What is skew deviation?;"• Supranuclear disorder due to disruption of vestibular input to brain stem responsible for vertical eye movements (cranial nerve 3 and cranial nerve 4) • Arises from cerebellar or brainstem pathology • Often manifests as weakness of bilateral inferior rectus muscles → alternating hypertropia (right hypertropia with right gaze; left hypertropia with left gaze) → localizes to cervicomedullary junction • Associated with downbeat nystagmus • ""Upright-supine test"" = hypertropia measured in upright position then again in supine position. In skew deviation, profound reduction in hypertropia when lying supine. No reduction in patients with cranial nerve 4 palsy" What is the difference between congenital nystagmus and latent nystagmus?;"• Congenital nystagmus: slow phase velocity accelerates over time • Latent nystagmus: slow phase velocity either constant velocity or decelerating velocity" What was the optic neuritis treatment trial and its findings?;"• Patients with first episode of optic neuritis, evaluated within 8 days of symptoms onset • 65% had retrobulbar optic neuritis (no papillitis) • 35% had anterior optic neuritis (papillitis) • Randomized to (1) Intravenous steroids (Solu-Medrol 250mg q6h x 3 days) followed by oral prednisone 1mg/kg/day x 11 days then quick taper (2) Oral prednisone 1mg/kg/day x 14 days (3) Placebo • All groups has excellent visual recovery without difference in visual outcome at 6 months • Steroid treatment (IV and PO) reduced risk of MS in first 2 years after treatment • PO prednisone group → higher rate of recurrence than both the other groups • IV steroid group → least likely to progress to MS at 2 years • Overall 50% of patients with optic neuritis developed clinically definite MS over next 15 years • Overall optic neuritis is presenting symptom in 25% of MS patients • Major predictor for risk of progression to MS = presence of one or more white matter lesions on MRI of brain • 25% risk of MS in the next next 15 years if no lesions on MRI • 72% risk of MS in the next 15 years if 1 or more lesions on MRI" Which diseases are associated with vascular compression induced neuropathy by aberrant, ectatic, or dilated intracranial artery compressing on cranial nerve?;"• Superior oblique myokymia • Trigeminal neuralgia • Hemifacial spasm" What is superior oblique myokymia?;"• Most common cause: idiopathic • Other causes: neoplasm, vascular, acquired • Irritation of cranial nerve 4 • Spontaneous firing of superior oblique on one side causing ""jumping"" of vision in one eye (high frequency, low amplitude oscillation) and binocular diplopia • MRI can show vascular compression of 4th cranial nerve on affected side" What is trigeminal neuralgia?;"• Unilateral facial pain described as electric shock-like • Transient, duration lasting seconds to minutes • Main cause is vascular compression (superior cerebellar artery) of trigeminal nerve • Treatment: spacer (sponge) between trigeminal nerve and superior cerebellar artery" What is hemifacial spasm?;"• Usually unilateral • Abnormal spasms of facial muscles supplied by cranial nerve 7 • Causes: stroke, demyelination, tumors, AVMs, idiopathic • Common etiology: vascular compression of facial nerve root by distal branches of anterior inferior cerebellar artery or vertebral artery • Work up: MRI brain, rule out CPA tumor • Treatment: botox, microvascular decompression surgery" What is benign essential blepharospasm?;"• Bilateral • Episodic contraction of orbicularis oculi muscles • Related to underlying basal ganglia dysfunction • Many have functional or stress related comp• onent • Exacerbated by corneal surface disease (dry eye) • Treatment: botox" What is the differential diagnosis for alternating hypertropia?;"• Skew deviation: right hypertropia with right gaze; left hypertropia with left gaze • Bilateral cranial nerve 4 palsy: right hypertropia with left gaze; left hypertropia with right gaze • Partial cranial nerve 3 palsy (if right sided): left hypertropia with upgaze and right hypertropia with down gaze • Bilateral inferior rectus restriction (thyroid-associated ophthalmopathy): Left hypertropia on right gaze; Right hypertropia on left gaze" What is Riddoch phenomenon?;"• In cortical blindness, ability to see moving targets but unable to see any stationary targets" What is Anton syndrome?;"• Patient with cortical blindness that is in denial of their blindness" What is Pulfrich phenomenon?;"● Illusion that an object is moving perpendicular to persons line of sight that is usually moving towards or away from them ● Seen after recovering from optic neuritis ○ Due to relative conduction delay within optic nerve that has been affected by demyelinating optic neuritis compared to contralateral unaffected optic nerve" What is Charles Bonnet syndrome?;"• In a patient with severe bilateral visual loss of any type (i.e. macular degeneration, glaucoma, cortical blindness) • Leads to formed and unformed visual hallucinations • Patient is aware hallucinations are not real" What are the most common visual abnormalities associated with optic disc drusen?;"• Visual field defects (75%-90%): most commonly enlarged blind spot • Transient visual obscurations (5-10%) due to transient optic disc ischemia" What are the ocular findings in Parkinson's disease?;"• Apraxia of eyelid opening • Square wave jerks • Convergence insufficiency • Blepharospasm • Dry eye • Decreased blink rate" What is the ocular effect of progressive supranuclear palsy?;"• Vertical saccades predominantly" What is ocular neuromyotonia?;"• Spasms of some or all muscles supplied by cranial nerves 3, 4, or 6 • Episodic binocular diplopia • Lasts seconds to minutes • Due to tonic discharges of offending cranial nerve related to some sort of prior insult (radiation, surgery, etc) • Treatment: carbamazepine, gabapentin" What is autosomal dominant optic atrophy?;"• Most common hereditary optic neuropathy (1 in 50,000) • Presents in the first 10 years of life but can present much later • Mutation of OPA1 gene on chromosome 3 → interrupts mitochondrial membrane integrity and function • Visual acuity range 20/40 to 20/100 • Temporal optic atrophy • Maculopapular bundle defects • Central and cecocentral scotoma • Blue dyschromatopsia (tritanopia)" What visual field (VF) defects are associated with mild to moderate temporal pallor?;"• Associated with many different VF defects, including isolated central visual field defects" What percentage of the general population has physiologic anisocoria?;"• Approximately 20% • Usually <1 mm difference in pupils • Anisocoria symmetric in light and dark or slightly greater in the dark" What work up should be done for optic nerve hypoplasia?;"• MRI brain • Endocrine evaluation" What are optic nerve drusen?;"• Proteinaceous nodules that become calcified • As the drusen progress towards nerve fiber layer → block axoplasmic flow → progressive visual field defects • Disrupt normal architecture of retina and nerve head → can cause choroidal neovascular membrane formation • <10% have transient visual obscurations lasting seconds due to transient ischemia of optic nerve head" What is the differential diagnosis for optociliary shunt vessels (retinochoroidal collaterals)?;"• Optic nerve sheath meningioma • Optic nerve glioma • Chronic papilledema • Chronic glaucoma" What is the definition of optociliary shunt vessels (retinochoroidal collaterals)?;"• Dilation of naturally occuring veins that drain from peripapillary retinal circulation into choroidal circulation • Shunt vessels occur in response to chronically poor drainage of central retinal vein" What are the findings in cranial nerve 6 nucleus lesion?;"• Ipsilateral abduction defect and contralateral adduction defect due to interneuron connection with contralateral MLF • Any lesion affecting 6th cranial nerve nucleus almost always causes ipsilateral facial palsy" What are the ocular findings and etiology of ethambutol toxicity?;"• Optic neuropathy in approximately 1.5% of patients who use it • Mitochondrial toxicity of retinal ganglion cells RGCs → RGCs whose axons decussate in the chiasm are more susceptible to mitochondrial toxicity • Bitemporal hemianopia and cecocentral pattern on visual field • Early decrease in color vision and visual acuity" What are the most common causes of retrochiasmal visual loss adults and children?;"• Adults: stroke (60%), trauma (10%), hemorrhage (10%) • Children: trauma and tumors" What is downbeat nystagmus?;"• Abnormal drift of eyes upward followed by corrective downward fast saccade • Most pathology localizes to cervicomedullary junction or vestibulocerebellum • Seen with Arnold-Chiari I malformation" What is morning glory disc?;"• Congenital • Unilateral • Females > males • MRI/MRA brain should obtained in all patients with morning glory disc 3 associations: • Serous RD • Transsphenoidal basal encephaloceles -mimic nasal polyps on exam • Moyamoya disease: bilateral stenosis or occlusion of arteries around circle of Willis (chronic progressive cerebrovascular disease)" What is digoxin toxicity-related chromatopsia?;"• Yellow or yellowish green hue throughout visual field • Reversible with cessation of decreased or decreased dose of drug • Can occur despite ""normal"" serum level of digoxin on lab testing • ERG: cone dysfunction" What is sildenafil-related chromatopsia?;"• Blue chromatopsia" What is the cause of convergence-retraction nystagmus?;"● Seen in dorsal midbrain syndrome (aka Parinaud's syndrome) ○ Abnormal co-contraction of medial and lateral rectus muscles on attempted upgaze ○ Not rhythmic ○ Does not have fast or slow phase ○ Does not involve abnormal drift from fixation ○ Elicited by holding down going OKN drown" What are the ocular effects of methanol and ethylene glycol toxicity?;"• Occur immediately after ingestion • Devastating acute, diffuse vision loss with associated optic disc edema" What are the most common causes of chronic toxic and nutritional neuropathy?;"Toxic: • Ethambutol • Ethanol • Chloramphenicol • Hydroxyquinolones • Penicillamine • Cisplatin • Vincristine Nutritional deficiency: • Vitamin B12 • Folate • Thiamine" What is latent nystagmus?;"• Develops early in life • Likely occurs from early disruption of binocular vision • Common in children with infantile strabismus • Asymptomatic for most patients • Can result in poor visual acuity on monocular vision testing • Bilateral jerk nystagmus that generally only manifests upon occlusion on one eye • Fast phase of nystagmus beats towards viewing eye" What is FLAIR MRI?;"• Subset of T2 • Attenuates bright signal of CSF allowing for better visualization in periventricular regions adjacent to CSF (i.e. multiple sclerosis)" What is DWI MRI?;"• Diffusion weighted imaging • Highlights recent vascular perfusion abnormalities (i.e. recent stroke)" What is T1 MRI?;"• Best for visualization of anatomy • Fat is bright • CSF, vitreous, and air are dark" What are square wave jerks and their common causes?;"• Type of saccadic intrusion • Lack fast or slow phase • Small amplitude • Bilateral small horizontal shifts from fixation during examination followed by pause and then quick return to fixation Common causes: • Cerebellar dysfunction • Parkinson's disease • Supranuclear palsy" What is polyopia?;"• Seeing 3 or more images • Unilateral monocular polyopia → functional or optical • Bilateral monocular polyopia → migraine, epilepsy, stroke • Often functional but when organic it is due to disturbance of occipital lobes, accompanied by homonymous VF defect" What is the sympathetic pathway to the eye?;"• Axons never decussate • First order neuron: from posterior lateral hypothalamus, descend through brainstem to intermediolateral cell column between C7 and T2 where they synapse with cell bodies of second order neurons • Second order sympathetic neurons project from intermediolateral cell column between C7 and T2 to paravertebral sympathetic chain where fibers ascend to superior cervical ganglion and synapse with third order neurons • Third order neuron cell bodies lie in superior cervical ganglion and pass superiorly into cranial cavity with the internal carotid artery (ICA) along wall of artery (carotid plexus). Ocular sympathetics continue with ICA as it passes in cavernous sinus. While in cavernous sinus, third order axons travel from ICA to abducens then join nasociliary branch of V1. Then travel with nasociliary V1 into orbit and through ciliary ganglion to extend to dilator muscle" What are the causes of first-order Horner syndrome?;"• Wallenberg (lateral medullary syndrome) • Thalamic hemorrhage/stroke • Tumors • Cervical disease • Demyelination • Pathology of cervical spinal cord" What are the causes of second-order Horner syndrome?;"• Pancoast tumor or apical lung malignancies • Brachial plexus injuries • Thoracic surgery with disruption of superior portion of paravertebral sympathetic chain" What are the causes of third-order Horner syndrome?;"• Internal carotid artery pathology (dissection) • Cavernous sinus compressive or inflammatory lesions • Trigeminal autonomic cephalgia type headache" What are low flow dural sinus fistulas?;"• Abnormal connection between small dural based arteries and cavernous sinus • Occur spontaneously without inciting event • Can resolve spontaneously without surgical intervention • Clinical findings: elevated IOP, proptosis, ocular motor palsy, arterialization, injection of conjunctival vessels, choroidal effusions, ischemic optic neuropathy, and pain" What are high flow carotid-cavernous fistulas?;"• Usually traumatic origin • Cranial bruit • Abnormal connection between internal carotid artery and cavernous sinus • Require urgent angiography with thrombosis • Clinical findings: elevated IOP, proptosis, ocular motor palsy, arterialization, injection of conjunctival vessels, choroidal effusions, ischemic optic neuropathy, and pain" How does one test ocular ductions?;"• Test ocular motility under monocular conditions" How does one test ocular versions?;"• Test ocular motility under binocular conditions" What are the causes of bilateral small pupils?;"• Opioid narcotics • Increasing age • Chronic tonic pupils (initially large, shrink over time) • Parasympathomimetic (i.e. use of pilocarpine) • Sleep • Pontine hemorrhages" What hormone is most commonly deficient in septo-optic dysplasia?;"• Growth hormone" What are the causes of infiltrative optic neuropathy?;"• Optic nerve gliomas • Leukemia • Lymphoma • Sarcoidosis • Syphilis • TB • Fungal • Meningeal-carcinomatosis" What is ocular dysmetria?;"• Overshoot or undershoot of eyes upon refixation to new target • Suggestive of cerebellar pathology" What is the inheritance and what mutations are involved in Leber's hereditary optic neuropathy?;"• Maternally inherited disease • Mitochondrial dysfunction • Point mutations in mitochondrial DNA • >90% cases due to 11778, 14484, and 3460 mutations" What is Wolfram syndrome?;"• Autosomal recessive or sporadic • Diabetes insipidus plus diabetes mellitus plus optic atrophy plus sensorineural deafness" What is Behr syndrome?;"• Variant of autosomal recessive optic atrophy • Associated neurologic abnormalities: ataxia, pyramidal and extrapyramidal dysfunction, hypertonia, intellectual disability, urinary incontinence, pes cavus" What is the most accurate means of quantifying afferent pupillary defect?;"• Neutral density filters" What is one-and-a-half syndrome?;"• Internuclear ophthalmoplegia with ipsilateral horizontal gaze palsy • Etiology: stroke of pontine region (includes MLF, PPRF, cranial nerve 6 nucleus)" What is the cause and presentation of ERM-induced diplopia?;"• Caused by dragging of the fovea in the affected eye • Prisms are ineffective • ERM peel often ineffective because fovea does not return to physiologic location" What is Duane retraction syndrome and what are the different types?;"• Congenital eye movement disorder • Agenesis or dysgenesis of 6th cranial nerve nucleus • Type 1: abnormal abDuction (one D) • Type 2: abnormal aDDuction (two D’s) • Type 3: both abDuction and aDDuction (three D’s)" What is the presentation of diabetic papillopathy?;"• Mild optic neuropathy • Minimal visual manifestations • Optic nerve with telangiectasia • Most common in older patients with diabetes mellitus type 2 • Visual field with enlarged blind spot • Fluorescein angiogram: leakage without rising into vitreous" What is the presentation of junctional scotoma?;"● Ipsilateral side with central vision loss and contralateral deficit in superotemporal field ● Compressive lesion localized to optic nerve junction with anterior chiasm(Willbrand's knee) ○ Willbrand’s knee = inferonasal retinal ganglion decussate in optic chiasm, before entering contralateral optic tract, they loop anteriorly for short segment in to contralateral optic nerve ○ Ipsilateral optic nerve compromise results in central scotoma" What are the causes of decreased and increased IOP in ocular ischemic syndrome (OIS)?;"Causes of decreased IOP • Hypoperfusion to ciliary body Causes of elevated IOP • Hemorrhages in anterior chamber or vitreous • Angle closure from neovascularization of angle • Reperfusion IOP spike after carotid endarterectomy" What is Weber syndrome?;"• Midbrain lesion affecting cranial nerve 3 and cerebral peduncle • Cranial nerve 3 palsy • Contralateral hemiparesis" What is Benedikt syndrome?;"• Lesion in midbrain affecting cranial nerve 3, red nucleus, and substantia nigra • Cranial nerve 3 palsy • Contralateral ""rubral"" tremor (slow tremor present with activity and rest)" What is Claude syndrome?;"• Damage to dorsal midbrain • Affecting superior cerebellar peduncle and cranial nerve 3 • Cranial nerve 3 palsy and contralateral ataxia" What are the causes of posterior ischemic optic neuropathy?;"• Hypoperfusion • Anemia • Poor cardiac output • Hypovolemia • Cardiopulmonary instability • Rare causes: giant cell arteritis, SLE, polyarteritis nodosa, VZV" What are the clinical findings associated with optic nerve pits?;"• Visual acuity is normal (unless serous macular RD) • Serous macular RD 25-75% • Associated with nerve fiber bundle visual field defects (arcuate scotoma)" What is Sherrington's law?;"• Monocular principle • Increased contraction of the innervated muscle results in equal relaxation the antagonist muscles" What is Hering's law?;"• Binocular principle • Equal and simultaneous innervation of yoke muscles • Violation of this law → dissociated vertical deviation (DVD); dissociated horizontal deviation (DHD); spread of comitance" What is the most commonly involved cranial nerve in neurosarcoidosis?;"• Most common is cranial nerve 7 • Second most commonly involved is cranial nerve 2, including disc edema secondary to meningeal infiltration or optic nerve perineuritis or optic nerve infiltration" What is the differential diagnosis for the combination of uveitis and facial nerve palsy?;"• Lyme disease • Sarcoidosis" What is the anterior visual pathway?;"• Axons from retinal ganglion cell nuclei → NFL → optic nerves → optic chiasm → optic tract → lateral geniculate nuclei where they synapse" What is cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR)?;"• CAR and MAR are paraneoplastic • Progressive vision loss with photopsias and nyctalopia • Normal fundus until late in the disease course → attenuated vessels, thinning of RPE, optic nerve atrophy • Cone and rod dysfunction • Most common malignancy in CAR: small cell lung cancer, also associated with other lung cancers, breast cancer, endometrial cancer, cervical cancer" What is the definition and causes of palinopsia?;"• Persistence or recurrence of images after removal of original stimulus Causes: • Most common cause is lesion of parieto-occipital region • Topiramate, clomiphene, trazodone, hallucinogenic drugs (LSD, mushrooms) • Migraine" What is the definition and cause of light-near dissociation?;"• Pupillary constriction upon near gaze greater than pupillary constriction to light • Pathophysiology: bilateral loss of afferent input from retina/optic nerves to pupillary light reflex Causes: • Argyll-Robertson pupils (disruption of Edinger-Westphal) • Adie's tonic pupils (aberrant regeneration of ciliary body parasympathetic input responsible for accomodation) • Parinaud's (dorsal midbrain) syndrome (disruption of Edinger-Westphal)" What is the ideal length for a temporal artery biopsy?;"• 2 to 3 cm • Rate of false negative temporal artery biopsy 3-9% • Obtain contralateral temporal artery biopsy in highly suspicious cases" How does a lesion of the MLF present?;"• Causes INO and skew deviation • Hypertropic eye on the side of lesion • Ipsilateral INO" How does ocular bobbing present?;"• Conjugate fast downward movement of both eyes followed by slow elevation of both eyes back to primary gaze • Indicates brainstem dysfunction -structural or metabolic" What is hemeralopia?;"• Deterioration of vision in higher light environments • Seen in cone dystrophy" What is the most common mutation in Leber's hereditary optic neuropathy?;"• 11778 mutation LHON" What mutation is most likely to show spontaneous late visual recovery in Leber's hereditary optic neuropathy?;"• 14484 mutation LHON" What size is the smallest aneurysm detectable by MRA/CTA?;"• 3 mm • Gold standard for cerebral aneurysm detection is catheter angiography" What features of acute optic disc edema distinguish elevated intracranial pressure from other causes?;"• Papilledema → bilateral optic disc swelling, retain good visual function (i.e. good visual acuity, central visual field, color vision) • Optic disc edema from other causes → prominent visual field and/or visual function deficits early in the disease" How many central degrees of visual field does amsler grid testing evaluate?;"• Central 20 degrees (10 degrees on either side of fixation)" What are the various types of humphrey visual field testing and the degrees they assess?;"• 10-2: central 20 degrees • 24-2: 48 degrees vertical, 54 degrees horizontal • 30-2: 60 degrees" What is the differential diagnosis for optic disc edema?;"• Most common: papilledema, optic neuritis, AION, pseudopapilledema • Somewhat common: CRVO, diabetic papillopathy • Less common: posterior uveitis, hypotony, malignant hypertension, Leber's hereditary optic neuropathy, optic nerve infiltration (i.e. sarcoidosis, lymphoma)" What visual field defects are expected to result from a pituitary mass?;"• Bilateral mostly supratemporal defects that respect vertical midline→ extend to complete bitemporal hemianopia late in disease • Exception to this → craniopharyngioma which arises from above chiasm and compresses superior aspect of chiasm → bitemporal defects greater inferiorly" What is Gradenigos syndrome?;"• Inflammation of petrous portion of temporal bone • Affects cranial nerves 5, 6, and 7 • Severe ipsilateral trigeminal pain and cranial nerve 6 and 7 palsy" What is the blood supply to lateral medulla?;"• Posterior inferior cerebellar artery" What is the cause of binocular diplopia after cataract surgery?;"• Rare • Seen with retrobulbar injection → mechanical needle trauma to inferior rectus → hemorrhage or nerve damage to inferior rectus • May resolve over time or may cause chronic fibrosis of inferior rectus" In optic neuritis, what is the appearance of the optic nerve and how does it correlate with the risk of developing MS?;"• Based on optic neuritis treatment trial • Severe optic disc edema with peripapillary retinal hemorrhage inversely related to risk of MS development • Patients with peripapillary hemorrhage and normal MRI → none developed MS at 10 year mark • All patients with optic neuritis and normal brain MRI → 20% chance of MS by 10 years • Patients with optic neuritis and massive disc edema or heme → usually have other etiologies for optic nerve dysfunction" What is the best test to distinguish between AION and NAION?;"• Fluorescein angiography → patchy choroidal perfusion with AION → commonly secondary to GCA → infarct posterior ciliary artery → presents as vision loss with normal fundus exam" What is the role of apraclonidine in Horner's syndrome?;"• Apraclonidine is a alpha-adrenergic receptor agonist, has more of an effect on alpha-2 receptors than alpha-1 receptors • Horner’s syndrome has up-regulation of post-synaptic alpha-1 receptors (usually after 2-5 days of injury) • Normal pupil has no reaction to apraclonidine • Horner’s pupil dilates to apraclonidine due to upregulation of alpha-1 receptor" What is the cause of benign episodic pupillary mydriasis?;"Migraine headache" What is Brown syndrome?;"• Inability to elevate the eye when adducted • Unilateral or bilateral • Type of restrictive strabismus • Cause of congenital Brown syndrome: due to short superior oblique tendon • Causes of acquired Brown syndrome: trauma, inflammation from idiopathic orbital inflammation, rheumatoid arthritis, Sjogren's syndrome, neoplasm" What is Tolosa Hunt syndrome?;"• Idiopathic sterile inflammation of cavernous sinus • Severe ipsilateral periorbital pain along with deficits of any of the nerves passing through the cavernous sinus (cranial nerve 3, 4, V1, V2, 6, sympathetics) • Diagnosis of exclusion→ first rule out infection, lymphoma, cancer" What is Miller Fisher syndrome?;"● Oculomotor predominant variant of Guillain-Barre syndrome (GBS) ● Affects eye movements first→ progresses to systemic motor and sensory neuropathy typical of GBS ● Tetrad of Miller-Fisher ○ Facial diplegia ○ Ophthalmoplegia ○ Ataxia ○ Areflexia" What is Heimann-Bielschowsky phenomenon?;"• Monocular vertical pendular nystagmus that occurs in poorly seeing eyes (often in children) • Can occur after vision loss from optic nerve gliomas, amblyopia, trauma, or other causes of vision loss • Any child with monocular nystagmus → neuroimage if cause of vision loss is unknown" What is the duration of vision loss in the following conditions: migraine aura, amaurosis fugax,transient visual obscurations (TVO)?;"• Migraine aura: 10-60 minutes • Amaurosis fugax: 1-10 minutes • TVO: few seconds" What are the clinical findings and cause of a lesion to the central tegmental tract?;"● Central tegmental tract runs from inferior olivary nucleus in the medulla to the red nucleus in the midbrain ● Results in oculopalatal myoclonus ○ Bilateral acquired, vertical and pendular nystagmus ○ Rhythmic movements of face, soft palate, pharynx, tongue, larynx, diaphragm, extremities ● Caused by brain stem stroke" What is Marcus Gunn jaw-winking syndrome?;"• Aberrant innervation connection (synkinesis) between muscles of (pterygoid) and levator muscle • Congenital • Autosomal dominant • Variant of congenital ptosis with fibrosis Types: 1. Aberrant connection between EXTERNAL pterygoid and levator palpebrae superioris → ptotic eyelid elevated with opening of mouth, or movement of jaw to contralateral side, or jaw protrusion 2. Aberrant connection between INTERNAL pterygoid and levator palpebrae superioris → ptotic eyelid elevated with closure of mouth" What is the normal levator function (LF)?;"• More than 12-16 mm • LF < 12 mm → myopathic or neurogenic cause of ptosis" What is the definition and causes of paradoxical pupillary reaction?;"• Immediate pupillary constriction instead of dilation upon turning exam room lights off • Highly suggestive of optic nerve or retinal pathology • Also seen with children with poor vision Differential diagnosis • Congenital stationary night blindness • Congenital achromatopsia • Best disease • Dominant optic atrophy" What work up should be done for neuroretinitis?;"• Serology work up: bartonella henselae, syphilis, sarcoidosis, toxoplasmosis, lyme • If serologic workup negative → idiopathic neuroretinitis AKA Leber’s idiopathic stellate neuroretinitis • Neuro-imaging is not necessary" What is the most specific sign for optic neuritis?;"• Eye pain, worse with eye movement • Present in 92% of patients with optic neuritis, per optic neuritis treatment trial" In thyroid-associated ophthalmopathy, what is the threshold of difference in IOP in primary gaze and upgaze to be considered significant?;"5 mm Hg higher in upgaze" What is Balint syndrome?;"• Bilateral dorsal parieto-occipital lesions • Difficult with visuospatial tasks and visual processing Classic triad: 1. Optic ataxia (poor hand eye coordination) 2. Ocular motor apraxia (difficulty initiating saccades) 3. Simultagnosia (failure to understand entire meaning of picture despite retaining ability to recognize isolated objects within picture) -most prevalent" What is periodic alternating nystagmus (AKA central vestibular instability nystagmus)?;"• Bilateral horizontal jerk nystagmus that periodically changes direction (usually 2-4 minutes) • Potentially alternating head turn • Null point alternates • Etiology: posterior fossa pathology in brainstem or cerebellum • Any surgery that would shift null point to primary gaze should be avoided" What is spasmus nutans?;"• Benign, self limited • High frequency, low amplitude nystagmus • Dysconjugate • Associated torticollis and head nodding • Usually horizontal and bilateral; can be unilateral, vertical or rotary • Presents within first 2 years of life and resolves within 1-2 years • Resembles Heimann-Bielschowsky • Obtain neuroimaging to rule out anterior optic pathway glioma" What is eight-and-a-half syndrome?;"• One-and-a-half syndrome (internuclear ophthalmoplegia with ipsilateral horizontal gaze palsy) plus ipsilateral facial palsy" How is chronic progressive external ophthalmoplegia diagnosed?;"• Skeletal muscle biopsy • Biopsy positive for high number of cytochrome C oxidase negative fibers with ragged red fibers on Gomori’s trichrome stain" What are the clinical findings in Holmes-Adies syndrome?;"• Adie tonic pupil plus decreased deep tendon reflexes (hyporeflexia)" What is the etiology and clinical findings in acute optic disc edema?;"• Caused by impaired axoplasmic flow in ganglion cell axons Clinical findings • Elevated appearance of optic nerve head • Blurring of optic nerve head margins and blood vessels as they traverse border of optic nerve • Peripapillary retinal venous dilation and tortuosity • Peripapillary hemorrhages and exudates • Hyperemic vasculature of optic nerve head • Chorioretinal folds • Macular edema" Ishihara color plates test what type of color vision deficiency?;"Red-green deficiency" Lanthony tritan plates test which type of color vision deficiency?;"Blue-yellow deficiency" What is the 10-20-40-60 rule?;"• Obtained from optic neuritis treatment trial • Isolated optic neuritis, negative MRI: 22%risk of MS development in 10years • All patients with single bout of clinically isolated optic neuritis (not taking into account MRI findings): 38% risk of MS development in 10 years • Optic neuritis plus one or more white matter lesions on MRI: 56% risk of MS development in 10years" What structures sit in the sella turcica and above the sella turcica?;"• Pituitary gland sits inside sella turcica • Optic chiasm is approximately 10 mm superiorly" What bone makes up the sella turcica?;"Sphenoid bone" What are the similarities and differences between Pseudo Foster Kennedy (PFK) syndrome and Foster Kennedy syndrome (FK)?;"Common among both PFK and FK • One side with atrophic optic nerve and another side with swollen optic nerve Differences between PFK and FK • PFK:bilateral sequential attacks of NAION. First optic nerve affected becomes pale 4-8 weeks after attack, then second optic nerve becomes affected and acutely swollen. 5-year risk of involvement of fellow eye with NAION is approximately 15% • FK: intracranial mass on one side compressing ipsilateral optic nerve causing optic atrophy and thus optic nerve pallor. While also raising intracranial pressure and causing contralateral optic disc to swelling" What are the clinical findings in Heerfordt syndrome (uveoparotid fever)?;"• Uveitis • Facial palsy due to parotid gland swelling • Fever • Seen in sarcoidosis" What are the clinical findings in Lofgren syndrome?;"• Erythema nodosum • Bilateral hilar adenopathy • Arthritis • Seen in sarcoidosis" Where is the lesion in Millard-Gabler syndrome localized and what are the clinical findings?;"• Lesion located in ventral pons affecting cranial nerve 6, 7 and corticospinal tract • Ipsilateral abduction deficit • Contralateral spastic hemiplegia • Ipsilateral facial weakness" What is the indication for use of fingolimod?;"• Treat relapsing remitting multiple sclerosis" What is the cause of gaze-evoked amaurosis?;"• Intermittent occlusion of retinal or optic nerve vasculature caused by intermittent compression from orbital mass when eye is turning in a certain position • Most common gaze is downgaze" What is dissociated nystagmus?;"• Amplitude of wave form of nystagmus differs in each eye (nystagmus more pronounced in one eye than the other eye)" What is disconjugate nystagmus?;"• AKA disjunctive nystagmus • Direction of nystagmus differs between eyes (i.e. right beating nystagmus OD; left beating nystagmus OS)" What is the null point of nystagmus?;"• Point of gaze where nystagmus is least severe" How is photostress recovery time measured and which two pathologies does it distinguish?;"• Measure BCVA, then shine bright light into undilated pupil for 10 seconds. Then measure the time required to read BCVA line or one line larger • > 90 seconds indicates significant maculopathy • Distinguishes photoreceptor dysfunction related maculopathy from optic neuropathy" Which neurological disorders are associated with irregularly shaped pupils?;"• Midbrain corectopia: diffuse damage to midbrain, selective inhibition of pupillomotor fibers from Edinger-Westphal nucleus • Migraine associated: ""tadpole pupils"" occurs in one eye, sectoral dilation or spasm of pupil lasting few minutes" According to the ischemic optic neuropathy decompression trial follow up study, what was the rate of contralateral involvement after five years in patients with unilateral NAION?;"14.7% in 5 years" What is the rate of contralateral involvement in untreated monocular vision loss from arteritic anterior ischemic optic neuropathy (GCA)?;"54-95% within weeks" Which drugs can cause drug-induced intracranial hypertension?;"• Vitamin A and derivatives (i.e. isotretinoin) • Tetracyclines • Cyclosporine • Oral contraceptives • Prednisone, especially with sudden withdrawal • Synthetic growth hormone" What are the causes of superior ophthalmic vein dilation?;"• High flow carotid cavernous fistulas • Low flow dural sinus fistulas • Cavernous sinus thrombosis" What is rebound nystagmus?;"• Occurs seconds after eyes brought to primary gaze following prolonged eccentric gaze • Fast phase nystagmus opposite to the previous gaze direction" What is gaze evoked nystagmus?;"• Inability to maintain fixation in eccentric gaze • Eyes drift back to midline and corrective saccade is generated to reposition eyes in eccentric target • Fast phase is always direction of eccentric gaze" What are the findings and associations with segmental optic nerve hypoplasia?;"● Usually superior half of the optic disc is hypoplastic ● Papillomacular bundle spared ● Normal visual acuity and color vision ● Associations: ○ Maternal diabetes during pregnancy ○ Isolated finding" What is a tonic pupil?;"• Females 70% • Unilateral 80% • Majority are idiopathic and isolated • Disruption of post-ganglionic parasympathetic pupillomotor fibers from ciliary ganglion • Hypersensitive to parasympathomimetic • Light near dissociation, segmental constriction, hypersensitivity to dilute pilocarpine 0.125%, loss of accommodation • Anisocoria greater in light • Associations: neurosyphilis, diabetes, chronic alcoholism, dysautonomia" What is the most common location of aneurysm causing pupil-involving cranial nerve 3 palsy?;"Junction of posterior communicating artery and internal carotid artery" What diseases are associated with agenesis of one or more cranial nerve nuclei in the brainstem?;"• Congenital fibrosis of extraocular muscles: congenital agenesis of cranial nerve 3, levator subnuclei and bilateral superior rectus subnuclei • Duane syndrome: agenesis or dysgenesis of cranial nerve 6 and nucleus • Mobius syndrome: agenesis of 6th and 7th cranial nerve" What is the cardinal feature of internuclear ophthalmoplegia?;"Slow adduction on the affected side compared to abduction velocity of contralateral eye" What is ophthalmoplegic migraine?;"• Episodic, localized orbital pain or headache associated with partial or complete paralysis of cranial nerve 3 • This is rare cause of episodic cranial nerve 3 palsy • Most common in childhood" What is the Goldmann perimetry nomenclature for target size, intensity, and filter?;"Target size presented with roman a number: 0 to V • 0: 1/16 mm2 • I: 1/4 mm2 • II: 1 mm2 • III: 4 mm2 • IV: 16 mm2 • V: 64 mm2 Brightness presented with an arabic number: 1 to 4 • 1: least intense • 4: most intense Filter presented by alphabets: A to E • E is the most commonly used filter V4e = largest and brightest target" What are the various presentations of giant cell arteritis?;"• Anterior ischemic optic neuropathy • Posterior ischemic optic neuropathy • Central retinal artery occlusion • Branch retinal artery occlusion • Choroidal infarction • Ocular ischemia • CNS stroke • Diplopia secondary ischemia of extraocular muscles or ocular motor nerves • Vasculitis of elastin containing large and medium sized arteries of head and neck" What are the clinical findings in Leber’s hereditary optic neuropathy?;"• Optic disc swelling without leakage on fluorescein angiogram • Peripapillary telangiectasia • Thickening of peripapillary retina • Tortuosity of medium sized retinal arterioles • Cecocentral visual field defect • Fellow eye with similar progress over weeks to months" What is the treatment for Leber’s hereditary optic neuropathy?;"• No treatment • Avoiding smoking and excess alcohol use is prudent to decreasing oxidative demand" What is numb-chin syndrome?;"• AKA mental nerve neuropathy • Highly concerning for malignancy • Most common associated malignancies: lymphoma and metastatic breast cancer • Sarcoidosis also been described as cause of mental nerve neuropathy" What is the standard "cycle" for periodic alternating nystagmus?;"• Duration: 2-4 minutes • One cycle: from static phase through 2 nystagmus phases in different directions and back to static phase" What conditions are associated with small optic disc drusen?;"• Retinitis pigmentosa • Pseudoxanthoma elasticum • Caucasian race" Which secreting pituitary adenoma is most likely to present with optic chiasm compression?;"Prolactin secreting adenoma" What are the best tests for posterior cortical atrophy?;"• Functional MRI • PET scan SPECT → test highlights hypometabolism and hypoperfusion in the brain" What is the differential diagnosis, etiology, and workup for nystagmus-like eye movements without intersaccadic interval?;"Ocular flutter • Repetitive purely horizontal saccades • Bursts of small-amplitude • Very high frequency (10-15 Hx) Opsoclonus (saccadomania) • Dramatic bilateral eye movements -high frequency with larger amplitude • Involuntary multidirectional saccades Etiology of ocular flutter and opsoclonus • May relate to dysfunction of omnipause neurons of pons or to connections with these cells Workup • Rule out paraneoplastic etiology. Children → most commonly secondary to neuroblastoma. Adults → most commonly secondary to small cell carcinoma of lung, breast cancer or ovarian cancer • In young adults, evaluate for multiple sclerosis" Which extraocular muscles are innervated by the right cranial nerve 3 nucleus?;"• Right inferior oblique, right inferior rectus, right medial rectus, LEFT superior rectus • Fibers innervating superior rectus muscle decussate and supply contralateral eye. The decussating fibers pass through the opposite superior rectus nucleus thus damage to right cranial nerve 3 nuclear result in bilateral superior rectus muscle involvement" Which extraocular muscles are innervated by the right cranial nerve 4 nucleus?;"• Left superior oblique, only cranial nerve to exit dorsal aspect of brainstem and decussates before exiting" What is the innervation of the levator palpebrae superioris muscle?;"• Muscles on both sides are innervated by centrally located common 3rd nerve subnucleus" Which extraocular muscles would be affected by a lesion of the 3rd nerve nucleus?;"• Ipsilateral palsy of inferior rectus, inferior oblique, medial rectus, superior rectus, levator palpebrae superioris • Contralateral palsy of superior rectus and levator palpebrae superioris" What kind of nystagmus is associated with hypertrophy of inferior olivary nucleus in the medulla on MRI?;"Oculopalatal myoclonus related pendular nystagmus" How does one conduct visually evoked potentials and what is their utility?;"• Rarely useful clinically • Present visual stimulus to one eye, then measure electrical brain waves over occipital cortex → rough estimate of function of entire afferent visual pathway • Good for estimating visual function in patients unable to communicate • Pattern-reversal VEP are useful for estimating actual visual acuity and require good cooperation" What are the indications for surgical intervention in idiopathic intracranial hypertension?;"• Progressive visual loss: optic nerve sheath fenestration → scar quickly, 15% success rate at 6 years • Intractable headaches despite maximal medical therapy → VP shunts → successful in relieving headache but high complication rate. Revisions in approximately 50%" What is pituitary apoplexy?;"• AKA hemorrhage into pituitary gland Cause of hemorrhage • Pituitary adenoma • Enlarged necrotic pituitary in peripartum period, known as Sheehan syndrome • MRI brain is the study of choice" What is the rate of morbidity of catheter angiography?;"1% to 2.5% due to risk of ischemia from emboli and vasospasm" What is oculomasticatory myorhythmia?;"• Very rare • Eye and facial movements seen in Whipple disease with CNS involvement • Use of muscles of mastication causes ocular oscillation • Vertical saccadic deficiencies • Chronic disease due to duodenal infestation with tropheryma whippeli • Associated with unintentional weight loss, fevers, lymphadenopathy" What is the definition of elevated opening lumbar puncture pressure?;"> 25 cmH2O" What is volitional nystagmus?;"• Voluntary nystagmus • Capable by 8% of popular • Bilateral • Conjugate • High frequency, low amplitude • Usually horizontal, can be vertical" What is Melkersson Rosenthal syndrome?;"• Triad: recurrent unilateral or bilateral facial paralysis (30%); chronic orofacial edema (usually lips); lingua plicata (fissured tongue, image below) • Cause unknown and no well defined treatment • Diagnose with lip biopsy → granulomatous infiltration " Which bone makes up the optic canal?;"Lesser wing of sphenoid" What are the contents of the optic canal?;"• Optic nerve • Ophthalmic artery • Sympathetic nerve" What are the clinical findings and work up for ocular ischemic syndrome?;"● Light induced amaurosis -characteristic ● Orbital or periocular pain that improves with laying down ● Hypotony ● Corneal edema ● Anterior chamber flare ● Mid-peripheral dot blot hemorrhage in the retina ● Retinal neovascularization ● Work up: ○ Fluorescein angiogram (delayed arm to eye time; areas of retinal, optic nerve neovascularization) ○ Carotid dopplers (stenosis) ○ CTA/MRA" What percentage of microvascular or vasculopathic cranial nerve 3 palsies demonstrate some degree of pupil involvement?;"20%" How long after initiating steroid treatment can temporal artery biopsy be delayed in cases of giant cell arteritis before compromising diagnostic yield?;"1 to 2 weeks" How many ganglion cell axons combine to form the optic nerve?;"1.2 million" What are the features of congenital nystagmus?;"""CONGENITAL"" Convergence and eye closure dampen nystagmus Oscillopsia absent Null zone present Gaze position does not change direction of nystagmus Equal amplitude and frequency in each eye Near acuity is good because convergence dampens nystagmus Inversion of optokinetic nystagmus Turning of head or abnormal head posture to allow eyes to enter null zone leads to better visual acuity Absent nystagmus during sleep Latent nystagmus occurs" Macular sparing homonymous hemianopia is specific to lesion in which cerebral lobe and most likely caused by stroke of which artery?;"● Occipital lobe ● Posterior cerebral artery ○ Posterior cerebral artery supplies majority of primary visual cortex ○ Middle cerebral artery supplies very posterior tip of primary visual cortex of occipital lobe responsible for contralateral macular portion of hemifield" What are the most common symptoms of idiopathic intracranial hypertension (IIH) and most common visual field defects in IIH based on the IIH treatment trial?;"● Symptoms: ○ Headache (most common; 84%) ○ Transient visual obscurations ○ Pulse tinnitus ○ Back pain ○ Diplopia ● Visual field defects: ○ Partial arcuate defects with and without enlarged blind spot (70-80%) ○ Enlarged blind spot alone" What is Ramsay Hunt syndrome?;"Herpes zoster involving cranial nerve 7" What is Foville syndrome?;"• Lesion of dorsal pons • Ipsilateral cranial nerve 5, 6, and 7 palsy without body weakness" What is dorsal midbrain syndrome?;"• Compression of posterior midbrain • Paralysis of upward gaze • Convergence retraction nystagmus • Involuntary convergence • Light near dissociation • Positive Collier sign -bilateral lid retraction • Increase in square wave jerks" What is sagging eye syndrome?;"• Seen in older patients • Lateral rectus-superior rectus band degenerates with inferior displacement of lateral rectus muscle • Prolapse of connective tissue supratemporally • Mild to moderate angle esotropia due to divergence insufficiency • Normal lateral gaze • Limited supraduction • Deep superior sulci • High lid creases • Ptosis secondary to levator aponeurosis dehiscence" What are the clinical findings in Susac syndrome?;"• Hearing loss • “Boxcar” retinal segmentation of retinal arteries • Corpus callosum with “snowball lesions” -signs of microinfarcts • Leptomeningeal enhancement" What are some multiple sclerosis medications and their side effects?;"• Natalizumab: altered mental status, hemianopia visual field defect due to progressive multifocal leukoencephalopathy in patients who are JC virus positive; nausea, infection, liver dysfunction • Fingolimod: macular edema • Interferon: flu-like symptoms, liver function tests changes, bone marrow suppression, thyroid dysfunction • Glatiramer: skin irritation, skin lipoatrophy, panic attack-like events" What are the types of glial cells and their function?;"• Types: oligodendrocytes, astrocytes, microglial cells, muller cells • Overall function: support function of CNS. Proliferate in response to retinal trauma • Oligodendrocytes → maintain myelin • Astrocytes → support and nutrition • Microglial cell (histiocytes of CNS) → phagocytic function • Muller cells: footplates form inner limiting membrane. Attachment between photoreceptors and Muller cells creates external limiting membrane" What is measured with multifocal ERG?;"• Direct measure of macular function and central retinal cone-system function • More objective index of macular function than pattern ERG" What is measured with pattern ERG?;"• Measure of central retinal ganglion cell function • Less objective index of macular function than multifocal ERG" What is measured with electrooculogram (EOG)?;"• Assess health of RPE by measuring changes in corneo-retinal standing potential during light adaptation • Arden ratio: ratio of light peak (Lp) to dark trough (Dt); determines the normalcy of the results • Arden ratio 1.80 or greater is normal, 1.65 to 1.80 is abnormal, and < 1.65 is significantly abnormal" What is the anatomy of cranial nerve 6?;"• Starts at nucleus of dorsal pons → ascends along clivus → crosses petrous apex → passes below petroclinoid ligament → center cavernous sinus • Takes 90 degree turn over tip of clivus and under petroclinoid ligament → vulnerable to compression from high or low intracranial pressure" What is prosopagnosia?;"• Inability to recognize familiar faces • Congenital or acquired from stroke in bilateral inferior occipitotemporal lobes or right inferior occipital lobe" Damage to which structure leads to alexia without agraphia (cannot read but can write)?;"Selenium of corpus callosum and left occipital lobe" What are the clinical findings and cause of lateral medullary syndrome?;"• Although lateral medulla is supplied by posterior inferior cerebellar artery, this syndrome is secondary to occlusion of vertebral artery • Ipsilateral Horner syndrome • Ipsilateral impairment of pain and temperature sensation over face (descending tract of cranial nerve 5) • Contralateral impairment of pain and temperature sensation (spinothalamic tract) of body • Ipsilateral cerebellar ataxia (spinocerebellar tract) • Ocular tilt reaction (head told toward side of lesion) • No extremity weakness • Lateropulsion (sensation of being pulled toward the side of lesion) • Dysarthria, dysphagia, vertigo" What VEP abnormality is present in optic nerve disease?;"• Peak-time delay in pattern reversal VEP • Increased latency in demyelination" What VEP abnormality is present in NAION?;"Amplitude reduction" What VEP abnormality is present in macular dysfunction?;"Delayed pattern reversal VEP" What is STIR MRI?;"• T1 images without confounding bright fat single • Useful for orbital and spinal cord evaluation" What is Uhthoff phenomenon?;"Worsening vision in multiple sclerosis patients when their bodies become overheated" What are the clinical findings in true infantile nystagmus (AKA congenital motor nystagmus)?;"• Paradoxical inversion of OKN response → right jerk nystagmus views OKN to the left, intensity of right jerk nystagmus increases • Velocity of slow-phase movement increases exponentially with distance and fixation • X-linked mutations in FERM domain-containing-7 gene" What does nystagmus that changes direction with change in fixation indicate?;"Fusion maldevelopment nystagmus syndrome (infantile-onset nystagmus) → left jerk nystagmus when right eye is occluded, right jerk nystagmus when left eye is occluded" Inferior rectus weakness is most evident in which position?;"Abducted position" Superior oblique weakness is most evident in which position?;"Adducted position" Which supranuclear ocular motor lesions produce strabismus and diplopia?;"• Convergence insufficiency or spasm • Divergence insufficiency • Ocular tilt reaction • Skew/alternating skew deviation • Thalamic esodeviation" What is the pathway of the pupillary light reflex?;"Retina → pretectal nuclei → Edinger-Westphal nuclei → cililary ganglion → pupilloconstrictor fibers " What is the optic nerve sheath meningioma triad?;"• Painless, slowly progressive vision loss • Optic atrophy • Optociliary shunt vessels" What is the path of sympathetic fibers in the cavernous sinus?;"Within cavernous sinus, fibers leave the carotid in junction with cranial nerve 6 for a few millimeters. Anteriorly in the cavernous sinus, fibers join nasociliary branch of cranial nerve V1."