The patient with diplopia

The Patient With Diplopia

The Patient With DiplopiaSiraj Safi

Diplopia is the most common symptom for which the patient needs urgent ophthalmic care

The two most common mechanisms for diplopia are ocular misalignment and ophthalmic aberrations

What does the faculty of BSV require?

Perfect ( or near perfect ) alignment of the visual axes simultaneously on the object of regardPerfect ( or near perfect ) retinal correspondencePerfect central ( or paracentral ) fusional capability. Perfect ( or near perfect ) alignment of the retinal receptorsPerfect ( or near perfect ) optics to allow only one image to be formed on the retina and the same single image to be formed on the other

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What is Diplopia ?

It is when more than one image ( two ) of the object of regard are seen simultaneously

Physiological Pathological

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Monocular vs Binocular DiplopiaKey question

Is the double vision present even on monocular eye closure?

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Monocular diplopiaMore than one image of the object of regard is formed in the retinae of one or both eyes..Irregular astigmatism ( corneal scars, haze, corneal distortion)Subluxated clear lensesPoorly fitting contact lensesEarly cataractMacular disorders edema, CNVM etc

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Binocular DiplopiaThe eyes lose their simultaneous alignment with the object of regard in one or more directions of gazeKey cluesAnomalous Head PositionVision Blurry in one gaze position, better in anotherObviously misaligned eyes, proptosisPresence of partial ptosisNystagmus

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History: A detailed history will be required to enquire about the patient current status:i.e. Either the diplopia eliminates after closing one eye (binocular diplopia)The diplopia remains after closing one eye (monocular diplopia)Monocular diplopia must not be confused with metamorphopsia secondary to maculopathy

Questions about the nature of the particular condition:Key questionsIs the diplopia more for distance or near? Is the diplopia predominantly horizontal or vertical?In which direction of gaze are the images maximally separated?To which eye does the outer image belong? Is there a predominant tilt? In which position of gaze does the tilt increase maximally?

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Physical examination:Movement of both eyesExternal examination for proptosis /ptosis, rednessDuctions VersionsAssessment of eye movements in all position of gazesComparison between near and far fixation

Identifying muscle/s involvedAHPPredominant face turn horizontal rectiPredominant chin elev/dep vertical recti, pattern strabismusPredominant tilt Obliques

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Purpose:Wether the misalignment is comitant or Incomitant

Comitant strabismus are congenital in nature while Incomitant gives a picture of acquired abnormality / disorder

Initial Assessment:Gross examinationAlternating cover test in all 9 position of gazesMaddox rod testDouble Maddox rod test (torsional misalignment)Red/Green gogglesPrism cover test

Differentiating Paretic From Restrictive Etiologies of Diplopia

Restrictive causes:ProptosisEnophthalmosOrbital traumaThyroid ophthalmopathyPost surgery

Differential diagnosis:Restrictive disorderParetic disorderSaccadic movements are normalSaccadic movements are slowerMechanical restriction on FDTFDT is normalIncrease IOP at eccentric fixationNo change in IOP at any gazeDuction =VersionDuction more than version

Causes of Diplopia:SupranuclearNuclear InternuclearInfranuclearMyopathicRestrictiveOrbital

Supranuclear causes:It includes any afferent input to the ocular motor nervesIt controls vestibular input to adjust the relative position of the eyes within the headIt allow accurate calculation of saccadic velocity and directionMost supranuclear disorders affect both eyes equally and do not cause diplopia

Continue:It involves:Skew DeviationThalamic esodeviationVergence Dysfunction

Skew Deviation

Skew deviation is an acquired vertical misalignment of the eyes resulting from asymmetric disruption of supranuclear input from the otolithic organs (the utricle and saccule of the inner ear, both of which contain otoliths, which are tiny calcium carbonate crystals).These organs sense linear motion and static head tilt via gravity and transmit information to the vertically acting ocular motoneurons, as well as to the interstitial nucleus of Cajal (INC), all of which are in the midbrain.

Skew Deviation

An alternating skew deviation on lateral gaze usually manifests as hypertropia of the abducting eye (ie, right hypertropia on right gaze) that switches when gaze is directed to the opposite side (ie, becoming left hypertropia on left gaze). Responsible lesions are located in the cerebellum, cervicomedullary junction, or dorsal midbrain. This disorder must be distinguished from bilateral fourth nerve palsies,

Thalamic EsodeviationThalamic esodeviation is an acquired horizontal strabismus that may be observed in patients with lesions near the junction of the diencephalon and midbrain, most often thalamic hemorrhage. The esodeviation may develop insidiously or acutely and, in the case of expanding tumors, may be progressive. It is especially important to consider the possibility of a central nervous system lesion in children being evaluated for strabismus surgery.

Vergence Disorders

Although vergence disorders are common, their diagnosis can be challenging because convergence depends heavily on patient effort. These disorders most commonly are classified as convergence insufficiency, convergence spasm, or divergence insufficiency. Unlike other supranuclear disorders, vergence disorders usually result in diplopic symptoms.

Convergence paralysisConvergence paralysis may be secondary to various organic processes such as encephalitis, diphtheria, multiple sclerosis and occlusive vascular disease involving the rostral midbrainPatients usually present with exotropia and diplopia for near fixation.They will have normal adduction of either eyeThe deviation can be said to be concomitant across the field of gaze.

Common supra nuclear disorders

INO

INO is a lesion of the Medial longitudinal fascicules (MLF) resulting in a palsy of the MR muscle and a dissociated gaze evoked nystagmus in the abducting eye .

INOINO may be unilateral or bilateral.Aetiology:Multiple Sclerosis in younger patients.Brainstem tumorTrauma etc.

INOFor right lateral gaze: The PPRF signals the right CN VI (Abducens) nucleus to turn the right eye outwardsIt also signals the left CN III (Occulomotor) nucleus, via the left MLF to simultaneously turn the left eye inwardsThus, a lesion of the left MLF, prevents the impulse from reaching the left medial rectusThe right eye abducts, left eye does not adductAnd nystagmus is seen in the right eye

LOOK TO THE RIGHT!!!

IIIIIIVIVIPPRFPPRF

RIGHTLEFT

INTERNUCLEAR OPHTHALMOPLEGIA

Left internuclear ophthalmoplegia

INOSymptoms and Signs: Painless onset of visual disturbance Diplopia usually not in the primary position horizontal diplopia in the lateral gaze Convergence usually normal

INOClinical and diagnostic features:The principle feature is abducting nystamus.Limitation of the MR of the affected eye in adduction during conjugate movement.There is usually an exophoria or exotropia in the primary position. Which increases in the direction of affected MR in horizontal gaze movement.

Defective left adductionNormal left gazeIntact convergence INO involving left MLF

INODeferential diagnosing

Myasthenia gravisMedial wall blow out fractureDuane's retraction syndromeMR palsy

Bilateral INOLesions that affect the interneurones running in the MLF from both sixth nerve nuclei result in bilateral INOWith loss of adduction of each eye on attempted contralateral versionAbducting nystagmus is present in each eye on lateral gazeProvided the lesion is limited to the interneurones, convergence is retained.Bilateral INO is often asymmetric

INOManagementNeurological investigation.Identify and treat the underlying cause.Monitor the condition.

One and a half eye syndrome

Description:One-and-a-half syndrome consists of a unilateral internuclear ophthalmoplegia and a contralateral horizontal gaze palsy.Causes include demyelination, vascular, tumour and inflammation

One and a half eye syndrome lesion involving MLF and ipsilateral 6-nerve nucleus and PPRFIpsilateral gaze palsyDefective ipsilateral adductionNormal contralateral abduction with ataxic nystagmus

FeaturesThe only remaining horizontal movement is abduction by the unaffected lateral rectus, which is associated with the typical abducting nystagmusWhen the patient attempts to fixate with this eye in the primary position, the nystagmus will reduce or cease. There is therefore a palsy of conjugate gaze on one side and an INO on looking to the other side

Defective left gazeDefective left side adduction with normal right side abduction

Left side 1 Syndrome

Nuclear palsies

Oculomotor Nerve Palsy

Nuclear PortionAnatomy

Nucleus lies at the level of superior colliculus in the periaqueductal gray mater of midbrain.

It is a complex of subnuclei

Levator muscles show projection form single midline subnucleus

Superior rectus subnucleus has a crossed projection Edinger-westphal nucleus gives parasymathetic input to ipsilateral pupil

Nuclear Portion Causes

Infraction Hemorrhage Neoplasia Abcess

NUCLEAR PORTIONClinical features Symptoms of iii cn dysfunction Diplopia mixed horizontal and vertical, binocular) Ptosis of varying degrees) Glare due to pupil dilatation

In nuclear lesions Ptosis should be bilateral or absent Sr muscle may be Involved contralaterally and spared ipsilaterally But commonly affected bilaterally

FASCICULUS Anatomy Passes through the red nucleus, and exits the brain stem through the medical portion of each cerebral peduncle

Causes InfractionHemorrhageNeoplasia

FASCICULUS: CLINICAL FEATURE III CN palsy may be associated with

Cerebellar ataxia (Nothnagel syndrome)Ipsilateral flapping hand tremor (rubral tremor) and contralateral sensory loss (Benedikt syndrome)

Contralateral hemiplegia or hemiparesis (Weber syndrome)

SUBARACNOID PORTION: ANATOMY

Passes between posterior cerebral and superior cerebellar arteriesParallels the tentorial edge and the posterior communicating artery Pupillomotor fibers are located superficially and derive their blood supply from pial blood vessels while the main trunk is supplied by vasanervosum

SUBARACNOID PORTION:CAUSESAneurysm (of post communicating artery) (most common cause)Infectious meningitis Bacterial, fungal/parasitic, viral Granulomatous inflammation (sarcoidosis, lymphomatoid granulomatosis, WegenerMeningeal infiltration (carcinomatous, lymphomatous or leukemic)Head trauma resulting in temporal lobe herniation.

SUBARACNOID PORTION: CLINICAL FEATURE III CN palsy may be associated with Signs and symptoms of subaracnoid hemorrhage like severe headache, stiff neck and loss of consciousness. (bleeding from berry aneurysm) Sign and symptoms of meningitis (basal meningeal infection) Progressive involvement of other cranial nerves (inflammatory and neoplastic infiltration)Early pupil involvement in cases of compressive lesions and relative sparing in cases of microangiopathies.

INTRACAVEROUS PORTION: ANATOMY Runs dorsal to IV CN in the lateral wall of cavernous sinus Causes Tumor pituitary adenoma, meningioma, craniopharyngiona, metastatic carcinoma Gaint intracavernous aneurysm Carotid artery-cavernous sinus fistula Cavernous sinus thrombosis Ischemia from microvascular disease in vasa nervosa Inflammatory Tolosa-Hunt syndrome (Idiopathic or granulomatous inflammation)

INTRACAVEROUS PORTION: CLINICAL FEATURES

III CN palsy is often accompanied by other cranial nerve palsies in the vicinity like IV, VI & V cranial nerve.An important exception is nerve infraction associated with microvascular disease. Significant pupillary involvement may occur in 10% of cases.

ORBITAL PORTION: ANATOMY Enters the orbit through superior orbital fissure and divides into :

Superior branchSR & L.P. Superiors muscles

Inferior branchIR, MR, IO muscles Parasympathetic fibers to the ciliary ganglion

ORBITAL PORTIONCAUSESTrauma Inflammatoryorbital inflammatory pseudotumor Orbital myositis Endocrine thyroid orbitopathyTumors hemangioma, lymphangioma, meningioma CLINICAL FEATURES III CN palsy may be associated with inflammatory signs e.g. lid swelling, conjunctival injection and chemosisIV & VI CN may be involved.

posterior communicating artery aneurysm and third cranial nerve palsy

. Complete right third-nerve palsy resulting in hypotropia, exotropia, and pupillary mydriasis. The ptotic eyelid is manually elevated.

Right third nerve palsyshowing limitation of elevationSame patient as in Figure 2 showinglimitation of adductionSame patient showing limitation of depression

Table 1. Acquired lesions of the oculomot or nerveAnatomic LocalizationCauseAssociated SymptomsDiagnostic EvaluationTherapyNuclearInfarction, mass, infection, inflammation, compressionBilateral ptosis and paresis of the contralateral superior rectus; lid function may be sparedMR imagingStroke resuscitation, antiplatelet therapy, coumadinWernicke-Korsakoff syndromeAtaxia, abducens palsy, nystagmus, altered mentationStroke workup*ThiamineFascicularInfarction, mass, infection, inflammation, compressionContralateral hemiparesis or tremor; pupil may be sparedMR imagingStroke resuscitation, antiplatelet therapy, coumadinDemyelinationStroke workup*Interferon-1, CopaxoneSubarachnoid spaceAneurysmHeadache, stiff neck, pupil-involved, aberrant regenerationMR imaging, MR angiography, angiogramInterventional radiology, surgical clippingVasculopathicPupil generally spared, no aberrant regenerationCheck BP, serum glucose, ESR, RPR, cholesterolSpontaneous recoveryMeningitisHeadache, cranial nerve involvement, meningismus, feverLP, MR imagingAntibiotics, steroids, supportive careMiller-Fisher syndromeAreflexia, ataxia, previous viral illnessNCS/EMG, LPPlasmapheresis, IVIGMigraineHeadache, positive family history, pediatric age groupMR imaging on initial eventRocovery generally goodUncal herniationEarly pupil involvement, altered mentation, ipsilateral hemiparesisEmergent CT scanEmergent hyperventilation, osmolar therapy

Uncal herniationEarly pupil involvement, altered mentation, ipsilateral hemiparesisEmergent CT scanEmergent hyperventilation, osmolar therapyCavernous sinusNeoplasmCSS, pain, sensory changes, potential sympathetic involvementMR imagingSurgery, radiation therapy, possible hormonal modulationFistulaExophthalmos, bruit, chemosisMR imaging, angiographySurgical ligation interventional radiologyThrombosisPrevious infection/trauma, pain, exophthalmos, chemosisMR imaging, angiographyAntibiotics, thrombolysisTolosa-Hunt syndromePain, pupil may be sparedMR imaging, evaluate for collagen-vascular diseaseSteroidsApoplexyHeadache, bilateral ophthalmoplegia, altered mentation, vision lossMR imaging, electrolyte and hormonal workupSurgical decompression, electrolyte maintenanceSuperior orbital fissureNeoplasmSuperior division involvement (ptosis and superior rectus paresis)MR imagingSurgery, radiation therapy, possible hormonal modulationNeuromuscular junctionMyasthenia gravisNo pupil involvement, frequent fluctuation, ptosis, ophthalmoparesis, orbicularis oculi weakness, dysarthriaTensilon test, electrodiagnostics, antiacetylcholine receptor antibody titerImmunosuppression, mestinon* B

TROCHLEAR NERVE: (IV CN)Underlying etiology of the congenital IV CN palsy remains obscure Acquired IV CN palsy may be Idiopathic Traumatic Microvasculopathy secondary to diabetes, atherosclerosis or hypertension Thyroid ophthalmopathy Myasthenia gravis Iatrogenic injury Tumors (pinealoma, teratoma) Aneurysms

IV CN PALSY: CLINICAL FEATURE Patient reports vertical, torsional or oblique diplopia. It is worse on downgaze & gaze away from the side of affected muscle Pt. often adopts characteristic head tilt away from affected side Bielschowsky head tilt test is extremely useful Double maddox rod test can be used to measure excyclotorsion 3o 10o excyclotorsion unilateral IV CN palsy > 100 excyclotorsion Bilateral IV CN palsy

A 2-year-old girl with compensatory left head tilt due to congenital right superior oblique palsy. Patient with traumatic bilateral superior oblique palsy; note right hypertropia on right head tilt and left hypertropia on left head tilt.

IV CN NUCLEUS Lies in periaqueductal gray mater in the midbrain Nuclear lesions may involve the descending sympathetic fibers leading to contralateral IV CN palsy with ipsilateral Horner syndrome

SUBARACHNOID COURSE From the dorsal brainstem it runs just below the tentorial edge and may be damaged by neurosurgical procedure and head trauma.

Table 2. Acquired lesions of the trochlear nerveAnatomic LocalizationCauseAssociated SymptomsDiagnostic EvaluationTherapyNuclearInfarctionInternuclear ophthalmoplegia, Horner's syndrome, afferent pupillary defectMR imaging, stroke workup*Stroke resuscitation, antiplatelet therapy, coumadinTruma, tumor, infection, inflammationAs aboveMR imaging, LPDepends on causeFascicularInfarctionInternuclear ophthalmoplegia, Horner's syndrome, afferent pupillary defectMR imaging, stroke workup*Stroke resuscitation, antiplatelet therapy, coumadinTrauma, trmor, infection, inflammationAs aboveMR imaging, LPDepends on causeDemyelinationIsolated or with midbrain signs aboveMR imagingInterferor-1, CopaxoneSubarachnoid spaceTrauma, hydrocephalusMay be bilateralNeuroimagingNeurosurgical consultVasculopathicUsually isolatedCheck BP, serum glucose, ESR, RPR, cholesterolSpontaneous recoveryMass lesionContralateral hemiparesis or ipsilateral ataxiaMR imagingNeurosurgical consultCavernous sinusAs in third nerve palsyCavernous sinus syndromeHerpes zoster ophthalmicusRash, trigeminal sensory loss in the V1 or V2 distributionAntiviral medicationsOrbitInflammation, trauma, tumorOculomotor, abducens, optic nerve dysfunction, proptosisMR imaging of the orbit or orbital ultrasound* V

Anatomic LocalizationCauseAssociated SymptomsDiagnostic EvaluationTherapyNuclearInfarctionInternuclear ophthalmoplegia, Horner's syndrome, afferent pupillary defectMR imaging, stroke workup*Stroke resuscitation, antiplatelet therapy, coumadinTruma, tumor, infection, inflammationAs aboveMR imaging, LPDepends on causeFascicularInfarctionInternuclear ophthalmoplegia, Horner's syndrome, afferent pupillary defectMR imaging, stroke workup*Stroke resuscitation, antiplatelet therapy, coumadinTrauma, trmor, infection, inflammationAs aboveMR imaging, LPDepends on causeDemyelinationIsolated or with midbrain signs aboveMR imagingInterferor-1, CopaxoneSubarachnoid spaceTrauma, hydrocephalusMay be bilateralNeuroimagingNeurosurgical consultVasculopathicUsually isolatedCheck BP, serum glucose, ESR, RPR, cholesterolSpontaneous recoveryMass lesionContralateral hemiparesis or ipsilateral ataxiaMR imagingNeurosurgical consultCavernous sinusAs in third nerve palsyCavernous sinus syndromeHerpes zoster ophthalmicusRash, trigeminal sensory loss in the V1 or V2 distributionAntiviral medicationsOrbitInflammation, trauma, tumorOculomotor, abducens, optic nerve dysfunction, proptosisMR imaging of the orbit or orbital ultrasound* V

ABDUCENS NERVE PALSY:

VI CN PALSY: CAUSES

Idiopathic Trauma Ischemic Aneurysm inflammatorygaint cell arteritis Neoplastic demyelinating disorders intracranial pressure

VI CN PALSY: CLINICAL FEATURES

Esotropia Face turn Diplopia Vision lossPain Hearing loss

abducens palsy caused by vasculopathic injury. There is a large angle esotropia in left lateral gaze.

VI CN PALSY: NUCLEUS

It contains both motor neurons and interneurons that project along the contralateral MLF to the contralateral MR subnucleus, so lesions result in gaze palsy.

VI CN PALSY: FASCICULES

If the damage to the fasciculus occurs in the ventral pons the pyramidal tract is involved causing contralateral hemiplegia (Millard-Gubler syndrome) In subarachnoid space Trauma may affect as it ascends the clivus or as it crosses the petrous pyramid e.g. Basilar skull fracture Raised intracranial presure can cause streching of VI CN Lesions arising in the cerebellopontine angle may involve e.g. Acoustic neurinomas and meningioms

VI CN PALSY: PETROUS PYRAMID

Can be compressed by dilated inf. Petrosal sinus in Dorellos canal e.g. CC Fistula Petrositis secondary to otitis media or mastoiditis (Gradenigo syndrome)

INTRA CAVERNOUS

May be associated with postganglionic Horner syndrome

Table 3. Acquired lesions of the abducens nerveAnatomic LocalizationCauseAssociated SymptomsDiagnostic EvaluationTherapyNuclearInfarctionIpsilateral facial paralysis, INOMR imaging, stroke workup*Stroke resuscitation, antiplatelet therapy, coumadinInfiltration, trauma, inflammationMR imagingVaries with causeWernicke-Korsakoff syndromeAtaxia, nystagmus, altered mentationThiamineFascicularInfarction, tumor, inflammation, MSIpsilateral facial nerve paralysis and contralateral hemiplegia (Miller-Gubler syndrome)MR imaging, stroke workup*Stroke resuscitation, antiplatelet therapy, coumadin, surgical treatment, interferon-1 or CopaxoneAnterior inferior cerebellar artery infarctionIpsilateral facial paralysis, loss of taste, ipsilateral Horner's syndrome, ipsilateral trigeminal dysfunction, and ipsilateral deafness (Foville's syndrome)

Subarachnoid spaceMassContralateral hemiparesisMR imagingIschemiaUsually isolatedMR imaging if no recovery after 3 monthsTraumaPapilledema, headacheDiamox, ventriculoperitoneal shuntIntracranial hypertensionHeadacheCSF study Blood patchIntracranial hypotensionPetrous apexMastoiditis, skull fracture, lateral sinus thrombosis, neoplasms, tumorIpsilateral facial paralysis, severe facial painCT or MR imagingAntibiotics, neurosurgical intervention, anticoagulationCavernous sinusAs with third nerve palsyCavernous sinus syndrome* I

NeuromyotoniaNeuromyotonia, a rare but important cause of episodic diplopia, is thought to be neurogenic in origin. Prior skull-base radiation therapy, typically for neoplasm (eg, meningioma), is the most common historical feature. Months to years post radiation, patients experience episodic diplopia lasting typically 30-60 seconds. Neuromyotonia may affect any of the ocular motor nerves or their divisions.

NeuromyotoniaDiplopia is often triggered by activation of the affected nerve, during which overaction of the nerve produces ocular misalignment( eg, abducens nerve neuromyotonia episodes produce abduction of the involved eye and attendant exotropia). The disorder generally responds quite well to medical therapy; carbamazepine and its derivatives are the first-line treatment

Myopathic, Restrictive, and Orbital Causes of DiplopiaThyroid Eye DiseaseThe most common cause of restrictive strabismus in adults is thyroid eye disease (TED).Any of the extraocular muscles may be involved, but the inferior and medial recti are most commonly affected.

Posttraumatic Restriction

Blowout fractures of the orbit often cause diplopia. The most typical presentation involves fracture of the orbital floor with entrapment of the inferior rectus muscle or its fascial attachments to the orbital tissues.

Post- Cataract Extraction Restriction

Binocular diplopia can result from injury to or inflammation within the inferior rectus orother muscles after retrobulbar injection for cataract or other ocular surgery. The onset of vertical diplopia just after surgery initially suggests nerve damage or myotoxicity from the local anesthetic.

Orbital MyositisIdiopathic inflammation of one or more extraocular muscles typically produces ophthalmoplegia and pain, often with conjunctival hyperemia, chemosis, and sometimes proptosis.The pain may be quite intense and is accentuated by eye movementsOrbital myositis related pain usually responds within 24 hours to systemic corticosteroid therapy, whereas diplopia may take longer to resolve.

Neoplastic InvolvementInfiltration of the orbit by cancer, especially from the surrounding paranasal sinuses, can impair eye movements because of either extraocular muscle infiltration or involvement of the ocular motor cranial nerves. Relative enophthalmos or associated eyelid "hang-up" on downgaze may accompany the diplopia. Occasionally, extraocular muscles may be the site of a metastatic tumor.

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Brown Syndrome

Brown syndrome is a restrictive ocular motor disorder that produces limited up gaze when the affected eye is in the adducted position (Fig 8-14). This pattern of motility is usually congenital but can be acquired. which produces an ipsilateral hypodeviation that increases on upgaze to the opposite side Acquired cases result from damage or injury to the trochlea, which may cause a "click" that the patient can feel.Caused by Inflammatory disease, or trauma, but it may, rarely, be a manifestation of a focal metastasis of a neoplasm to the superior oblique muscle.

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