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Flashcards Neuro
Flashcards Neuro
Flashcards Neuro
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"
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 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 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 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 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 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 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"
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 infiltrative optic neuropathy?;"• Optic nerve gliomas
• Leukemia
• Lymphoma
• Sarcoidosis
• Syphilis
• TB
• Fungal
• Meningeal-carcinomatosis"
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 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 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 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 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"
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 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 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"
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 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 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"
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"
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 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"
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 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"
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 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 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 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 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 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"
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 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 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 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 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 is the optic nerve sheath meningioma triad?;"• Painless, slowly progressive
vision loss
• Optic atrophy
• Optociliary shunt vessels"