Cerebral Palsy: The Optometrists Role

Ashley Schuelke, OD | Southern College of Optometry

Background
Cerebral Palsy (CP) designates a large group of non-progressive sensory and motor impairment disorders that are the result of brain lesions occurring early in development.1 The condition can be classified into three main physiologic subtypes: spastic, dyskinetic (or athetoid), and ataxic.2 Each type is thought to affect different brain areas leading to the varied manifestations. Spastic CP encompasses the majority of cases (70-80%), which results in stiffness of muscles reflecting damage specifically to the brains periventricular white matter.2,3 Affecting 10 to 15% of CP patients, dyskinetic or athetoid CP is associated with damage to the basal ganglia, causing uncontrolled, slow, writhing movement.2,3 Ataxic CP is characterized by difficulty with balance and coordination due to cerebellar damage, affecting less than 5% of people with CP.2,3 Cerebral Palsy can also be designated as one of three anatomical subtypes: hemiplegia, diplegia and quadriplegia.4 Hemiplegia affects 20-30% of patients resulting in dysfunction in the right or left side of the body.3 Diplegia refers to reduced abilities in the lower or upper limbs (30-40%) with the lower extremities being affected with much greater frequency. The most severe anatomical dysfunction is quadriplegia, which affects all four limbs and trunk (10-15%). Spastic hemiplegia is the overall most common manifestation.3,5 Beyond the main physiologic and anatomical subtypes, researchers have further classified cerebral palsy patients based on gross motor and fine motor abilities. The Gross Motor Function Classification System (GMFCS) has proved to be reliable and repeatable as a diagnostic tool in assessing gross motor ability.1,4,6-8 More recently, the Bimanual Fine Motor Function classification system (BFMF) was developed to correspond with the five levels of the GMFCS.6,7 The exact criterion of these two functional scales can be seen in Table 3 and 4.6 A strong correlation between the two systems has been repeatedly demonstrated by multiple international researchers.6,7 A 2002 population-based study performed in Ontario, Canada compared eight functional health status domains to the Gross Motor Function Classification System (GMFCS). The eight areas of measure included: mobility, dexterity, speech, vision, hearing, cognition, emotion and pain.9,8 A statistically significant association was found between the GMFCS levels and the functional limitations of mobility, dexterity, speech and vision.9 Low correlations were found with hearing and cognition, while neither emotion nor pain were associated with degree of functional limitation as described by GMFCS. A 2007 National Institutes of Health (NIH) cross-sectional designed study found a similar relationship that the probability of debilitating visual deficits was greater in children with higher GMFCS scores.1,2,4 A 2001 article from the European Journal of Paediatric Neurology regarding visual disorders in children with brain lesions states: Given the high prevalence of visual abnormalities in children with most types of cerebral palsy and the very important role of vision for all areas of development, we consider that a comprehensive and early visual assessment is mandatory in any child suspected of having a motor disorder of central origin.10 As the most common cause of motor disability in children, occurring in ~3 in 1000 live births, it is important that optometrists are aware of the visual impact of Cerebral Palsy.6,8 With the use of therapeutic lenses and visual rehabilitation therapy, optometrists should be a critical member of the CP health care team. Multiple investigative studies have demonstrated that the cerebral palsied population is more likely to have deficient visual skills than the general population. Visual anomalies common to these patients include: strabismus, amblyopia, visual field defects, saccadic and pursuit dysfunction, accommodative insufficiency and reduced visual perceptual abilities. Regardless of intelligence, the visual deficits alone can lead to reduced reading success. As optometrists, we play a critical role in the day to day functional abilities of these patients.

Exam Elements
Visual Acuity
Assessing visual acuity in children and adults with cerebral palsy (CP) can be challenging, especially those patients at higher levels on the Gross Motor Function Classification Scale (GMFCS).8 Reduced mental capacity, speech impairments, and reduced gaze control may impede accurate testing. In 2009, Ghasia et al., examined 76 children at the St. Louis Childrens Hospital to determine the probability of obtaining quantitative visual acuities in CP children with different levels of motor dysfunction. Compared to age-matched norms, the CP group averaged two lines worse in Snellen-equivalent acuities. Of the CP children tested, logMAR acuities were obtainable in 88% with either spatial-sweep visually evoked potentials (SSVEPs) or optotypes. A correlation was found between reduced levels of visual acuity and increased deficits on the GMFCS, but logMAR acuities were still obtainable in 56% of children with the most severe disease.1,8 As optotypes require a verbal or motor response, SSVEPs circumvent this problem.8 The use of the sweep-VEP for the measurement of visual acuity in these patients allows for more precise and reliable data collection and should be utilized if readily available to the optometric practitioner.1

Treatment
Bifocal / Prism Prescription
As accommodative deficiency has a higher prevalence among the cerebral palsy population it is important to address these limitations in the management plan.2,12,18,19 The use of a bifocal is often necessary, but motor abilities must be taken into consideration. Two prescriptions may be needed for distance and near if oculomotor abilities are limiting proper use of a bifocal. As progress is made in vision therapy, the patient can graduate into using one pair of glasses instead of two. A case report from a 2000 article in Developmental Medicine and Child Neurology describes the successful use of a no-line bifocal for a 10-year-old patient with dyskinetic CP.18 Intact, accurate control of vertical eye movements would be necessary for this to be considered and would have limited use in most cases.

Case
Subjective
Patient: Age: Race: Gender: Chief Complaint: GH Seven years White Female One eye (usually OD) turns out constantly. Patient complains that eyes are sore. Has seen other optometrists and ophthalmologists with no improvements and wants another opinion. Not sure when the turn started due to custody change. Glasses have not improved turn. (+) CP uses walker or wheelchair. Will not walk with assistance (holding hand, etc.) from person. Cerebral infarct was localized to the parietal-occipital brain junction. (+) Asthma (+) Hx of Pneumonia with Hospitalizations (+) Joint Pain Twin 2nd grade, reads above grade level, poor handwriting Baclofen Wearing most recent SpecRx full-time x 2 mos. Negative Negative

Vision Therapy Activities

From the Journal of the American Optometric Association (Jan 1987)19 by Robert H. Duckman

PMHx:

OCULAR MOTOR ACTIVITIES Tube Rotations

a. Child in supine position with room totally darkened b. Occlude one eye and hold a paper towel tube to the other eye c. At the other end of the tube, present a flashlight and move slowly d. Rotate the tube to keep up with the flashlight, slowly pulling it away from the eye as skills improve e. Increase room illumination and increase difficulty of flashlight pattern with practice

Stereo
Stereopsis assessment may result in similar limitations when a verbal or motor response is required. The use of an optokinetic nystagmus drum can be a helpful tool in evaluating whether gross stereopsis is present or not.11 The drum should be used to measure nasal to temporal and temporal to nasal tracking abilities in each eye. It has been postulated that symmetry between these measurements is indicative of a gross level of stereoscopic vision.

Accommodation
In a 2008 article in Developmental Medicine & Child Neurology, researchers evaluated the near pupillary response in comparison to dynamic retinoscopy as a measure of accommodative ability.12 Pupil response was classified as normal, reduced or absent by subjective observation. They discovered that patients with reduced or absent pupillary responses demonstrated significantly lower accommodative ability as measured with dynamic retinoscopy. Although not a replacement for more formal accommodative testing, the near pupil response can provide another insight into the functional level of CP patients. McClelland et al., examined 90 children with CP and found that 57.6% demonstrated an accommodative lag outside of normal limits at one or more distances.2 The greatest accommodative deficits were associated with more severe motor and intellectual impairments. They also found that the subjects with dyskinetic (a.k.a. ataxic) CP had significantly more reduction than the spastic CP subtype. It is essential that a thorough accommodative evaluation is performed on all cerebral palsy patients to best determine the appropriate lens management.

Mirror Rotations
a. Can be done sitting or supine b. Move a small mirror in front of the childs eye, they are to use their own eye as a target c. Feedback should be given when not following accurately

Objective

BirthHx: School Hx: Medications: POHx: FMHx: FOHx:

Spelling Flashlight
a. Therapist and child in a darkened room facing a blank wall b. The therapist slowly writes a letter on the wall using a flashlight c. The patient is asked to say what symbol was printed on the wall d. Difficulty can be increased by using more letters, shapes or whole words

Flashlight/Chalkboard Racetrack
a. Draw a racetrack on a chalkboard to the childs level of difficulty b. The therapist can hold the flashlight as the child directs them around the track and tells them when mistakes are made c. The child can do the driving themselves if able

Perceptual Abilities
European researchers examined 70 children, 36 of which had CP.13 The L94 visual perceptual battery was performed and compared to the performance age obtained on non-verbal intelligence subtests. Five computer tests were performed: visual matching (VISM), overlapping line drawings (OVERL), line drawings occluded by noise (NOISE), a De Vos task (DE VOS) and unconventional object views (VIEW). The specific task characteristics can be found in Table 5. Of the areas tested, the visual matching task was found to be the easiest, while the De Vos task was the most difficult. Of the patients found to have a perceptual visual impairment, 62% had cerebral palsy. Similar perceptual deficits have been repeatedly found in other research studies.14,15

Flashlight Tag
a. When the child can manipulate a flashlight, they follow the therapists light slowly around the room b. Goal is to keep the flashlights together at all times

Table 1: Physiologic Classifications of Cerebral Palsy

Subtypes Spastic 70-80% Dyskinetic/Athetoid 10-15% Ataxic <5% Mixed Characteristics Stiffness of muscles; damage to periventricular white matter Uncontrolled, slow writhing movement; damage to basal ganglia Difficulty with balance and coordination; damage to cerebellum Blend of two or more of the above forms

Finding Objects
a. In sitting position, hold the childs head and ask them to locate various objects in the room by using their eyes only

Table 5: L94 Visual Perceptual Battery Subtests

Task Subtype Visual Matching (VISM) Overlapping Line Drawings (OVERL) Line Drawings with Noise (NOISE) De Vos Task (DE VOS) Unconventional Object View (VIEW) Characteristics Ten items with one target white line drawing on a black background are presented for one sec. Four alternatives are then shown, of which only one matches. Six drawings in which two, thre, or four line drawings are presented on top of each other. Target drawing is presented for six sec then followed by a matching screen containing the overlapping drawings and two distracter drawings. Child is to identify all the overlapping drawings by naming or pointing. Six items in which the target drawing is occluded by a random grid of small squares. The target drawing is shown for two sec. Each item starts with 60% occlusion. Occlusion is reduced until the child is able to recognize the target drawing. Forty-three items, which the child has to identify by naming. Objects or presented while embedded in context, partly deleted, only contour-line drawn, typical part left out, or from a different orientation. 20 items in which the target drawing is seen from an unconventional viewpoint. Each target drawing is presented for 3 sec after which the drawing can be presented in less unconventional circumstances.

Blackboard Saccadics
a. Draw symbols in the corners of the blackboard b. The therapist must call the name of the symbol and the patient find it c. Can number the symbols and place a random list of numbers in the center of the board, the patient must then read the number and find the corresponding symbol

Entering VAs: OD +4.50-1.00x180 Dist 20/30 Near 20/30 OS +4.50-1.00x180 Dist 20/30 Near 20/30 Pupils: PERRL(-)APD EOMs: FROM OU CVF: FTFC OD, OS CT: Dist 35 CAXT c OS fixation preference Near 35 CAXT c OS fixation preference BI Assessment: Put 10 BI loose prism in front of one eye and began to see the eyes move out of the exotropic posture with moments of alignment. Removed the glasses to apply Fresnel press-on prism and the eyes were completely straight, but with reduced VA. Uncorrected VAs: OD 20/60 Dist 20/50 Near ***While taking near VAs, RET emerged OS 20/60 Dist 20/50 Near Next Question: What lens power would allow for more binocular stability while maintaining a functional VA? Trial Framing: OD +2.50 D 20/30 N 20/30 OS +2.50 D 20/30 N 20/30 Maintained VAs and reduced frequency and degree of XT. Yoked Prism Evaluation: Trialed 15 yoked BU/BD/BR/BL with Marsden ball tracking and bean bag toss Subjective and objective improvements noted with yoked BU Final Rx: OD +2.50 1 BU OS +2.50 1 BU

http://emedicine.medscape.com/article/310740-overview3

Table 2: Anatomical Classifications of Cerebral Palsy

Subtypes Hemiplegia 20-30% Diplegia 30-40% Quadriplegia 10-15% Characteristics Affecting RIGHT or LEFT side of body Affecting UPPER or LOWER extremities (usually lower) All four limbs and trunk are affected

Grid Activities
a. Arrange pictures/words/symbols in a 2x2 matrix on the chalkboard b. Code each row with a number (or color) and each column with a letter (or shape) c. The therapist should name one of the pictures and the child indicates the row and column d. Increase difficulty with more cells in the matrix

Assessment Plan

1. IAXT 2. Oculomotor Dysfunction 1-2. New SpecRx given, sent home c push/pull activities, tracking skills and ball play. Start office based VT with goals to: a. Increase the amount of time per day that her eyes are straight and comfortable b. Improve ocular motilities c. Gain better control of accommodation d. Improve eye-hand coordination and visual motor integration

ACCOMMODATION Monocular Push Out

a. Slowly move a target from the eye outward b. The patient indicates when it is clear and then the target removed c. The patient must then tell what it was d. Record the distance, trying to decrease it with successive attempts

http://emedicine.medscape.com/article/310740-overview3

Table 3: Gross Motor Function Classification System (GMFCS)

Level 1 2 3 4 5 Functional Capability/Limitation Walks without assistance; limited advanced motor skills Walks without assistance; limited advanced walking Walks with assistance walking device Self-mobility with a transporter Self-mobility with a transporter; severely limited

VT Activities

Monocular Minus Lens Rock

a. Introduce a minus lens monocularly while the child looks at a target at 40 cm b. Have the child signal when the target is clear c. Then remove the lens and the target and the patient must say what it was d. Continue increasing the power & push through plateaus

Marble Roll Marsden Ball WSF Yoked Prism Walk

Look Hard/Look Soft Flashlight Tag/Racetrack MIT Alternate Flash Dotting Os

Pegboard Rotator Luster Activities R/G Coloring Book R/G Playing Cards

Bubble Popping Eye Control Wall Saccades Brock String

Oculomotor
One-hundred and five CP children with IQ levels between 70 and 100 were evaluated in Bulgaria using the Developmental Eye Movement (DEM) Test.16 Only 19% were found to have normal function, 20.9% exhibited purely oculomotor dysfunction, 32.4% had a perceptual problem, and 27.7% had a combined oculomotor and perceptual problem. According to the researchers, Eye tracking skills are very important in reading. A harmonic pattern of eye movement is needed for reading to be effective. As such, it is appropriate to include the DEM or NSUCO test as part of the visual evaluation of the cerebral palsied patient.

Near-Far Hart Chart

Current Status
Mom reports that eye is out only 10% of time at home, usually when sick or tired. During activities, fused 100% of time. Walking with assistance for the 1st time!

PERCEPTUAL MOTOR ACTIVITIES Touch & Go

a. Therapist touches a part of the body and asks the child to move only the part touched a specified amount b. In order of difficulty: homologous (both arms or both legs), monolateral (one arm or one leg), ipsilateral (arm and leg on same side), contralateral (arm and leg on opposite sides) c. Therapist may move the body part for the CP patient if they are unable to do so in order to reinforce body awareness

Table 4: Bimanual Fine Motor Function (BFMF)

Level 1 2 3 4 5 Functional Capability/Limitation One hand: manipulates without restrictions Other hand: manipulates with restrictions or limitations in more advanced fine motor skills. a. One hand: manipulates without restrictions; Other hand: only ability to grasp or hold. b. Both hands: limitations in more advanced fine motor skills. a. One hand: manipulates without restrictions; Other hand: no functional ability. b. One hand: limitations in more advanced fine motor skills; Other hand: only ability to grasp or worse. a. Both hands: only ability to grasp. b. One hand: only ability to hold; Other hand: only ability to hold or worse. Both hands: only ability to hold or worse.

Prism Evaluation
Deficient postural control is one of the major components of reduced function in cerebral palsy patients. Vision plays an important role in controlling the position of the head in space. On the other hand, head stability is important for vision, as it fosters gaze stability and therefore image stability on the retina, facilitating the processing of visual information.17 Yoked prism may provide postural benefits that can improve visual processing and set-up better conditions for vision therapy treatment. Using a moderate amount of yoked prism (1015), evaluate performance with the base placed in all four directions. Use an activity that is appropriate for the patients functional level. This may include hitting, throwing or watching a ball, handling eating utensils or writing. When subjective and objective improvement is seen, prescribing a small amount of prism (1-4) may be warranted.

Directional Arrows
a. Make arrows of different directions on the blackboard (four rows of four each) b. Patient reads the arrows in order, correctly naming the direction c. Can increase difficulty by naming opposite of arrow direction or using oblique

Directional Maps
a. Use a simple line drawing of a starting position and where the child would like to go b. The child verbally gives instructions on how the therapist should move a pencil to drive the road c. Therapist should follow all directions, even wrong ones so the patient is able to correct them. Must also give directions to stop at a corner, etc. d. The therapist and child can then move around the room on the basis of the childs verbal instructions