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Palsies/Opthalmoplegias
Final-lectures 16-19
186
Medical
Graduate
07/28/2013

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Cards

Term
3 forms of ophthalmoplegia caused by sphenocavernous syndrome
Definition
Cavernous Sinus Thrombosis
Carotid-Cavernous Fistula
Tolosa-Hunt Syndrome
Term
Which type of ophthalmoplegia is caused by infection of the facial sinuses
Definition
Cavernous Sinus Thrombosis
Term
Treatment of Cavernous Sinus Thrombosis
Definition
Sinus drainage
IV steroids
IV broad-spectrum antibiotics
Heparin
Term
Involvement of Cavernous Sinus vs Orbit
Which is more likely to have VA loss and exophthalmos
Definition
Orbital Involvement
Term
3 symptoms that may precede Cavernous Sinus thrombosis
Definition
Headache, fever and malaise
Term
Carotid-Cavernous Fistula can happen anteriorly or posteriorly, which is more urgent
Definition
Anterior
Term
Carotid-Cavernous Fistula can happen anteriorly or posteriorly, which is more commonly caused by head trauma
Definition
Anterior
Term
Ocular presentation of Carotid-Cavernous Fistula
Definition
Red eye
Corkscrew shaped vessels
Proptosis
increased IOP
ophthalmoplegia
Term
Carotid-Cavernous Fistula may be mistaken for what routine ocular condition
Definition
Pink Eye since the eye is so red
Term
Which cause of ophthalmoplegia can be confirmed with a stethoscope
Definition
Carotid Cavernous Fistula
Term
Direct vs. Indirect Fistula
which tends to occur spontaneously
Definition
Indirect
Term
Direct vs. Indirect Fistula
Which is less likely to recover spontaneously
Definition
Direct
Term
Which type of ophthalmoplegia is caused by nonspecific inflammation and can happen in the cavernous sinus and superior orbital fissure
Definition
Tolosa-Hunt syndrome
Term
Which of these three ophthalmoplegias often remits and relapses
Cavernous Sinus Thrombosis
Carotid Cavernous Fistula
Tolosa Hunt Syndrome
Definition
Tolosa Hunt Syndrome
Term
3 presenting symptoms of Tolosa Hunt Syndrome
Definition
Pain
Ptosis
Diplopia
Term
In Tolosa Hunt Syndrome the pupils are often involved, how will they present if the internal carotid artery is involved
Definition
Miotic-since the sympathetic plexus wraps around the interal carotid
Term
Treatment of choice of Tolosa Hunt
Definition
IV steroids
Term
4 Neuropathies that cause ophthalmoplegia
Definition
Guillain-Barre Syndrome
Miller-Fisher Syndrome
Abetalipoproteinemia
Refsum Disease
Term
What is the most frequent cause of acute paralysis
Definition
Guillain- Barre Syndrome
Term
Campylobacter jejuni is associated with which syndromes
Definition
Guillain Barre
Miller-Fisher
Term
Ocular presentation of Guillain Barre
Definition
Bilateral ptosis
Optic neuritis/papilledema
Poor near tried response
Term
Which nerve is most frequently involved in Guillain Barre syndrome
Definition
Abducens
Term
3 ways to diagnose Guillain Barre
Definition
Lumbar puncture (proteins & WBC in CSF)
Electromyography-(demyelination?)
Nerve conduction (slow or blocked?)
Term
Prognosis of Guillain Barre
Definition
70% complete recovery
20% residual disability
10% mortality
Term
Treatments of Guillain Barre syndrome
Definition
IV immunoglobulin
Plasmapheresis
Term
Classic Miller-Fisher triad
Definition
Ophthalmoplegia
Ataxia
Areflexia
Term
Ptosis in Miller-Fisher may mimic what other condition
Definition
Myasthenia Gravis
Term
Describe the ataxia in Miller Fisher Syndrome
Definition
Truncal ataxia
Difficulty with tandem gait
positive Romberg
Term
Which direction of gaze is affected first in Miller Fisher
Definition
Upgaze first, then horizontal and down
Term
These ocular motor findings correlate with which syndrome
INO
One-and-a half syndrome
Pretectal Syndrome
Spasm of near reflex
Divergence paralysis
Definition
Miller Fisher
Term
True or False
Miller Fisher syndrome has detectable autoimmune antibodies
Definition
True in 90%
Term
4 methods of diagnosing Miller Fisher
Definition
Lumbar puncture
Look for Demyelination on EMG
Look for slow nerve conduction
IgG antibodies in bloodwork
Term
Which syndrome is a subset of Guillain Barre
Definition
Miller Fisher
Term
Treatment of Miller Fisher
Definition
IV immunoglobulin
Plasmapheresis
(Same as GBS)
Term
How does absence of apolipoprotein B affect metabolism
Definition
malabsorption of fat-soluble vitamins (D,A,K, E)
Term
Irritable Bowel, Ulcerative Colitis, Celiac and Crohn's all are similar to what condition that causes ophthalmoplegia
Definition
Abetalipoproteinemia
Term
Non-ocular motor findings of abetalipoproteinemia
Definition
Steatorrhea
Cerebellar ataxia
Areflexia
Loss of proprioception
Pigmentary retinopathy
Term
Ocular motor findings of abetalipoproteinemia
Definition
Ophthlamoplegia
Ptosis
Slow saccades
Dissociated nystagmus (fast adduction, slow abduction)
INO
Term
How may abetalipoproteinemia affect vision
Definition
Night blindness
Dyschromatopsia
Pigmentary retinopathy
Term
How is abetalipoproteinemia acquired
Definition
Autosomal recessive (thus congenital)
Term
How will abetalipoproteinemia manifest on small intestine biopsy
Definition
Lipid deposits in mucosa (PATHOGNOMONIC)
Term
How will abetalipoproteinemia manifest in blood work (2 ways)
Definition
spiny RBCs
very low total cholesterol
Term
Treatment of abetalipoproteinemia
Definition
Oral or IV vitamin A, E, K and iron
Term
3 possible results of lack of treatment of abetalipoproteinemia
Definition
Nyctalopia
Retinitis Pigmentosa
Intellectual disability
Term
which condition is caused by malabsorption of phytanic acid
Definition
Refsum's disease
Term
Ocular manifestations of Refsum's Disease
Definition
Ophthalmoplegia
Loss of pursuits
Early-onset cataracts
RP-like retinopathy
visual field defects
Term
4 systemic effects of Refsums Disease
Definition
Peripheral polyneuropathy
Cerebellar ataxia
anosmia
hearing loss
Term
Retinopathy associated with Refsums disease resembles what other form of retinopathy
Definition
Retinitis Pigmentosa
Term
Which ophthalmoplegia-causing condition is associated with skin changes
Definition
Refsums Disease, which causes ichthyosis
Term
2 ways to diagnose Refsums Disease
Definition
Blood serum levels of phytanic acid
abnormalities of the 3rd and 4th metatarsal and metacarpal
Term
Which foods should be avoided by those with Refsum's Disease
Definition
Dairy
Ruminant Animal meat
Fatty fish
Term
term for a dyskinesia caused by spontaneous discharge of the trochlear nerve
Definition
Superior Oblique Myokymia
Term
DDX of Superior Oblique Myokymia
Definition
Superior Oblique Palsy
Term
4 causes of Superior Oblique Myokymia
Definition
Fatigue
Caffeine
Aberrant regeneration
Vascular compression at root of trochlear nerve
Term
Describe the direction of problematic eye movements in Superior Oblique Myokymia
Definition
Torsional, vertical rotations
Term
3 triggers of superior oblique myokymia
Definition
Blinking
tilting head towards side of affected eye
Downgaze
Term
4 meds for treatment of SOM
Definition
Carbamazepine
gabapentin
Baclofen
Propanolol
Term
Surgery to treatment of SOM
Definition
Superior Oblique Tenotomy with ipsi IO
Term
How may ocular neuromyotonia be precipitated
Definition
Holding eyes in eccentric gaze, especially adduction
Term
Episodes of diplopia caused by involuntary contractions of extraocular muscles
Definition
Ocular Neuromyotonia
Term
What is the most common disorder of the neuromuscular junction
Definition
Myasthenia Gravis
Term
OCULAR myasthenia is more common in ______ whereas general MG is more commen in __________ (under 40yo)
Definition
OCULAR myasthenia is more common in men whereas general MG is more commen in _women__ (under 40yo)
Term
Fluctuating asymmetric external ophthalmoplegia with ptosis and weak eye closure is diagnostic of which condition
Definition
Ocular myasthenia gravis
Term
Name 2 symptoms that 2/3 of myasthenia gravis pts present with
Definition
Ptosis
Diplopia
Term
Pathophysiology of myasthenia gravis
Definition
acetylcholine receptor antibodies block post-synaptic acetylcholine receptors, preventing the excitatory effects of acetylcholine on the muscles.
Term
what direction of gaze may cause ptosis to worsen in MG
Definition
Upgaze
Term
Describe the curtain sign in MG
Definition
Manual elevation of one eyelid in bilateral ptosis leads to worsening of ptosis in fellow eye
Term
Cogan's lid-twitch sign
Definition
pt looks down for 15sec then back to primary gaze, upper lid overshoots upward then slowly resettles back to ptotic position
Term
Peek sign in MG
Definition
sustained eyelid closure leads to widening of palpebral fissure
Term
3 forms of pediatric MG
Definition
Juvenile
Neonatal
Congenital
Term
Describe saccades in MG
Definition
Hypermetric small saccades
Hypometric large saccades
Hyperfast saccades
Term
Nystagmus in MG
Definition
Gaze-evoked centripetal drift or fatigue nystagmus
Term
Classification of MG
Rapid progression of severe symptoms with respiratory crisis and poor drug response, high incidence of thymoma and high mortality.
Definition
Class III Severe
Term
Classification of MG
Slow progression, drug responsive, no crisis
Definition
Class IIa mild
Term
Pathophysiology of the tensilon test on Myasthenia Gravis
Definition
The edrophonium chloride temporarily blocks Ach reuptake
Term
3 methods of diagnosing Myasthenia Gravis
Definition
Tensilon Test
Single-fiber electromyography
Blood work for antibodies
Term
5 Treatments of Myasthenia Gravis
Definition
Prisms
Immunosuppressants
Plasmapheresis/ IV immunoglobulin
Mestinon (cholinesterase inhibitor)
Thymectomy
Term
3 benefits of thymectomy in patients with MG
Definition
Clinical Improvement
Drug-free remission
Reduced med requirements
Term
presynaptic disorder of neuromuscular transmission due to impaired release of acetylcholine
Definition
Lambert Eaton Myasthenic Syndrome (LEMS)
Term
2/3 of all cases of LEMS are caused by__________________________
Definition
2/3 of all cases of LEMS are paraneoplastic
Term
Where do the autoantibodies attach to, to cause LEMS
Definition
auto-antibodies attach to voltage gated calcium channels at the pre-synaptic neuromuscular junction
Term
3 symptoms of LEMS related to the autonomic nervous system
Definition
Blood pressure changes
Dizziness upon standing
Dry mouth
Term
3 autoimmune diseases associated with Lambert Eaton Myasthenic Syndrome
Definition
Pernicious Anemia
thyroid disease
Sjogren syndrome
Term
Muscles most severely affected by LEMS
Definition
Proximal Leg muscles
Pelvic girdle
Term
Why does LEMS cause muscle wasting
Definition
Trick Question
It does not generally cause wasting, just hyporeflexia
Term
2 diagnostic tests for LEMS
Definition
Electromyography
Nerve Conduction Velocity
Term
4 Treatments of LEMS
Definition
Plasmapheresis
Prednisone
Anticholinesterase meds
3,4-diaminopyridine
Term
Which recreational drug is associated with botulism
Definition
black tar heroin from Mexico
Term
Systemic features of Botulism
Definition
Nausea, vomiting, dysphagia and weakness of proximal muscles
Term
Ocular features of botulism
Definition
Ptosis
Ophthalmoplegia
dilated non reactive pupil
Term
Ocular eye movements with botulism
Definition
quivering hypometric multistep saccades with backward drifts
Term
Treatment of foodbourne botulism
Definition
Remove stomach contents, administer antitoxin
Term
Treatment of wound botulism
Definition
debride wound
administer antitoxin and antibiotic
Term
How does INO in MG present differently than in a regular INO
Definition
In MG it is partial INO, the adduction is slower than the abduction. In normal INO there is complete abduction.
Term
2 forms of ophthalmoplegia caused by mitochondrial myopathies
Definition
Chronic Progressive External Ophthalmoplegia
Kearns-Sayre Syndrome
Term
a subtype of Chronic Progressive External Ophthalmoplegia caused by a single mitochondrial DNA deletion
Definition
Kearns-Sayre Syndrome
Term
How is pigmentary retinopathyin Kearns-Sayre syndrome different from RP
Definition
KS retinopathy=mostly on posterior pole with salt and pepper appearance, mild nyctalopia
RP=peripheral and mid-peripheral with bone spicule appearance, severe nyctalopia
Term
Diagnosis of Kearns-Sayre Syndrome
Definition
Biopsy and staining of the orbicularis shows red ragged fibers which are a marker for dysfunction of mitochondrial DNA
Term
Treatment of Kearns-Sayre
Definition
Dietary-high lipids, low-carb, supplemental CoQ10, vitamins B1, and B7
Term
2 meds that should be specifically avoided in Kearns-Sayre syndrome
Definition
Valproate Phenobarbitol
Term
Differentiate CPEO vs. Myasthenia
Definition
CPEO-saccades are slow throughout the entire movement
Myasthenia-initial high speed of saccades
Term
What is the hallmark feature of MELAS syndrome
Definition
Stroke-like episodes before age 40
Term
MELAS syndrome
Definition
Mitochondrial Encephalomyopathy, Lactic Acidosis and Stroke-like episodes
Term
How does mitochondrial dysfunction manifest in MELAS syndrome
Definition
manifested as lactic acidosis or ragged-red muscle fibers
Term
Encephalopathy due to MELAS is characterized by 3 things
Definition
Developmental delay
dementia
seizures
Term
3 ocular manifestations of MELAS syndrome
Definition
Ophthalmoplegia
optic atrophy
pigmentary retinopathy
Term
T of F MELAS is associated with diabetes mellitus and hearing loss
Definition
True
Term
MNGIE syndrome
Definition
Mitochondrial Neuro-Gastro-Intestinal Encephalomyopathy syndrome
Term
Opthalmoplegia, Peripheral neuropathy, Leukoencephalopathy and recurrent nausea, vomiting or diarrhea with intestinal dysmotility
Definition
MNGIE syndrome
Term
SANDO syndrome
Definition
Sensory Ataxic Neuropathy Dysarthria and Ophthalmoplegia
Term
Which syndrome causes Ataxic neuropathy, dysarthria, ophthalmoplegia and sensory loss
Definition
SANDO syndrome
Term
what causes SANDO syndrome
Definition
multiple deletions of mitochondrial DNA
Term
Subacute Necrotizing Encephalomyelopathy is also known as ___________ syndrome
Definition
Leigh syndrome
Term
Name the 2 types of mutations that can cause Leigh Syndrome
Definition
Mutations of mitochondrial DNA (mitochondrial inheritance)
Mutations of nuclear DNA (autosomal recessive)
Term
Pathophysiology of Leigh syndrome due to mitochondrial mutation
Definition
deficiency of enzyme in respiratory chain leads to lactic acidosis, leads to respiratory or cardiac failure
Term
Ophthalmic findings in Leigh Syndrome
Definition
Optic atrophy
retained cell nuclei in lens cortex
Term
Ocular motility findings in Leigh syndrome
Definition
Gaze evoked, seesaw & upbeat nystagmus
Ocular tilt reaction
horizontal/vertical gaze palsies-->total ophthalmoplegia
Term
Ocular manifestations of Myotonic Dystrophy
Definition
bilateral ptosis
bilateral external ophthalmoplegia
miotic pupils
Visual-perceptual problems (due to intellectual disability)
Term
Cataract found in 100% of pts with myotonic dystrophy
Definition
Christmas Tree Cataract
Term
Which of the following was not listed in association with Myotonic Dystrophy
a. pigmentary retinopathy
b. subluxated lens
c. iris neo
d. corneal epithelial dystrophy
Definition
b. subluxated lens,
All the others may be seen in myotonic dystrophy
Term
What is the most prevalent neuromuscular disease in adults
Definition
Myotonic Dystrophy 1
Term
Which condition is especially common the in Sanguenay region of Quebec, Canada
Definition
Myotonic Dystrophy 1
Term
5 methods of diagnosing Myotonic Dystrophy
Definition
SLE for Christmas tree cataract
Electromyography
Electrocardiography->bradycardia
Muscle biopsy
Genetic testing
Term
5 treatments for Myotonic Dystrophy
Definition
Phenytoin for the myotonia
Cataract surgery
Pacemaker
Ptosis surgery
Prisms for diplopia
Term
Facial features associated with Myotonic dystrophy
Definition
Frontal balding
Hollowing of temporalis & masseter
Facial weakness
slackened mouth and long face
thin neck (wasting of SCN)
Term
The largest group of Americans with Oculopharyngeal Muscular dystrophy live where
Definition
Hispanics living in New Mexico
Term
Pts with oculopharyngeal Muscular Dystrophy often have bilateral ptosis, how do they compensate for this
Definition
They can develop the "Astrologist's posture" which is retroflexion of the head and downward gaze posture
Term
4 descriptions of ptosis associated with OMD
Definition
Acquired
Bilateral
Complete (almost)
Dramatic
Term
2 methoods of diagnosing Oculopharyngeal Muscular Dystrophy
Definition
High Creatine Phosphokinase levels
Abnormal vacuoles in muscle biopsy
Term
How is Oculopharyngeal Muscular Dystrophy differentiated from MG
Definition
Abnormal vaculoles in muscle biopsy in OMD and not in MG
Term
Treatment of oOMD
Definition
no cure, just treat symptoms, so ptosis surgery and gastrostomy for dysphagia
Term
2 conditions that can cause fibrotic dystrophy
Definition
Graves' Disease
Myositis
Term
do fibrotic dystrophies cause internal or external ophthalmoplegia
Definition
External
Term
Which extraocular muscles are affected by Graves' disease first
Definition
Inferior muscles (IR and IO)
Term
3 methods of diagnosing Graves' disease
Definition
Ultrasound/MRI of orbit
Thyroid function tests
Radioactive iodine scanning
Term
Ultrasound is used to diagnose Graves' disease, what are they looking for
Definition
Enlargement of EOMs with sparing of the tendons
Term
What substances are analyzed in thyroid function tests for Graves'
Definition
#!-Thyrotropin receptor antibodies (diagnostic)
free 13
free T4
TSH
Thyrotropin
antithyroglobulin
Term
Treatment of Graves' disease:
For hypothyroidism
Definition
Replacement therapy
Term
Treatment of Graves' disease:
for hyperthyroidism
Definition
radioablation therapy
Term
Treatment of Graves' disease:
For strabismus
Definition
Prisms or surgery
Term
Treatment of Graves' disease:
For exposure keratitis
Definition
Nocturnal taping
orbital decompression
Term
Treatment of Graves' disease:
For optic neuropathy
Definition
Corticosteroids
Radiation therapy
Orbital decompression
Term
Another term for orbital myositis found in the literature
Definition
Myositic form of idiopathic inflammatory orbital pseudotumor
Term
Manifestation of Orbital myositis
Definition
sudden onset of:
Diplopia w pain
conjunctival chemosis and edema
proptosis
Term
What do we see in ultrasound/CT/MRI of Orbital myositis
Definition
Enlargement of EOMS AND the tendons
Term
Differentiate imaging results of Graves' vs. Orbital myositis
Definition
Graves'- enlargement of EOMs but not tendons
Myositis-enlargement of EOMS and tendons
Term
Idiopathic inflammatory Orbital Pseudotumor may occur in association with lupus and….. (name 3 more)
Definition
RA
Sarcoidosis
Wegener's granulomatosis
Term
How boes bacterial myositis often occur (cause?)
Definition
After trauma, bacteria enters orbit via paranasal sinuses
Term
T or F
Infectious myositis causes ocular motility disturbances due to muscle invasion
Definition
False, rather it is caused by generalized inflammation with soft-tissue edema, not muscle invasion
Term
Ocular motility manifestations of Brown Superior Oblique Tendon Sheath Syndrome
Definition
Deficient elevation on adduction
normal or near normal elevation on abduction and in primary gaze
Term
Forced duction test results in Brown Superior Oblique Tendon Sheath syndrome
Definition
Mechanical restriction of upward and nasalward motion
Term
2 causes of congenital browns syndrome
Definition
Congenitally short or inelastic tendon
abnormal tendon-trochlear complex
Term
Treatment of congenital browns syndrome
Definition
SO tendon tenotomy with IO recession
Controlled weakening of SO using a silicone expander
Term
4 causes of Acquired Browns syndrome
Definition
Iatrogenic (SO surgery or RD surgery)
trauma
Paranasal sinus disease
RA
Term
Treatment for Acquired Browns syndrome
Definition
Same surgeries as for congenital
Local injection of corticosteroids
Term
Appearance of Grade 1 Third nerve palsy
Definition
Exotropia
Term
Appearance of Grade 2 Third nerve palsy
Definition
Exohypotropia
Term
Appearance of Grade 3 Third nerve palsy
Definition
Exohypotropia with ptosis
Term
Appearance of Grade 4 Third nerve palsy
Definition
Exohypotropia with ptosis and pupil involvement
Term
How will the eye be rotated in a CN III palsy
Definition
Intorted due to unopposed action of SO
Term
Appearance of pupil in Grade 4 CN III palsy
Definition
Dilated, poorly reactive
Term
Which directions of eye movement are affected in CN III palsy
Definition
adduction
elevation
depression
Term
5 causes of CN III palsy
Definition
ISCHEMIA
Hemorrhage
Infiltration or tumour
inflammation
compression
Term
Most common cause of CN III palsy
Definition
Ischemia-embolic or thrombotic occlusion of portions of basilar artery
Term
Syndrome with ipsilateral third nerve palsy plus contralateral facial hemiparesis
Definition
Weber syndrome
Term
Syndrome with ipsilateral third nerve palsy plus ipsilateral cerebellar ataxia and contralateral tremor
Definition
Claude syndrome
Term
In Claude syndrome, the cerebellar ataxia is due to involvement of the ___________ and tremor is due to involvement of the _____________________
Definition
In Claude syndrome, the cerebellar ataxia is due to involvement of the superior cerebellar peduncle and tremor is due to involvement of the red nucleus
Term
3 forms of congenital cranial dysinnervation syndrome
Definition
Congenital fibrosis of EOMS
Duanes
Mobius
Term
Why is congenital fibrosis of the EOMs not termed ophthalmoplegia
Definition
because the problem is in the orbit
Term
Syndrome that is thought to be due to congenital absence of one 6th nerve nucleus
Definition
Duane's syndrome
Term
Eye movement abnormalities in Duanes
Definition
marked limitation of abduction and variable adduction
Term
Which directions of gaze are involved in Duanes type II
Definition
aDDuction
Term
Syndrome characterized by palsies of one Adbucens and one Facial nerve
Definition
Mobius
Term
Which type of Duanes syndrome looks most like Mobius?
Definition
Type II Duanes
Term
Systemic effects of mobius
Definition
limb abnormalities/deformities, most commonly affecting the feet
Term
Pseudo-Graefe sign
Definition
retraction and elevation of eyelid on attempted downgaze
Term
If when a pt attempts to look down, instead their eyelid retracts and elevates, what nerve misdirection may have occurred
Definition
Misdirection of fibers for inferior rectus to levator palpebrae
Term
If when a pt attempts to adduct instead their eyelids eleavte, what nerve misdirection may have occurred
Definition
Misdirection of fibers for medial rectus to levator palpebrae
Term
What may cause occasional retrtaction of the globe on attempted vertical gaze
Definition
simultaneous contraction of elevators and depressors
Term
Oculomotor Nerve Synkinesis may cause what pupil abnormality
Definition
Pseudo-Argyll Robertson Pupil
Term
What nerve fiber misdirection can cause Pseudo-Argyll Robertson Pupil
Definition
Misdirection of fibers to medial rectus to pupillary sphincter muscles
Term
All vertical deviations are due to ___________ ___________ palsy until proven otherwise
Definition
Superior oblique palsy
Term
Most common cause of 4th nerve paresis
Definition
Head trauma
Term
2 ways to DDX Trochlear Palsy and Skew Deviation
Definition
Skew deviation-magnitude of deviation is same in all positions of gaze
Trochlear-magnitude of deviation is greater in some positions of gaze

Head tilt- contralateral in Trochlear, in same direction as cyclotorsion in skew
Term
Neoplasm is commonly a cause of which nerve paresis
Definition
6th nerve
Term
4 Treatments for EOM Palsies
Definition
Prisms
Botox
Surgery
Vision Therapy
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