Download Alexandra Talaber - American Academy of Optometry

Diagnosis and Management of a Pediatric Patient with Infantile Nystagmus
Authors: Alexandra Talaber O.D., Brian M. Rodrigues O.D., Barry Tannen O.D., F.C.O.V.D,
F.A.AO., M.H. Esther Han O.D, F.C.O.V.D, F.A.A.O.
Abstract: Guidelines on diagnostic testing for infantile nystagmus, along with optometric
treatment modalities to increase visual acuity, binocular stability, and improve cosmesis will be
explored.
I. Case History: AM is a nine year old Hispanic male referred to the SUNY State College of
Optometry University Eye Center at five years of age for the treatment of congenital nystagmus.
Symptoms were reported as decreased vision and irregular eye movements. Ocular history is
significant for infantile nystagmus, status post nystagmus surgery at seven years of age. AM has
bilateral anisometropic refractive amblyopia with two years of OD patching. Medical history is
significant for later birth delivery, increased insulin levels at birth resulting in hospitalization,
and asthma. Current medication is albuterol rescue inhaler.
II. Pertinent findings

Entering comprehensive exam from 2008:

Habitual Rx: OD +3.00-1.25x005 / OS +4.00-1.50x005; ADD +4.00

Distance VA without correction: OD 20/80, OS 20/100, OU 20/80

Distance VA with correction: OD 20/70, OS 20/80, OU 20/80

Near VA without correction: OD 20/30, OS 20/40, OU 20/30,

Near VA with correction: OD 20/100, OS 20/100, OU 20/100

Retinoscopy (variable): OD +4.25-2.00x010 / OS +2.50-1.50x180

EOM: horizontal pendular nystagmus with dampening upon convergence,
superior null point, high frequency latent component, Inferior left head tilt
 Hirschberg reflex: IRXT
 Accommodation: +1.75 lag OU
 Fundus examination: No retinal or optic nerve pathology noted
III. Differential diagnosis
 Infantile nystagmus (also termed congenital nystagmus)
o Idiopathic (motor pathway or feedback deficit)
o Visual input disorder (leber’s congenital amaurosis, cone dystrophy, congenital
stationary night blindness, optic nerve hypoplasia/atrophy, anterior segment or
media opacity, ocular albinism)
o Neurological
 Spasmus Nutans
o Onset typically between 5 months and 3 years of age
o Spontaneous resolution between 2 and 8 years of age
o Varied presentation with different nystagmoid patterns and directions but
typically small frequency and low amplitude
o Normal fundus examination and normal imaging studies
o Typically does not require treatment, not pathological entity
 Acquired Nystagmus
o Due to disease process, trauma or neurological complication; typically after
infantile period
o Characteristically due to vestibular pathway pathology
o Imaging studies of cranium, orbit or auditory system, and/or a neurological
consult must be performed to rule out or identify pathological entities
IV. Diagnosis and discussion
 Infantile nystagmus is considered a pathological process that can be of an idiopathic
origin or associated with other visual disorders such as albinism, retinal disease, low
vision and neurological childhood diseases. Nystagmus causes decreased visual acuity
due to movement of images away from the foveal area of the retina. The prevalence of
nystagmus is reported to be 24/10,000 and the impact of nystagmus is significant.
 Characteristics:
o variable waveform nystagmus, usually horizontal, medium amplitude and
frequency, symmetrical
o Head tilt, turn, or oscillation
o latent component, nystagmus worsens with fixation, (+) null point
 Tests: ERG, look for high hyperopia (leber’s), myopia and male (X-L congenital
stationary night blindness), astigmatism (ocular albinism), low amplitude with high
frequency (cone dystrophy)
 Pediatric populations are at risk for nystagmus due to the susceptibility and fragility of
their neurological development. Nystagmus often presents as a significant deterrent to
optimal vision and visual performance any may isolate children due to poor performance
or cosmesis. Many optometrists find themselves referring their patients with nystagmus
because they are uncertain of the proper diagnostic testing and treatment options for this
patient population.
V. Treatment
 Vision Therapy –binocularity, suppression control, biofeedback
o Patient AM began Vision Therapy at age 6 in 2008 to improve oculomotor skills,
accommodative insufficiency and convergence excess. Distance VA (DVA) with
correction OU was 20/80 at the start of therapy. After 40 sessions DVA OU was
measured at 20/100, although accommodative amplitudes and binocularity were
improved to above normal and the patient was graduated due to immaturity for
biofeedback training at the time. AM began biofeedback therapy at age 8,
completed 8 sessions in 2010 and was dismissed thereafter due to time constraints
and scheduling conflicts. AM returned to the clinic in January 2012; Entering
DVA with correction OU was 20/70. DVA with correction OU was 20/30 after 6
months of biofeedback therapy. Readalyzer™ fixation recordings were performed
throughout to measure improvements in fixation. These recordings show a
marked reduction in nystagmus amplitude and frequency, along with reduced
convergence for an extended time period compared with initial reading. AM is
continuing biofeedback therapy weekly, including some oculomotor and visualmotor skills. Subjective improvements are pronounced since starting biofeedback
therapy along with visual skills therapy, along with measureable improvement in
visual acuity over time. Due to increase in frequency of nystagmoid movements
under monocular conditions, therapy was performed binocularity in order to
provide the greatest amount of oculomotor control and least amount of retinal
slip. Biofeedback: research supports reduction of amplitude of nystagmus and
frequency (to a lesser extent) with the use of auditory biofeedback therapy.
Kirschen demonstrated improved visual acuity and contrast sensitivity during the
technique. All patients report subjective improvement after VT.
 Explanation of auditory feedback
 Visagraph readings from 1/5/12 and 4/12/12 (see below). Interpretation
and additional recordings will be present in final presentation or poster.
o Table 1. Visual acuity over time
Date
6/11/2008
7/15/2008
9/16/2008 (began VT)
1/20/2009
3/24/2009
9/15/2009
3/9/2010 (s/p surgery)
1/5/12
3/15/2012 (start of
biofeedback therapy)
7/12/2012
7/19/12
8/30/12



DVA OU
20/80
20/200
20/80
20/50
20/60+2
20/100
20/80
20/70+1
20/60-1
20/40 (near)
20/30+1 (near)
20/25 (near)
20/40+1
Prism
o BO to increase convergence or yoked prism to alter visual direction.
o AM was given varying degrees of vertical prism in the range of 15-9 base down
in order to elevate primary gaze to a superior position in order to attempt to bring
AM gaze towards his null point. Father reported initial decrease in head tilt with
regression in 1-2 months of wearing prismatic correction upon examination.
Surgical
o AM underwent combined nystagmus and strabismus surgery in February 2010.
Immediately following surgery, DVA without correction were OD and OS
20/100, OU 20/80. Father reported improvement in head tilt and eye movements
initially but nystagmus regressed over the period of one year with return in head
tilt downward.
Other treatment options:
o Rigid gas permeable contact lenses
o Pharmacological, CNS depressants
o Botulinum toxin injections
VI. Conclusion

Careful case history to review age of onset, severity, previous treatments, family history
of condition.








Rule out any neurological or pathological conditions that may manifest with nystagmus
Attempt to achieve best correctable visual acuity by first documenting binocular VA and
allowing patient to assume habitual head posture (examining at null point)
Correct any refractive error with spectacles: Binocular refraction preferred by fogging
one eye when refracting fellow eye to minimize effects of latent component
Careful documentation of nystagmus eye movements such as fast and slow phase
direction, conjugation, amplitude, frequency, effect of convergence and positions of gaze
and latency.
Treat any amblyopia if present
Attempt to align primary gaze with null point with use of prism
Improve any additional deficits that may impede academic learning such as
accommodative deficiencies, saccadic and pursuit deficiencies and abnormal
binocularity.
Perform Biofeedback training via visual, auditory or oculomotor awareness therapy to
improve intrapersonal cognizance and control of ocular deviations and oscillations.
Resources:
Dell’Osso, L.F., and Z.I. Wang. Extraocular proprioception and new treatments for infantile
nystagmus syndrome (2008). Progress in Brain Research, Vol 171, pp. 67-75.
Hertle, Richard W. Nystagmus in Infancy and Childhood (2008). Seminars in Ophthalmology,
23:307-317.
Mclean and Gottlob. An Update on Nystagmus (2012). Advances in Clinical Neuroscience &
Rehabilitation Neuro-ophthamology Series. Vol 11: 6, pp. 17-18.
Sharma, P., Tandon, R., Kumar, S., and S. Anand. Reduction of congenital nystagmus amplitude
with auditory biofeedback (2000). Journal of AAPOS. Vol 4:5, pp. 287-290.
Tao and Flickinger. How to Assess and Treat Infantile Nystagmus (2012). Ophthalmic Pearls:
Neuro-Ophthalmology. American Academy of Ophthalmology. 29 Aug. 2012
http://www.aao.org/publications/eyenet/200511/pearls.cfm
Biofeedback Recordings using Readalyzer™
Initial Recordings performed 1/15/2012
Mid Therapy Recordings performed 4/12/2012