Neuro-ophthalmology Flashcards
(200 cards)
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

