State-of-the-Art Review
Therapy for Nystagmus
Matthew J. Thurtell, MBBS, FRACP, R. John Leigh, MD
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Abstract: Pathological forms of nystagmus and their vi-
sual consequences can be treated using pharmacologi-
cal, optical, and surgical approaches. Acquired periodic
alternating nystagmus improves following treatment with
baclofen, and downbeat nystagmus may improve follow-
ing treatment with aminopyridines. Gabapentin and
memantine are helpful in reducing acquired pendular
nystagmus due to multiple sclerosis. Ocular oscillations
in oculopalatal tremor may also improve following treat-
ment with memantine or gabapentin. The infantile nys-
tagmus syndrome (INS) may have only a minor impact
on vision if ‘‘foveation periods’’ are well developed, but
symptomatic patients may benefit from treatment with
gabapentin, memantine, or base-out prisms to induce
convergence. Several surgical therapies are also reported
to improve INS, but selection of the optimal treatment
depends on careful evaluation of visual acuity and nys-
tagmus intensity in various gaze positions. Electro-optical
devices are a promising and novel approach for treating
the visual consequences of acquired forms of nystagmus.
Journal of Neuro-Ophthalmology 2010;30:361–371
doi: 10.1097/WNO.0b013e3181e7518f
Ó 2010 by North American Neuro-Ophthalmology Society
R ational therapy of nystagmus rests on several basic
principles (1). A clear percept of an object requires that
its image be held steadily within about 0.5 degrees of
the center of the fovea. For objects with higher spatial
frequencies, such as Snellen optotypes, retinal image slip
should be less than 5 degrees per second.
Department of Neurology, University Hospitals Case Medical Cen-
ter, Cleveland, Ohio; and Neurology Service and Daroff-Dell’Osso
Ocular Motility Laboratory, Veterans Affairs Medical Center, Cleve-
land, Ohio.
Supported by the National Institutes of Health R01-EY06717, the
Office of Research and Development, the Medical Research Service,
the Department of Veterans Affairs, and the Evenor Armington
Fund.
Address correspondence to Dr. R. John Leigh, MD, Department of
Neurology, University Hospitals Case Medical Center, 11100 Euclid
Avenue, Cleveland, OH 44106-5040; E-mail: rjl4@case.edu
Thurtell and Leigh: J Neuro-Ophthalmol 2010; 30: 361-371
Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.
Patients with infantile nystagmus syndrome (INS), for-
merly called congenital nystagmus, often have a brief epoch of
stillness called the ‘‘foveation period’’ during each cycle of the
nystagmus, which is sufficient to provide clear vision (Fig. 1).
It appears that they can suppress the visual consequences of
rapid image motion at times other than during the foveation
period, and therefore seldom complain of oscillopsia (2).
There are 3 gaze-holding mechanisms that promote clear
vision. Visual fixation mechanisms reduce eye drifts that take
the eyes away from the target and suppress unwanted
saccades. The vestibulo-ocular reflex (VOR) generates eye
movements to compensate for head perturbations at short
latency, being especially important for stabilizing gaze
during locomotion. An eccentric gaze-holding mechanism is
important to withstand the elastic pull of the orbital fascia
that tends to bring the eye back to the center position.
Malfunction of each of these gaze-holding mechanisms, as
well as other abnormal inputs to the ocular motor system,
may cause drifts of the eye away from the target (slow
phases) with interspersed corrective quick phases (saccades)
that constitute pathological nystagmus.
Based on these principles, one goal of therapy is to abolish
abnormal ocular oscillations and leave normal gaze-holding
eye movements intact. For example, treatments to stop the
eyes from moving altogether, such as botulinum toxin in-
jections, may abolish the ocular oscillations, but provide no
net improvement, since patients then complain of blurred
vision when they move their head (due to absent VOR) and
diplopia (due to absent vergence, which normally aligns the
eyes). Treatments that suppress the abnormal ocular oscil-
lations without affecting normal eye movements are therefore
preferred. Furthermore, some forms of nystagmus, such as
the gaze-evoked nystagmus common with drug intoxications
and cerebellar disease (1,3), do not usually cause sufficient
visual disturbance to require treatment. It should be noted
that inappropriate saccades, including intrusions and oscil-
lations, may also impair vision, but treatments for these are
reviewed elsewhere (1,4–6).
We will review, in turn, drug treatments for pathological
nystagmus, optical devices that negate the visual consequences
of ocular oscillations, and surgical procedures. We will briefly
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 State-of-the-Art Review

TABLE 1. Treatments for nystagmus and its

visual consequences
Medications
Gabapentin (8,9,16,45,77)
Memantine (8,9,33,47,77)
4-Aminopyridine (20,23)
and 3,4-diaminopyridine (19,24,21)
Baclofen (14,16,29,37)
Clonazepam (12)
Valproate (43)
Trihexyphenidyl (17,18,58)
Benztropine (17)
Scopolamine (17,61,151)
Isoniazid (44)
Carbamazepine (152,153)
Barbiturates (154)
Alcohol (73)
Acetazolamide (66,67,70)
Cannabis (78,79,155)
Optical Devices
Contact lenses (86)
FIG. 1. Waveform of infantile nystagmus. During fovea- Prisms: base-in or base-out (89–91)
tion periods (arrows), when the fovea is briefly pointed at Retinal image stabilization (92,94)
a visual target located at 0 degrees, the eye is relatively Electro-optical devices that selectively cancel the
still (almost a horizontal line), so that retinal image mo- ocular oscillations (97,98)
tion is minimized. (Positive values indicate eye rotations Surgical Procedures
to the right.) Anderson-Kestenbaum procedure to shift null
point (99,100,102,103,105,106)
Cüppers divergence procedure (88,107,108)
Recession of horizontal rectus muscles (110,111)
discuss botulinum toxin and alternative methods to treat Tenotomy and reattachment of the extraocular
nystagmus (Table 1). We caution that although many muscles (122,125)
treatments have been proposed for nystagmus, few have been Botulinum Toxin
evaluated with controlled clinical trials (7–9). Injected either into selected extraocular muscles
(134) or into the retro-bulbar space (135–137,156)
DRUG TREATMENTS Other Measures
Acupuncture (144,145)
Biofeedback (146–148)
Nystagmus of Peripheral Vestibular Imbalance Cutaneous head and neck stimulation (142)
This form of nystagmus usually resolves over the course
of a few days. Medications, mainly used to treat associated
vertigo, nausea, and vomiting, are helpful only during the
acute phase of the illness (1,10). Nystagmus associated with drug produced a consistent improvement and that, in some
benign paroxysmal positional vertigo is better treated with patients, the nystagmus was made worse (16).
repositioning procedures, such as the Epley maneuver (11), An interest in using anticholinergic agents was generated
than with medications. by the observation that intravenous scopolamine reduces
downbeat nystagmus (17). However, a controlled trial of
Downbeat Nystagmus the oral anticholinergic agent trihexyphenidyl produced
This type of nystagmus is largely a feature of diseases only modest improvement and side effects that were poorly
affecting the vestibulocerebellum. Several hypotheses have tolerated (18).
been proposed for its pathogenesis, most invoking an up- The potassium channel blockers 3,4-diaminopyridine
down asymmetry that affects the inhibition of projections of and 4-aminopyridine are promising for the treatment of
the vertical semicircular canals, vertical smooth pursuit, or downbeat nystagmus. Both medications have been shown
otolithic influences (1). Many medications have been re- to suppress downbeat nystagmus in some patients (19–22).
ported to improve downbeat nystagmus, including the Although they are generally well tolerated, they can cause
GABAA agonist clonazepam (12,13) and the GABAB ago- seizures. How do they suppress nystagmus? Because
nist baclofen (14,15). However, a double-masked com- potassium channels are abundant on cerebellar Purkinje
parison of baclofen and gabapentin showed that neither cells, the aminopyridines may increase their discharge. The

362 Thurtell and Leigh: J Neuro-Ophthalmol 2010; 30: 361-371

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enhanced Purkinje cell activity could then restore normal
levels of inhibition of vertical vestibular eye movements,
leading to suppression of the nystagmus (22). However,
4-aminopyridine suppresses upbeat nystagmus in some
patients (23), and it may occasionally cause downbeat
nystagmus to convert to upbeat nystagmus (24). Alterna-
tively, 4-aminopyridine could modulate otolithic mecha-
nisms that influence vertical nystagmus (24). Whatever the
mechanism, many patients are likely to benefit from
treatment with 4-aminopyridine, which is generally better
tolerated than 3,4-diaminopyridine (21,25,26).
Upbeat Nystagmus
This form of nystagmus usually occurs with brainstem
lesions. Although it may produce pronounced visual
symptoms during the acute period, upbeat nystagmus often
resolves spontaneously or converts to downbeat nystagmus.
There are few clinical trials evaluating pharmacological
treatments for this form of nystagmus, although 1 recent
study has shown that it may be suppressed with memantine
(8). Treatments similar to those for downbeat nystagmus,
such as the aminopyridines (23), are also worth considering
in patients with persistent upbeat nystagmus.
Periodic Alternating Nystagmus
This form of nystagmus consists of spontaneous horizontal
nystagmus that reverses direction approximately every 100–
120 seconds. The acquired form of periodic alternating
nystagmus (PAN) is a rare but well understood form of
central vestibular nystagmus. A monkey model has been
produced following surgical lesions of the cerebellar nod-
ulus and ventral uvula (27). Such lesions may cause excessive
vestibular responses (velocity storage), which, in turn,
stimulate adaptive mechanisms that cause the ocular os-
cillations (28). The nystagmus of most patients (and the
monkey model) is decreased following treatment with the
GABAB agonist baclofen (29–32). Improvement following
treatment with memantine has also been reported (33). The
infantile form of PAN, which has a more variable cycle
length, probably has a different pathogenesis and only oc-
casionally improves with baclofen treatment (34–37).
Acquired Pendular Nystagmus Associated With
Multiple Sclerosis
This form of nystagmus usually causes visual impairment
and oscillopsia, for which most affected patients seek
therapy. These patients often have coexisting internuclear
ophthalmoparesis and impaired visual function due to optic
neuropathy. Indeed, the amplitude of the nystagmus is
often greater in the eye with poorer vision, prompting the
hypothesis that delays in visual pathway conduction give rise
to the oscillations (38). However, other investigations have
suggested that an instability in the gaze-holding mechanism
(neural integrator) may be responsible (39–41). Suspicion
of neural integrator dysfunction led to the testing of
Thurtell and Leigh: J Neuro-Ophthalmol 2010; 30: 361-371
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State-of-the-Art Review
medications with presumed effects on GABA-mediated and
glutamate-mediated mechanisms (41,42). In early studies,
GABAergic agents, such as clonazepam, valproate, and
isoniazid, were found to decrease the nystagmus in some
patients (43,44). In a multicenter double-masked study of
15 patients with acquired pendular nystagmus (APN) (16),
gabapentin, an anticonvulsant initially thought to have
GABAergic action, was compared to baclofen, a GABAB
agonist. Visual acuity improved significantly with gaba-
pentin, but not with baclofen. Gabapentin reduced median
eye speed in all 3 planes, but baclofen did so only in the
vertical plane. In 10 of the 15 patients, the suppression of
nystagmus with gabapentin was substantial, and 8 patients
chose to continue taking the drug. However, some patients
in the study showed no response to either medication. An
important side effect of gabapentin was increased ataxia.
Three subsequent trials, 1 comparing gabapentin with the
anticonvulsant Vigabatrin (45) and the other 2 comparing it
with memantine (8,9), have confirmed that gabapentin is an
effective treatment for APN. Vigabatrin, which is more
purely GABAergic than gabapentin, was ineffective in the
first of these trials (45), suggesting that gabapentin sup-
presses APN by a non-
GABAergic mechanism. Gabapentin is now known to exert its
effect by binding to the calcium channel subunit a2d-1 (46).
Memantine, a noncompetitive N-methyl-D-aspartate
receptor antagonist that has been used for more than 25
years in Germany as a therapy for a variety of neurological
symptoms, including treatment of spasticity in multiple
sclerosis (MS), was recently approved by the United States
Food and Drug Administration at a dose of 20 mg/d for the
treatment of memory failure in Alzheimer disease. At doses
of 40 mg/d, memantine has been reported to reduce or
abolish APN in patients with MS (8,9,47). Memantine
also shows some antagonistic effects at 5-hydroxytryptamine
and nicotinic acetylcholine receptors (48). Memantine may
reduce nystagmus in some patients in whom gabapentin has
proven ineffective (8,9,49). However, at doses of 30 mg/d,
patients with MS may develop blurred vision, fatigue, severe
headache, increased muscle weakness, or gait instability
(50), so that gabapentin may be the preferred treatment
when APN is due to MS.
While recent clinical trials have compared the relative
efficacy of gabapentin and memantine for APN (8,9),
further trials are required to determine if combinations of
gabapentin and memantine have an additive effect and to
establish whether these drugs may be useful adjuncts to sur-
gical treatments for APN, as suggested in case reports (51,52).
The same is true for other medications that have been reported
to suppress nystagmus in individual patients with APN, but
have not been studied in controlled trials (Table 1).
Oculopalatal Tremor
Previously called oculopalatal myoclonus, oculopalatal
tremor (OPT) usually develops in the weeks following
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 State-of-the-Art Review

brainstem or cerebellar strokes that interrupt projections

from the deep cerebellar nuclei. These projections run in the
superior cerebellar peduncle, bending (but not synapsing)
near the red nucleus, before descending in the central
tegmental tract to contact the inferior olivary nuclei (53). In
health, inferior olivary neurons, which possess gap junctions
(connexins) on their dendrites, discharge asynchronously.
Following degenerative hypertrophy of the inferior olives,
gap junctions also develop on the cell bodies of olivary
neurons (54), producing electrotonic coupling between
them. Thereafter, ensembles of inferior olivary neurons
begin to fire in synchrony at a frequency of about 2 Hz and
serve as ‘‘pacemakers’’ projecting via climbing fibers to the
cerebellum, where maladaptive learning takes place (55,56).
The entire process results in spontaneous oscillations of the
eyes, palate, and other branchial muscles at a frequency of
about 2 Hz.
Nystagmus may be the only clinical manifestation of
OPT. Some patients show partial suppression of their
nystagmus with gabapentin (16) or memantine (Fig. 2) (8).
However, neither drug noticeably suppresses the palatal
tremor, which is usually asymptomatic. While the nystag-
mus of OPT can respond dramatically to gabapentin or
memantine in occasional patients (8), it is generally more
refractory to treatment than is APN secondary to MS.
The hypertrophied inferior olivary nucleus of patients
with OPT also shows increased acetylcholinesterase activity
(57), prompting trials of anticholinergic agents. Some
patients with pendular nystagmus may show a response to
trihexyphenidyl (58,59), but a clinical trial of this medi-
cation showed only modest effects (18). A discrepancy
between the effects of intravenous scopolamine (17) and
oral trihexyphenidyl in certain pendular forms of nystagmus
might be due to the more selective antagonism of musca-
rinic receptors by trihexyphenidyl (60). Intravenous sco-
polamine clouds consciousness and is not a practical therapy
for pendular forms of nystagmus. Furthermore, transdermal
scopolamine is not a reliable therapy for pendular forms of
nystagmus and can make the nystagmus worse or induce
confusion (61).
Novel therapies for OPT may target the unusual elec-
trotonic coupling of the inferior olivary neurons by con-
nexins (62–64). These connexins can be inhibited by certain
antimalarial agents (65). Other medications reported to aid
patients with OPT are listed in Table 1.

Familial Episodic Vertigo and Ataxia Type 2

Nystagmus in this disorder, which is due to a calcium
channelopathy, usually responds to acetazolamide (66–68),
although associated cerebellar symptoms are occasionally
made worse (69). The potassium channel blocker 4-
aminopyridine is also an effective treatment for episodic
ataxia type 2 in some patients (70). Some patients with
spinocerebellar ataxia type 6 who have episodic attacks of
FIG. 2. Waveform of vertical component of OPT prior to
and during treatment. A. Wide-amplitude pendular-like
nystagmus is evident prior to treatment. B. After this
patient has been treated with gabapentin 1,200 mg/d,
the nystagmus amplitude is reduced. C. After the same
patient has been treated with memantine 40 mg/d, the
nystagmus amplitude is also reduced.

364 Thurtell and Leigh: J Neuro-Ophthalmol 2010; 30: 361-371

Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.

vertigo and nystagmus benefit from acetazolamide (71).
Studies of animal models for these channelopathies are
likely to produce a clearer rationale for therapy (72).
Seesaw Nystagmus
This form of nystagmus may be suppressed by alcohol
(73,74) and clonazepam (75). We have observed im-
provement of hemiseesaw nystagmus in single patients
treated with gabapentin or memantine (8).
Heimann-Bielschowsky Phenomenon
This ocular motor disorder, which consists of slow—and
sometimes symptomatic—vertical oscillations in an eye
with visual loss (1), may be improved with gabapentin (76).
Nystagmus of Early Childhood
The nystagmus of some patients with INS has improved
with gabapentin or memantine. In a randomized, con-
trolled, double-masked trial comparing the 2 medications,
nystagmus intensity and visual acuity improved in both
treatment groups (77). However, there was only a small
effect in patients with abnormal afferent visual system
function or structure compared to those with normal af-
ferent visual systems. INS may also be reduced by smoking
cannabis (78,79).
Gene therapy holds the potential for treatment of nys-
tagmus associated with retinal disorders. For example, in an
animal model of Leber congenital amaurosis, successful
gene therapy restored vision and reduced the associated
nystagmus (80–83).
OPTICAL TREATMENTS
Correction of refractive error is worthwhile in most patients
with infantile or acquired forms of nystagmus and may pro-
duce an appreciable improvement in vision (84,85). Contact
lenses may suppress INS (86), suggesting a mechanism beyond
refractive correction (discussed further in the final section of
this review). The main therapy for latent nystagmus (fusional
maldevelopment nystagmus syndrome) consists of measures to
improve vision, such as patching for amblyopia (87).
Patients whose nystagmus is suppressed by convergence
may benefit from wearing spectacle prisms that require
convergence for single vision of far targets (88). Adequate
convergence may be produced by a pair of 7 prism-diopter
base-out prisms with 21 diopter spherical power added to
compensate for the accommodation that accompanies the
induced convergence (89). (The spherical correction may
not be required in individuals with presbyopia.) With base-
out prisms, some individuals with INS experience an im-
provement of vision that is sufficient to qualify them for
a driving license. Occasional patients with acquired nys-
tagmus may benefit from prisms (90). Those whose nys-
tagmus is worse during near viewing may respond to base-in
prisms, which reduce convergence effort (91). Patients with
Thurtell and Leigh: J Neuro-Ophthalmol 2010; 30: 361-371
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State-of-the-Art Review
INS whose nystagmus is of lower amplitude when the eyes
are placed in an eccentric null position rarely report a
benefit from conjugate prisms that shift gaze.
An alternative approach has been to develop optical
devices that negate the visual effects of the nystagmus. One
approach consists of using high-plus spectacle lenses in
combination with high-minus contact lenses (92). The
underlying principle is that stabilization of images on the
retina can be achieved if the power of the spectacle lens
focuses the primary image close to the center of rotation of
the eye. However, such images are defocused, requiring
a contact lens to extend the focus back onto the retina.
Because the contact lens moves with the eye, it does not
negate the effect of retinal image stabilization due to the
spectacle lens. With such high-positive spectacle lens and
high-negative contact lens combinations, it is possible to
negate about 90% of the visual effects of eye movements
(93). However, this approach impairs all eye movements,
including the VOR and vergence, so that it is only useful
when the patient is stationary and viewing monocularly.
Other disadvantages are that the field of view is limited and
patients with ataxia may have difficulty inserting the contact
lens. Gas-permeable or even soft contact lenses may,
however, achieve lesser degrees of image stabilization that
are beneficial to the patient (94,95). Thus, in selected pa-
tients, this approach may prove useful for limited periods
of time, such as for the duration of a movie.
A more recent approach is to develop an electro-optical
device that measures the ocular oscillations and negates their
effects (96). This approach is best suited for pendular
nystagmus, which can be electronically distinguished from
normal eye movements, such as voluntary saccades. Figure 3
summarizes the image-shifting optics that are being used to
develop a portable battery-driven device (97,98), a pro-
totype of which is shown in Figure 4.
SURGICAL TREATMENTS
Surgical procedures for the treatment of nystagmus have
mainly been developed for patients with INS. The
Anderson-Kestenbaum operation aims to move the at-
tachments of the extraocular muscles, so that the null point
is shifted to the straight-ahead gaze position (99,100). Se-
lection of patients who will benefit most entails measuring
visual acuity and nystagmus intensity in different gaze
positions (101). The surgeon can then calculate what is
required surgically to shift the position of the null point
(102,103). The Anderson-Kestenbaum procedure not only
shifts and broadens the null zone, it decreases nystagmus
intensity outside of the null zone, and may improve head
posture (104–106).
A second surgical approach, suitable for patients whose
nystagmus suppresses with convergence, aims to diverge the
eyes, thereby requiring the patient to converge during far
viewing (107,108). Some surgeons have reported that
365
 State-of-the-Art Review
FIG. 3. A 3-lens image-shifting device that nulls the visual
effects of ocular oscillations. The line diagrams summa-
rize the optical principles used in the device. Starting with
the eyes and optics in a neutral position (upper panel),
light from a distant target is brought to the fovea of the
retina. If the eye is rotated downward (middle panel), the
image is displaced from the fovea. However, if the central
lens is moved downward by the appropriate amount (lower
panel), the image is brought back onto the fovea (97).
combining the Anderson-Kestenbaum operation with a di-
vergence procedure may produce a better visual outcome
than either alone (102,107,109).
A third approach involves large recessions (weakening)
of the horizontal rectus muscles, which may cause im-
provement of vision and head posture (110–115). However,
experimental procedures to weaken the extraocular muscles
induce adaptive changes that restore muscle force (116).
Such changes might cause the nystagmus to increase in
severity following an initial improvement. Thus, controlled
studies are required to evaluate the long-term effects of this
recession approach.
An observation of Dell’Osso (117), that some suppres-
sion of nystagmus and broadening of the null zone follows
almost every surgical procedure for INS, led to the
366
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FIG. 4. Electro-optical device with servo-controlled optics
to measure ocular oscillations and negate their effects.
The central movable lens is positioned by 2 voice coil
motors (contained in the lower ‘‘arms’’ of the x-shaped
optics package). Lens position is sensed by 2 precision
sensors contained in the upper arms of the package and
fed back to the motor drive electronics. Eye position is
sensed by a miniature video camera contained in the
housing behind the visible optics. This device can com-
pensate for eye oscillations with amplitudes up to 65
degrees. The optics package is heavy enough to require
external support, making it more appropriate as an office-
based tool than as a portable treatment device (courtesy
of John S. Stahl, MD, PhD).
suggestion that simply detaching the muscles, dissecting the
perimuscular fascia, and reattaching them (‘‘tenotomy and
reattachment’’) at the same site on the globe might suppress
INS. Experimental studies using a canine model support
this hypothesis (118). The operation may have its effects
by disrupting extraocular proprioceptive feedback signals
(119). Recent work has shown that the brain not only
receives proprioceptive inputs from extraocular muscles
(120), but appears to use that information at a cortical level
(121). Clinical trials have indicated that some patients
treated with tenotomy and reattachment show improve-
ment in some measures of visual and ocular motor function
following horizontal rectus surgery (122–124), but not all
reports agree (125).
Aside from the need to conduct masked trials, other
challenges to evaluate the effectiveness of surgical therapies
for INS arise from the inherent variability of the nystagmus
waveform and the complex relationship between waveform
and visual acuity in any 1 individual. Thus, measurements
of the duration of the foveation period from eye movement
records (126) may appear to improve more with surgery
than do conventional measurements of visual acuity, which
Thurtell and Leigh: J Neuro-Ophthalmol 2010; 30: 361-371

is highly variable in INS. Carefully selected patients with
INS may benefit from surgical treatments that are geared to
their individual visual and ocular motor findings: 1) if there
is a narrow eccentric null zone, then the Anderson-
Kestenbaum operation should be considered; 2) if the
nystagmus is greatly reduced with convergence, then a bi-
lateral medial rectus recession procedure often damps the
nystagmus; and 3) if neither of these conditions apply, then
tenotomy and reattachment may help some patients by
broadening the null zone. Patients with INS and associated
afferent visual system abnormalities, such as oculocutaneous
albinism, are less likely to benefit from surgery (127).
Extraocular muscle surgery has also been tried as
a treatment for acquired nystagmus, either alone or in
combination with medication therapy, sometimes with
success (52,128–131). However, formal clinical trials are
needed to determine whether surgery has a role in the
treatment of acquired nystagmus.
BOTULINUM TOXIN
Botulinum toxin has been injected into the extraocular
muscles or retrobulbar space to temporarily reduce or abolish
acquired nystagmus (132,133). Although some patients have
reported improved vision (134–137), common side effects
include ptosis and diplopia, which are usually more trou-
blesome to the patient than were the visual consequences of
the nystagmus itself. Less often, botulinum toxin has been
used to treat infantile or latent nystagmus (138,139).
Another drawback of botulinum toxin treatment for
nystagmus is that it also impairs normal eye movements
(140,141). Compromised function of the VOR causes
patients to complain of blurred vision or oscillopsia when
they walk. In patients who habitually view with their in-
jected (paretic) eye, adaptive changes may take place such
that the nystagmus increases in the noninjected eye (135).
Thus, botulinum toxin may abolish nystagmus and
improve vision in some patients and may be acceptable to
patients who are prepared to view monocularly. However,
its limited period of action and side effects limit its ther-
apeutic value.
OTHER TREATMENT APPROACHES
After the observation that wearing contact lenses may
suppress INS (86), it was documented that electrical
stimulation or vibration over the forehead may suppress
the oscillations in some patients (142). Such effects may be
exerted via the trigeminal system, which receives afferent
(proprioceptive) signals from the extraocular muscles (143).
Acupuncture to the neck muscles may suppress INS in some
patients, perhaps by a similar mechanism (144,145). Bio-
feedback has also been reported to help some patients with
this condition (146,147), but without sustained effects
(148). At present, a definite benefit from any of these
treatments is yet to be demonstrated via controlled trials.
Thurtell and Leigh: J Neuro-Ophthalmol 2010; 30: 361-371
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State-of-the-Art Review
FUTURE DEVELOPMENTS
As more becomes known about the pharmacology of the
ocular motor system, new medications may emerge for the
treatment of acquired and infantile forms of nystagmus.
Ideally, these drugs should be evaluated in controlled
masked trials. Better understanding of the proprioceptive
control of eye movements may make it possible to hone
surgical treatments, such as tenotomy and reattachment
(149), or even develop medication therapies that act at the
insertions of the extraocular muscles (119). Since INS ap-
pears to be genetically determined in many individuals
(150), specific treatment directed toward the abnormal
protein or channel may be effective. Gene therapy offers
great promise for those individuals with hereditary retinal
disorders that are associated with nystagmus (83). In re-
fractory acquired forms of nystagmus, electro-optical de-
vices may negate the visual consequences of the nystagmus if
individualized digital filtering of nystagmus waveforms can
be achieved and the devices can be miniaturized (97).
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