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TRIPLE PTOSIS
ABSTRACT
This is a case in which aberrant regeneration after a remote facial palsy confounds the diagnosis of
an early ipsilateral third nerve palsy.
CASE HISTORY
A 54 year old Caucasian male presented to the emergency department for an acute increase in left
ptosis x 1 day. The patient had a previously existing left ptosis residual from a left lower motor
neuron facial palsy in 2004.
Initial assessment by the ER physician revealed left eye ptosis with accompanying left retrobulbar
pain. Pupils and extraocular motilities were recorded as normal. Other cranial nerve testing was
remarkable for left facial weakness involving the upper and lower face. No other focal neurological
deficits observed. CBC, electrolytes plus and head CT were obtained. (See Laboratory/Radiology
Studies). CT of the head without contrast revealed no acute intracranial hemorrhage, infarct or mass.
The patient was admitted to neurology service for workup of recurrent left facial lower motor neuron
palsy vs acute facial nerve palsy of infectious etiology vs. ptosis of unclear etiology (unlikely
myasthenia gravis). The patient was consulted to optometry for an evaluation of the ptosis.
OPTOMETRY CASE HISTORY
The patient presented to the optometry clinic complaining of an exacerbation of longstanding left
ptosis upon wakening x 1 day with accompanying significant left retrobulbar pain which started 3
days prior. He denied double vision, blurry vision, photophobia, eyelid swelling, variability of ptosis
size. He noted no additional facial weakness from baseline and reported the ability to fully close the
left eye. His wife who accompanied him to the exam claimed that the change in his facial
appearance was limited to the left lid. He additionally denied recent trauma or MVA, new rash or
history of shingles, changes in hearing, tick bite, difficulty swallowing, dysarthria, numbness or
tingling.
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CASE HISTORY CONTINUED
Ocular History
h/o left lower motor neuron facial palsy 2004 with longstanding left ptosis
Mild nonproliferative diabetic retinopathy OU
H/o classic migraines diagnosed at 18 years old. Frequency: 2- 3x/year
Choroidal Nevus OD
Mild Myopia with Presbyopia OU
Medical History
Diabetes Mellitus
Type II x 20 years
Hypertension
Hyperlipidemia
Coronary Artery
Disease
Obseity
Fibromyalgia
PTSD
Medications
Insulin
Metformin
Clopidogrel
Metoprolol
Lovastatin
Valsartan
Amitriptyline
Cetirizine
Hydrochlorothiazi
de
Gabapentin
Nabumenton
EXAMINATION
BCVA
OD 20/20, OS 20/20
Confrontation Fields
Full OD, OS
Pupils
Equal, round reactive to light OD, OS (-)APD
EOMs
OD: full
*SEE PHOTOS*
OS: partial limitation supraduction, adduction
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EXAMINATION continued
Alternating Cover test:
Cranial Nerve
Testing
CN VII: 1+ action left frontalis
3+ action orbicularis oculi
1+ orbicularis oris
CN II, IV, V, VI, VIII, IX, X, XI, XII unremarkable.
CNI not tested
Eyelid Position
Palpebral Fissure
OD 9mm OS 3mm
Marginal Reflex Distance OD 3mm OS -2mm
Levator Function
OD 14mm OS 12mm
Worsening of left ptosis on mouth opening and
puffing cheeks *SEE PHOTOS*
Hertel Exophthal.
OD 15mm
OS 13mm
Resistance to
Retropulsion
Negative bilaterally , left globe tender to touch
Photo1: Resting eyelid position
Base 96mm
Photo 2: Complete closure of eye on
opening of mouth
Photo 3:Complete closure
of eye on puffing checks
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EXAMINATION continued
Anterior Segment
Unremarkable OU (-)Cogan lid twich
Handheld Goldman
Applanation Tonometry
OD: 10 mmHg
OS: 11mmHg @ 5:01p
Dilated Fundus Examination
Optic nerves with distinct borders, pink healthy rim 360
OU. No evidence of papilledema.
Choroidal nevus 1/3DD temporal to macula OD flat with
distinct borders.
No evidence of hemorrhages, cotton wools spots, IRMA,
venous beading, neovascularization OU
Humphrey Visual Field 24-2
Unreliable OU, non-neurological field
LABORATORY/RADIOLOGY STUDIES
CBC
Test Name
WBC
RBC
HGB
HCT
MCV
MCH
MCHC
RDW-CV
PLT
MPV
NEUT %
LYMPH %
MONO %
EOS %
BASO %
NEUT, ABS
MONO, ABS
EOS, ABS
BASO, ABS
Result
5.65 K/cmm
4.28 L M/cmm
13.1 L g/dL
38.4 L %
89.7 fL
30.6 pg
34.1 gm/dL
12.5 %
159 K/cmm
10.0 fL
66.0 %
25.1 %
5.5 %
2.7 %
0.5 %
3.73 K/cmm
0.31 K/cmm
0.15 K/cmm
0.03 K/cmm
Ref Range
4.5 - 10.0
4.7 - 5.7
14 - 17
40 – 51
80 - 101
27 - 34
31.0 - 36.0
11.5 - 15.0
130 - 450
9.0 - 12.0
40.0 - 75.0
10 - 55
2 - 12
0.0 - 7.0
0-2
1.8 - 6.5
0.11 - 0.59
0.0 - 0.7
0 - 0.2
Sed Rate 38
CRP 1.28 mg/L
HB A1C 8.9
CT head without contrast
MRI of brain with and
without gadolinium
There is no evidence of intra-axial or extra-axial mass,
midline shift, acute hemorrhage or infarct. The sulci,
ventricles and cisterns are normal. The orbits are
unremarkable. Skull and extracranial soft tissues are
unremarkable.
Conclusion: No acute intracranial abnormality.
Impression: No retrobulbar mass identified. Unrevealing
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DIFFERENTIAL DIAGNOSIS of chief complaint: acute unilateral ptosis
CN III palsy
Brow ptosis from facial palsy (pseudoptosis)
Myasthenia Gravis
Acute Horner’s Syndrome
Mechanical ptosis (ie acute hordeolum)
DIAGNOSIS : TRIPLE PTOSIS
1. History of left lower motor neuron facial palsy 2004 with known longstanding left ptosis
secondary to aberrant regeneration
2. Synkinesis manifesting as increasing left ptosis when patient opens mouth widely or puff
cheeks following facial palsy
3. Painful, partial, pupil-sparing left CNIII palsy, primarily involving supraduction and adduction
We were unable to clinically observe an infraduction deficit of the left eye however cover
test inferiorly revealed a small left hyperphoria suggesting sub-clinical limitation.
This is a case in which aberrant regeneration after a remote facial palsy confounded the diagnosis of
an early ipsilateral third nerve palsy. To complicate the diagnosis further, the palsy was partial and
manifested as subtle EOM limitations with a spared the pupil. Another confusing variable was that
the patient initially denied diplopia which is usually one of the presenting complaints in an acute CN
III palsy. The most common reason that diplopia is not elicited upon an acute CN III palsy is because
the ptosis covers the pupil. The other less common reasons are that the extraocular muscles are so
minimally affected that the patient has some fusional vergence to hold the eye into partial alignment
or that the images are so far apart that the patient is unaware of the second image. The patient
denied diplopia in primary gaze even with the lid held but could be elicited in all other directions of
gaze. All of these variables led to this diagnosis being overlooked by multiple physicians prior to
optometry evaluation.
Therefore the “triple ptosis” diagnosis was formulated from the following patient characteristics:
Ptosis One: from aberrant regeneration following facial palsy
Ptosis Two: increased ptosis with facial animation via synkinesis
Ptosis Three: from acute CN III palsy
DISCUSSION
In a discussion revolving around ptosis it is important to review eyelid anatomy. Normal eyelid
movements are supplied by 3 entities:
1. Facial Nerve (CN VII) innervates the orbicularis oculi to close both the upper and lower lid
2. Oculomotor nerve (CN III) innervates the levator palpebrae superioris to elevate the lid
3. Sympatheics innervate the superior tarsal muscle (Mueller) to augment opening of the lid
and also the inferior tarsal muscle
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It is commonly known that during an acute facial palsy a “brow ptosis” can occur secondary to
weakness of the frontalis muscle forcing the brow to sit at or below the superior orbital rim.
Aberrant regeneration, on the other hand, is an under recognized cause of ptosis following a facial
palsy. During regeneration and repair of neurons after a palsy, some fibers may take an unusual
course and connect to other muscles innervated by CN VII. Aberrant regeneration following a facial
palsy may give rise to an ipsilateral ptosis because of increased orbicularis tone. Signs of aberrant
regeneration include reduced palpebral aperture, with reduced upper and lower marginal reflex
distance. 30% of patients with Bell’s palsy experience sequelae of the paralysis which can include
incomplete motor or sensory regeneration and aberrant regeneration.1
Ptosis following a facial palsy can be exacerbated by synkinesis. Synkinesis refers to the
abnormal involuntary facial movement that occurs with voluntary movement of a different facial
muscle group. Marin-Amat syndrome describes an acquired synkinesis of the orbicularis oculi and
jaw movements. It manifests as an involuntary eyelid closure when the patient is asked to open the
mouth widely or to puff the cheeks. The reported incidence of synkinesis in facial nerve palsy varies
greatly from 9 to 55%.2 Yamamoto et al found that synkinesis occurs most frequently 24-39 weeks
following the onset of facial palsy.3 It is not known when our patient developed ptosis since he
transferred care to the clinic several years his palsy. We had on record an identification photo taken
from 2009 which was 5 years after the initial incident.
CN III is also responsible for eyelid position and paralysis can lead to a partial or complete
ptosis. In addition to innervating the levator palpebrae superioris, CN III innervates 4 of the 6
extraocular muscles (superior rectus, inferior rectus, medial rectus and inferior oblique) and also
supplies parasympathetic innervation to the sphincter muscle of the iris and to the ciliary body. CN
III palsies are either classified as complete or incomplete and either pupil sparing or pupil involving.
Complete paralysis refers to total palsy of all four extraocular muscles and levator. Pupil sparing is
defined as less than 1 mm of anisocoria and normal pupillary light reflex.4 For the sake of this
discussion, we will refer to acquired, non-traumatic and isolated (without associated neurological
findings) third nerve palsies only. This subset of CN III palsies can be further classified.
Third nerve palsy with:
1. Normal pupil with completely palsied EOM
2. Normal pupil with incomplete palsied EOM
3. Abnormal pupil with partial or complete EOM palsy
Our patient’s profile fits category 2. He had a subtle partial paralysis of at least 3 of the 4 muscles
innervated by CN III. It has been documented that most patients with extraocular muscle and levator
involvement in pupil sparing, incomplete third nerve palsies of vasculopathic origin have a diffuse
pattern of paresis.5 This partial and diffuse limitation was also the reason the diagnosis of an acute
CN III palsy was that much more difficult to spot, especially to the non-optometric trained eye.
This patient also presented with retrobulbar pain. Headache and periocular pain, excruciating
enough to overshaow the ptosis and diplopia, occur in at least 50% of patients with microvascular
ischemic CN III palsy.6 Ischemic damage to the trigeminal fibers in CN III may be the source of pain in
ischemic diabetic CN III palsy.7 The most common location of the pain has been documented around
the ipsilateral brow and eye. Literature indicates that 11-33% of patients have pain preceding the
onset of visual symptoms. 8 This finding is also consistent with our patient’s presentation.
Ischemic lesions of CN III often spare the pupil in theory because the lesion is confined to the
core of the nerve and does not affect the peripherally situated pupillary fibers. By contrast, a
compressive lesion (ie tumor or aneurysm) would compress the superficial pupillary fibers and impair
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their function. Pupil involvement occurs in as many as 20% of patients with ischemic CN III palsy but
anisocoria of more than 1.5mm is exceedingly rare. 9 Incomplete third nerve palsies with pupillary
sparing require an MRI to rule out a mass. According to Jacobson and Trobe, for this subset of CN III
palsies even those greater than 40 years old, who have vasculopathic factors present, it is
recommended to obtain MRI followed by MRA if the MRI doesn’t disclose a non-aneurysmal cause. 10
MRA was recommeded for our patient but was not ordered by neurology.
TREATMENT AND MANAGEMENT
Plan:
1. Obtain MRI followed by MRA if the MRI is unremarkable. Rule out giant cell arteritis as a
cause of ischemic CN III palsy.
2. Address underlying vasculopathic risk factors and patient education
3. Dispense a patch which can be useful for alleviating diplopia short term. Patient educated
regarding depth perception
4. Patient educated not to drive until diplopia is resolved
Initial management of an acute, incomplete pupil sparing CN III palsy is to order appropriate
imaging as described above to rule out a posterior communicating artery aneurysm and to monitor
progression of ophthalmoplegia and/or pupil involvement. The patient was re-examined 1 day and 1
week after initial presentation to the eye clinic. During this time the patient was advised to self
monitor his pupils. No changes were detected on subsequent visits. For maximal ophthalmoplegia,
the average time from onset has been documented as 34 to 10 days11. Our patient has not yet
recovered at 2.5 weeks after onset.
Therapy for CN III palsy is directed at the underlying etiology. For this patient, underlying
microvascular disease needed to be addressed and vascular risk factors treated. The majority of
ischemic third nerve palsies improve within 3 months- 6 months. In a study of 28 patients with
ischemic CN III palsy, 68% resolved in four weeks, 96% in eight weeks, and 100% in 12 weeks.12 The
most common therapeutic modalities for the treatment of facial synkinesis and aberrant
regeneration include botulinum toxin and surgical correction.
CLINICAL PEARLS: TRIPLE PTOSIS
1. Aberrant regeneration is an under recognized cause of ptosis following a facial palsy
2. Observation of synkinesis requires facial animation in office
3. Careful attention must be given to extraocular muscles in the setting of an acute unilateral
ptosis with or without diplopia. Incomplete CN III palsies can be subtle and missed.
4. If an incomplete CN III palsy presents with a normal pupil, pupil involvement may still evolve
and needs close monitoring. Maximal ophthalmoplegia should be present in 10 days.
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References
1. Marsk E, Bylund N, Jonsson L, Hammarstedt L, Engström M, Hadziosmanovic N, et al. Prediction
of nonrecovery in Bell's palsy using sunnybrook grading. Laryngoscope. Apr 2012;122(4):901-6.
2. Monteserra L,Benito M. Facial synkinesis and aberrant regernation of facial nerve.In:Jankovic J
and Tolosa E (eds). Advances in Neurology. Raven Press: New York, 1998 p211-224
3. Yamamoto E, Nishimura H, Hirono Y. Occurrence of sequelae in Bell's palsy. Acta Otolaryngol
Suppl. 1988;446:93-6. 1988;446:93-6.
4. Lee AG, Hayman LA, Brazis PW. The evaluation of isolated third nerve palsy revisited: an update
on the evolving role of magnetic resonance, computed tomography, and catheter angiography.
Surv Ophthalmo 2002l, pp. 47(2); 137-57
5. Sanders S, Kawasaki A, Purvin VA. Pattern of extraocular muscle weakness in vasculopathic pupilsparing, incomplete third nerve palsy. J Clin Neuroophthalmol, 2001, Vols. 21:256-259.
6. Smith CH. Nuclear and infranuclear ocular motility disorders. In: Miller NR, Newman NJ, eds.
Walsh & Hoyt’s Clinical Neuro-ophthalmology. 5th edu Baltimore. 1998;1:1205
7. Bortolami R, D’AlessandroR, Manni E. The origin of pain in ischemic –diabetic third nerve palsy.
Arch Neurol 1993; 50:785
8. Wilker SC, Rucker JC, Newman NJ, Blousse V, Tomask RL. Pain in ischemic ocular motor cranial
nerve palsies. BrJ Ophthalmol2009.Dec;93:1657-1669
9. Jacobson DM. Pupil involvement in patietns with diabetes-associated oculomotor nerve palsy.
Arch ophthalmol 1998;116:723-7.
10. Jacobson DM, Trobe JD. The emerging role of magnetic resonance angiography in the
management of patients with third cranial nerve palsy. Am J Ophthalmol 1999, pp. 128;94-96.
11. Renowden SA, Harris KM, Hourihan MD. Isolated atraumatic third nerve palsy: Clinical features
and imaging technqiues. Br J Radiol 1993;66:1111-1117
12. Capo H, Kupersmith WF. Evolution of oculomotor nerve palsies. J Clin Neuro-ophthalmol
1992;12:21-25
13. Trobe J. Searching for brain aneurysm in third cranial nerve palsy.ED J Neuro-Ophthal Vol 29.
No3, 2009; 171-173
14. Chen C, Malhotra R, Muecke J, Davis G, Selva D. Aberrant facial nerve regeneration: an under
recognized cause of ptosis. Eye 2004.18,159-162.
15. Wood, S. Normal pupil findings can give a false sense of security in a presumed cranial nerve III
palsy patient: A unique case report. Optometry (2010) 81;505-509
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