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iplopiaseeing doubleis a symptom with many potential causes, both neurological and
ophthalmological. Accurate diagnosis and appropriate plans of management can be
achieved with careful history taking and clinical examination. In this article we review the
practical points for clinicians dealing with diplopia. The general approach is demonstrated in fig 1.
A diagnosis of functional diplopia should not be entertained based simply on the absence of
gross ocular misalignment, because sometimes very subtle misalignment of the ocular axes,
which are difficult to elucidate at the bedside, may require more sensitive tests. On the other
hand, one should not be surprised to see gross ocular misalignment without diplopia as brain
plasticity usually takes over if diplopia is longstanding and the image from one eye is suppressed.
HISTORY TAKING
Careful history taking has an important role in the management of diplopia and details of the
history can be used to focus examination on areas of relevance. The following points in the history
should be elucidated.
Is the diplopia monocular or binocular?
Whether the diplopia is monocular or binocular should be determined first. Should the symptom
of diplopia persist with one eye occluded, the patient has monocular diplopia, the causes of which
are usually ophthalmological with refractive error being the most common (table 1). Binocular
diplopia resolves when one eye is occluded and it is caused by misalignment of the visual axes,
and can be due to many different aetiologies.
It is very important to distinguish monocular from binocular diplopia because their possible
causes differ significantly. Monocular diplopia is usually caused by intraocular pathology,
therefore detailed ophthalmological assessment is required. The characteristics of the images may
help to localise the problem. For example, a combination of glare and impaired visual acuity can
be seen in monocular diplopia caused by cataract. Patients with macular disease causing
monocular diplopia may also describe bent or warped images. Rarely, monocular diplopia
may be seen in disorders of the primary or secondary visual cortex, especially when they are
bilateral or the patient sees more than two images (cerebral polyopia). This group of patients may
also describe palinopsiamultiple images appearing in the visual scene soon after gaze has been
turned away from an object or after it has been removed from view.
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Alignment of the images and the direction that results in the most separation
Questions should be asked to identify if the diplopia is horizontal, vertical, or oblique. Horizontal
diplopia, without vertical separation, is related to the impaired neural control or function of the
medial rectus muscle, the lateral rectus muscle, or both. The direction of gaze that increases the
separation of the images can be helpful in determining which extraocular muscle is involved.
The images are maximally separated when the direction of gaze is in the region of action of the
paretic muscle. For example, left trochlear nerve palsy causes most image separation on right
and downward gaze. Horizontal diplopia that occurs almost exclusively at near distance is
strongly suggestive of convergence insufficiency.
Oblique separation with one image slightly tilted is indicative of either superior or inferior
oblique muscle dysfunction. Pronounced image tilting (90 or 180) may be seen in lateral
medullary syndrome. Aniseikonia, a condition caused by notable discrepancy of the refractive
errors between two eyes, gives rise to diplopia because one image is smaller than the other and
superimposed.
Onset and progression of the symptoms
The onset of diplopia by its very nature is almost always sudden. Therefore, an abrupt onset of
diplopia does not necessarily imply a vascular cause. Progression and the temporal sequence of
symptoms provide much useful information. Diplopia that reaches the maximum image
separation instantly and improves spontaneously is usually caused by a single event, often
NEUROLOGY IN PRACTICE
Diplopia
Monocular diplopia
Repetitive images
Cerebral polyopia
Non-organic
Binocular diplopia
Ghosting image
Improved with
blinking or pinholes
Refractive errors
Retinal disease
No misalignment
Misalignment
Comitant strabismus
Dissmilar image
Macular disease
Marked refractive asymmetry
Incomitant strabismus
Similar images
Constant
Intermittent
Childhood strabismus
Restrictive disease
Acquired
vergence paresis
skew deviation
Cranial neuropathy
Supranuclear disorder
Physiologic
MS
Ocular myopathies
Non-organic
Myasthenia gravis
Neuromuscular junction
disorder
Decompensating
phoria
Figure 1 General approach to diplopia.
Associated symptoms
Periorbital pain or pain on eye movements may be indicative
of inflammatory causes. In the absence of obvious ptosis, a
history of intermittent ptosis should be identified. Visual loss
from optic neuropathy is usually seen in an orbital apex
lesion. However, poor vision does not necessarily indicate
optic nerve involvement because a pure oculomotor nerve
Table 1 Causes of monocular diplopia
c Refractive error
c Corneal disease (for example, irregular astigmatism)
c Iris injury
c Cataract
c Media opacity
c Macular disease
c Primary or secondary visual cortex disorder (usually bilateral
monocular diplopia)
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IO
LR
MR
IR
SO
Left eye
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NEUROLOGY IN PRACTICE
IO
SR
SO
IR
MR
Right eye
NEUROLOGY IN PRACTICE
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NEUROLOGY IN PRACTICE
Sup. rect.
Inf. obl.
Inf. obl.
Sup. rect.
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Sup. rect.
temp
Lat. rect.
Inf. rect.
Inf. obl.
Inf. obl.
Lat. rect.
Inf. rect.
Sup. obl.
nasal
Med. rect.
Med. rect.
Sup. obl.
Sup. obl.
Sup. rect.
Lat. rect.
Lat. rect.
temp
Inf. rect.
Sup. obl.
Inf. rect.
Figure 3 Hess chart plotted from the patients perspective. Range of eye movement is smaller in the right eye, therefore, it is a paretic eye. The
diagnosis is right superior oblique palsy.
INVESTIGATION
The investigation of an adult usually consists of assessment
of their eye position and movement. The Hess chart is
commonly used to provide a pictorial and reproducible record
of the patients eye movements (fig 3). The patient is seated
facing the screen being plotted, with the head centred on the
fixation spot. Each eye is plotted in turn for the central fixation spot and targets at 15 and 30 eccentricity, respectively.
Some general rules for interpreting a Hess chart are as
follows:
c The smaller field belongs to the paretic eye
c Neurogenic pareses will show the largest underaction in
the direction of paretic muscle and the largest over-action
is seen in the contralateral synergist
c Mechanical defects show a compressed field without
obvious over-action.
The advent of clinical imaging, the computed tomographic
(CT) and MRI scan, opened a new era in clinical neurology
and also clinical neuro-ophthalmology. In order to get the
best out of imaging, scans should be requested to look for
specific regions of the brain guided by the clinical findings.
For example, it would be more appropriate to request an MRI
scan of the cavernous sinuses and orbits instead of the whole
brain in a case of cavernous sinus syndrome. For suspected
orbital disorders a thin section CT scan through the orbit may
be more useful than an MRI scan.
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NEUROLOGY IN PRACTICE
Smooth pursuit
Both horizontal and vertical pursuit eye movements can be
examined by instructing the patient to track a small target at
about 1 m distance, while keeping the head still. The target
should be moved at a slow and uniform speed and the
examiner should observe the smoothness of the following eye
movement. Broken pursuit is a non-specific sign if present in
both directions and may occur with fatigue and anticonvulsant drugs. However, if broken pursuit is seen in only
one direction, it may indicate a focal posterior cortical lesion
ipsilateral to the direction of broken pursuit.
Vergence
The vergence system can be examined by asking the patient
to track an object that is gradually brought closer to the
patients nose. Alternatively, vergence eye movement can also
be examined by telling the patient to look to the distance and
then focus onto a close object. Normal subjects should be able
to keep the image single up to the tip of the nose and the
resulting convergence should be accompanied by accommodative reflexes. However, inability to converge is not always
pathologic as many people are not good convergers.
Patients with convergence insufficiency usually present in
early adulthood with eye strain, double vision, and difficulty
maintaining near visual function that may respond to
convergence exercise therapy. This condition is usually
benign but may be caused by closed head trauma or viral
encephalitis. On the other hand, convergence paralysistotal
failure of convergence with normal adduction that results in
constant diplopia at nearusually requires further investigation to exclude midbrain pathology and toxic encephalopathy.
Optokinetic nystagmus (OKN)
The optokinetic system cannot be properly assessed at
bedside because the OKN drum commonly used examines
smooth pursuit and not the optokinetic system. A full-field
revolving striped drum is required to elicit true OKN.
Vestibular system
This is discussed by Davies (see p iv32).
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C
NEUROLOGY IN PRACTICE
eye movements, although impairment of vertical eye movements may also be seen.
Internuclear ophthalmoplegia
A lesion of the medial longitudinal fasciculus (MLF) results
in an internuclear ophthalmoplegia (INO), in which there is a
disturbance of adduction ipsilateral to the side of the lesion
and also nystagmus of the contralateral abducting eye. In a
partial INO, adduction will be slowed but it will be
completely absent in a complete lesion. Convergence should
be examined and intact convergence usually indicates a more
caudal lesion (posterior INO). In contrast, absent convergence is seen in a rostral lesion (anterior INO) as the medial
rectus subdivision of the contralateral oculomotor nucleus
will also be affected. The most common causes of INO
include demyelination and vascular infarction. Bilateral INO
is very suggestive of a demyelinating process and it can cause
in addition impaired vertical pursuit and vestibular eye
movements, and impaired vertical gaze holding with gaze
evoked nystagmus on looking up and down. More extensive
lesions affecting both MLFs and the abducens nucleus on one
side only results in a one-and-a-half syndrome. The only
preserved horizontal eye movement is abduction of the
contralateral eye. The main causes of one-and-a-half syndrome are brainstem ischaemia, haemorrhage, and tumour.
Abnormalities of vertical eye movements
Disturbances of vertical gaze are usually associated with
damage to either the posterior commissure, the vertical
equivalent of the PPRF, the rostral interstitial nucleus of the
medial longitudinal fasciculus (riMLF), and the interstitial
nucleus of Cajal, or a combination of these structures.
Interpretation of limited vertical gaze should be undertaken
with care because restricted upward gaze is seen in the
normal elderly. Downward gaze palsy is usually pathologic
and may be seen in brainstem lesions and certain neurodegenerative disorders such as progressive supranuclear palsy
or adult Niemanns-Pick disease. Dorsal midbrain syndrome
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Central vestibular
c Combination of horizontal,
c Unidirectional
vertical and torsional nystagmus
c Associated with severe vertigo
c Mild or no vertigo
NEUROLOGY IN PRACTICE
saccadic abnormalities is not yet available but these conditions may resolve with time or once the cause has been
treated.
Nystagmus
Nystagmus can be classified into two categories based on the
velocity of both phases. When the slow movement that takes
the eye away from the target is followed by a fast phase, it is
called jerk nystagmus, and if both phases have equal velocity,
pendular nystagmus is observed. Patients with nystagmus
usually have other brainstem, cerebellar, and vestibular
symptoms and signs, which should be considered for
diagnosis purposes. Although nystagmus almost always
prompts the clinician to identify the cause, symptomatic
treatment of nystagmus is indicated only when oscillopsiaillusion of movement of the seen worldis present.
The most common form of jerk nystagmus is vestibular
nystagmus. It is most frequently a result of damage to the
vestibular apparatus that may be either peripheral (labyrinth,
vestibular nerve) or central (brainstem). These two categories
can be distinguished clinically by careful observation of the
features described in table 2.
Downbeat nystagmus, a jerk nystagmus with the quick
phase moving downwards, is seen in bilateral disturbance of
the cerebellar flocculus and lesions at the cranio-cervical
junction, such as Chiari malformation type 1. Other causes
include cerebellar degeneration, anticonvulsant and lithium
intoxication, and intra-axial brainstem lesions; however, no
cause can be identified in half of the patients. Symptomatic
treatment for downbeat nystagmus is rather limited, but
gabapentin and 3, 4-diaminopyridine are worth trying.
Upbeat nystagmus, when present in the primary position, is
usually associated with focal brainstem lesions in the
tegmental grey matter. The most common causes are multiple
sclerosis, tumour, infarction, and cerebellar degeneration.
Torsional jerk nystagmus is usually seen in combination
with other types of nystagmus. However, pure torsional
nystagmus indicates a lesion of the lateral medulla involving
the vestibular nuclei. Periodic alternating nystagmus (PAN)
is a primary position horizontal nystagmus that changes
direction in a crescendodecrescendo fashion, characteristically every 90 seconds, with a null period of 010 seconds
between each directional change. This condition can be
congenital or associated with various lesions at the brainstem
and cranio-cervical junction, and anticonvulsant intoxication. Baclofen has been shown to be effective in PAN and the
response is usually dramatic.
Gaze evoked nystagmus, a jerk nystagmus present only on
eccentric gaze but not in the primary position, is commonly
seen and has limited localising value. Bilateral horizontal and
vertical gaze evoked nystagmus commonly occurs with
structural brainstem and cerebellar lesions, diffuse metabolic
disorder, and drug intoxication.
Pendular nystagmus can be either congenital or acquired.
The acquired form is usually seen in multiple sclerosis, visual
loss (including unilateral optic neuropathy), brainstem
ischaemia, and spinocerebellar degenerations. It can affect
one eye or both, equally or unequally, and often results in
oscillopsia. When it is present in association with palatal
myoclonus, oculopalatal myoclonus, the lesion usually
involves the red nucleus, dentate nucleus, and inferior olivary
nucleus (Mollarets triangle). In see-saw nystagmus, one eye
intorts and rises while the other eye extorts and falls in a
..................
Authors affiliations
REFERENCES
1 Miller NR, Newman NJ, eds. Walsh & Hoyts clinical neuro-ophthalmology
the essentials, 5th ed. Baltimore: Williams & Wilkins, 1998.
2 Leigh RJ, Zee DS. The neurology of eye movements, 3rd ed. Oxford University
Press, 1999.
3 Liu G, Volpe N, Galetta S. Neuro-ophthalmology: diagnosis and
management, Saunders, 2000.
4 Kennard C. Normal and abnormal eye movements. In: Luxon L, ed. A textbook
of audiological medicine: clinical aspects of hearing and balance. London:
Martin Dunitz, 2003:78196.
5 Leigh RJ, Kennard C. Using saccades as a research tool in the clinical
neurosciences. Brain 2004;127(Pt 3):46077.
6 Stahl JS, Plant GT, Leigh RJ. Medical treatment of nystagmus and its visual
consequences. J R Soc Med 2002;95:2357.
7 Halmagyi GM, Rudge P, Gresty M, et al. Treatment of periodic alternating
nystagmus. Ann Neurol 1980;8:60911.
8 Strupp M, Schuler O, Krafczyk S, et al. Treatment of downbeat nystagmus with
3, 4-diaminopyridine: a placebo-controlled study. Neurology
2003;61:16570.
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