Cortical Visual Impairment and Blindness Functional Implications and Rehabilitation

Kia B. Eldred, OD, FAAO Diplomate in Low Vision Michael E. DeBakey VAMC and University of Houston College of Optometry

Course Objectives
Participants will be able to define Cortical Blindness and Visual Impairment and identify the different types of presentation of acquired vs. congenital. Participants will recognize the clinical findings and functional implications of cortical vision loss, as well as strategies to enhance vision. Learners will understand less commonly seen cortical visual changes. The importance of rehabilitation and education will be emphasized for this population of patients.

Definition of Cortical Blindness and Visual Impairment Congenital and Acquired Blindness or Visual Impairment (CVI) due to bilateral damage to the occipital cortex (Hoyt, 2003). The absence or reduction of vision in either eye while still maintaining a normal pupillary response to light and a normal ocular examination. It can occur both congenitally and it can be acquired, with many fewer cases acquired.

Vision Loss in Children in the U.S.

Cortical Visual Impairment -22% Retinopathy of Prematurity 14% Optic Nerve Hypoplasia 10% Coloboma 7% Cataracts -6% Albinism - 5% Optic Atrophy -2%

Congenital CVI Diagnosis and Evaluation

Diagnosis of exclusion and based on history information. Perinatal hypoxia/ischemia, Traumatic Brain Injury (TBI), encephalitis and meningitis. About 75% have accompanying neurological deficits in a recent study.

Common Causes of CVI in Children

Hypoxic Ischemic Encephalopathy (HIE) term infant Periventricular Leukomalacia (PVL) pre-term infant TBI shaken baby, accidental head injuries, meningitis, shunt failure, severe seizures, cardiac arrest, CNS developmental delays

Demographics of Congenital CVI

Congenital cortical visual impairment is more common in wealthier nations. Considered to constitute between 0.07% and 0.22% of the total school population in the US.

Congenital CVI Common Signs and Symptoms

Vision appears variable, even hour to hour Children with CVI may be able to use their peripheral vision more efficiently than their central vision. One third are photophobic, others are light gazers. Color vision is generally preserved. The vision of children with CVI has been described as looking through a piece of Swiss cheese. May exhibit poor depth perception, influencing their ability to reach for a target. Vision may be better when either the visual target or the child is moving.

Congenital CVI Common Signs and Symptoms

Blunted or avoidant social gaze Brief fixations, intermittent following Reduced visual acuity Visual field loss - generalized constriction, inferior altitudinal, hemianopic defect Reduced responses to visual stimuli when music, voices, and other sounds are present, and often, when the child is touched

Dorsal Visual Systems disrupted with CVI

Dorsal where is it pathway associated with posterior parietal (occipital) lobe lesions cause
Visual motor disturbances deficits in fixing direct visual attention to an object shifting fixation and gaze to new stimulus fine motor tasks such as copying and drawing Visual Spatial disturbances localization of objects judgment of direction and distance objects orienting the body to the physical world

Ventral Visual Systems disrupted with CVI

Ventral what is it aspect of vision inferior temporal lobe lesions
Discrimination Recognition Integration of visual images and objects

Team Players for Rehabilitation

Physical Medicine Rehabilitation Physician Pediatrician Neurologist Physical therapist Occupational therapist Speech therapist Occupational Therapist Teacher of the Visually Impaired Optometrist Orientation and Mobility Specialist

Congenital CVI Management and Intervention

Case Example
CS 12 year old Caucasian male

28 week premature, birth weight 1 lb, 15 oz. Central apnea, ototoxicity (Gentamicin) with hearing loss Cochlear implants at age 3 Currently in good health, wheelchair bound with head rest (cannot sustain erect head posture) Inclusion sixth grade, resource and lifeskills, OT, PT and ST at school. TVI and O and M Uses a Dynavox for communication, Smart board

Assessment
Cardiff Cards (preferential viewing) were utilized for visual acuity with contact lens correction: OD, OS 20/40 (Previous exams spanning 7 years started with VA of OD 20/130, OS 20/130 with gradual improvement) 30^ Alternating Exotropia Vertical gaze palsy with more restriction in downgaze than upgaze, horizontal eye movements are intact Full visual fields in each eye

Assessment
Normal color vision with Pease Allen preferential viewing plates Intact contrast with Hiding Heidi cards 1.25% Normal accommodative response (MEM retinoscopy) Refractive error : Right eye: +1.75 1.25 x 170 Left eye: +0.75 -0.50 x 170 Ocular health evaluation demonstrated intact structures internal and external

Plan
CS Qualifies for vision services Position objects at midline horizontally due to the vertical gaze palsy. Color coding may be used for education. Not necessary to use high contrast materials for education. Continuation of contact lenses is recommended. Lenses ordered after Medicaid approval will be as follows: Acuvue Oasys Right eye: +1.75 -1.25 x 170, 8.6, 14.4, Left eye: +0.50 -0.75 x 170, 8.6, 14.4. Materials for near should be at least inch at 16 inch working distance. Return in one year for annual evaluation.

Causes of Adult Cortical Visual Impairment

Cerebrovascular Accidents (CVA) 700,000 per year in the US
The largest group of visual disorders after acquired brain injury are homonymous hemianopsia (HVFD). Approximately 20-30% of all patients with CVA requiring treatment in a rehabilitation center have HVFD.

Traumatic Brain Injury - 85,000 people suffer long term

disabilities In the U.S., more than 5.3 million people live with disabilities caused by TBI

It should be noted that 40% of HVFD recover after stroke, especially within 2 months of the stroke

Acquired CVI Diagnosis and Evaluation

MRI/CAT scan would indicate location of damage which would lead to diagnosis of CVI Varied levels of improvement of visual field and visual acuity after sometimes total loss of vision with CVA or TBI

Team Players for Adult Rehabilitation

Physical Medicine Rehabilitation Physician Neurologist Internal medicine physician Physical therapist Occupational therapist Speech therapist OT/CLVT Optometrist Orientation and Mobility Specialist

Acquired CVI Common Symptoms

Most common vision loss is hemianopsia Less common is bilateral occipital loss with near or total blindness Usually no strabismus or other ocular disorder Anton Syndrome the patient may deny the vision loss entirely, may be aware of color Patients may be able to navigate the environment despite serious vision loss Blindsight

Blindsight
Existence of 2 extrastriate pathways for objects and spatial vision
Ventral stream projecting to the inferior temporal lobe the what pathway Dorsal stream projecting to the parietal lobe the where pathway

Recent studies of patients with lesions in V1 provide some evidence for existence of connections (remaining perception of motion) or the Riddoch phenomenon Cat study demonstrated transient deficit with recovery in a matter of days, probable relocation to similar structures.
Rushmore RJ, Bertram P, Valero-Cabre A Recovery of function following unilateral damage to visuospatial cortex Exp Brain Res (2010) 203: 693-700

Rehabilitation of Blind Areas

Jobke S, Kasten E, Sabel B Vision Restoration Through Extrastriate Stimulation in Patients with Visual Field Defects: A Double-Blind and Randomized Experimental Study Neurorehabilitation and Neural Repair 2009;23:246255 Study with 21 subjects with lesions older than 1 year in trial Crossover study design Standard Vision Restoration Therapy (VRT) (using single point visual stimulation in areas of residual vision) and Extrastriate VRT (visual stimulus activating extrastriate pathways) were utilized Extrastiate VRT utilized a massive moving spiral to address motion perception throughout the entire defective visual field (absolute blind region only)

Rehabilitation of Blind Areas

Evaluation tools High Resolution Perimetry Conventional Perimetry NEI VFQ Trained hour daily with large spiral stimulus for 90 days Control group trained with standard VRT for the same time Improvement in detection performance of 4.2% compared to standard VRT, improvement also found deep in the blind area Reasons? Magnocellular pathway direct connection from lateral geniculate nucleus to V5 in humans is under investigation Possible evidence for direct thalamic functional pathway to extrastiate visual cortical motion in humans which bypasses primary visual cortex (Schoenfeld et al 2002)

Acquired CVI Case 1

LJ 61 year old AAM +HTN, +DM Admitted 10/8/09 for progressively worsening HA Ischemic stroke with conversion to hemorrhagic stroke
Angiogram demonstrated complete occlusion of left vertebral artery Bilateral hemianopsia is reported

Acquired CVI Case 1

OT/CLVT notes 10/29/09
Patient reports peripheral vision is fuzzy like an unfocused TV screen. Denies difficulty with central vision or with locating objects in hospital room. Could read short paragraph without errors Denied difficulties with mobility or participation in therapy due to vision Recommended low vision evaluation, patient was told by physician he could not drive

Acquired CVI Case 1

Exam with OD 01/25/10
Reports vision has improved since the stroke. It is still blurry. Previously couldnt identify information in front of him, now peripheral vision is better as well. He does have difficulty with steps, particularly stepping up. Glare outdoors. Goals: Driving, reading with less work

Acquired CVI Case 1

Distance VA sc
20/20 each eye

Near VA sc
OD 20/40, OS 20/30 Mild compound hyperopic refractive error, presbyopia Eyes are aligned with cover test Meibomian gland disease

Acquired CVI Case 1

Visual fields assessed with gross confrontations and Humphrey visual fields with only the inferior left quadrant appearing intact in each eye or
Right homonymous hemianopsia with superior left quadrantopsia

Plan
New spectacles Re-evaluate visual fields on follow-up, Dynavision, Reading evaluation No driving at this time. Warm compresses, Refresh tears

Acquired CVI Case 1

OD 3/22/10 LJ reports vision still isnt the same in periphery as prior to the stroke, but it is much better. Warm compresses and drops are helping, glasses are working well. Central distance vision is stable Visual fields on HVF Kinetic and Estermann
Fairly intact far peripheral visual fields with constriction in paracentral area in each eye

Acquired CVI Case 1

OT/CLVT 3/22/10 Pepper reading test
Corrected reading rate (WPM) 52.9 Contextual reading rate (WPM) 84.65

Dynavision
135 total hits, 1.77 average reaction time per hit Upper left 1.81 Upper right 2.24 Lower left 1.50 Lower right 1.35

Less than 175 hits, questionable safety with driving, cautioned no driving at this time. Return in 3 months for reassess, hasnt returned

Dynavision

Acquired CVI Case 2

RA 78 year old CM Diagnosed with optic atrophy secondary to past CVA, strong history of carotid disease with history of right carotid endarterectomy, also history of error in medication with over dose of hypertensive medication O&M evaluation Lives in a high rise, difficulty with curbs, if he moves reports when he moves he can make out objects in the environment, sees colors well. Cane training was started at the next visit.

Acquired CVI Case 2

Evaluated by OT/CLVT reports difficulty writing, mobility, using microwave Dynavision completed with great difficulty with most difficulty in upper left and lower right quadrant
Worked with OT/CLVT on caning training and required moderate verbal cues in order to scan in systematic fashion Lighting eval completed to assist with lighting in home

Acquired CVI Case 2

Evaluated by OD
Reports keyhole of vision in the right eye with good vision in that area, his vision in the left eye has improved from nothing to light and dim objects. He can read words and letters, but they run together. Eyes water and hurt. Goals improve reading ability and find information at longer distance. Distance VA OD 20/25+2, OS 3/350 (NLP 2 months prior in eye clinic). Refraction compound hyperopic astigmatism Near vision Right eye .3/.4M Contrast Peli Robson 1.2

Acquired CVI Case 2

Low Vision Devices
Appreciated demonstration of -5.00 minifier for near to increase visual field CCTV appreciated for increase reading with reversed contrast and stage movement 2.8x Hand-held telescope used as reverse system with VA 20/40 Superficial punctate staining of cornea

Acquired CVI Case 2

2.8x Telescope, -5.00 Minifier, CCTV were ordered dispensed and trained Next visit BITA lens was prescribed later dispensed with success. Veteran met all vision rehabilitation goals.

Rare Disorders with Cortical Visual impairment

Balints syndrome paralysis of gaze with haphazard scanning. Damage to the posterior parietal cortex. Damage to the posterior superior watershed areas or parietal-occipital vascular border zone or temporaloccipital damage.
Optic Ataxia incoordination of hand and eye movement Oculomotor Apraxia -the inability to voluntarily guide eye movements/ change to a new location of visual fixation Simultanagnosia - the inability to perceive more than one object at a time, even when in the same place

Treatment includes compensatory strategies

Rare Disorders with Cortical Visual Impairment

Blindsight
Type 1 patient has no awareness of any stimuli, but can report accurately location or movement of an object.
Type 2 when subjects have some awareness of movement within the blind field, but not able to report what the object is.

Hemispatial Neglect also called hemineglect, spatial neglect or neglect syndrome

Damage to the parieto -occipital area of typically right side of brain Deficit in attention to and awareness of one side of space

Alien hand syndrome the hand has a mind of its own

Caused by lesion of corpus callosum, could be secondary to surgery for epilepsy

Tips for work with CVI

Control visual input to avoid over stimulation Eliminate extraneous noise or visual distractions from environment Present one item at a time Touch should be used to cue Use language to label objects and describe objects Movement may assist child or adult to find objects (i.e. allow them to move or rock, bring food into mouth of child in an arc when feeding)

References
Bouwmeester L, Heutink J, Cees,L The effect of visual training for patients with visual field defects due to brain damage: a systematic review Zhang X, Kedar S, Lynn MJ,Newman NJ, Biousse V Natural history of homonymous hemianopsia Neurology 2006;66:901-905 Rushmore RJ, Payne B, Valero-Cabre A Recovery of function following unilateral damage to visuoparietal cortex Experimental Brain Research (2010) 203:693-700 www.Current Perspectives by Luisa Mayer, PhD Boyle N, Jone DH, Hamilton R, Spowart K, Dutton GN Blindsight in Children does it Exist and can it be used to help the child? Observations on a case series Developmental Medicine and Child Neurology 2005, 47:699-702 Jobke S, Kasten E, Sabel B Vision Restoration Through Extrastriate Stimulation in Patients with Visual Field Defects: A Double-Blind and Randomized Experimental Study Neurorehabilitation and Neural Repair 2009;23:246- 255 Schoenfeld MA, Heinze HJ, Wodorff MG. Unmasking motion-processing activity in human brain area V5/MT + mediated by pathways that bypass primary visual cortex. Neuroimage.2002;17:769-779 Schoenfeld MA, Noesselt T, Poggel D, et al. Analysis of pathways mediating preserved vision after striate cortex lesions. Ann Neurol. 2002;52:814-824.

Thanks!
Kia B. Eldred, OD, FAAO Diplomate in Low Vision kia.eldred@va.gov