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Practical Neurology 2011;11:332–340. doi:10.1136/practneurol-2011-000026
Blepharoptosis, ‘the fallen eyelid’, is a
clinical sign that neurologists encoun-
ter regularly. Knowledge of the anat-
omy and the normal physiology of the
eyelid makes it easier to understand
the various ways in which ptosis may
present. The aetiology of ptosis can
be divided into structural abnormali-
ties affecting the eyelid muscles and/
or surrounding tissues in the orbit,
myogenic causes, neurogenic causes,
disorders of the neuromuscular junc-
tion and central causes. Differentiating
between these causes can often be
achieved by a carefully directed his-
tory and examination. Investigation
depends on the clinical assessment
and hence the likely underlying cause.
Treatment is usually directed at the
underlying pathology but occasionally
oculoplastic surgery is appropriate.
This review summarises these aspects
and provides a guide to the clinical
assessment of ptosis.
Ptosis is a lowering of the eyelid
to below its normal position. The
word ‘ptosis’ derives from the Greek
πτωσις’, which translates as ‘to fall’.
It is an abbreviation of ‘blepharopto-
sis’—a fallen eyelid—but this longer
version is now almost never used. The
normal palpebral fissure measures
12–15 mm. The distance between
the corneal light reflex and the upper
eyelid margin is termed the upper
marginal reflex distance. These two
measurements are used for objective
assessment of ptosis (figure 1). The
official definition of ptosis is an upper
marginal reflex distance below 2 mm
or an asymmetry of more than 2 mm
between the eyes. Ptosis has many
causes and is a presenting symptom
in both emergency and outpatient
settings. While most ptosis presents
to ophthalmologists, neurologists
often see cases in day to day practice.
Ptosis may point towards something
as dramatic as a leaking aneurysm or
something as mundane as a soft tissue
injury from rubbing the eye. Careful
clinical assessment will prevent unnec-
essary investigations but may also save
a patient’s life. This review focuses on
acquired ptosis in adults, starting with
an overview of the normal anatomy
and function and then a discussion of
possible causes. We end with a sug-
gested clinical approach to the patient
with ptosis.
Anatomy and function
The eyelid anatomy is shown in fig-
ure 2. The levator palpebrae supe-
rioris (LPS) is the primary muscle
responsible for lid elevation. It arises
from the back of the orbit and extends
forwards over the cone of eye muscles.
It inserts into the eyelid and the tarsal
plate, a fibrous semicircular structure
which gives the upper eyelid its shape.
The LPS is supplied by the superior
division of the oculomotor nerve. The
way that the LPS attaches to the tar-
sal plate is modified by the underly-
ing Müller’s muscle. This involuntary
muscle, comprising sympathetically
innervated smooth muscle, has the
capacity to ‘tighten’ the attachment
and so raise the lid a few millimetres.
Two other muscles affecting the final
position of the eyelid are the frontalis
muscle and the orbicularis oculi, both
supplied by the facial nerve. Frontalis
contraction helps to elevate the lid
by acting indirectly on the surround-
ing soft tissues, while orbicularis oculi
contraction depresses the eyelid.
The eyelid is held open when awake
by the tonic action of LPS, punctu-
ated intermittently by blinks. The
upper eyelid normally covers the top
Department of Clinical
Neurosciences, Western General
Hospital, Edinburgh, UK
Department of Ophthalmology,
Princess Alexandra Eye Pavilion,
Edinburgh, UK
Department of Neurology, The
Canberra Hospital and Australian
National University, Canberra,
Correspondence to
Dr K Ahmad, Department of
Clinical Neurosciences, Western
General Hospital, Edinburgh
EH4 2XU, UK;
Kate Ahmad,
Mark Wright,
Christian J Lueck
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Practical Neurology 2011;11:332–340. doi:10.1136/practneurol-2011-000026
retraction or lid lag. Fatigability is assessed by
detecting any lowering of the eyelid during sus-
tained upgaze for at least 60 s.
Causes of ptosis
The commonest cause of ptosis is disinsertion of
the LPS tendon from the tarsal plate (lid dehis-
cence or aponeurotic ptosis). This causes the
whole eyelid to sit low, even though the normal
range of movement is preserved. It is important to
distinguish this condition from ptosis due to mus-
cle weakness. Ptosis may also result from reduced
activity of the muscles that elevate the eyelid. In
some situations, the lid may appear lower than
normal because of structural abnormalities affect-
ing either the eyelid itself or the surrounding orbit,
or because of active contraction of the orbicularis
oculi. Table 1 summarises the causes of ptosis but
we briefly discuss each in turn.
Structural causes
It is possible for an eyelid with normal muscle
function to appear to sit lower than normal. This
20% of the cornea but its exact position is deter-
mined by several factors. It rises and falls with
vertical eye movements but it is also affected by
the horizontal position of the eye, being slightly
lowered when the eye is either adducted or
abducted. The state of arousal influences lid posi-
tion: fatigue is associated with reduced LPS activ-
ity but the overall level of sympathetic activity
affects the tone in Müller’s muscle so the eyelid
sits slightly higher in circumstances associated
with increased arousal.
The range of eyelid movement from full ele-
vation to closure (‘eyelid excursion’) is usually
greater than 12 mm.
This range can easily be
measured and forms an important part of the
assessment of any patient with ptosis (figure 1).
LPS function is assessed by measuring the dif-
ference in eyelid margin position in upgaze and
downgaze (while holding the eyebrow down to
prevent frontalis activity). The patient should
then be asked to pursue an object slowly from
upgaze to downgaze in order to detect lid
Figure 1 Measurement of eyelid excursion. The top left image shows the margin of the closed eyelid at a level of 20 mm. When the
eyelids are fully elevated (bottom left), this increases to 33 mm, giving a normal eyelid excursion of 13 mm. The right-sided image
shows a normal eyelid crease (A), upper marginal refl ex distance (B) and palpebral fi ssure (C).
Figure 2 Anatomy of the upper eyelid. (Reprinted from
Spalton D et al
with permission.)
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Practical Neurology 2011;11:332–340. doi:10.1136/practneurol-2011-000026
usually results from structural problems but can
be from overactivity of eye closure. Structural
problems can include those affecting the globe,
the eyelid or the tissues above the eye.
Any process causing the eye to retract into the
orbit may give the appearance of ptosis because
the eyelid slips down over the retracted eye.
Congenital microphthalmos rarely presents to
the adult neurologist but Duane’s retraction
syndrome may do so. This is a congenital ocu-
lar motility disorder which causes restriction of
eye movement, usually of adduction. It is char-
acterised by globe retraction during movement
in the opposite direction to the restriction, due
to co-contraction of extraocular muscles.
retraction narrows the palpebral fissure. Of the
acquired conditions causing globe retraction,
damage to the orbital floor by trauma or malig-
nancy is the most common. Note that extraoc-
ular muscle palsy impairing elevation may also
give the appearance of ptosis.
Any condition causing the upper eyelid to swell
may cause ptosis, as the enlarged lid narrows
the palpebral fissure. Inflammation or infection
is usually relatively obvious, but tumours, most
commonly neurofibromata, may be more subtle.
‘Floppy lids syndrome’ is a recently described
syndrome occurring mainly in overweight men
with obstructive sleep apnoea and/or hyperten-
The normal stiffness of the upper eyelids is
reduced and they are easily everted. The lids are
often lengthened, giving the appearance of pto-
sis, and there is often accompanying conjunctival
irritation due to eyelashes pointing in the wrong
direction (‘lash ptosis’). Note that any cause of
lid elevation in the contralateral eye may give the
appearance of ptosis.
Surrounding tissues
In dermatochalasia, the upper orbital skin becomes
lax and hangs over the true eyelid, sometimes even
impairing vision. This is not uncommon in elderly
people and is easily mistaken for ptosis.
Table 1 Causes of ptosis
1. Congenital
Isolated congenital ptosis
Congenital myasthenic syndromes
Transient neonatal myasthenia (myasthenic mother)
Anomalous synkineses (eg, jaw winking)
Blepharophimosis and branchial arch syndromes
2. Structural
Levator dehiscence (age, trauma, contact lenses)
Eyelid or orbital tumours
Eyelid swelling (allergy, infection)
3. Sympathetic supply (Horner’s syndrome)
4. Muscle/neuromuscular junction
Myasthenia gravis
Myotonic dystrophy
Chronic progressive external ophthalmoplegia
Other mitochondrial cytopathies
(inherited and acquired)
Oculopharyngeal muscular dystrophy
Graves’ disease
5. Oculomotor nerve
See table 2
6. Central
‘Cortical’ ptosis
Hemifacial spasm
Essential blepharospasm
Apraxia of eyelid opening
7. Miscellaneous
Fatigue, lethargy, drowsiness, coma
Hysteria, ‘functional pseudoptosis’
Figure 3 Levator dehiscence af
fecting the left eye. Note the
eased distance between the lid margin and the skin crease
on that side with preserved range of movement.
Figure 4 Right-sided Hor
s syndrome after lateral medullary
infarction, demonstrating the subtlety of this abnormality.
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Levator dehiscence (aponeurotic ptosis)
This is the most common cause of a lowered eye-
lid. It typically occurs in middle aged to elderly
patients and is caused by a disinsertion of the LPS
tendon from the tarsal plate. As a result, the lid
sits lower than normal but has a normal range of
movement. Lid dehiscence may appear relatively
suddenly following trauma or ocular surgery
(usually cataract extraction) but its onset is usu-
ally subacute in the elderly. It can be caused by
wearing hard contact lenses and may even follow
rubbing the eye. Clinically, it is characterised by
a high skin crease (more than 7 mm from the lid
margin) and a rather thinned eyelid but a rela-
tively normal range of lid movement (figure 3).
Failure to recognise this diagnosis may result in
unnecessary tests. It can be corrected by a simple
surgical procedure if functionally disabling.
Reduced sympathetic activity
Any condition interrupting the sympathetic sup-
ply to Müller’s muscle may cause ptosis. This
almost always occurs in the context of a Horner’s
syndrome (figure 4). The ptosis is only ever partial
and is often very subtle, as the maximum excur-
sion of the muscle is 3 mm. It may be accompa-
nied by minor elevation of the lower lid due to
involvement of the much smaller sympathetically
innervated muscle in the lower eyelid; this can
give the eye a ‘sunken’ appearance. The pres-
ence of other features of a Horner’s syndrome
(ie, a miotic pupil, anhidrosis and facial vaso-
motor abnormalities) depend on the site of the
lesion. With congenital or very chronic lesions,
there may be iris depigmentation. Lesions caus-
ing Horner’s syndrome are traditionally classified
according to the three neurons involved in the
sympathetic supply (hypothalamus to spinal cord,
spinal cord to sympathetic ganglion and sympa-
thetic ganglion to end organ). Lesion localisation
is often determined by the presence or absence
of accompanying clinical abnormalities and by
radiological investigation but pharmacological
testing of the pupil is occasionally useful. Further
investigation and treatment (if any) depend on
the likely site of the lesion. A comprehensive list
and details of pharmacological pupil testing can
be found elsewhere.
The conditions causing myogenic or neurogenic
ptosis are most easily considered on the basis
of anatomy. We discuss these in terms of lesions
affecting muscle, the neuromuscular junction, the
oculomotor nerve and the CNS.
Several conditions affect the LPS itself. These are
often bilateral and may also affect the extraocu-
lar muscles, causing disordered eye movements.
If the onset is slow, as in many of the congenital
syndromes, patients are often unaware of their
ptosis and may not experience diplopia, even
though their eyes are manifestly malaligned. As
the ptosis becomes more severe, patients increas-
ingly have to adopt a characteristic backward
head tilt to prevent the ptotic lids from obscur-
ing vision.
Mitochondrial disorders
Because the eyelids and extraocular muscles have
a relatively large number of mitochondria due to
high levels of metabolic activity, mitochondrial
disorders often cause ptosis. The most common
of these is chronic progressive external ophthal-
moplegia, in particular Kearns–Sayre syndrome,
although mitochondrial neurogastrointesti-
nal encephalopathy (MNGIE), mitochondrial
encephalomyopathy, lactic acidosis and stroke-
like episodes (MERRF) and several other mito-
chondrial function mutation disorders may cause
Figure 5 Myasthenia gravis: before treatment (left) and after 3 days of intravenous immunoglobulin (right). Note previous
blepharoplasty scars.
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Figure 6 Ptosis props are rarely effective.
Figure 7 Partial right oculomotor nerve palsy with mild ptosis,
mydriasis and a ‘down and out’ eye.
Table 2 Causes of oculomotor nerve palsy (reprinted from Lueck C
with permission)
Common Uncommon Rare
Localisation by anatomical site
Fascicular Head trauma Brainstem tumour
Brainstem infarction Brainstem haemorrhage
Multiple sclerosis
Subarachnoid Head trauma Neurosurgical complication Arteriovenous malformation
Aneurysm (posterior
communicating artery)
Aneurysm (basilar artery, superior cerebellar
Ectatic basilar or posterior communicat-
ing artery
Tentorial herniation
Subarachnoid haemorrhage Oculomotor nerve tumour
Meningitis (infective, infl ammatory or neoplastic) Post lumbar puncture/ myelography
Hypertrophic pachymeningitis
Superfi cial siderosis
Idiopathic intracranial hypertension
Cerebral venous sinus thrombosis
Cavernous sinus/
superior orbital fi ssure
Head trauma Carotico-cavernous fi stula Pituitary apoplexy
Tumour, lymphoma
Internal carotid artery aneurysm, occlusion or
Cavernous sinus thrombosis
Tolosa–Hunt syndrome
Wegener’s granulomatosis
Herpes zoster
Orbit Head trauma Tumour, lymphoma Infections (eg, mucor mycosis)
Retrobulbar anaesthesia
Paget’s disease of skull
Localisation uncertain
Congenital ‘Congenital oculomotor nerve palsy’ Aplasia
Congenital fi brosis of the extraocular
Vascular Nerve infarction (diabetes mellitus,
hypertension, etc)
Nerve infarction (giant cell arteritis,
Carotid artery stenosis
Protein S defi ciency
Hyperviscosity syndrome
Cocaine, sildenafi l
Infl ammatory/infective Sarcoidosis Sjögren’s syndrome, Systemic lupus
Ophthalmoplegic migraine
Metabolic/toxic Wernicke’s encephalopathy
Drug/substance toxicity
Miscellaneous Idiopathic Dental anaesthesia
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ptosis. Because some mitochondrial proteins are
coded for by mitochondrial DNA while others are
coded for by nuclear DNA, some of these con-
ditions are inherited maternally while others are
inherited autosomally. In those that are mater-
nally inherited, there may be a variable degree of
penetrance due to heteroplasmy. There is often a
family history but the absence of affected relatives
does not exclude mitochondrial disease. Patients
typically present in childhood or early adult life
with progressive bilateral ptosis and ophthal-
moparesis. The abnormalities may be restricted
to the eyes and eyelids, or there may be more
generalised abnormalities such as myopathy, sco-
liosis, pigmentary retinopathy and cardiac con-
duction defects (as in Kearns–Sayre syndrome). It
is now possible to detect many genetic causes of
mitochondrial dysfunction on blood tests. If these
are negative but mitochondrial disease is still sus-
pected, further investigation in the form of mus-
cle biopsy may be appropriate because histology
may show abnormalities of mitochondria—for
example, ragged red fibres—and further genetic
testing may be possible on the muscle tissue itself.
Importantly, mitochondrial dysfunction may
be acquired, particularly from drug treatment.
A recent report highlighted ptosis occurring in
patients treated with highly active antiretroviral
treatment for HIV.
Muscular dystrophies
The eyelids are usually spared in the dystrophi-
nopathies and the limb girdle group of muscu-
lar dystrophies. However, some other muscular
dystrophies are associated with ptosis. People
with myotonic dystrophy, particularly DM1,
commonly have ptosis. It is usually mild but can
become severe enough to impair vision as the dis-
ease progresses. If ptosis occurs in facioscapulo-
humeral dystrophy, it is a late finding despite facial
weakness being characteristic. Oculopharyngeal
muscular dystrophy is a rare condition due to
abnormalities of the polyadenine binding protein
nuclear 1 gene on chromosome 14q11. It is char-
acterised by progressive ptosis, dysphagia and
proximal limb weakness.
Symptoms usually start
in mid-life and are commonly initially mistaken
for ocular myasthenia gravis (MG, see below).
Several infantile onset muscular dystrophies,
including muscle–eye–brain disease, can cause
ptosis; these conditions are associated with other
severe neurological abnormalities and are rarely
encountered by adult neurologists.
Myopathies rarely affect the eyelid. Most drug
induced myopathies affect large muscles and
ptosis would be highly atypical. Inflammatory
myopathies, including polymyositis, dermatomy-
ositis and inclusion body myositis almost never
cause ptosis. Isolated ocular myositis is rare but
may result in ptosis if the LPS/superior rectus
muscle complex is affected. Rarely, amyloidosis
may affect the LPS, causing a (usually unilateral)
ptosis, often preceded by an orbital mass.
Dysthyroid eye disease
This is typically associated with lid retraction.
However, it can cause ptosis, usually through
mechanical disruption of the LPS as it is stretched
by the proptosed globe and hypertrophied mus-
cles. The diagnosis is usually fairly obvious in a
patient with disease severe enough to cause pto-
sis. However, also consider MG if a patient with
hyperthyroidism develops ptosis.
Neuromuscular junction
It is well known that patients with MG often
have ptosis (figure 5), which may be demonstra-
bly fatigable. Several signs in MG relate to eyelid
function, including Cogan’s lid twitch sign, cur-
taining and enhanced ptosis. Cogan’s lid twitch
is seen after a patient looks down. It appears as a
brief overshoot of the upper eyelid as the patient
attempts to look back up to the primary position
(straight ahead). This means that the sclera is
transiently visible above the cornea. ‘Curtaining’
Figure 8 Mild right-sided ptosis in Miller Fisher syndrome. Note the mid-dilated pupils and the left upper lid retraction as attempted
compensation for the ptosis. (B) The ptosis worsens when the retracted lid is elevated manually, so-called enhanced ptosis.
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and ‘enhanced ptosis’ refer to the phenomenon
of contralateral eyelid lowering when an eyelid is
manually elevated. ‘Curtaining’ occurs when the
ptotic eyelid is elevated resulting in a ptosis of
the normal eyelid. ‘Enhanced ptosis’ occurs when
the normal eyelid is lifted resulting in further
drooping of the ptotic eyelid. Both are explained
by Hering’s law of equal innervation to paired
yoke muscles (ie, the eyelids). Note that these
signs are not specific to myasthenia and may be
seen in other conditions, including Miller Fisher
syndrome (figure 6) and botulism (although in
both Miller Fisher syndrome and botulism the
pupils are often involved, and typically dilated).
Lambert–Eaton myasthenic syndrome is fre-
quently associated with ptosis but almost never
in isolation or as the presenting feature.
Congenital myasthenic syndromes
This is a heterogenous group of rare disorders of
the neuromuscular junction, usually inherited in
an autosomal recessive pattern. Fatigable ptosis is
very common. Multiple identified genetic defects
can result in acetylcholine receptor deficiency, ace-
tylcholinesterase deficiency or kinetic abnormali-
ties of the acetylcholine receptor ion channels.
The clinical phenotype depends on the genotype,
and each type may also have characteristic neuro-
physiological features. These syndromes usually
present in childhood but occasionally in adult-
hood when they may mimic MG or a muscular
dystrophy. Congenital myasthenia is worth con-
sidering in seronegative myasthenic patients or
those with an unexplained muscle disease.
Oculomotor nerve palsy is a common presenta-
tion to neurologists. The classical signs are a pto-
sis (ranging from subtle to complete), mydriasis
and ophthalmoparesis with the eye resting ‘down
and out’. However, partial lesions are common
(figure 7). Oculomotor nerve lesions may arise
anywhere along its course, including the nucleus,
the fascicles in the midbrain tegmentum and the
nerve itself as it passes through the subarachnoid
space, the cavernous sinus, the superior orbital
fissure and the orbit (table 2).
The nuclear supply of the LPS arises from the
central caudal subnucleus of the oculomotor
nucleus. This is a single midline structure supply-
ing the LPS on both sides. Hence ptosis resulting
from lesions of the oculomotor nucleus is typically
bilateral. Because there may be damage to adjacent
neural pathways such as the corticospinal tract or
red nucleus, fascicular lesions of the oculomotor
nerve are often associated with contralateral hemi-
paresis or contralateral limb ataxia.
The oculomotor nerve divides into superior and
inferior divisions and these can be damaged indi-
vidually. The LPS is supplied by the superior divi-
sion. Superior divisional palsies therefore cause
ptosis and elevation failure (due to superior rec-
tus involvement), while inferior divisional palsies
cause ophthalmoparesis, frequently with pupil
involvement but spare the LPS.
There are many possible causes of oculomotor
nerve damage. These include giant cell arteritis, or
trauma and compression by an aneurysm (usually
of the posterior communicating artery). Infection,
other causes of inflammation, thrombosis or tumour
can affect the oculomotor nerve in the cavernous
sinus, superior orbital fissure or orbit. Similarly, the
it may be affected by several metabolic or inflam-
matory disorders such as diabetes mellitus or the
Miller Fisher syndrome, associated with antibodies
to ganglioside GQ
(figure 8). In Miller Fisher syn-
drome, extraocular muscle involvement is the rule
but there are reports of isolated ptosis.
Space does
not permit a more detailed discussion of oculomo-
tor nerve palsies here and the interested reader is
referred elsewhere.
Central causes
Cerebral cortex
Cortical (or supranuclear) ptosis is unusual but
well recognised. Exactly how this happens is not
clear, granted that the central caudal nucleus
innervates the LPS on both sides. When it occurs,
the ptosis is usually contralateral to the lesion, but
ipsilateral and bilateral cases have been reported.
It occurs most frequently in large non-dominant
hemisphere lesions.
Basal ganglia
Apraxia of eyelid opening may occur in several
neurological conditions, most commonly pro-
gressive supranuclear palsy. It manifests as a
Figure 9 Left functional ptosis.
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(transient) inability to open the eyes although the
eyelids can be held open normally once they are
open. Patients often have to open their eyes with
their fingers to overcome the apraxia. As eyelid
function is normal, this is not a true ptosis.
Excessive orbicularis activity
This may be mistaken for ptosis. The excessive
activity may be involuntary, as in hemifacial
spasm, aberrant regeneration following Bell’s
palsy, or essential blepharospasm. Alternatively, it
may be voluntary as in functional pseudoptosis.
Functional ptosis is recognisable by normal leva-
tor function with apparent weakness of the fron-
talis and overcontraction of the orbicularis oculi
(figure 9).
Clinical management
The history in a patient with ptosis should cover
the following points:
Is one or are both eyes affected?
Are there associated symptoms, specifically pain,
malaise, visual disturbance, diplopia, dysphagia or
muscle weakness elsewhere?
What was the speed of onset, the duration and
extent of progression of the ptosis? Does the ptosis
fluctuate? Are there any obvious relieving and
exacerbating factors?
Does the patient have any comorbidities? In
particular, are there vascular risk factors, a history of
injury to the head, neck or chest, a history of HIV or
other cause of immunosuppression, features of the
metabolic syndrome, cancer or ocular disease? Are
there any systemic features of giant cell arteritis?
Is there a history of trauma, ophthalmic surgery
or rubbing of the eyelid? Does the patient wear
contact lenses?
Has the patient had a blepharoplasty in the past?
Is the patient taking any medications, regular or
Is there a family history of ptosis or of other muscle
Patients with ptosis should undergo a general sys-
temic and neurological examination. Of particular
importance are the pupils, visual acuity and fields,
funduscopy, extraocular and facial movements
and other cranial nerve function. Ideally, patients
should have an ocular examination, looking specif-
ically for inflammation in the anterior chamber.
The eyelids should be inspected for symme-
try, visible lesions, thickening, discolouration
and involuntary movement. The position of the
skin crease should be noted. The palpebral fis-
sure, upper marginal reflex distance and eyelid
excursion should be measured as in figure 1. The
behaviour of the eyelid during eye movements
should then be noted.
Evidence of fatigability can be sought by ask-
ing the patient to maintain fixation in upgaze
for 60 s and remeasuring the palpebral fissure
immediately afterwards. An ice or rest test
may demonstrate reversibility of the ptosis in
myasthenia. Eye closure is frequently weak in
ocular myasthenia.
Most causes of ptosis can be diagnosed clinically.
Unequal pupil size suggests a Horner’s syndrome
or an oculomotor nerve palsy. An elevated skin
crease with thinned eyelid but a normal range
of movement suggests levator dehiscence. If the
ptosis is fatigable, or if there are supportive sys-
temic features, it may be possible to diagnose MG.
Dysfunction of extraocular muscles suggests a
myopathy or muscular dystrophy if longstanding,
and an oculomotor nerve palsy or a neuropathy
(such as Miller Fisher syndrome) if of recent onset.
Pain at the onset of ptosis suggests the possibility of
an aneurysm compressing the oculomotor nerve.
Other clinical features such as visual loss or sys-
temic symptoms may suggest giant cell arteritis.
Investigation and management
The causes of ptosis are so numerous that it is
impossible to give an exhaustive list of appropri-
ate diagnostic tests. However, imaging may well
be important to look for a possible aneurysm or
other structural abnormalities. Blood tests (espe-
cially erythrocyte sedimentation rate), neurophysi-
ology, lumbar puncture or muscle biopsy may each
be appropriate, depending on the likely cause and
whether or not there are accompanying symptoms
or signs suggesting a specific diagnosis.
Similarly, the management of ptosis depends on
its cause. In many cases, ptosis improves with time
or with treatment of the underlying condition.
When a reasonable amount of time has passed
without improvement and further improvement
is felt to be unlikely, referral to an oculoplastic
surgeon may be considered. The most common
surgical techniques used to treat ptosis are short-
ening of the LPS or insertion of tendon slings.
Ptosis props are rarely effective (figure 6).
Ptosis has a myriad of causes but only a hand-
ful are seen frequently. The clinical examination
is easily understood once the underlying anatomy
is known. To avoid unnecessary investigations, it
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is useful to try to determine whether the cause
is likely to be structural, myogenic, neurogenic,
neuromuscular or central. A directed history and
examination often allows the clinician to localise
the cause of the ptosis to a particular anatomical
area, if not to reach a specific diagnosis. This in
turn permits appropriate investigations and treat-
ment to be instituted. The management of ptosis
is very much dependent on the underlying cause
but oculoplastic surgery should be considered for
patients with static lesions.
Acknowledgements The authors thank Dr Jon Stone
who provided the image of functional ptosis. This article
was reviewed by Dr Mark Lawden, Leicester, UK.
Learning points
Ptosis is common, and knowledge of how to examine
a ptotic eyelid is useful to neurologists.
The most common cause of ptosis is levator dehis-
cence. Other common causes are myasthenia gravis,
Horner’s syndrome and oculomotor nerve palsies.
Pseudoptosis refers to the appearance of ptosis un-
related to eyelid dysfunction. Dermatochalasia is the
most common cause. Be aware of other conditions
that give the appearance of ptosis such as contralat-
eral lid retraction or excessive orbicularis activity.
Horner’s syndrome may cause a very subtle ptosis,
and pharmacological testing of the pupil may be re-
quired to confirm this diagnosis.
Oculoplastic surgery should be reserved for cases
where the diagnosis is clear and improvement is not
Competing interests None.
Patient consent Obtained.
Contributors KA was the primary author of the manuscript.
CJL made significant additions. MW provided most
of the images. All authors edited the manuscript.
Provenance and peer review Commissioned;
not externally peer reviewed.
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02_practneurol-2011-000026.indd 34002_practneurol-2011-000026.indd 340 11/17/2011 2:45:29 PM11/17/2011 2:45:29 PM
... De differentiaaldiagnose van een ptosis is breed (tabel). 1 In dit artikel richten we ons op de differentiaaldiagnose van een wisselende ptosis, zoals die zich voordeed bij de twee besproken patiënten. Zowel bij apraxie van de ooglidopening als bij blefarospasmen wordt een 'geste antagonique' of 'sensory trick' beschreven, waarmee wordt bedoeld dat een bewuste aanraking de afwijkende stand van het ooglid verbetert. ...
Background: A variable ptosis may point towards serious neurological disorders and is presented to general practitioners, ophthalmologists and neurologists. Case description: Two patients presented at the neurology outpatient clinic with a ptosis confined to awakening from sleep. There were no other neurological complaints and neurological examination was normal. The diagnosis 'awakening ptosis' was made. Conclusion: Awakening ptosis is a benign, but rare disorder. The exact pathophysiology remains unclear. In the case of a classic clinical picture of awakening ptosis, additional examinations are not indicated.
Myasthenia gravis is a rare autoimmune disease characterised by autoantibodies preventing normal function of acetylcholine receptors at the post-synaptic membrane of the neuromuscular junction. This causes weakness of skeletal muscles that can be variable and fatigable, and often manifests as ptosis and/or diplopia, with 60% of patients demonstrating ocular features at onset, and thus may present initially to eye care practitioners. Approximately 15% of patients have ocular myasthenia gravis, where symptoms remain restricted to this distribution. The majority of patients have blocking antibodies against the acetylcholine receptor, but antibodies directed against other related targets account for a smaller proportion and are associated with specific phenotypes. Associations with both thymoma and with other autoimmune phenomena (particularly thyroid disease) can occur. Clinical examination can identify characteristic findings including fatigable ptosis and Cogan's lid twitch sign. Investigations to confirm the diagnosis include simple office-based procedures such as the ice test, and testing for serum autoantibodies, as well as electrophysiological testing such as repetitive nerve stimulation and single-fibre electromyography. The management of ocular myasthenia gravis is discussed, including non-pharmacological options, pyridostigmine, corticosteroids, other immunosuppressive agents, and thymectomy. The goals of management are to alleviate symptoms, and where possible prevent chronic disability or progression to generalised myasthenia gravis.
Patients presenting with disorders affecting the pupils, eyelids, or orbits may have a relatively benign etiology (e.g., tonic pupil, levator dehiscence, mild thyroid eye disease), or symptoms/signs may signify a potentially life-threatening disorder (e.g., aneurysmal third nerve palsy [NP], Horner’s syndrome from carotid artery dissection, aggressive orbital fungal infection). Therefore, the clinician must be able to recognize the most dangerous etiologies, but also those benign etiologies that may only require reassurance. This chapter covers each of these anatomic regions individually, first with an emphasis on the focused history and examination, followed by a case-based approach to the most common disorders. Or, if your patient has anisocoria and/or ptosis and you do not know where to start and do not have the time to read through the individual sections, begin with the “Help me now!” tables for a symptom (or sign)-based approach.
Purpose: To examine whether Müller’s-muscle-conjunctival-resection (MMCR) changes the position of the lower eyelid. Methods: Retrospective controlled-cohort study. All patients who underwent MMCR (study group) or blepharoplasty (control group) between January 2016 and September 2018 were recruited. The data retrieved from the patients’ medical records included demographics, visual-acuity, eyelid parameters and dry-eye parameters before and 3 months after surgery. Frontal photographs of the patient’s eyes in primary position were taken preoperatively and at 3 months postoperatively. The margin-reflex-distance 1 (MRD1) and MRD2 were evaluated. The preoperative and 3 months postoperative MRD1, MRD2,and dry-eye signs and symptoms were compared. Results: Sixty-nine patients underwent MMCR and 54 patients underwent blepharoplasty during the study period. There were significant changes in MRD2 after MMCR surgery compared to preoperative values (P < .01, paired t-test), but no significant changes in MRD2 after blepharoplasty surgery (P = .091, paired t-test). The mean changes in MRD2 (delta MRD2) were 0.51 in the MMCR group versus (−0.10) in the blepharoplasty group (P = .04, t-test). Conclusions: The position of the lower eyelid was altered significantly in patients that underwent MMCR surgery. This sequela should be discussed with the patients before surgery and should be considered by physicians when planning ptosis surgery.
Purpose: To shed light upon the possible role of the levator aponeurosis (LA) developmental fibrotic changes as an added etiology for simple congenital ptosis, which causes limitation of the levator function (LF). Methods: This retrospective cohort study included patients with simple congenital ptosis who underwent skin approach LA resection as a primary intervention with an intraoperative photographic documentation of LA fibrotic changes. Preoperative demographics and clinical data were reviewed. The effect of LA fibrotic changes on the LF was assessed in different LA fibrotic changes with or without levator palpebrae superioris (LPS) muscle fatty infiltration. Results: A total of 56 eyelids of 49 patients with a mean age (±SD) 6.7 (±3.2) years were enrolled in this study. The fibrotic changes of LA were observed as a sheet of fibrosis (19 eyelids) or fibrous bands (23 eyelids). Fatty infiltration of LPS was noticed in 28 eyelids, either with or without fibrotic changes of LA. Preoperative LF was diminished in LPS fatty infiltration compared with LA fibrotic sheets (P = 0.026). Postoperative LF improved significantly in both LA fibrotic sheets and LA fibrotic bands (9.4 ± 2.5 mm and 9.6 ± 2.8 mm, respectively) compared with LPS with fatty infiltration (6.4 ± 1.8 mm) (P = 0.004). Conclusions: Although our data are inconclusive due to lack of embryologic studies, the observed LA fibrotic changes may suggest a complex pathogenesis of simple congenital ptosis. The meticulous observation of the LA and the releasing of any adhesion or band to the surrounding structures could improve postoperative LF.
Conventional levator aponeurosis plication is a widely accepted technique for correction of mild to moderate ptosis. However, this method is associated with a high recurrence rate. The objective of this study was to investigate the clinical efficacy of levator aponeurosis posterior layer plication technique for correction of mild to moderate ptosis.A convenience sampling approach was used to recruit 450 patients with mild to moderate blepharoptosis at the Guangzhou Eye-Nose-Face Aesthetic Plastic Surgery Hospital between August, 2015 and December, 2017. All participants were treated with levator aponeurosis posterior layer plication technique. The primary outcome was the postoperative change in marginal reflex distance 1 (MRD1). The paired t test was used to determine the clinical efficacy. Outcomes were assessed at 1 week, 1 month, 3 months, and 6 months after surgery.The mean preoperative MRD1 was 1.7 ± 0.5 mm, and the mean postoperative MRD1 at 6-month follow-up was 3.7 ± 0.4 mm (P < .0001). According to the postoperative survey, 427 (94.9%) patients were satisfied with surgical outcomes.This modified levator aponeurosis plication technique is a simple and effective procedure for correction of mild to moderate blepharoptosis. It results in good MRD1 and high patient satisfaction.
Purpose: To assess the effect of releasing the central attachment between the Whitnall's ligament (WL) and the levator palpebrae superioris muscle on the postoperative levator function (LF), eyelid lag, and degree of lagophthalmos. Methods: This retrospective case-control study included patients with moderate and severe simple congenital ptosis who underwent skin approach levator aponeurosis resection (LR) as a primary procedure with a minimum of 6-month follow up. Patients were divided into 2 groups; the first group underwent LR without WL release (control group) while the second group underwent LR with WL release. Preoperative demographics and clinical data were reviewed. Postoperative LF, eyelid lag, and degree of lagophthalmos as well as surgical outcomes were compared and analyzed in both groups. Results: A total of 81 patients (88 eyelids) were included in this study. There were 50 males (61.7%). The mean age was ±SD 12.0 ± 9.5 years. The first group included 43 eyelids while the second had 45 eyelids. There was no statistical difference in demographics and preoperative data between both groups. The postoperative LF was higher in the second group (10.7 ± 2.1 mm) with less consecutive eyelid lag compared with the control group (7.8 ± 1.9 mm) (p < 0.001). The control group had acquired more postoperative lagophthalmos compared with the second group (p < 0.001). Complete surgical success was achieved in 82.2% in the second group compared with 60.5% in the control group (p = 0.024). Conclusions: Releasing the central attachment between WL and levator palpebrae superioris muscle has achieved an improvement in LF with minimal postoperative eyelid lag, lagophthalmos, and corneal complications.
A 31-year-old woman, 27 weeks pregnant with twins but otherwise without a remarkable medical history, presented to the emergency ward with a right-sided drooping eyelid that had appeared shortly after vomiting. She noted that she had never vomited with that much force. She initially noticed a dull pain behind the right eye, which faded within an hour. On neurological examination, a partial ptosis was observed without any other signs of cranial nerve dysfunction (Figure 1). Importantly, her pupils were equal and reactive, and her eye movement was unrestricted and painless. No headache or visual impairment was reported. Her vital signs were normal.
Introduction: Levator function is classically estimated by measuring upper eyelid excursion (ULE) with digital brow stenting. The purpose of this study is to compare ULE with and without brow stenting in normal and ptotic eyelids. Methods: In this prospective observational study, normal and ptotic eyelids were recruited. Subjects were photographed with and without digital brow stenting in primary position, downgaze, and upgaze. Measurements were conducted on digital photographs. The primary outcome measure was ULE (distance travelled by the eyelid margin between downgaze and upgaze). Normal and ptosis (MRD1 ≤ 2.5 mm or asymmetry ≥ 1 mm) subgroups were defined. Independent one-way ANOVA and independent samples t-tests were performed. This study was powered to detect a 1 mm difference in the primary outcome measure, assuming SD = 1 mm, with alpha = 0.05 and beta-error = 0.95. Results: Twenty-eight normal eyelids of 22 subjects and 28 ptotic eyes of 18 subjects were included. Stenting significantly (p < 0.01) increased ULE in the overall sample (+0.9 mm) and in controls (+1.2 mm), but not (p > 0.05) in ptotic eyelids (+0.5 mm). Post hoc analysis revealed a beta-error of 0.08 in the latter. Conclusion: ULE was significantly higher with brow stenting in normal eyelids (approximately +1.2 mm) but not in ptotic eyelids, possibly due to increased levator tone secondary to increased effort in the coupled frontalis.
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Neuro-ophthalmological signs and symptoms are common in the emergency department but are a frequent source of diagnostic uncertainties. However, neuro-ophthalmological signs often allow a precise neuro-topographical localization of the clinical problem. A practical concept is presented how to perform a neuro-ophthalmological examination at the bedside and to interpret key findings under the aspect of emergency medicine with limited resources. © 2018 Journal of Neurosciences in Rural Practice | Published by Wolters Kluwer - Medknow.
Full-text available
A new syndrome associated with a deficiency of acetylcholine receptor (AChR) and a short open-time of the AChR channel in a 5 year-old girl with myasthenic symptoms since birth is reported from the Neuromuscular Research Laboratory, Mayo Clinic, Rochester, MN.
Oculopharyngeal muscular dystrophy is usually a dominant late-onset myopathy associated with unique intranuclear inclusions caused by short (GCN)11-17/polyalanine expansions in the polyadenylate-binding protein nuclear 1 gene (PABPN1). The intranuclear inclusions are known to sequester many RNA-binding proteins and components of the ubiquitin proteome system. The pathological role of the intranuclear inclusions is still unclear. There is growing evidence that the inclusion free soluble expanded PABPN1 is the key culprit in the pathology.