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eye disease
kundart2
68
Pathology
Graduate
03/30/2014

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Term
wallenburg synrome
Definition
i. Lateropulsion (sensation of being pulled toward lesion) from involvement of inferior cerebral peduncle
ii. Cause: occlusion of ips vertebral artery
iii. Ipsipulsion of saccades
1. Hypermetric ipsilesional saccades, hypometric contralesional.
2. Ipsipulsion of vertical saccade causeing ablique tragectorory for vert
iv. Lateropulsion – deviation of eyes toward lesion. Closed eyes- dark- blinks
v. Impaired contralesional smooth pursuits.
vi. Ocular tilt reaction
1. Ipsiletional hypotropia, ipsilesional head tild
vii. Spontaneous nystagmus in central position.
1. Horizontal tortional
Term
inferior olivary nucleus
Definition
i. Oculopalatal tremor
1. Vertical pendular nystagmus – triangle of guillain and molleret (inferior olivary, red nucleus in midbrain, contralateral dendate nucleus in cerebellum.
ii. Synchromous movement of other body parts
iii. Infarct
iv. Treat with gabapentin
Term
perimedian medulary lesion
Definition
i. Vesibular nuclei, NPH, ventral tegmental tract
ii. Upbeat nystagmus
iii. Horizontal nystagmus with gaze evoked component.
Term
abducens
Definition
i. Send signal to ipsilateral lateral rectus and contralateral MR through MLF)
ii. Affects saccades, pusuits, and VOR
iii. Ipsilesional horizontal gaze palsy.
iv. Contralateral gaze deviation.
v. Spares vertical and vergence.
vi. Horizontal gaze evoked nystagmus may occur when looking contralesional. Quick phase away from lesion.
Term
PPRF
Definition
i. Unilateral lesion
1. Ipsilesional horizontal saccade palsy. Often leave pursuits intact.
2. Contralesional gaze deviation
3. Horizontal gaze evoked nystagmus on looking contralesionally. Quick phase away.
ii. Bilateral lesion
1. Total horizontal gaze palsy (both ways). Slowing of vertical saccades.
Term
MLF
Definition
i. MS
ii. Unilateral INO
1. Ispilesional adducting deficit. (MR weakness)
2. Abducting nystagmus of contralateral eye.
3. Skew deviation – hypotropia of contralesional eye.
4. Dissociated vert nystagmus
iii. Bilateral INO
1. Same as unilateral + abnormal vert movements. Vert gaze evoked nystagmus, impaired vert pusuits vor cancelations.
2. Saccadic intrusions.WEBINO, bilateral INO that may result in EXO fromloss of convergence.
Term
one and a half syndrome
Definition
i. MLF and ipsilesional abducens palsy. Abduction of contralesional eye is only preserved movment. 3 of 4 horizonatal recti appear to be broken.
ii. Paralystic pontine exotropia (contralateral eye deviated out, because its unopposed.
iii. MS
Term
NRTP
Definition
i. Abnormal saccades with torsional errors that violate listings law.
ii. Impaired convergence.
iii. Rostral NRTP lesion- upward defecits
iv. Ablation of Cerebral vermis- esodeviation
Term
RIMFL / basilar syndrome
Definition
i. Unilateral
1. Mild slowing of downward saccades
2. Torsional nystagmus beating contralateral to lesion.
3. Vertical one and a half syndrome- upgaze palsy in both eys, and monoc paresis of depression ipsilateral or a downgaze palsy in both eyes and monoc paresis of elevation in ipsilateral
ii. Bilateral
1. Loss of vertical and torsional saccade.
Term
Interstitial nucleus of Cajal
Definition
i. Unilateral
1. Impaired gaze holding in vert and torsional.
2. Ocular tilt reaction: skew deviation: hypo contra and contra head turn
3. Torsional nystagmus with ipsilesional quick phase
ii. Bilateral
1. All vertical impaired
2. Upbeat nystagmus
Term
Parinaud syndrome
Definition
i. From posterior commisure
ii. Vert gaze palsy and loss of vert gaze holding.
1. Upward movements affected most.
2. Downbeat nystagmus
iii. Convergence retraction on upgaze
iv. Light near dissociation of pupils
v. Lid retraction for straight ahead vision. (colliers lid retraction)
vi. Skew deviation
vii. Problems with vergence.
Term
central mesenphelic reticular formations.
Definition
i. Ipsilateral gaze shif
ii. Hypometric ipsilateral and hypermetric contralateral.
iii. Square wave jerks.
Term
thalmic esotropia
Definition
i. Esotropia that is a false CN 6 palsy
ii. Lesion on thalamus
iii. Wrong way deviation- deviation away from lesion
iv. Sustained downward deviation.
Term
Progressive supranuclear palsy
Definition
i. Vert saccades impaireds. Slow early, absent later.
ii. Horizontal saccades slow.
iii. Inaccurate antisaccades./ saccadic intrusions/ hypometria
iv. Poor pursuits
v. Loss of convergence
vi. Hummingbird sign due to midbrain atrophy
vii. Applause sign
viii. Fatal within 6 years.
Term
Whipple Disease
Definition
i. Vertical gaze palsy. Slow.
ii. Oculomasticatory myorhythmia.
1. Pendular convergence divergence oscillations.
iii. Contration of facial muscle.
iv. Treat with antibiotics like trimethoprim sulfmethozole
Term
MS
Definition
i. Optic neuritis, INO, neck shock
ii. INO, usually bilateral
iii. Gaze evoked nystagmus
iv. Acquired pendular nystagmus
v. Steroids for acute, beta interferon for chronic
Term
Wernickes encephalopathy
Definition
i. B1 deficiency
ii. Triad of opthalmoplegia, ataxia and global confusion state
iii. Horizontal and vert gaze palsies that may progress.
iv. INO, gaze evoked nystagmus, abduction problems, poor VOR. Upbeat nystagmus.
v. Treat with intravenous thiamine (B1)
Term
Alzeimers
Definition
i. Most common cause of dementia
ii. Progressive memory loss.
iii. Language disorders
iv. Impaired visuospatial skils
v. Impaired executive functions
vi. Large saccadic intrusions –frontal lobe
vii. Parietal lobe- cant shift attention, increased saccade latency.
viii. Secondary areas in visual cortex: impaired smooth pursuits.
Term
Cruetzfeild Jacob disease
Definition
i. Most common prion disease. Fatal in 8 months
ii. Triad of progressive dementia, myoclonic jerks, typical EEG changes..
iii. Slow horizontal and vertical saccades.
iv. PAN
v. Centripetal nystagmus, gaze evoked nystagmus, skew deviation.
vi. Balints syndrome
vii. Unable to close lids
Term
Aids dementia complex
Definition
i. Cd4 below 200
ii. Cognitive changes, clumsiness, behavioral changes.
iii. Frontal lobe
1. Increased latency of saccades, fixation instability, increased error with antisaccades, acquired oculomotor apraxia.
iv. Brainstem or cerebellar
1. Gaze evoked nystagmus, dissociated nystagmus, slow saccades., ocular flutter, decreased or asymetic slow pursuit gain.
Term
flocculus and parafloculus
Definition
a. Impaired ipsilateteral gaze holding, downbeat nystagmus, poor pursuitds, impaired VOR needs catch up saccades.
Term
nodulus and uvula
Definition
a. PAN, Abnormal velocity storage mechanism/ broke VOR. Downbeat nystagmus
Term
dorsal vermis
Definition
a. Hyptometric ipsilestional saccade. Poor pursuits toward lesion.
Term
fastigial nucleus
Definition
a. Hypermetric ipsilesional saccade, poor contra pursuits.
Term
chiari malformations
Definition
a. 3 types. Brainstem sinking through foramen magnum.
b. Downbeat nystagmus that is worse on lateral gaze.
c. Gaze evoke, horizontal, pan, seesaw…
d. INO, strabismus,
e. Poor OKN- slow.
f. Abnormal VOR
g. Saccadic dysmetria
h. Type 1
i. Adult: cerebellar vermis herniation
ii. Headaches, dizziness
iii. positional
i. Type 2
i. Child, herniation of cerebellar vermis, medulla ad 4th ventricle
ii. Myelomenigocele – hole in spine, 4th venrtical swelling
j. Type 3
i. Part of medulla and cerebellum lie withing the cervico-occipital meningomyelocele
k. Treat with surgical decompresssion
Term
dandy walker
Definition
a. Congenital enlargement of 4th ventrical, absence of corpus colosum and most of cerebellar vermis.
b. Developmental delay.
c. Balance problems
d. Enlarged head
e. Hydrocephalus
f. Mild saccadic dysmetria
g. Nystagmus and strabismus.
Term
walker warburg
Definition
a. Hydrocephalus, retinal dysphasia. Lethal within 1st few months of life.
b. Microphtalmos, coloboma,
c. Hypoplasia of cerebellar vermis, hydrocephalus, dandy walker malformations,
d. Muscular dystrophy, genital anomalies.
e. Persistant hyperplastic primary vitreous.
Term
epesodic ataxia type 1
Definition
a. 2-20 yo
b. Attacks seconds to minutes,
c. Stress, excersize trigger
d. Myokymia in periorbital muscles and fingers.
e. Acetazolamide treats in 50%
Term
epesodic ataxia type 2
Definition
a. 20-30
b. Hours
c. Stress excersize
d. 50% migraine
e. Mild ataxia, nystagmus (gaze evoked, rebound, downbeat) dysmetric saccades with normal velocity. Impaired pursuits, OKN, VOR cancelatoins. Increaded VOR gain.
f. 90% acetazolamide. Calcium channel blacker may work.
Term
benign recurent vertigo
Definition
a. 2-50 yo
b. Minutes to hours
c. Stress, sleep deprivations
d. 75% migrains
e. Trigeminal neuralgias
f. Vestibulopathy, vertigo, peripheral vestibular nystagmus
g. 70% respond to acetezolamide
Term
cogan syndrome
Definition
a. apraxia
b. Children turning their head instead of initiating eye movements. Hypometric saccades with long latencies.
c. Eyes deviated opposite to the turn.
d. Eye head coordination is normal.
e. Saccadic amp in small with a long latency.
Term
joubert syndrome
Definition
a. Underdeveloped cerebellar vermis. (contols balance and coordination)
b. Apraxia. With retinal dystrophy.
c. Molar tooth sign on MRI
d. Strabismus and pendular or seesaw.
e. Alternating skew deviation.
f. Treat iris colobomas with contact lenses. Can do surgery for ptosis.
Term
caudate nucleus
Definition
a. Hypometric slow memory guided saccades. Gaze preffernce, ipsilesional.
Term
sustantia nigra
Definition
a. Saccadic intrusions contralateral to lesion
Term
FAS
Definition
a. Facial dysmorphology
i. Epicanthus, smooth philtrum.
b. Growth deficiency
c. CNS disfunction
d. Microcornea
e. Optic nerve hypoplasia
f. Strabismus
g. Congenital cataracts
h. Saccadic disfunction
Term
huntingtons
Definition
a. Adult
b. Chorea
i. Uncontrollable flailing of extremities.
ii. Progressively replaced by systonia, causing twisted abnormal postures and parkinsonism causing rigity.
iii. Dementia and depression.
c. Saccades
i. Acquired oculomotor apraxia
ii. Difficulties suppressing reflexive saccades. To novel stimuli.
iii. Difficulty initiating saccades
iv. Slow saccades, especially vertical.
d. Poor pursuits
e. Saccadic intrutions.
f. Treatment:
i. For chorea: benzodiadepines, valproic acid, dopamine depleting agents. Neuroleptics
ii. For parkinsonism: levodopa or dopamine agonist.
iii. For depression:antidepressents.
Term
lewy body dementia
Definition
a. Either predominately parkinsons or predominantly dementia.
b. Vertical gaze palsy.
c. Lewy bodies associated with dementia
Term
parkinsons
Definition
a. Resting tremor, pill rolling,
b. Rigitiy, increased resinstance to passive movement.
c. Bradykinesia. Difficulty with rapid alternating movement.
d. Postural instability, shuffling gate.
e. Lewy bodies, loss of pigmented dopaminergic neurons in sustantia nigra
f. Square wave jerks.
g. Vertical Saccades especially upward early: hypometric. Advanced: slow
h. Impaired pursuits
i. Vor impaired
j. CI
k. Oculogyric crisis: eyes dev upward. – treat with anticholinergic drugs.
l. Lid abnormalities.
m. Treatment- levodopa and carbidopa
n. Monoamine oxidase b inhibitor for neuroprotections
o. Dopamine agonist (bromocriptine
p. Anticholinergics
q. Surgeries on thalmus
Term
PSP
Definition
a. Prominent akinesia, truncal rigity, subcortical dementia.
b. Slow vert saccades, especially downward
c. Neurofibril tangels and neuronal loss of subcortical and brainstem.
Term
tourrettes
Definition
a. Intermittent involuntary gaze deviations
b. Impaired sequencing of memory guided saccades.
c. Increased latency and decreased peak velocity of antisaccades.
d. Blepharospasm and eye tics.
e. Treat with clonidine
Term
frontal eye fields
Definition
a. Saccade problems,
b. Ipsilestional gaze deviation.
c. Hypometric saccade in contralateral side
d. Cant suppress inappropriate saccades.
e. Impaired pursuits
Term
supplementary eye fields
Definition
a. Inaccurate memory guided saccades.
Term
dorsolateral prefrontal cortex
Definition
a. Impaired predictice and memory guided saccade. Impaired antisaccades
Term
parietal lobe/ bilateral=balints syndrome
Definition
a. Unilateral
i. Ipsilesional gaze deviation
ii. Contralateral inatentiventess
iii. Increased latency for saccades
iv. Impaired pursuits
b. Bilateral
i. = balints syndrome
ii. Difficulty making visually guided saccades. Increased latency and decreased accuracy.
iii. Periphera visual inattention (simultagnosia)
iv. Inaccurage arm pointing. Optic ataxia
Term
primary visual cortex V1
Definition
a. Impaired ability to generate saccades or smooth pursuits in response to visual stimuli presented to a blind hemifield
Term
middle temporal visual area V5
Definition
a. Scotoma of motion, decreased smooth pursuit speed and dysmetric saccades.
Term
MST
Definition
a. Decreased pursuits toward vf defect.
Term
Chronic large hemispheric lesion
Definition
a. Shifting eyes in fixation.
b. Square wave jerks
c. Slow inaccurate saccades
d. Decreased pursuit gain.
e. Unsemetric VOR
Term
cavernous sinus thrombosis
Definition
a. Infection of cavernous sinus secondary to sinus infection
b. Headache, fever malaise
c. Periorbital edema and chemosis
d. Proptosis
e. Increased iop
f. Incomplete or complete opthalmoplegia. Including cn 346
g. Spread infection to the other cavernous sinus.
h. Signs of sepsis.
i. Treatment- aggressive broad spectrum antibiotics. Antichoagulants, corticosteroids, drainage of primary sourse of infection.
Term
carotid cavernous fistula
Definition
a. Corcrew conj veins, proptosis, orbital bruit, increase iop, eom paresis
b. Direct
i. High flow, high pressure
ii. Head trauma
iii. Fed by internal caratid artry in cavernous sinus
iv. Spontaneous recovery uncommon
v. Complications visual loss, stroke, subarachnoid hemmorage.
vi. Requires urgent treatment
1. Surgery to block fistula, ligation of caratid
c. Indirect.
i. Low pressure low flow
ii. Spontaneous
iii. Fed by dural branches
iv. Spontaneous recover
v. Complications- stroke subarachnoid hemmorage
vi. Treat- observation.
Term
tolusa hunt
Definition
a. Painful opthalmoplegia caused by nonspecific inflamaion of the cavernous sinus or superior orbital fissure.
b. Acute onet of seer, steady, retrobital periorbital pain and diplopia.
c. Involvement of CN2 and trigeminal nerve.
d. Can cause dilated or constricted pupil
e. Ptosis
f. Treatment: IV corticosteroids
Term
guillian barre
Definition
a. Acute inflammatory demyelinating neuapathy that is most frwquent cause of paralysis in adults and kids
b. Progressive muscle weakness
c. areflexia
d. Ptosis
e. Opthalmoplegia (abducens most)
f. Dilated pupil
g. Accomadative paralysis
h. Optic neuritis
i. Papilledema
j. Treatment: support, plasmapheresis or intravenous immunoglobulin.
Term
miller fisher
Definition
a. Subtype of guilian barr. Rapidly progressive triad of areflexia, ataxia, and opthalmoplegia.
b. Areflexia
c. Ataxia- truncal ataxia, difficulty with tandem gait, and positive Romberg test.
d. Opthalmoplegia- upgaze 1st then horizontal and downgaze
e. INO, 1 and a half, pretectal syndrome, spasm of near reflex, divergence paralysis
f. Ptosis and dilated pupil. Cold relieves ptosis like in MG
g. Treatment: supportive, plasmapheresis or intravenous immunoglobulin.
Term
abetalipoproteinemia
Definition
a. Lack of apolipoprotein B causing malabsorbtion of all fat soluble vitamins, including ADEK.
b. Steatrorrhea
c. Cerebellar ataxia.
d. Areflexia
e. Loss of proprioception
f. Pigmentary retinopthay
g. Opthalmoplegia and ptosis
h. Slow saccades
i. Dissociated nystagmus on lateral gaze. Fast adduction with little range of motion and slow abduction with full range of motion.
j. INO
k. Treatment: oral or intravenous A E K as well at iron.
Term
refsum disease
Definition
a. Defect in the metabolism or phylanic acid that leads to accumulation of phylantic acid in plama and tissue.
b. Neurologic manifestations: pheripheral neuraopathy cerebellar ataxia
c. Ocular: cataracts, pigmentary retinopathy, visual field constriction
d. Opthalmoplegia
e. Cardiomyopathy
f. Sensorinrual deafness
g. Treatment: diet low in phytanic acid and phtol (low in milk products, animal fats and green leafy vegetables)
Term
CN3
Definition
a. Limits adduction, elevation, and depression.
b. Incyclorotated and exo
c. Ptosis
d. Dilated pupil
e. Loss of accomadation
f. Cause- aneurism if it involves pupil, and ischemia if it doesn’t.
g. Treat with occlusion, prsim, botox to lateral rectus. Strab surgery, ptosis surgery.
Term
CN4
Definition
a. Hypertropia and excyclotorsion
b. Diplopia
c. Contralateral head tilt
d. Cause trauma
e. 75%congenital
f. Treat with prsm, occlusion and surgery
Term
CN 6
Definition
a. Limited abduction, esotropia
b. Treat with occlusion prism, nd surgery
Term
duane syndrome
Definition
a. Type 1 limited abduction
b. Type 2 limited adduction
c. Type 3 limited abduction and adduction.
d. Narrowing of palp fissure and globe retraction when you adduct
Term
LEMS
Definition
a. Presynaptic disorder tof neuromuscular transmission in which quantal release of acetylchoine is impaired.
b. 2/3 are paraneoplastic. 1/3 is autoimmune
c. Weakness and fatigability. – legs and pelvic girdle.,, bulbar muscle and ptosis
d. Hyporeflexia
e. Autonomic disfuntion- dry mouth, loss of pupillary refles,
f. Treatment: identify and treat malignancies. Meds: 3,4-diaminopyridine, cholinesterase inhibitors. Immunotherapy (steroids). Plasmaphereseis and intravenous immunoglobulin therapy.
Term
MG
Definition
a. Most common disorder affecting neuromuscular junction.. ocular 1st in 75%
b. Antibodies against acetylcholine receptors.
c. Ptosis, diplopia, muscle weaknes,
d. Ptosis gets worse on sustained upgaze
e. Lifting one lid will make ptosis worse
f. Cogans lid twitch from looking down.
g. Abnormal saccades
i. Hypermetria of small saccades and hypometria of large saccades.
h. Diplopia,
i. Workup: tensilon test. Ice test, sleep test.
j. Treatment: patching, prisms. Mestinon. Thymectomy. Immunosuppressive agents.plasmaphereisis, or intervenous immunoglobulin.
Term
Botulism
Definition
a. Caused by closteridium botulinum.
i. Food born
ii. Wound botulism
iii. Infantile botulism
b. Interferes with release of acetylcholine vesicles after stimulus induced influx of calcium into the nerve terminal.
c. Ptosis, general weakness, dizziness, dry mouth, blur, diplopia
d. Dysphagia, semetrical decnding flaccid paralysis and repiratory failure.
e. Nausea vomiting, quivering eye movements.
f. Black tar heroine injections, honey.
g. Treatment: bivalent or trivalent antitoxin.
h. Romove stomach contents., debribe wound.
Term
superior oblique myokymia
Definition
a. Dyskinesia caused by spontaneous discharge of the troclear nerve and eye rotates.
b. Intermittent uniocular microtremor consisting of spasm of torsional vertical rotations.
c. Causes diplopia , monocular oscilopsia
d. Lasts for seconds and occurs in clusters
e. Triggered by blinking, tilting head, downgaze
f. No underlieing disease necessary.
g. Treatment: may resolve spontaneously. Meds: carbamazepine, gabapentin, baclofen, propranolol. Surgery.
Term
brown
Definition
a. Deficient elevation on adduction .
b. Mechanical restriction of SO, cant get through trochlea or muscle is too short
c. Treatment: SO tendon tenotomy with inferior oblique recession. Controlled weakening fo the SO with silicone expander.. may try local infjections of steroid for acquired
Term
graves
Definition
a. Hyperthyroidism due to circulating autoantibodies
b. Females
c. Tachycardia, palptations. Tremor, weigh loss, heat intoleracnc.. easily fatigued
d. Ptotosis, lid retraction,
e. Eom involvement/fibrosis. Im slo
f. Exposure keratitis
g. Optic nruropathy
h. T4 level test, iodine testing
i. Treatment:
i. hypo? – replacement therapy
ii. hyper- radioablation with iodine, concurrent low dose steroids.
iii. For irritation and swelling-Steroids
iv. For ptosis- orbital decompression if seer
v. For strab- prism, surgery
vi. For exporsure keratitis, lubrication, nocturnal lid tape, orbital decompresion
vii. Optic neuropathy- steroid, radiation therapy, orbital decompression
Term
infectious myostitis
Definition
a. Infection
b. Ocular motility disturbances are usually caused by generalized orbital inflammation with soft tissue edema
c. Often occurs after trauma, then bacteria can sneak in. via infected sinuses
d. Bacterial, viral, parasitic, fungal
Term
kearne sayer syndrome
Definition
a. Chronic progressive external opthalmoplegia occurs in 90% of patients with mitochondrial myopathy. Slowly progressive ptosis and opthalmoplegia. Ptosis comes 1st.
b. Kearne sayre is a subtype of CPEO. – deletions of mitochondrial DNA.
c. Severe before age 20
d. Pigmentary retinopathy- mostly post pole/ salt pepper fundus/ mild nyctalopia.
e. Need one of the following:
i. Cardiac: heart blcok
ii. Neurologic: increased ptotein in cerebrospinal fluid, cerebellar ataxia. Deafness, dementia.
iii. Endocrine: short stature, hypoparathyroid, gonadal dysfunction, diabetes,
iv. Muscular: facial muscle weakness
f. Treatment: none. High lipid, low carb diet. Coenzyme Q10. Biotin, thiamine. Avoid meds like valproate and phenobarbital.
Term
myotonic dystrophy
Definition
a. Most common adult onset muscular dystrophy. Increased intensity as generations go by.
b. Myotonia of skeletal muscles. (involuntary delayed relaxation folloing contraction.)
c. Progressive weakness of distal muscles
d. Fontal balding, facial weekness. Long face, thin neck
e. Cardiac conduction defect.
f. Ptosis and opthalmoplegia.
g. Christmas tree Cataract.
h. Treatment: phenytoin. Cataract surgery, pacemaker, ptosis surgery, prism
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