Добавил:
kiopkiopkiop18@yandex.ru t.me/Prokururor I Вовсе не секретарь, но почту проверяю Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз: Предмет: Файл:

Ординатура / Офтальмология / Английские материалы / Pickwell's Binocular Vision Anomalies 5th edition_Evans_2007

.pdf
Скачиваний:
0
Добавлен:
28.03.2026
Размер:
6.78 Mб
Скачать

18 PICKWELL’S BINOCULAR VISION ANOMALIES

Table 18.1 Clinical observations of nystagmus (modified after Grisham 1990)

 

Characteristic

Observations

 

 

 

 

 

General observations

General posture, facial asymmetries, head posture

 

Type of nystagmus

Pendular, jerk, or mixed (N.B. this is the apparent

 

 

 

type, possibly different to the actual type as

 

 

determined by eye movement recording)

 

Direction

Horizontal, vertical, torsional, or combination

 

 

Amplitude

Small ( 2°), moderate (2–10°), large ( 10°;

 

 

 

cornea moves by more than 3 mm)

 

Frequency

Slow ( 0.5 cycles per second (Hz)), moderate

 

 

 

(0.5–2 Hz), fast ( 2 Hz)

 

Constancy

Constant, intermittent, periodic

 

 

Conjugacy

Conjugate (both eyes’ movements approximately

 

 

 

parallel), disjunctive (eyes move independently) or

 

 

monocular

 

Latent component

Does nystagmus increase or change with occlusion

 

 

 

of one eye? If so, does it always beat towards the

 

 

uncovered eye?

 

Field of gaze changes

Null point: does nystagmus increase or decrease in

 

 

 

any field of gaze or with convergence?

 

 

 

 

Evaluation

Children with new nystagmus, or nystagmus that has not been previously investigated, should be referred. It is disturbing that half of children diagnosed as having CN receive neither visual evoked potential or electroretinogram testing (Budge & Derbyshire 2005). These tests are required for the full evaluation of CN, and optometrists’ referrals should therefore recommend that the patient should be seen in a tertiary centre with the appropriate facilities (e.g. Great Ormond Street Hospital or an eye hospital with paediatric facilities). An important clinical judgement for the optometrist is whether the nystagmus is congenital, latent or acquired. The characteristic features of these conditions are summarized in Table 18.2 to help with differential diagnosis.

Management

 

 

 

There is no cure for nystagmus and an apparent improvement following

 

330

 

intervention for any condition could be attributable to a placebo effect.

 

 

Patients with CN may be particularly vulnerable to placebo effects, since

NYSTAGMUS 18

Table 18.2 Characteristic features of congenital, latent and acquired nystagmus to aid differential diagnosis

Congenital nystagmus

Latent nystagmus

Acquired nystagmus

 

 

 

Presents in first 6 months

Usually presents in first

Onset at any age and

of life

6 months of life and almost

usually associated

 

always in first 12 months

with other symptoms

 

 

(e.g. nausea, vertigo,

 

 

movement or balance

 

 

disorders)

Family history often present (X-linked or, less commonly, autosomal modes of inheritance)

May be family history of

History may include

underlying cause (e.g.

head trauma or

congenital esotropia)

neurological disease

 

such as cerebellar

 

degeneration or

 

multiple sclerosis

Oscillopsia absent or rare

Oscillopsia absent or rare

Oscillopsia common;

under normal viewing

under normal viewing

may also have

conditions

conditions

diplopia

 

 

 

Usually horizontal;

Always horizontal and, on

Oscillations may be

although small vertical

monocular occlusion,

horizontal, vertical or

and torsional movements

saccadic, beating away from

torsional depending

may be present. Pure

the covered eye

on the site of the

vertical or torsional

 

lesion

presentations are rare

 

 

 

 

 

The eye movements are

Oscillations are always

Oscillations may be

bilateral and conjugate

conjugate

disconjugate and in

to the naked eye

 

different planes

 

 

 

Eye movement recordings

Decelerating slow phase

Jerk, pendular or saw-

show accelerating slow

 

toothed waveform

phase

 

 

 

 

 

May be present

Usually occurs secondary to

Results from

with other ocular

an early-onset interruption

pathological lesion or

conditions: albinism,

of binocular vision,

trauma affecting

achromatopsia,

particularly congenital

motor areas of brain

aniridia, optic atrophy

esotropia; may be associated

or motor pathways

 

with dissociated vertical

 

 

deviation (p 136)

 

A head turn may be present, usually (Repka 1999) to utilize a null zone, although nystagmus is present in all directions of gaze

May be a head turn in the direction of the fixating eye

There may be a gaze direction in which nystagmus is absent, and a corresponding head turn

(continued) 331

18 PICKWELL’S BINOCULAR VISION ANOMALIES

Table 18.2 (continued )

Congenital nystagmus

Latent nystagmus

Acquired nystagmus

 

 

 

Intensity may lessen on convergence but is worse when fatigued or under stress

More intense when the fixating eye abducts, less on adduction

Pursuit and optokinetic

Peripheral vestibular disease

reflexes may be

(e.g. Ménière’s disease)

‘inverted’

usually generates linear slow

 

phases and worsens if fixation

 

is removed

 

 

patients are likely to become more relaxed with each subsequent measurement of their visual acuities, causing an improvement simply because they are less stressed. It is therefore important that any treatment for nystagmus should be evaluated with double-masked randomized placebo-controlled trials (RCTs). A recent review (Evans 2006b) revealed only one RCT of a nystagmus treatment, intermittent photic stimulation (Evans et al 1998). One other intervention, the use of contact lenses, has been shown, by an elegant experimental design, to be very likely to be more than just a placebo (Dell’Osso et al 1988). Other research and theories described in this section await validation with RCTs and should therefore be considered as unproven.

Even when an improvement is shown during or immediately after treatment, the patient has only really been helped if this improvement transfers into everyday life.

Goals of the treatment of nystagmus and aetiology of poor vision

 

 

 

The four goals of nystagmus treatment are to improve the visual acuity, to

 

 

 

improve the cosmesis from the ocular oscillation, to improve the cosmesis

 

 

 

from any abnormal head position and (principally in acquired nystagmus)

 

 

 

to reduce oscillopsia. An informal survey by me at an open day of the

 

 

 

Nystagmus Network demonstrated that the first of these, an improvement in

 

 

 

visual acuity, was by far the highest priority of most people with nystagmus.

 

 

 

Whatever the underlying aetiology of the nystagmus, some of the

 

 

 

reduced visual acuity is likely to be attributable to the constant oscillation

 

 

 

of the eyes, with the reduced foveation time (Bedell et al 1989). Treatment

 

 

 

of this motor element should be aimed not just at reducing the nystagmus

 

 

 

but also at changing the waveform to one (pseudocycloid) with a longer

 

 

 

percentage foveation time per cycle (Dickinson & Abadi 1985). Since CN

 

 

 

occurs during the sensitive period, this reduced acuity can cause meridional

 

332

 

amblyopia (Abadi & King-Smith 1979). As the child becomes older the

 

 

amplitude of nystagmus usually reduces (Harris 1997a), so that the residual

NYSTAGMUS

18

reduced vision may be attributable in part to the ocular oscillation and in

 

 

part to amblyopia that occurred secondary to the oscillation (Spierer 1991,

 

 

Chung & Bedell 1995, 1996).

 

 

One interesting feature of CN is that most patients do not experience

 

 

oscillopsia: they are unaware that their eyes are ‘wobbling’ (Bedell 1992).

 

 

This is in most respects advantageous but the lack of feedback about their

 

 

nystagmus might be one reason why they are unable to control their ocular

 

 

oscillations (Abplanalp & Bedell 1983). Many forms of putative treatment

 

 

aim to provide this feedback.

 

 

Refractive management: spectacles and contact lenses

 

 

Patients with CN often have better vision with contact lenses than with

 

 

spectacles. The improvement may be attributable to optical factors and to

 

 

the contact lenses providing a form of biofeedback (Abadi 1979). The lenses

 

 

seem to provide tactile feedback from the inner eyelids that dampens CN

 

 

and results in better acuity (Dell’Osso et al 1988). Dell’Osso et al’s study used

 

 

soft lenses, although one might expect a greater improvement from rigid

 

 

lenses and this concurs with clinical experience at the Institute of Optometry.

 

 

It is possible that, when the lenses are removed, there may be a ‘rebound

 

 

phenomenon’ of dizziness and oscillopsia for 5–20 min (Safran & Gambazzi

 

 

1992). This phenomenon appears to be rare.

 

 

Dell’Osso (1994) recommended that patients with a convergent null pos-

 

 

ition could benefit from prisms with –1.00 overcorrection to create accom-

 

 

modative-convergence. In other cases, pre-presbyopic patients may require

 

 

a positive reading addition (Evans 2001d). The important point is to evalu-

 

 

ate whether patients are capable of binocular single vision and, if so, to care-

 

 

fully investigate the effect of refractive correction on their binocular status

 

 

(Evans 2001d).

 

 

Prismatic

 

 

It was noted above that the intensity of CN is sometimes reduced in near

 

 

vision. This effect is not mediated by convergence or accommodation but is

 

 

determined solely by the angle between the visual axes (either symmetrical

 

 

or asymmetrical): binocular viewing is not necessary (Abadi & Dickinson

 

 

1986). This suggests that one treatment approach, prescribing base-out prisms,

 

 

can help in these cases. This is not a universal treatment: most cases of CN

 

 

do not show a reduced nystagmus at near (Abadi & Dickinson 1986) and

 

 

there may even be an increase in intensity at near in some cases (Ukwade &

 

 

Bedell 1992).

 

 

Some cases of CN benefit from the correction of small vertical devi-

 

 

ations (Evans 2001d). Yoked prisms can also be used in nystagmus to cause

 

 

a version movement so that the eyes look through the null gaze position

 

 

without an anomalous head position, or with a reduced anomalous head

333

 

position.

 

18 PICKWELL’S BINOCULAR VISION ANOMALIES

Eye exercises/vision therapy

Latent nystagmus is often an insuperable barrier to conventional occlusion therapy for strabismic amblyopia. Stegall (1973) reported that the latent nystagmus can be overcome by using a narrow band transmission red filter over the unoccluded eye. He also described two studies that found a reduction in latent nystagmus in the unoccluded eye when a cycloplegic was instilled. In addition to penalization methods, Scheiman & Wick (1994) recommended using anaglyph techniques to treat amblyopia in latent nystagmus and suggested that vision therapy can be effective at reducing latent nystagmus, supporting Healy (1962). Leung et al (1996) reported improvements in a few case studies of CN following vision therapy but there have been no RCTs (Evans 2006b).

Auditory biofeedback

Apparatus has been developed that measures eye movements and translates these into auditory signals (Abadi et al 1980, 1981). Eye movements to the right or left can be converted to sounds in the appropriate earphone and the pitch of the sound made proportional to the magnitude of the eye movement (Abplanalp & Bedell 1983). Case studies and open trials have suggested that treatment using this approach may be effective in albinotic nystagmus (Abplanalp & Bedell 1987), congenital idiopathic nystagmus (Kirschen 1983), sensory defect nystagmus, latent nystagmus and acquired nystagmus (Ciuffreda et al 1982). Clearly, neither the foveal hypoplasia in albinism nor the optic disc hypoplasia in Ciuffreda et al’s (1982) sensory defect nystagmus case can be treated, suggesting that some of the visual loss may have been secondary to the nystagmus, not from the original pathology. There have been no RCTs of auditory feedback (Evans 2006b).

Visual (after-image) biofeedback

A simple form of visual biofeedback can be achieved using an after-image (Stegall 1973, Stohler 1973), and this may be more effective at translating into everyday life than treatment solely based on auditory biofeedback (Abplanalp & Bedell 1983). People with nystagmus usually spontaneously comment that they perceive an after-image to be ‘wobbling’. This movement is related (but not equal in magnitude; Kommerell et al 1986) to their eye movements and it has been suggested that patients can improve their nystagmus by trying to reduce the movement of the after-image (Mallett, personal communication).

An alternative after-image technique (Stegall 1973) is to allow the patient to adopt a head position to reduce the ‘wobble’ as they slowly straighten their head. Goldrich (1981) described a perceptual effect, emergent textual contours, which he claimed allowed nystagmus patients to monitor their nystagmus as an alternative to an after-image method.

Active amblyopia therapy: intermittent photic stimulation

334

People with CN may have some level of amblyopia associated with their

nystagmus (Abadi & King-Smith 1979, Currie et al 1993). Mallett (1983)

 

 

 

NYSTAGMUS

18

0.75

 

 

 

 

 

 

 

Placebo

 

0.65

 

 

Experimental

 

 

 

 

 

0.55

 

 

 

 

VA

 

 

 

 

0.45

 

 

 

 

0.35

 

 

 

 

0.25

Second

Third

Post-treatment

 

First

 

 

 

Assessment

 

 

Figure 18.2 Graph of high-contrast Bailey–Lovie visual acuity (VA) at each research visual assessment (error bars represent 1 standard error of the mean). VA is in LogMAR units, so that smaller figures represent better VA (0.4 represents 6/15 and 0.5 represents 6/18). VA was measured three times before treatment, to investigate the practice effect, and once after treatment. (Reproduced with permission from Evans et al 1998.)

described the use of intermittent photic stimulation (IPS) for the treatment of congenital idiopathic nystagmus. Scheiman & Wick (1994) described a case study where IPS had been used to treat nystagmus successfully. An RCT of this treatment is described below.

Combining treatment approaches

The greatest chance of success will probably be obtained by combining

 

two or more of the above methods. Ciuffreda et al (1982) described a com-

 

bination of auditory and visual biofeedback. Mallett & Radnam (1992)

 

found a combination of after-image feedback and IPS treatment to be opti-

 

mal for congenital (including albinotic) nystagmus.

 

Evans et al (1998) carried out an RCT of the combined treatment described

 

by Mallett & Radnam (1992). They studied 38 subjects, which, according

 

to a statistical sample size calculation, should have been enough for a clin-

 

ically significant treatment effect to reach statistical significance. The

 

visual acuity (VA) and contrast sensitivity (CS) were assessed three times

 

before undergoing treatment for 6 weeks and then once more after treat-

 

ment. An improvement in VA occurred, but this was not significantly dif-

 

ferent in the two groups (Fig. 18.2). The improvement in CS was greater in

 

the experimental than in the control group but the difference failed to

 

reach significance in most statistical tests.

 

Evans et al’s (1998) RCT clearly demonstrates that the improvement in

 

high-contrast VA of the group receiving the experimental treatment is not

335

significantly different to the improvement in those receiving a placebo

18 PICKWELL’S BINOCULAR VISION ANOMALIES

0.4

0.39

0.38

0.37

0.36

0.35

 

 

 

Initial VA

Final VA

 

 

 

Figure 18.3 Bar chart representing the improvement in LogMAR VA of the experimental

 

 

 

group of Evans et al (1998). VA is in LogMAR units, so that smaller figures represent better

 

 

 

VA. Note: This figure deliberately misrepresents the overall results of the study to illustrate

 

 

 

the dangers of researching therapies for CN without using an RCT design (see text).

 

 

 

(Adapted with permission from Evans et al 1998.)

 

 

 

 

treatment. If we just look at the data for the experimental group, we can

 

 

 

investigate what the result of the study would have been if it had been a

 

 

 

non-controlled trial, like most other studies of treatments for nystagmus.

 

 

 

Figure 18.3 illustrates the improvement in VA of the experimental group from

 

 

 

the first VA measurement to the final, post-treatment, assessment. A matched

 

 

 

pairs t-test on the preand post-treatment data in Figure 18.3 shows that the

 

 

 

apparent improvement in VA is statistically significant (p 0.031). Yet Figure

 

 

 

18.2 shows that this improvement is attributable to practice and placebo

 

 

 

effects. This demonstrates the risks of research that does not use an RCT design

 

 

 

and must raise questions about whether any safe conclusions can be drawn

 

 

 

from research in this field that has not employed an RCT (Evans 2006b).

 

 

 

Surgery

 

 

 

 

Abadi & Whittle (1992) showed that, in carefully selected cases of congenital

 

 

 

idiopathic nystagmus with an eccentric null zone, surgery to shift the null

 

 

 

position to the primary position (Kestenbaum procedure) may be effective. A

 

 

 

different approach, based on using a silicone band to create a new anatom-

 

 

 

ical insertion for the recti muscles, may reduce the amplitude but has little

 

 

 

effect on vision (Richman et al 1992). Successive publications on ‘null point

 

 

 

surgery’ seem to recommend ever more surgery and Harris (1997a) recom-

 

 

 

mended reserving this intervention for cases with significant symptoms.

 

 

 

Another technique, ‘artificial divergence surgery’, has been used to reduce

 

336

 

the effect of the medial rectus muscle, resulting in more adduction innerv-

 

 

ation, which, in some cases, reduces the nystagmus (Spielmann 1994). Repka

NYSTAGMUS

18

(1999) reviewed various surgical approaches to nystagmus, which can be

 

 

combined (Graf 2002). An additional recent technique is horizontal rectus

 

 

tenotomy (Hertle et al 2003). A recent review notes that there is a lack of

 

 

RCTs for any of these approaches (Evans 2006b).

 

 

Botulinum toxin can be used as a temporary measure to investigate the

 

 

likely effect of this type of procedure (Spielmann 1994). Botulinum toxin

 

 

can also be injected into two or four recti muscles as a treatment but has

 

 

to be repeated every 4 months and doubts have been raised over this inter-

 

 

vention (Dell’Osso 1994, Repka 1999).

 

 

Other treatment approaches

 

 

Leigh et al (1988) used an electronic device to stabilize the retinal image

 

 

and reduce oscillopsia in patients with acquired nystagmus due to neuro-

 

 

logical disease. This can be used to calculate the power required for a tele-

 

 

scopic contact lens system (high minus contact lens with high plus spectacle

 

 

lens) that provides partial optical stabilization of the retinal image (Yaniglos &

 

 

Leigh 1992).

 

 

Pharmacological agents have been used to treat nystagmus, most com-

 

 

monly acquired nystagmus (Grisham 1990, Richman et al 1992) and may

 

 

also have a role in CN (Shery et al 2006). Other approaches include hypno-

 

 

sis (Chase 1963), electrical or vibratory stimulation of the ophthalmic div-

 

 

ision of the trigeminal nerve (Dell’Osso 1994, Sheth et al 1995) and visual

 

 

stimuli designed from dynamical systems analysis (Abadi et al 1997).

 

 

It was noted above that many people with CN suffer a worsening of their

 

 

nystagmus and visual acuity when they are under stress (e.g. in academic

 

 

examinations). The may be why the placebo effect seems to be so large in

 

 

nystagmus. Hypnosis is ‘an empirically-validated, non-deceptive placebo’

 

 

(Kirsch 1996) and it is possible that this could be used as a sort of ‘focussed

 

 

relaxation’ to help patients whose nystagmus is particularly troublesome

 

 

in certain stressful situations. Once again though, it is stressed that there

 

 

have been no RCTs of the approaches described in this ‘other treatments’

 

 

section (Evans 2006b).

 

 

Counselling

 

 

Three sorts of counselling can be helpful to patients with nystagmus. First,

 

 

as is often the case after an appointment in a busy hospital department,

 

 

patients are discharged with a diagnosis but with many unanswered ques-

 

 

tions (Budge & Derbyshire 2005). If the diagnosis is clear then the commu-

 

 

nity optometrist can explain what the diagnosis means. For example, a

 

 

diagnosis of congenital idiopathic nystagmus does not mean that the infant

 

 

is, or will go, blind. Although the nystagmus will always be present it usually

 

 

reduces a little as the child ages (Harris 1997a) and the level of vision should

 

 

be enough to allow the person to do most everyday activities, usually going

 

 

to a normal school, although driving will probably not be possible.

 

 

The second type of counselling is genetic counselling to discuss whether

337

 

an underlying pathology or idiopathic nystagmus are likely to be passed on

 

18 PICKWELL’S BINOCULAR VISION ANOMALIES

to future generations. This should be provided by appropriate experts, genetic counsellors, who are usually found in major hospitals and who will accumulate the necessary facts before giving their advice.

The third type of counselling is to give patients or their parents advice that will help them deal with the nystagmus on a day to day basis. For example, if there is a null zone in a certain direction of gaze then a child should be allowed to sit at a position in class that takes advantage of this. People with nystagmus or their families can often receive considerable support from talking to other people with the condition. The Nystagmus Network provides this type of support, and has excellent literature for those affected and their family (www.nystagmusnet.org).

Clinical Key Points

The main types are congenital nystagmus (CN; onset in first 6 months), latent nystagmus (usually associated with early onset strabismus) and (more rarely) acquired (neurological) nystagmus

CN can occur secondary to a sensory defect, including albinism, or idiopathic where there is assumed to be a motor defect

The apparent pattern of nystagmoid eye movements cannot be used with certainty to predict the aetiology of the nystagmus or the level of vision

All new cases in adults need referral to a neuro-ophthalmologist; new cases in children need referral ideally to a specialist paediatric hospital eye clinic

Nystagmus often varies over time and at different gaze positions and fixation distances

There may be an anomalous head position so that the gaze is in a ‘null position’

High refractive errors are common and contact lenses often improve vision more than spectacles

Orthoptic problems should be treated if causing symptoms, but patients with albinism are probably unable to exhibit true binocular vision

There is no cure for nystagmus and claims about treatment should be viewed sceptically: the only randomized controlled trial of a treatment for CN showed the therapy to be no better than a placebo

338

APPENDICES

Appendix 1 Confusing aspects of binocular vision tests

There are several potentially confusing aspects of binocular vision tests. This appendix aims to remove some of this confusion and to provide useful mnemonics to help students remember essential ‘rules’.

Binocular vision tests: practitioner’s perspective

Rule

Mnemonic

 

 

 

 

Light is deviated towards the base

Imagine a prism standing on its base with

 

of a prism

light coming along parallel to the ground:

 

 

imagine it being deviated down by gravity

 

 

 

 

If eye is OUT need base IN,

OUT – IN IN – OUT

 

i.e. EXO needs base in to correct

IN ‘XS’ (in excess)

 

 

 

 

If eye is UP need base DOWN,

UP – DOWN DOWN – UP

 

i.e. R hyper needs base down

 

 

General rule: (1) Prism base to correct a deviation is in opposite direction to the deviation or (2) ‘the base of the prism must be on the side of the inefficient muscle’ (Percival 1928).

Binocular vision tests: patient’s perspective

Rule

Mnemonic

 

 

EXO deviations give crossed disparity

eXo X cross(ed)

exo: RE image is seen on left of LE image

 

eso: RE image is seen on right of LE image

 

R hyper: RE image is seen below LE image

 

FD: if RE image goes to the left then exo and

 

need base in

 

FD: if RE image goes up then R hypo and

 

need base-up RE

 

Maddox rod: if RE rod goes to left of LE spot

LOSEX: L of spot if exo

then exo and need base in

ROSES: R of spot if eso

Maddox rod: if RE rod goes up, R hypo, need base-up RE

(continued )

340