Texas School for the Blind and Visually Impaired Outreach Programs www.tsbvi.edu |512-454-8631 | 1100 W. 45th St. | Austin, Texas 78756 2012-13 Texas Focus Nystagmus in Children: Causes, Effects and Strategies March 31, 2013 8:30-10:00 AM or Presented by Dr. Ana Perez, OD, FAAO Low Vision Rehabilitation Michael E. DeBakey VAMC Developed for Texas School for the Blind & Visually Impaired Outreach Programs Nystagmus in Children: Causes, Effects and Strategies – Perez, 2012 – 2012 Texas Focus Conference 0 Nystagmus in Children: Causes, Effects, and Strategies Ana M. Perez, OD, FAAO Low Vision Rehabilitation, Michael E. DeBakey VAMC, University of Houston College of Optometry Objectives 1. Participants will discover the causes and treatments for nystagmus. 2. Participants will review how nystagmus affects visual performance in both near and distance visual activities, as well as ways to improve visual performance. Nystagmus Condition in which the eyes make repetitive, uncontrolled movements, often resulting in reduced vision Types of movement Side to side: horizontal nystagmus Up and down: vertical nystagmus Rotary: torsional nystagmus Nystagmus is a sign of a problem Ocular structure Pathways that connect the eye to the parts of the brain that deal with eye movement It is not really a condition in its own right! Two main types of nystagmus Congenital nystagmus or early onset nystagmus It appears in the first months of life Acquired nystagmus Develops later in life Nystagmus affects one in 1500 people Nystagmus in Children: Causes, Effects and Strategies – Perez, 2012 – 2012 Texas Focus Conference 1 Causes of Nystagmus Physiological nystagmus – normal part of how our eyes work If you watch someone’s eyes as they are watching a train passing, their eyes follow the train then flick back to a starting point; this is repeated over and over OKN drum Pathological nystagmus – If the eye or the visual pathway (where visual information is processed) are damaged Idiopathic nystagmus – condition starts early in life and the cause is unknown Congenital Nystagmus Occurs in the first couple of years of life Caused by two things: 1. A problem with the eye structure 2. A problem with the visual pathway from the eye to the brain New born babies All children are born with a visual system that is not fully developed Vision continues to develop in the first few years of life as a result of the eye and brain being stimulated Eye conditions affecting vision Congenital cataracts – lack of good stimulation to the retina Congenital glaucoma – damaged optic nerve Optic nerve hypoplasia – under developed optic nerve Albinism – under developed macula Visual system will not develop normally Nystagmus in Children: Causes, Effects and Strategies – Perez, 2012 – 2012 Texas Focus Conference 2 Severity of the congenital nystagmus Depends on the severity of the sight loss caused by the underlying condition Vision tends to worsen when: o The eye movements of the nystagmus increases Not being at the null point o Patient is stress and/or fatigue o There is occlusion of an eye Congenital Nystagmus It is not painful It does not lead to progressive vision loss For the most part, patient do not see the world jumping Typically, vision tend to improve until it stabilizes at age five or six. Giving children plenty of visual stimulation in the early years leads to best use of visual function Acquired Nystagmus Typically, develops in adulthood Causes Multiple sclerosis Brain tumor/ brain injury Effects of a drug: Dilantin (anti-seizure med) Thiamine or Vit B12 deficiency Patients are aware of the movement: Oscillopsia. Typically, very disabling Drug Induced Nystagmus Dilantin (phenytoin) – an anti-seizure medication Excessive alcohol Sedating medications Nystagmus in Children: Causes, Effects and Strategies – Perez, 2012 – 2012 Texas Focus Conference 3 Classifying Nystagmus Describing eye movements Jerk nystagmus: movement is quick in one direction and slow in another Pendular: the speed of movement is equal in both directions Direction of the nystagmus Vertical Horizontal Circular/tortional Jerk and Pendular Nystagmus Video Clip Null Point A particular head position that results in the slowest eye movement Reductions in the movement (amplitude) of the nystagmus usually means the vision is at its best Visual acuity is variable 20/30 to 20/200 Null point Video clip Nystagmus Treatment Nystagmus cannot be cured Muscle surgery o Reduces the amount of head turn needed to reach the null point o Cosmetic/Postural Medication: Botox (baclofen) o Temporary effect Biofeedback training o Making patients aware of the movement thru visual and audio signals Nystagmus in Children: Causes, Effects and Strategies – Perez, 2012 – 2012 Texas Focus Conference 4 Pharmacologic, Optical, and Surgical Treatments Choice of treatment depends on the characteristics of the nystagmus and the severity of the associated visual symptoms Electro-optical devices are currently being developed (optical stabilizer) Different meds for different types of nystagmus Downbeat nystagmus o 4-aminopyridine, 3,4-diaminopyridine or clonazepam Upbeat nystagmus o Memantine, 4-aminopyridine or baclofen Torsional nystagmus o gabapentin Acquired pendular nystagmus o In multiple sclerosis: partially suppressed by gabapentin or mematine o In oculopalatal tremor: gabapentin, memantine or trihexyphenidyl Infantile nystagmus nystagmus o Gabapentin, memanine, acetazolamide, contact lenses, prisms, or surgery. Maximizing Visual Function with Nystagmus Early intervention Visual stimulation with engagement of the environment Because of the variability in patients with congenital nystagmus (with or without pathology) it is difficult to predict best VA Intervention: Active looking, searching, & selecting Visual curiosity o Visual o Tactile Explore environment o Mobility DISTANCE Video Clip Nystagmus in Children: Causes, Effects and Strategies – Perez, 2012 – 2012 Texas Focus Conference 5 Extending Visual Reach Having a desire/need to look at a distance o Typically, distance is of little consequence to a young child with visual impairments Creating curiosity for exploration Creating skills for safety Creating independence How do we make it interesting? Incorporating navigational skills o The child should learn landmarks and give directions o This is to be done without the use of optical devices which provide detail information like telescopes Use a “big wheel” and place cones to navigate a path Implications for Distance The use of telescopes (TS) in cases of congenital nystagmus is not contraindicated Patients with nystagmus generally perform very well with the use of TS Telescopes for distance Provide magnification by creating a larger image and placing it on the retina If the magnification is 4x, then the image movement through the TS is 4x faster If the patient has congenital nystagmus, they will NOT see the image jumping Recommendations for Distance Modified seating placement o Specific distance in meters or feet Introduction to the use of HHTS o Localization/Copying skills o TS power for in- classroom activities o TS power for outside activities (may be different) NEAR/READING Video Clip Nystagmus in Children: Causes, Effects and Strategies – Perez, 2012 – 2012 Texas Focus Conference 6 Reading with Congenital Nystagmus Visual acuity is typically reduced It is not possible to predict with any amount of certainty the visual acuity by the amplitude/frequency of the nystagmus The null point is the position of least eye movement and best resolution The words on a page are NOT jumping when reading Foveation Periods Intervals in the CN waveform when the eye velocity is relatively slow and the target is imaged on or near the fovea. Initially, it was thought that patients with CN “sample” their environment only during foveation and suppressed visual input thru out the rest of the CN waveform; and in this way they do not have the perception of oscillopsia. Evidence has been found against this hypothesis. Jin YH, Goldstein HP, Reinecke RD. Absence of visual sampling in infantile nystagmus. Korean J Ophthalmology 1989 Implications for Reading Research-based information Patients with CN have equal maximum reading speeds when comparing rapid serial visual presentation (RSVP) and continuous text (CT) o 449 and 448 words/minute Patients with CN can read at rates faster than the frequency of nystagmus o This suggest that they are able to read during non-foveation periods. Woo S, Bedell H. Beating the Beat: Reading can be faster than the frequency of the eye movements in persons with congenital nystagmus. Optometry and Vision Science. Aug 2008 What does this mean for our visually impaired students…? Patients with congenital nystagmus need to have a reading reserve… They should not be reading at their threshold! Make sure that this has been address for each student Nystagmus in Children: Causes, Effects and Strategies – Perez, 2012 – 2012 Texas Focus Conference 7 Providing Reading Reserve Low Vision Optometrist can provide Print size recommendation Reading glasses/bifocals Incorporation of magnifiers Use of CCTV: with an enlargement recommendation LV Rehab Optometrist need to know… What is the patient‘s reading level? o Does it correlate with their grade level? What is the reading demand? o Size of textbook print: science/social studies o Size of math print o Is patient reading paperback books o Is patient reading off the computer screen Implications for School Best correction in place Allow patient’s prefer head position, so as to reach their null point which in turn provides best visual resolution There is no way of predicting visual acuity in patients with nystagmus Congenital nystagmus is not a progressive condition Patients with congenital nystagmus do not see the world moving Patients with nystagmus respond very well to the implementation of optical devices Nystagmus in Children: Causes, Effects and Strategies – Perez, 2012 – 2012 Texas Focus Conference 8 Texas School for the Blind & Visually Impaired Outreach Programs Figure 24 TSBVI logo. "This project is supported by the U.S. Department of Education, Office of Special Education Programs (OSEP). Opinions expressed herein are those of the authors and do not necessarily represent the position of the U.S. Department of Education. Figure 25 IDEA logo Nystagmus in Children: Causes, Effects and Strategies – Perez, 2012 – 2012 Texas Focus Conference 9