The patient with diplopia

The patient with diplopia

1. The Patient With Diplopia Siraj Safi

2. 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

3. What does the faculty of BSV require? Perfect ( or near perfect ) alignment of the visual axes simultaneously on the object of regard Perfect ( or near perfect ) retinal correspondence Perfect central ( or paracentral ) fusional capability. Perfect ( or near perfect ) alignment of the retinal receptors Perfect ( 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

4. What is Diplopia ? It is when more than one image ( two ) of the object of regard are seen simultaneously Physiological Pathological

5. Monocular vs Binocular Diplopia Key question Is the double vision present even on monocular eye closure?

6. Monocular diplopia More 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 lenses Poorly fitting contact lenses Early cataract Macular disorders – edema, CNVM etc

7. Binocular Diplopia The eyes lose their simultaneous alignment with the object of regard in one or more directions of gaze Key clues Anomalous Head Position Vision Blurry in one gaze position, better in another Obviously misaligned eyes, proptosis Presence of partial ptosis Nystagmus

8. 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

9. Questions about the nature of the particular condition: Key questions Is 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?

10. Physical examination: Movement of both eyes External examination for proptosis / ptosis , redness Ductions Versions Assessment of eye movements in all position of gazes Comparison between near and far fixation

11. Identifying muscle/s involved AHP Predominant face turn – horizontal recti Predominant chin elev/dep – vertical recti, pattern strabismus Predominant tilt – Obliques

12. Purpose: Wether the misalignment is comitant or Incomitant Comitant strabismus are congenital in nature while Incomitant gives a picture of acquired abnormality / disorder

13. Initial Assessment: Gross examination Alternating cover test in all 9 position of gazes Maddox rod test Double Maddox rod test (torsional misalignment) Red/Green goggles Prism cover test

14. Differentiating Paretic From Restrictive Etiologies of Diplopia

15. Restrictive causes: Proptosis Enophthalmos Orbital trauma Thyroid ophthalmopathy Post surgery

16. Differential diagnosis: Restrictive disorder Paretic disorder Saccadic movements are normal Saccadic movements are slower Mechanical restriction on FDT FDT is normal Increase IOP at eccentric fixation No change in IOP at any gaze Duction =Version Duction more than version

17. Causes of Diplopia: Supranuclear Nuclear Internuclear Infranuclear Myopathic Restrictive Orbital

18. Supranuclear causes: It includes any afferent input to the ocular motor nerves It controls vestibular input to adjust the relative position of the eyes within the head It allow accurate calculation of saccadic velocity and direction Most supranuclear disorders affect both eyes equally and do not cause diplopia

19. Continue: It involves: Skew Deviation Thalamic esodeviation Vergence Dysfunction

20. 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.

21. 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,

22. Thalamic Esodeviation Thalamic 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 .

23. 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.

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

25. Common supra nuclear disorders

26. 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 .

27. INO INO may be unilateral or bilateral. Aetiology : Multiple Sclerosis in younger patients. Brainstem tumor Trauma etc.

28. INO For right lateral gaze: The PPRF signals the right CN VI ( Abducens ) nucleus to turn the right eye outwards It also signals the left CN III ( Occulomotor ) nucleus, via the left MLF to simultaneously turn the left eye inwards Thus, a lesion of the left MLF, prevents the impulse from reaching the left medial rectus The right eye abducts, left eye does not adduct And nystagmus is seen in the right eye

29. “LOOK TO THE RIGHT!!!” III III VI VI PPRF PPRF RIGHT LEFT INTERNUCLEAR OPHTHALMOPLEGIA

30. Left internuclear ophthalmoplegia

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

32. INO Clinical 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.

33. Defective left adduction Normal left gaze Intact convergence INO involving left MLF

34. INO Deferential diagnosing Myasthenia gravis Medial wall blow out fracture Duane's retraction syndrome MR palsy

35. Bilateral INO Lesions that affect the interneurones running in the MLF from both sixth nerve nuclei result in bilateral INO With loss of adduction of each eye on attempted contralateral version Abducting nystagmus is present in each eye on lateral gaze Provided the lesion is limited to the interneurones , convergence is retained. Bilateral INO is often asymmetric

36. INO Management Neurological investigation. Identify and treat the underlying cause. Monitor the condition.

37. One and a half eye syndrome

38. 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

39. One and a half eye syndrome lesion involving MLF and ipsilateral 6-nerve nucleus and PPRF Ipsilateral gaze palsy Defective ipsilateral adduction Normal contralateral abduction with ataxic nystagmus

40. Features The only remaining horizontal movement is abduction by the unaffected lateral rectus, which is associated with the typical abducting nystagmus When 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

41. Defective left gaze Defective left side adduction with normal right side abduction Left side 1 ½ Syndrome

42. Nuclear palsies

43. Oculomotor Nerve Palsy

44. Nuclear Portion Anatomy 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

45. Nuclear Portion Causes Infraction Hemorrhage Neoplasia Abcess

46. NUCLEAR PORTION Clinical 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

47. FASCICULUS Anatomy Passes through the red nucleus, and exits the brain stem through the medical portion of each cerebral peduncle Causes Infraction Hemorrhage Neoplasia

48. 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)

49. SUBARACNOID PORTION: ANATOMY Passes between posterior cerebral and superior cerebellar arteries Parallels 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

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

51. 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.

52. 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)

53. 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.

54. ORBITAL PORTION: ANATOMY Enters the orbit through superior orbital fissure and divides into : Superior branch SR & L.P. Superiors muscles Inferior branch IR, MR, IO muscles Parasympathetic fibers to the ciliary ganglion

55. ORBITAL PORTION CAUSES Trauma Inflammatory orbital inflammatory pseudotumor Orbital myositis Endocrine thyroid orbitopathy Tumors hemangioma , lymphangioma , meningioma CLINICAL FEATURES III CN palsy may be associated with inflammatory signs e.g. lid swelling, conjunctival injection and chemosis IV & VI CN may be involved.

56. posterior communicating artery aneurysm and third cranial nerve palsy

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

58. Right third nerve palsy showing limitation of elevation Same patient as in Figure 2 showing limitation of adduction Same patient showing limitation of depression

59. Table 1. Acquired lesions of the oculomot or nerve Anatomic Localization Cause Associated Symptoms Diagnostic Evaluation Therapy Nuclear Infarction, mass, infection, inflammation, compression Bilateral ptosis and paresis of the contralateral superior rectus; lid function may be spared MR imaging Stroke resuscitation, antiplatelet therapy, coumadin Wernicke-Korsakoff syndrome Ataxia, abducens palsy, nystagmus, altered mentation Stroke workup* Thiamine Fascicular Infarction, mass, infection, inflammation, compression Contralateral hemiparesis or tremor; pupil may be spared MR imaging Stroke resuscitation, antiplatelet therapy, coumadin Demyelination Stroke workup* Interferon-β1, Copaxone Subarachnoid space Aneurysm Headache, stiff neck, pupil-involved, aberrant regeneration MR imaging, MR angiography, angiogram Interventional radiology, surgical clipping Vasculopathic Pupil generally spared, no aberrant regeneration Check BP, serum glucose, ESR, RPR, cholesterol Spontaneous recovery Meningitis Headache, cranial nerve involvement, meningismus, fever LP, MR imaging Antibiotics, steroids, supportive care Miller-Fisher syndrome Areflexia, ataxia, previous viral illness NCS/EMG, LP Plasmapheresis, IVIG Migraine Headache, positive family history, pediatric age group MR imaging on initial event Rocovery generally good Uncal herniation Early pupil involvement, altered mentation, ipsilateral hemiparesis Emergent CT scan Emergent hyperventilation, osmolar therapy

60. Uncal herniation Early pupil involvement, altered mentation, ipsilateral hemiparesis Emergent CT scan Emergent hyperventilation, osmolar therapy Cavernous sinus Neoplasm CSS, pain, sensory changes, potential sympathetic involvement MR imaging Surgery, radiation therapy, possible hormonal modulation Fistula Exophthalmos, bruit, chemosis MR imaging, angiography Surgical ligation interventional radiology Thrombosis Previous infection/trauma, pain, exophthalmos, chemosis MR imaging, angiography Antibiotics, thrombolysis Tolosa-Hunt syndrome Pain, pupil may be spared MR imaging, evaluate for collagen-vascular disease Steroids Apoplexy Headache, bilateral ophthalmoplegia, altered mentation, vision loss MR imaging, electrolyte and hormonal workup Surgical decompression, electrolyte maintenance Superior orbital fissure Neoplasm Superior division involvement (ptosis and superior rectus paresis) MR imaging Surgery, radiation therapy, possible hormonal modulation Neuromuscular junction Myasthenia gravis No pupil involvement, frequent fluctuation, ptosis, ophthalmoparesis, orbicularis oculi weakness, dysarthria Tensilon test, electrodiagnostics, antiacetylcholine receptor antibody titer Immunosuppression, mestinon * B

61. 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

62. 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

63. 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.

64. 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.

65. Table 2. Acquired lesions of the trochlear nerve Anatomic Localization Cause Associated Symptoms Diagnostic Evaluation Therapy Nuclear Infarction Internuclear ophthalmoplegia, Horner's syndrome, afferent pupillary defect MR imaging, stroke workup* Stroke resuscitation, antiplatelet therapy, coumadin Truma, tumor, infection, inflammation As above MR imaging, LP Depends on cause Fascicular Infarction Internuclear ophthalmoplegia, Horner's syndrome, afferent pupillary defect MR imaging, stroke workup* Stroke resuscitation, antiplatelet therapy, coumadin Trauma, trmor, infection, inflammation As above MR imaging, LP Depends on cause Demyelination Isolated or with midbrain signs above MR imaging Interferor-β1, Copaxone Subarachnoid space Trauma, hydrocephalus May be bilateral Neuroimaging Neurosurgical consult Vasculopathic    Usually isolated Check BP, serum glucose, ESR, RPR, cholesterol Spontaneous recovery    Mass lesion Contralateral hemiparesis or ipsilateral ataxia MR imaging Neurosurgical consult Cavernous sinus As in third nerve palsy Cavernous sinus syndrome Herpes zoster ophthalmicus Rash, trigeminal sensory loss in the V1 or V2 distribution Antiviral medications Orbit Inflammation, trauma, tumor Oculomotor, abducens, optic nerve dysfunction, proptosis MR imaging of the orbit or orbital ultrasound * V

66. Anatomic Localization Cause Associated Symptoms Diagnostic Evaluation Therapy Nuclear Infarction Internuclear ophthalmoplegia, Horner's syndrome, afferent pupillary defect MR imaging, stroke workup* Stroke resuscitation, antiplatelet therapy, coumadin Truma, tumor, infection, inflammation As above MR imaging, LP Depends on cause Fascicular Infarction Internuclear ophthalmoplegia, Horner's syndrome, afferent pupillary defect MR imaging, stroke workup* Stroke resuscitation, antiplatelet therapy, coumadin Trauma, trmor, infection, inflammation As above MR imaging, LP Depends on cause Demyelination Isolated or with midbrain signs above MR imaging Interferor-β1, Copaxone Subarachnoid space Trauma, hydrocephalus May be bilateral Neuroimaging Neurosurgical consult Vasculopathic    Usually isolated Check BP, serum glucose, ESR, RPR, cholesterol Spontaneous recovery    Mass lesion Contralateral hemiparesis or ipsilateral ataxia MR imaging Neurosurgical consult Cavernous sinus As in third nerve palsy Cavernous sinus syndrome Herpes zoster ophthalmicus Rash, trigeminal sensory loss in the V1 or V2 distribution Antiviral medications Orbit Inflammation, trauma, tumor Oculomotor, abducens, optic nerve dysfunction, proptosis MR imaging of the orbit or orbital ultrasound * V

67. ABDUCENS NERVE PALSY:

68. VI CN PALSY: CAUSES Idiopathic Trauma Ischemic Aneurysm inflammatory gaint cell arteritis Neoplastic demyelinating disorders intracranial pressure

69. VI CN PALSY: CLINICAL FEATURES Esotropia Face turn Diplopia Vision loss Pain Hearing loss

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

71. 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.

72. 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

73. 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

74. Table 3. Acquired lesions of the abducens nerve Anatomic Localization Cause Associated Symptoms Diagnostic Evaluation Therapy Nuclear Infarction Ipsilateral facial paralysis, INO MR imaging, stroke workup* Stroke resuscitation, antiplatelet therapy, coumadin Infiltration, trauma, inflammation MR imaging Varies with cause Wernicke-Korsakoff syndrome Ataxia, nystagmus, altered mentation Thiamine Fascicular Infarction, tumor, inflammation, MS Ipsilateral facial nerve paralysis and contralateral hemiplegia (Miller-Gubler syndrome) MR imaging, stroke workup* Stroke resuscitation, antiplatelet therapy, coumadin, surgical treatment, interferon-β1 or Copaxone Anterior inferior cerebellar artery infarction Ipsilateral facial paralysis, loss of taste, ipsilateral Horner's syndrome, ipsilateral trigeminal dysfunction, and ipsilateral deafness (Foville's syndrome)

75. Subarachnoid space Mass Contralateral hemiparesis MR imaging Ischemia Usually isolated MR imaging if no recovery after 3 months Trauma Papilledema, headache Diamox, ventriculoperitoneal shunt Intracranial hypertension Headache CSF study †    Blood patch Intracranial hypotension Petrous apex Mastoiditis, skull fracture, lateral sinus thrombosis, neoplasms, tumor Ipsilateral facial paralysis, severe facial pain CT or MR imaging Antibiotics, neurosurgical intervention, anticoagulation Cavernous sinus As with third nerve palsy Cavernous sinus syndrome * † I

76. Neuromyotonia Neuromyotonia , 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.

77. Neuromyotonia Diplopia 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

78. Myopathic , Restrictive, and Orbital Causes of Diplopia Thyroid Eye Disease The 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.

79. 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.

80. Post- Cataract Extraction Restriction Binocular diplopia can result from injury to or inflammation within the inferior rectus or other 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.

81. Orbital Myositis Idiopathic 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 movements Orbital myositis related pain usually responds within 24 hours to systemic corticosteroid therapy, whereas diplopia may take longer to resolve.

82. Neoplastic Involvement Infiltration 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.

83. 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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