Quiz 25 on Ophthalmoplegia

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Published on August 18, 2009

Author: draswinikumars

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Quiz 25Ophthalmoplegia : Quiz 25Ophthalmoplegia Dr. S. Aswini Kumar. MD Professor of Medicine Medical College Hospital Thiruvananthapuram 1 01? : 01? In this 35 year-old man, there is third nerve paralysis of lower motor neuron type, on the right side. There is bilateral ptosis. There is also weakness of the contra-lateral superior rectus. Examination also showed evidences of brainstem lesion 2 Superior Rectus weakness Bilateral Complete Ptosis Right Third Nerve Palsy 01. Nuclear Third Nerve Palsy  : 01. Nuclear Third Nerve Palsy  III Nerve palsy plus Bilateral weakness of the Levator Palpebrae Superioris muscle Nucleus of the third nerve in the rostral portion of the midbrain in front of aqueduct is affected 3 01. Nuclear Third Nerve Palsy  : 01. Nuclear Third Nerve Palsy  There is bilateral ptosis in addition to a Lower Motor Neuron type third nerve palsy because the levator palpebrae superioris muscle is innervated by a single central subnucleus There is weakness of the contralateral superior rectus, because it is supplied by the oculomotor nucleus; occasionally both superior recti are weak Isolated nuclear oculomotor palsy is rare without involvement of either of these additional findings. Similarly involvement of other nuclei and tracts of the brainstem location may also be evident Lower motor neuron type of third nerve palsy usually results from damage in the midbrain portion of the brainstem due to either infarction, hemorrhage, tumor, or infection 4 02? : 02? In this 25 year-old male patient there is lower motor neuron type of oculomotor palsy on the right side and contra-lateral cerebellar ataxia. He has in addition to these, contra-lateral, chorea, and athetosis and tremor involving the left side of the body 5 cerebellar ataxia chorea, and athetosis tremor 02. Fascicular Third Nerve Lesion : 02. Fascicular Third Nerve Lesion The fascicles or the tracts of the third nerve are involved within the brain stem These lesions also affect the superior cerebellar peduncles and the red nucleus 6 02. Fascicular Third Nerve Lesion  : 02. Fascicular Third Nerve Lesion  Injury to structures surrounding fascicles of the oculomotor nerve descending through the midbrain has given rise to a number of classic eponymic designations. In Nothnagel's syndrome, injury to the superior cerebellar peduncle causes ipsilateral oculomotor palsy and contralateral cerebellar ataxia. In Benedikt's syndrome, there is injury to the red nucleus which results in ipsilateral oculomotor palsy and contralateral tremor, chorea, and athetosis. Claude's syndrome incorporates features of both the aforementioned syndromes, by injury to both the red nucleus and the superior cerebellar peduncle 7 03? : 03? In this 65 year-old woman there was acute onset of stroke with a dense left sided hemiplegia including an upper motor neuron type of facial paralysis. Clinical examination also revealed a contra lateral LMN type of third nerve paralysis on the right side 8 Rt LMN 3rd paralysis Lt UMN facial paralysis Lt UMN Hemiplegia 03. Weber Syndrome  : 03. Weber Syndrome  The Third nerve fascicles in the ventral portion of the midbrain is involved Along with it the cerebral peduncle or the corticospinal tracts are involved 9 03. Weber Syndrome  : 03. Weber Syndrome  In Weber syndrome, injury to the cerebral peduncle causes ipsilateral oculomotor palsy with contralateral hemiplegia. In other words it is a classical form of crossed hemiplegia These lesions may spare the pupils, because the fibers carrying this function of the third nerve are separate from the rest of the fibers supplying the extra ocular muscles This lesion may involve only one division of the nerve or even result in isolated lesion of the individual extra-ocular muscle supplied by the oculomotor nerve This lesion being in the midbrain also involves the supra-nuclear fibers to the lower half of the face on the opposite side; therefore there is a contralateral UMN paralysis of the VII nerve 10 04? : 04? In this patient there was a peculiar type of eye movement disorder. When the patient was looking to the right side, the movements were full in all directions. But when looking to the left side, the right eye failed to adduct and the left eye showed nystagmus. 11 Abnormality in gaze Normal Gaze Pathway Normal Conjugate Gaze 04. Inter-nuclear Ophthalmoplegia  : 04. Inter-nuclear Ophthalmoplegia  The conjugate gaze pathway in the brain stem, MLF is involved in a disconjugate manner The connection of the parapontine reticular formation to the III nerve nucleus on opposite side 12 04. Inter-nuclear Ophthalmoplegia  : 04. Inter-nuclear Ophthalmoplegia  It is a disorder of conjugate lateral gaze due to the involvement of the pontine connections of the cranial nerves, which can affect the right or left eye independently or both eyes simultaneously The affected eye shows impairment of adduction. The opposite eye diverges from the affected eye during abduction, producing diplopia; also during abduction, there is compensatory nystagmus The disorder is caused by injury or dysfunction in the medial longitudinal fasciculus, a tract connecting the para-pontine reticular formation of one side to oculomotor nucleus of the opposite side Causes are manifold; brainstem demyelinating diseases such as multiple sclerosis are frequently found to be the causal reason, especially if found to be bilateral 13 05? : 05? In this patient the only horizontal movements of the eye possible were right lateral movement. This also meant that the horizontal movements of the left eye were absent in either directions; but the elevation and depression of the eyes was possible to some extent 14 05. One and a Half Syndrome : 05. One and a Half Syndrome Paralysis of adduction in one eye plus conjugate gaze palsy to the same side This suggests a pontine lesion involving the PPRF on one side and the MLF on the same side 15 05. One and a Half Syndrome  : 05. One and a Half Syndrome  The one and a half syndrome is a rare cause of ophthalmoparesis characterized by "a conjugate horizontal gaze palsy in one direction and an inter-nuclear ophthalmoplegia in the other" The most common manifestation of this unusual syndrome is limitation of horizontal eye movement to adduction of one eye with no horizontal movement of the other eye Convergence is classically spared in “One and a half syndrome “ as Cranial Nerve III (oculomotor nerve) and its nucleus the Edinger Westphal nucleus is spared bilaterally The syndrome usually results from a single unilateral lesion of the para-median pontine reticular formation and also involving the ipsilateral medial longitudinal fasciculus 16 Brain Stem Lesions of Third Nerve : Brain Stem Lesions of Third Nerve 17 06? : 06? In this patient there is paralysis of up gaze; but the downward gaze is normally preserved. An accommodative paresis also present. The pupils are mid-dilated and shows light-near dissociation. There is also a Convergence-Retraction Nystagmus and eyelid retraction 18 paralysis of up gaze light-near dissociation accommodative paresis 06. Parinaud’s Syndrome  : 06. Parinaud’s Syndrome  Paresis of upward gaze in a patient with neoplasm of the third ventricle of brain Named after Henri Parinaud 1844-05 it is also known as the Dorsal midbrain syndrome 19 06. Parinaud’s Syndrome  : 06. Parinaud’s Syndrome  Paralysis of upgaze: Downward gaze is usually preserved. This vertical palsy is supra-nuclear, so doll's head maneuver should elevate the eyes, but eventually all upward gaze mechanisms fail Pseudo-Argyll Robertson pupils: Pupils become mid-dilated and show light-near dissociation. Accommodative paresis gradually ensues in most cases later Convergence-Retraction nystagmus: Attempts at upward gaze often produce this phenomenon. On fast up-gaze, the eyes pull in and the globes retract Eyelid retraction (Collier's sign), as well as Conjugate down gaze in the primary position other wise called the “Setting-sun sign” are sometimes present 20 07? : 07? In this 25 year old man there was a sudden onset of ptosis on the left side, a dilated pupil with sluggish reaction to light, both direct and consensual reflex being weak. It left the eye "down and out" because of the unopposed action of lateral rectus and superior oblique 21 sudden onset of ptosis a dilated pupil with sluggish reaction left the eye "down and out" 07. Total Third Nerve palsy : 07. Total Third Nerve palsy There is ptosis on the left side with a dilated pupil and the adduction, elevation found to be impaired This man has evidence of left sided partial ptosis, dilated pupils but eye is not shifted down & out 22 07. Total Third Nerve palsy : 07. Total Third Nerve palsy Total third nerve paralysis occurs out side the brainstem and therefore is not associated with any features of the nuclear type of lesion, red nucleus, cerebral or cerebellar peduncles affection The other site where the third nerve may be involved in a partial manner is within the orbit, where the nerve has already divided into the superior and inferior divisions The trunk of the nerve contains the pupillary fibers in the outer aspect and the fibers carrying the impulses for the extra ocular muscles inside In a lesion of the trunk of the nerve both these may be involved independently or together producing an internal ophthalmoplegia or external ophthalmoplegia or both 23 08? : 08? The pupil in this patient on right side started to constrict and became pin point then gradually dilated to the full extend over 24 hours. After wards the left pupil also showed constriction followed by dilation. The extra-ocular movements were not affected 24 R pupil constrict R pupil dilate L pupil constrict L pupil dilate 08. Internal Ophthalmoplegia : 08. Internal Ophthalmoplegia Supratentorial space occupying lesion pushing temporal lobe along tentorium cerebri Compressing the trunk of the outer placed pupillary fibers of the occulo- motor nerve 25 08. Internal Ophthalmoplegia : 08. Internal Ophthalmoplegia Internal ophthalmoplegia means a paralysis of the pupillary fibers of the oculomotor nerve with the sparing of paralysis of the extra ocular movements, at least early during the course It is sometimes an important sign of the presence of a supra-tentorial space occupying lesion compressing the outer pupillary fibers of the third nerve on the same side Initially there is irritation of the fibers supplying the pupil resulting in pupillary constriction which is followed by paralysis of the same fibers resulting in dilation of the pupils In a severe case the increased intra cranial pressure may pass on to the opposite side with pupillary paralysis of a similar nature occurring in due course on the opposite side – “Hutchinson’s pupil” 26 09? : 09? In this diabetic patient, one fine morning there appeared complete weakness of all muscles supplied by the third nerve on the right side. The pupil remained normal with normal reactions to light and accommodation reflex. The other eye was also not affected 27 weakness of all EOM pupil remained normal other eye not affected 09. External Ophthalmoplegia : 09. External Ophthalmoplegia It means that the extra-ocular muscles of III are paralysed and the eyes are displaced down and out It also means that the pupillary fibers are not involved, hence the size and reactions are normal 28 09. External Ophthalmoplegia : 09. External Ophthalmoplegia This is a classical type of lesion occurring in patients with diabetes mellitus producing a completely reversible unilateral third nerve palsy with paralysis of adduction, elevation and depression of eye This is due to the involvement of the vasa-nervosum affecting the inner most part of the nerve, which carries the nerve fibers for the extra ocular muscles The lesion spares the pupils completely, at least during the early phase because these fibers are situated on the outer aspect of the nerve and are involved late Similar paralysis of the third nerve can also occur in other diseases like vasculitis, again involving the vasa nervosum. How ever these may not recover readily as in the case of diabetes mellitus 29 10? : 10? This 55 year-old man presented with acute onset of severe unilateral throbbing type of headache on the left side followed by a complete third nerve paralysis on the left side. There was mild neck stiffness but no other accompanying neurological signs 30 headache complete third neck stiffness 10. Posterior com. artery aneurysm : 10. Posterior com. artery aneurysm Acute onset complete third nerve palsy with or with out accompanying headache Cerebral angiogram showing aneurysmal swelling of the posterior communicating artery 31 10. Posterior com. artery aneurysm : 10. Posterior com. artery aneurysm Posterior Communicating artery aneurysms occur at the origin of the artery from the internal carotid artery and compress the adjacent oculomotor nerve especially upon rupture and SAH The patient presents with sudden severe headache, vomiting and loss of consciousness. On clinical examination there is neck stiffness and positive Kernig’s sign The associated oculomotor palsy is isolated, may take three days to maximize and becomes complete with pupil dilation as the rule. CT Angiography is indicated in all isolated OM palsies MR angiography is an option when the index of suspicion is lower. For example a diabetic patient with oculomotor nerve palsy with slight dilation of the pupils 32 11? : 11? There is ptosis, chemosis, and cranial nerve palsies(III, IV, V, VI)in this patient along with sensory deficits of ophthalmic and maxillary branches of 5th nerve. Also, fever, tachycardia, sepsis, decreased vision, periorbital sensory loss & impaired corneal reflex 33 11. Cavernous Sinus Lesion : 11. Cavernous Sinus Lesion In the cavernous sinus, oculomotor nerve sits against the lateral wall along with the fourth nerve Also along with the upper 2 branches of trigeminal nerve. Abducens nerve is more medial 34 10. Cavernous Sinus Lesion  : 10. Cavernous Sinus Lesion  Cavernous sinus thrombosis can be a life-threatening, rapidly progressive infectious disease with high morbidity and mortality rates despite antibiotic use. Complications of untreated CST include extension of thrombus to other dural venous sinuses, carotid thrombosis with concomitant strokes, subdural empyema, brain abscess, or meningitis. Septic embolization from the thrombosed cavernous sinus may also occur to the lungs, resulting in Adult Respiratory Distress Syndrome, pulmonary abscess or empyema. Complications in un-treated patients include opthalmo-paresis, blindness and pituitary insufficiency. Prompt and appropriate therapy to be initiated in infections of face and para-nasal sinuses 35 12? : 12? This 45 year-old woman presented with a sudden and unusually severe headache, nausea, vomiting, vision impairment and loss of consciousness, Left eye showed paralysis of third nerve, proptosis and paralysis of the pupils 36 headache vomiting Visual loss III paralysis 12. Int. Carotid artery aneurysm : 12. Int. Carotid artery aneurysm The third nerve is closely related to the internal carotid artery in the cavernous sinus Hence a subject of compression by the Internal Carotid Artery aneurysms 37 12. Int. Carotid artery aneurysm  : 12. Int. Carotid artery aneurysm  Approximately 85% of cerebral aneurysms develop in the anterior part of the Circle of Willis, and involve the internal carotid arteries and their major branches that supply the sections of the brain The most common sites include the ACA(30-35%), the bifurcation of the internal carotid and PCA (30-35%), the bifurcation of the MCA(20%), and the remaining posterior circulation arteries (5%). Before a larger aneurysm ruptures, the individual may experience such symptoms as a sudden and unusually severe headache, nausea, vision impairment, vomiting, and loss of consciousness, Or the individual may be totally asymptomatic, experiencing no symptoms at all. One the rupture of the aneurysm occurs the clinical picture is dramatic producing signs of meningeal irritation 38 13? : 13? This 35 year-old woman presented with complaints of severe pain behind the right eye, a complete right third nerve palsy, and lateral rectus paralysis along with pain and numbness in the area of distribution of the first and second division of the fifth nerve 39 pain behind eye third nerve Sixth nerve Numb V1 V2 13. Superior orbital fissure lesion : 13. Superior orbital fissure lesion These include Tolosa Hunt Granulomatous inflammation, Pitiutary, nasopharyngeal Metastatic neoplasms and cavernous aneurysms. Herpes zoster may also produce it 40 13. Superior orbital fissure lesion : 13. Superior orbital fissure lesion Number of important anatomical structures pass through the fissure, and these can be damaged in orbital trauma. These structures are: superior and inferior divisions of oculomotor nerve (III) trochlear nerve (IV) lacrimal, frontal and nasociliary branches of ophthalmic nerve (V1) abducens nerve (VI) superior ophthalmic vein sympathetic fibers from cavernous plexus The abducens nerve is most likely to show signs of damage first, with the most common complaints being retro-orbital pain and involvement of cranial nerves III, IV, V1, and VI without other neurological signs or symptoms. 41 14? : 14? The illness began abruptly, in this 55 year-old man with eye movement disorder including ophthalmoplegia,  especially of the lateral rectus muscles, gaze palsies and nystagmus, gait ataxia, confusion, confabulation, and short-term memory loss 42 ophthalmoplegia gaze palsies ataxia, confusion memory loss 14. Wernickes Encephalopathy : 14. Wernickes Encephalopathy The classic triad for this disease is encephalopathy, ophthalmoplegia, and ataxia Damage occurs to the medial thalamic nucleii and the mamillary bodies 43 Long term memory Decay Gaze Palsies Encephalopathy Ophthalmoplegia Ataxia Korsaakoff’s Confusion 14. Wernickes Encephalopathy : 14. Wernickes Encephalopathy Wernicke encephalopathy is characterized by ophthalmoplegia, ataxia, confusion and short-term memory loss. Sometimes patients have features of psychosis (Korsakoff’s) also It is usually linked to damage to the medial thalamic nucleii, mammillary bodies, peri-aqueductal, and peri-ventricular brainstem nuclei and the superior cerebellar vermis. It is the result of inadequate intake or absorption of thiamine (Vitamin B1) coupled with continued carbohydrate ingestion. The most common cause of an onset is severe alcoholism, or malnutrition. Other causes of thiamine deficiency are carcinoma of the stomach, chronic gastritis, or continuous vomiting, all of indirectly produce a short term deficiency of thiamine 44 15? : 15? The subacute illness in this 25 year-old man manifested as a descending paralysis, proceeding in the reverse order of the more common form of GBS. It affected the ocular muscles first and presents as ophthalmoplegia, ataxia, and a-reflexia 45 ophthalmoplegia ataxia a-reflexia 15. Miller Fisher Syndrome : 15. Miller Fisher Syndrome This Syndrome is characterized by the triad of ophthalmoplegia, ataxia and areflexia. Anti-GQ1b IgG antibodies specifically bind to GQ1b antigen that is localized in the myelin 46 15. Miller Fisher Syndrome  : 15. Miller Fisher Syndrome  Miller Fisher Syndrome (MFS) is a rare variant of Gullain Barre Syndrome and manifests as a descending paralysis, proceeding in the reverse order of the more common form of GBS. It usually affects the ocular muscles first and presents as ophthalmoplegia, affecting the third, fourth and sixth cranial nerves, In addition there is severe ataxia, and a-reflexia. Infection precedes the onset of neuropathy in most cases. The syndrome follows an acute and self-limited course. IgG antibodies against ganglioside GQ1b are present in the acute phase sera of more than 90% of patients with this syndrome. Anti-GQ1b IgG antibody is also present in the acute phase sera from patients with Guillain-Barré syndrome with ophthalmoplegia. 47 16? : 16? The diplopia in this 45 year-old man is often intermittent, variable, and not confined to any single ocular motor nerve distribution. The pupils are always normal. Fluctuating bilateral ptosis is present. This patient has a purely ocular form of this systemic disease 48 Bilateral Asymmetric Ptosis Attempted down gaze Attempted distant gaze 16. Myasthenia Gravis : 16. Myasthenia Gravis Bilateral ptosis Edrophonium injection 49 Intravenous administration of edrophonium chloride (Tensilon, Reversol) or neostigmine (Prostigmin), drugs that block the breakdown of acetylcholine by cholinesterase and temporarily increases the levels of acetylcholine at the neuromuscular junction 16. Myasthenia Gravis : 16. Myasthenia Gravis It is a neuromuscular disease leading to fluctuating muscle weakness and fatiguability. Botulism from food or wound poisoning can mimic ocular myasthenia It is an autoimmune disorder, in which weakness is caused by circulating antibodies that block acetylcholine receptors at the post-synaptic neuromuscular junction, inhibiting the stimulative effect of the neurotransmitter acetylcholine. The diagnosis can be confirmed by an intravenous edrophonium injection or by an assay for antiacetylcholine receptor antibodies. Negative results from these tests do not exclude the diagnosis. It is treated medically with Plasmapherasis, IVIG in the acute phase; cholinesterase inhibitors or immunosuppressants, in the chronic phase and, in selected cases, thymectomy 50 17? : 17? 12 year-old boy presented with gradual progressive ptosis. His parents noted it as painless, progressive and constant over the last 3 years. The patient denied symptoms of diplopia, weakness, or speech or swallowing troubles. He was healthy as a young child 51 17. Chronic Progressive External Ophthalmoplegia : 17. Chronic Progressive External Ophthalmoplegia CPEO is a primary disease state caused by mitochondrial abnormalities Kearns-Sayre syndrome (KSS), severe form of CPEO with pigmentary retinopathy, CHB 52 17. Chronic PE Ophthalmoplegia : 17. Chronic PE Ophthalmoplegia Ptosis is typically bilateral, but may be unilateral for a period of months to years before the fellow lid becomes involved. As ptosis becomes complete, use offrontalis muscle help elevate the lids. Ophthalmoplegia or the inability/difficulty to move the eye is usually symmetrical. As such, diplopia is not often a complaint of these patients. In fact, progressive ophthalmoplegia is often unnoticed All directions of gaze are affected, however, downward gaze appears to be easily spared. This is in contrast to Progressive Supranuclear Palsy (PSP) which affects all directions of gaze Weakness of extraocular muscle groups including, the orbicularis oculi muscle as well as facial and limb muscles may be present in up to 25% of patients with CPEO. 53 18? : 18? This 15 year-old boy complained of binocular vertical diplopia, which worsens on down gaze. He felt maximum difficulty while climbing down steps. Examination of the case revealed hyperdeviation on the side of lesion that is worsened on down gaze and adduction 54 Vertical Diplopia Exacerbated by tilting the head to wards the side of Muscle Palsy and Alleviated by tilting it away: “Head Tilt Test” 18. Unilateral 4th Nerve Palsy  : 18. Unilateral 4th Nerve Palsy  Vertical diplopia seen is exacerbated by tilting the head toward the side with muscle palsy, And it is alleviated by tilting it away. This "head tilt test" is a cardinal diagnostic feature. 55 18. Unilateral 4th Nerve Palsy  : 18. Unilateral 4th Nerve Palsy  The fourth cranial nerve originates in the midbrain, just caudal to the oculomotor nerve complex. Fibers exit the brainstem dorsally and cross to innervate the contralateral superior oblique. Isolated trochlear nerve palsy occurs from all the causes listed above for the oculomotor nerve, except aneurysm. The trochlear nerve is particularly apt to suffer injury after closed head trauma. The free edge of the tentorium is thought to impinge upon the nerve during a concussive blow. Most isolated trochlear palsies are idiopathic and hence diagnosed by exclusion as "microvascular.“ Spontaneous improvement occurs over a period of months in most patients. A base-down prism may serve as a temporary measure to alleviate diplopia. If the palsy does not resolve, the eyes can be realigned by weakening the inferior oblique muscle. 56 19? : 19? In this patient , while looking to the right, the eyes move in a conjugate manner. While looking straight ahead, double vison appears with the left eye turned inwards; and on looking to the left the diplopia is maximum as the left eye completely fails to abduct 57 Looking to right Looking straight Looking to left 19. Unilateral 6th Nerve Palsy Lt side : 19. Unilateral 6th Nerve Palsy Lt side Along its subarachnoid course the Sixth nerve is susceptible to meningitis, tumor etc. Infiltration by Carcinomatous meningitis, SAH, trauma, and compression by are also possible 58 19. Unilateral 6th Palsy – Nuclear : 19. Unilateral 6th Palsy – Nuclear The sixth cranial nerve innervates the lateral rectus muscle. A palsy produces horizontal diplopia, worse on gaze to the side of the lesion. Rest of the extra ocular movements are normal Foville's syndrome following dorsal pontine injury includes lateral gaze palsy, ipsilateral facial palsy, and contralateral hemiparesis incurred by damage to descending corticospinal fibers. Millard-Gubler syndrome from ventral pontine injury is similar, except for the eye findings. There is lateral rectus weakness only, because the abducens fascicle is injured; but no gaze palsy, Infarct, tumor, hemorrhage, vascular malformation, and multiple sclerosis are the most common etiologies of brainstem abducens palsy of unilateral variety 59 19. Unilateral 6th Palsy – Trunk : 19. Unilateral 6th Palsy – Trunk Along its subarachnoid course it is susceptible to meningitis, tumor (meningioma, chordoma, carcinomatous meningitis), SAH, trauma, and compression by aneurysm or dolichoectatic vessels. At the petrous apex, mastoiditis can produce deafness, pain, and an ipsilateral abducens palsy, the condition is termed as Gradenigo's syndrome. After leaving the ventral pons, the abducens nerve runs forward along the clivus to pierce the dura at the petrous apex, where it enters the cavernous sinus. In the cavernous sinus, the nerve can be affected by carotid aneurysm, carotid cavernous fistula, tumors (pituitary adenoma, meningioma, nasopharyngeal carcinoma) and herpes infection 60 19. Unilateral 6th Nerve Palsy - Trt : 19. Unilateral 6th Nerve Palsy - Trt Treatment of abducens palsy is aimed at prompt correction of the underlying cause. However, the cause remains obscure in many instances, despite diligent evaluation. As for isolated trochlear or oculomotor palsy, most cases represent microvascular infarcts because they often occur in the setting of diabetes or other vascular risk factors. Some cases may develop as a post-infectious mononeuritis (e.g., following a viral flu). Patching one eye or applying a temporary prism will provide relief of diplopia until the palsy resolves. If recovery is incomplete, eye muscle surgery can nearly always realign the eyes, Patient with an abducens palsy that fails to improve should be reevaluated for an occult etiology (e.g., chordoma, carcinomatous meningitis, carotid cavernous fistula, myasthenia gravis). 61 20? : 20? In this 34 year-old man there was a sub-acute onset of headache associated with projectile vomiting and blurring of vision. There was bilateral lateral rectus weakness resulting in a medial squint and diplopia upon looking to the either lateral sides 62 bilateral lateral rectus weakness projectile vomiting blurring of vision 20. Bilateral 6th Nerve Palsy : 20. Bilateral 6th Nerve Palsy Unilateral or and more often bilateral abducens palsy is a classic sign of raised intracranial pressure . The diagnosis can be confirmed if papilledema is observed on fundus examination 63 20. Bilateral 6th Nerve Palsy  : 20. Bilateral 6th Nerve Palsy  Elevated intracranial pressure can result in downward displacement of the brainstem, causing stretching of the sixth nerve secondary to its anatomic location within the Dorello canal. This is believed to be the reason that about 30% of patients with pseudotumor cerebri have an isolated abducens nerve palsy withoutany associated neurological deficits. The same phenomenon accounts for abducens palsy from low intracranial pressure (e.g., after lumbar puncture, spinal anesthesia, or spontaneous dural cerebrospinal fluid leak). Subarachnoid space lesions can cause BL abducens palsy (eg, hemorrhage, tumor, cavernous sinus thrombosis. Inflammatory (eg, postviral, demyelinating, sarcoid, giant cell arteritis) 64 21? : 21? There is proptosis in this patient which is bilateral but asymmetric. There is lid retraction, conjunctival injection, restriction of gaze in all directions, mild diplopia, corneal exposure and visual loss from optic nerve compression. Patient is nervous with a fine distal tremor 65 proptosis conjunctival injection restriction of gaze in all directions 21. Grave’s Ophthalmopathy : 21. Grave’s Ophthalmopathy Bilateral proptosis is obvious with lid retraction and lid lag upon following down the gaze CT scan axial view of the patient showing hypertrophy of extra ocular muscles 66 21. Thyroid Ophthalmopathy : 21. Thyroid Ophthalmopathy Orbital inflammation and engorgement of the extraocular muscles, particularly the medial rectus and the inferior rectus, account for the protrusion of the globe. Graves' ophthalmopathy is treated with oral prednisone (60 mg/d) for 1 month.This is to be followed by a tapering dose over several months, Topical lubricants, eyelid surgery, eye muscle surgery, and in some patients orbital decompression. Radiation therapy is not effective. 67 22? : 22? Symptoms in this patient are pain, limitation of eye movements, proptosis, and congestion of conjunctiva. Evaluation for Hyperthyroidism,sarcoidosis, Wegener's granulomatosis, and other types of orbital vasculitis or collagen-vascular disease is negative 68 22. Orbital Pseudotumor : 22. Orbital Pseudotumor The lesion is orbital myositis presenting with swelling and restricted movements of eyes Axial contrast CT showing the hypertrophy of extra ocular muscles within the orbit 69 22. Orbital Pseudotumor : 22. Orbital Pseudotumor This is an idiopathic, inflammatory orbital syndrome. The Tolosa-Hunt syndrome may be regarded as an extension of orbital pseudotumor through superior orbital fissure into cavernous sinus. Imaging often shows swollen eye muscles due to orbital myositis with enlarged tendons. By contrast, in Graves' ophthalmopathy the tendons are usually found to be spared. The diagnosis of orbital pseudotumor is difficult. Biopsy of the orbit frequently yields nonspecific evidence of infiltration of the fatty tissues by lymphocytes, plasma cells, & eosinophils. It is frequently confused with graves Opthalmopathy. A dramatic response to a therapeutic trial of systemic glucocorticoids indirectly provides the best confirmation of diagnosis 70 23? : 23? In this patient there was pain, lid erythema, proptosis, conjunctival chemosis, restricted motility, decreased acuity, afferent pupillary defect, fever, and leukocytosis. A history of recent upper respiratory tract infection, acute sinusitis, was present 71 23. Orbital Cellulitis : 23. Orbital Cellulitis Bilateral painful eyes and proptosis along with conjunctival congestion & purulent discharge CT scan of Orbit of this patient shows swelling of the structures of the orbit 72 23. Orbital Cellulitis : 23. Orbital Cellulitis Blood cultures should be obtained, but they are usually negative. Most patients respond to empirical therapy with broad-spectrum intravenous antibiotics. Occasionally, orbital cellulitis follows an overwhelming course, with massive proptosis, blindness, septic cavernous sinus thrombosis, and meningitis, septicemia and fatal outcome To avert this disaster, orbital cellulitis should be managed aggressively in the early stages, with immediate antibiotic therapy and imaging of the orbits. Prompt surgical drainage of an orbital abscess or paranasal sinusitis is indicated if optic nerve function deteriorates despite antibiotics 73 24? : 24? This patient has a slowly progressive swelling of one eye which was totally painless in the early stages. But the swelling increased gradually and the whole eye was bulging out. There was also a painless restriction of all extra ocular movements. 74 24. Orbital Tumor : 24. Orbital Tumor Swelling of right eye which is progressive and painless which chemosis and congestion In this case the tumor was removed by surgical resection hoping for a permanent cure 75 24. Tumor of the Orbit : 24. Tumor of the Orbit Tumors of the orbit cause a sub acute onset of painless, progressive unilateral proptosis. The most common primary benign tumors are hemangioma, lymphangioma, neurofibroma, and dermoid cyst Most common primary malignant tumors are adenoid cystic carcinoma, optic nerve glioma, optic nerve meningioma, and benign mixed tumor of the lacrimal gland. Metastatic tumor to the orbit occurs frequently in breast carcinoma, lung carcinoma, and lymphoma. Diagnosis by fine-needle aspiration followed by urgent radiation therapy can sometimes preserve vision. 76 25? : 25? The combination of slight proptosis, diplopia, enlarged muscles, and an severely injected eye in this 75 year-old woman was mistaken for a thyroid ophthalmopathy. Surprisingly a bruit was heard upon auscultation of the head, also reported by the patient 77 25. Carotid Cavernous Fistula : 25. Carotid Cavernous Fistula Cork screw vessels seen in the conjunctiva are the characteristic lesions in this rare condition Internal carotid angiogram showing the presence of carotid cavernous fistula 78 25. Carotid Cavernous Fistula : 25. Carotid Cavernous Fistula Direct fistulas usually result from trauma. They are easily diagnosed because of the prominent signs produced by high-flow, high-pressure shunting. Indirect fistulas, or dural arterio-venous malformations, are more likely to occur spontaneously. The signs are more subtle and the diagnosis is frequently missed. They are common, especially in older women. Cork screw vessels seen in the conjunctiva are the characteristic lesions . Imaging with angiogram shows an enlarged superior ophthalmic vein in orbits The combination of slight proptosis, diplopia, enlarged muscles, and an injected eye is often mistaken for thyroid ophthalmopathy. These shunts can be eliminated by intravascular Embolization. 79 Slide 80: Thank You for the Patient Listening 80

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