visual input (primary visual pathway)
Temporal lobes – input from occipital lobes – Visual “library” – Words, numbers, shapes, landmarks – Faces – Color
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Apple!
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????
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Dorsal Stream
Occipital Lobe
Posterior Parietal
Motor Cortex
Frontal Cortex
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Dorsal Stream -“Where is it?” Vision for action - visual attention,
visually guided movement • Occipital - posterior parietal lobes
– Integration of sensory input with attention and during motor output, management of visual complexity
• Feedback from frontal cortices – Motor planning, head/eye movement, visual
guidance of movement
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I want it
Attend, Attend Fixate with
Eyes
Reach
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?????
Too Much Information!
!!!!!!!! Where do I look???
How do I move?
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Apple! I want it
It’s in front of me Reach with right hand
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?????
Too Much Information!
!!!!!!!! Where do I look???
How do I move?
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Injury at Pre-term or Full-term (infants)
vs
Traumatic Brain Injury or Acquired damage (disease)
Brain Damage
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History of CVI
1900’s WW I Veterans – posterior parietal lobe damage (visual field loss, decreased object avoidance, poor visually guided movement, poor eye movement, simultagnosia) Rudolph Balint & Holms
Early 1980’s
Cortical Blindness (occipital lobe damage)
Late 1980’s
Cortical Visual Impairment (post-chiasm to occipital lobe = reduced visual acuity)
* Does not include post-occipital lobe damage
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History of CVI
1990’s Increased knowledge of post-occipital lobe vision loss (Visual field loss, ocular motor, visual perception)
2000’s
Cerebral Visual Impairment
Increase in premature births (worldwide) Survival rate (2010) 14:15 million
Increased prevalence of CVI (developed countries)
10 - 22: 10,000 under age 16 Increased professional awareness
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Terminology
Visual Impairment – “visual impairment, including blindness,
means an impairment in vision that, even with correction, adversely affects a child’s educational performance. The term includes for partial sight and blindness”
(IDEA, 2004 P.L. 108-446)
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Terminology
Cortical Visual Impairment– “Visual impairment related to the cortical
area of the brain and/or optic radiations”
Roman, et al (2010) JVIB
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Cortical Visual Impairment
Characteristics – Light gazing or withdrawal – Better visual attention for:
• Moving vs. static objects • Familiar vs. novel objects • Simple vs. complex environments
– Difficulty integrating gaze with reach – Difficulty integrating looking with listening – Poor social gaze – Delayed visual (& other) responses
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Terminology
Cerebral Visual Impairment – “Visual malfunction due to retro-chiasmal
visual and visual association pathway pathology”
Philip & Dutton (2014) Clinical and Experimental Optometry
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Cerebral Visual Impairment
Characteristics – Post optic chiasm brain damage – Complex brain processing difficulties – Dorsal/ventral stream dysfunctions – May not be “legally blind”
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Classification of Vision Loss • Ocular
– Eye structures, to chiasm • Ocular motor
– Brain stem, basal ganglia, thalamus, cerebellum
• Cortical – Primary pathway (post-chiasm to occipital)
• Cerebral – Post-chiasm, complex brain processing areas
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Cerebral Visual Impairment Continuum
Profound Cortical Visual Impairment Minimal Functional Vision
Visual Impairment “Higher Order Visual
Processing Dysfunction”
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Patient M. Cerebral + Ocular & OM VI
– Premature birth (28 weeks gestation)
– Age 2 months: oxygen deprivation • Changes in
occipital cortex – Mild spastic diplegia
– Learning disabilities
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Ocular Hx
• Cerebral Vision Impairment (Dx @ 8 months)
• Nystagmus • Strabismus surgery for esotropia ~age 2 • Optic nerve pallor • Glasses for hyperopic astigmatism
Patient M. Cerebral + Ocular & OM VI
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Patient M. Cerebral + Ocular & OM VI
Bilateral inferior field defect
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Ocular Findings Distance Visual Acuity (isolated line)
Right eye Left eye Both eyes
20/80+2 20/150 20/60
OU 1.0M @ 40cm (isolated line)
5.0M @ 25cm (whole chart)
Near Visual Acuity
Patient M. Cerebral + Ocular & OM VI
AGE 19
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Patient M. Observations • Visual scanning? • Integration of visual & add sensory input? • Vision for action?
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Patient M. Impressions How to best characterize M’s visually
guided behaviors?
- Cerebral Visual Impairment (Dorsal) – Impaired vision for action – Impaired attention – Impaired visually guided movement – Impaired vision for complex visual scenes (crowding)
– Ocular & OM Visual Impairment • Visual acuity deficit + strabismus do not account
for behaviors • Eye turn & nystagmus does not account for level of
functional vision
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Patient M. Recommendations • Collaborative approach
– TVI & AT • Modifications for learning
–Enlarged print –Electronic technology
– O&M • integrate tactile/auditory information for
travel (multi-sensory approach) • Sequential instruction (repetition) • Contextual timing of tasks
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Patient L: Cerebral & Ocular VI
Medical Hx: –Prematurity (26 wks, 750 g) – Bilateral hemorrhages –Hypotonia of trunk & extremities
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Ocular Hx – Retinopathy of Prematurity (RE worse)
– Very high myopia & anisometropia (RE worse)
– RE amblyopia • Refractive and strabismic
Patient L: Cerebral & Ocular VI
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Presenting concerns Age: 18.5 years
• How much functional vision and how she uses it. • Proficiency for driving? • Symptoms:
– Difficulty walking with changes in terrain/steps – Slow reaction time – Eyes ache and tire easily with demanding near tasks
• Eye Dr: VA & VF adequate for school work and driving
• Prior TVI/O&M evals: Services not needed
Patient L: Cerebral & Ocular VI
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• Distance acuity with glasses – Both eyes viewing
Patient L: Cerebral & Ocular VI
FULL CHART ISOLATED LETTERS
20/60 20/40 -2 • Behaviors • Fatigued
• Head/body posture and tone • Color • Voice
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• Constricted Visual Fields – (nasal field more than temporal field)
Left Eye Right Eye
Patient L: Cerebral & Ocular VI
Normal
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Patient L Cerebral VI vs. Ocular VI Neuropsych eval
– Normal IQ – Processing speed delays and anxiety
• Driving evaluation – Visual cognitive assessment in moving vehicle
• Unable to manage & figure out what to do in complex situation (car tire blowout)
– In a driving simulator had great difficulty planning and successfully implementing a lane change
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Patient L Cerebral VI vs. Ocular VI • Driving evaluation
– “She does not currently have the life skills necessary to cross a busy street, manage herself independently at home or in the community.”
• Based on this data and observations – Mother & daughter each completed a Dutton
Inventory – MRI results requested
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April 2010
• Mild parietal – occipital area volume loss • Asymmetrical ventricles
Patient L: Cerebral & Ocular VI
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What other reported observations raised suspicions that there may be an additional basis for her visual impairment?
– Observation of use of vision during acuity testing
– Comments in recent OT driving evaluation
Patient L: Cerebral & Ocular VI
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DL Mayer 2.28.10
Ocular Ocular media, retina, optic nerve, to chiasm
Cerebral post-chiasm
Ocular Motor Brain stem, cerebellum
CLASSIFICATION OF VISUAL IMPAIRMENT
BY CAUSE
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• Decreased visual acuity • Constricted visual fields • Not “legally blind” • Patient and mother report functional
difficulties • Processing delays and anxiety • Does not possess safe travel skills • MRI shows damage (occipital, parietal,
ventricles)
Patient L: Summary
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• Ocular VI is NOT the primary cause of L.’s visual function deficits
• Ed. team and eye doc. DID NOT identify signs consistent with Cerebral VI
• MRI + exam observations + Dutton Inventory support Dx of Cerebral VI (dorsal + ventral)
Patient L: Conclusions
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Collaborative Approach • Stat eval by TVI and O&M specialist
• FVA, LMA, O&M, AT
• Coordinate transition to community college or to the community in general
Patient L Recommendations
*
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Where Are We Today? 1. Cerebral VI is a continuum
2. Cerebral VI can co-exist with Ocular dysfunction
3. Cerebral VI is #1 cause of pediatric vision loss in developed countries
4. No universally accepted term(s)
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Where Are We Today? 5. Identification/diagnosis of profoundly impaired children continues to be challenging
6. Methodology of assessing vision function can dramatically impact result
7. The physical environment directly impacts functional use of vision
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Where Are We Today?
8. Entitlement to vision-related services is contingent on visual acuity/visual field (legal blindness)
9. Education & Rehabilitation instructional strategies have yet to be fully developed and scientifically proven.
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Where Are We Today? 10. Additional training is needed by all team members!
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Resources and Images
Wikipedia - http://en.wikipedia.org/wiki/Brain Brain Injury Association - http://www.biasd.com/en_brain_map.html About Brain Injury www.waiting.com/brainanatomy.html#anchor2884157 Lueck, A (2010) Cortical or Cerebral Visual Impairment in Children: A Brief Overview. JVIB, AFB press. Dutton GN, Bax M, editors. Clinics in developmental medicine no. 186: visual impairment in children due to damage to the brain. London: Mac Keith Press; 2010
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Resources and Images
Hoyt CS. Visual function in the brain-damaged child. Eye. 2003;17:369–84. Dennison E, Hall, Lueck A eds. Proceedings Summit on Cerebral/Cortical Visual Impairment April 30, 2005 2006 AFB Press NY, NY Roman-Lantzy C.,Cortical Visual Impairment: An approach to assessment and intervention 2007 AFB Press NY, NY Philip S. & Dutton G.N. (2014) Identifying and characterising cerebral visual impairment in children: a review. Clinical and Experimental Optometry (97.3) Lueck, A. & Dutton G.N, (eds) (2015) Vision and the Brain: Understanding cerebral visual impairment in children. AFB Press:NYC
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Cortical/Cerebral Visual Impairment What is it?