Neuro-ophthalmologyNeuro-ophthalmology

sjtu ophthalmology sjtu ophthalmology 樊莹樊莹

Neuro-ophthalmologyNeuro-ophthalmology an important sensory end organan important sensory end organ intimatedly related to the brainintimatedly related to the brain

cranial nerves: cranial nerves: 5050 ％％ IIII 、、 IIIIII 、、 IVIV 、、 VV 、、 VIVI 、、 VIVIII

cranial nerve fiber: cranial nerve fiber: 3838 ％％ intracranial diseases: intracranial diseases: 6565 ％％

vision loss blindness diediagnosis including etiology and location

optic neuropathyoptic neuropathy

structure of retinastructure of retina

nerve fiber layer nerve fiber layer

sensory visual pathwaysensory visual pathway

anatomy of the optic nerveanatomy of the optic nerve

from optic disc to optic chiasmfrom optic disc to optic chiasm lengthlength ：： 45~50mm45~50mm includingincluding ：： intraglobeintraglobe 11 mm mm

intraorbitintraorbit 25~30 25~30 mmmm optic canaloptic canal 55 mmmm intracrania intracrania 1010 mmmm

histology of the optic nervehistology of the optic nerve

comprised by ganglion cell axonscomprised by ganglion cell axons covered by meninges (3 layers)covered by meninges (3 layers) lack of Schwann's cellslack of Schwann's cells hard to rebuildhard to rebuild

to valuate optic nerveto valuate optic nerve

morphologymorphology ：： ophthalmoscope ultrasonicophthalmoscope ultrasonic X-ray CT MRIX-ray CT MRI

functionfunction ：： vision acuity visual field vision acuity visual field pupil test VEP+ERGpupil test VEP+ERG dark adaption colour perceptiondark adaption colour perception

visual fieldvisual field testing testing

steady fixation steady fixation measure the comdined function of the retinameasure the comdined function of the retina

““peripheral vision”peripheral vision”

<30°central visual field<30°central visual field>30°peripheral visual field>30°peripheral visual field

kinetic perimetrykinetic perimetry

static perimetry static perimetry

electrophysiologic testingelectrophysiologic testing electrooculogram EOGelectrooculogram EOG electroretinogram ERGelectroretinogram ERG visual evoked potential VEPvisual evoked potential VEP

pupilpupil

light reflexlight reflex

normal

abnormal

optic neuropathyoptic neuropathy

inflammatoryinflammatory ：： optic neuritisoptic neuritis vascularvascular ： ： anterior ischemic optic neuropathy (AION)anterior ischemic optic neuropathy (AION) neoplastic infiltrationneoplastic infiltration ：： glioma, leukaemia, lymphoma, glioma, leukaemia, lymphoma, meningeal carcinomatosismeningeal carcinomatosis othersothers ： ： optic atrophyoptic atrophy 、、 papilledemapapilledema

Optic neuritisOptic neuritis

duo to a variety of causesduo to a variety of causesthe most common is the most common is demyelinationdemyelinationelse:else: immune-mediatedimmune-mediated direct infectionsdirect infections granulomatous optic nueropathygranulomatous optic nueropathy

contiguous inflammatory diseasecontiguous inflammatory disease

demyelinative optic neuritisdemyelinative optic neuritis clinical features:clinical features: chiefly in women(3:1) chiefly in whitechiefly in women(3:1) chiefly in white associated with multiple sclerosisassociated with multiple sclerosis subacute(2~7days)subacute(2~7days) color vision and contrast sensitivity impaired color vision and contrast sensitivity impaired pain(in eye 90%)(by eye movement 50%)pain(in eye 90%)(by eye movement 50%) abmormal in pupil testingabmormal in pupil testing change in VEP change in VEP a central scotoma in most casesa central scotoma in most cases brain MRI (necesssary)brain MRI (necesssary) including:including: papillitis papillitis

retrobulbar neuritisretrobulbar neuritis

PapillitisPapillitis in 35% casesin 35% cases hyperemia of the optic disk and large veins(ehyperemia of the optic disk and large veins(e

arly signs)arly signs) edema (nearly more than 3D) (common)edema (nearly more than 3D) (common) blurring of the disk margins (common)blurring of the disk margins (common) filling of the physiologic cup (common)filling of the physiologic cup (common) retinal exudates and edema (uncommon)retinal exudates and edema (uncommon) hemorrhage (uncommon)hemorrhage (uncommon) vitreous cells (uncommon)vitreous cells (uncommon)

PapillitisPapillitis

Fundus

PapillitisPapillitis

FFA

PapillitisPapillitis

Visual field

CT

Retrobulbar neuritisRetrobulbar neuritis

vision lossvision loss no obvious changes in the optic disk (at eno obvious changes in the optic disk (at e

arlier stage)arlier stage) secondary optic atrophysecondary optic atrophy

demyelinative optic neuritisdemyelinative optic neuritis

differential diagnosis:differential diagnosis: compressive optic neuropathycompressive optic neuropathy anterior ischemic optic neuropathyanterior ischemic optic neuropathy autoimmune optic neuropathyautoimmune optic neuropathy toxic amblyopiatoxic amblyopia Leber's hereditary optic neuropathyLeber's hereditary optic neuropathy vitamin Bvitamin B1212 deficiency deficiency papilledemapapilledema

demyelinative optic neuritisdemyelinative optic neuritis

treatmenttreatment:: steroid therapysteroid therapy can accelerate recovery of visioncan accelerate recovery of vision can not influence the ultimate visual outcomecan not influence the ultimate visual outcome may increase the risk of recurrencymay increase the risk of recurrency intravenous steroid therapyintravenous steroid therapy useful for multiple sclerosisuseful for multiple sclerosis

anterior ischemic optic anterior ischemic optic neuropathy (AION)neuropathy (AION)

due to infarction of the retrolaminar opdue to infarction of the retrolaminar optic nerve from occlusion or decreased tic nerve from occlusion or decreased perfusion of the short posterior ciliary perfusion of the short posterior ciliary arteriesarteries

vessels of the optic nervevessels of the optic nerve

AIONAION

causes:causes: arteriosclerosisarteriosclerosis diabetesdiabetes hypertensionhypertension hyperlipidemiahyperlipidemia intracranial strokeintracranial stroke vasculitis,migraine,inherited prothrombotivasculitis,migraine,inherited prothromboti

c states(in younger patients)c states(in younger patients) reduced cup,optic nerve head drusen,increreduced cup,optic nerve head drusen,incre

ased intraocular pressureased intraocular pressure

AIONAIONclinical features:clinical features:

in the sixth or seventh decadein the sixth or seventh decade vision loss without painvision loss without pain generally abrupt,progressivegenerally abrupt,progressive altitudinal vision field defects()altitudinal vision field defects() FFA:decreased perfusionFFA:decreased perfusion segmented the nonarteritic formsegmented the nonarteritic form diffuse the arteritic formdiffuse the arteritic form disk leakage the late phasedisk leakage the late phase

AIONAION

AIONAION

AIONAION

treatment:treatment: No treatment has been shown to provide long-teNo treatment has been shown to provide long-te

rm benefit.rm benefit.Low-dose aspirin may reduce the risk of involvemLow-dose aspirin may reduce the risk of involvem

ent of the fellow eye.ent of the fellow eye.identify the arteritic AION (high-dose systemic stidentify the arteritic AION (high-dose systemic st

eroids)eroids)

PapilloedemaPapilloedema

noninflammatory congestion due to raised inoninflammatory congestion due to raised intracranial pressurentracranial pressure

causescauses intracranial massintracranial mass abscessesabscesses subdural hematomasubdural hematoma arteriovenous malformationarteriovenous malformation subarachnoid hemorrhagesubarachnoid hemorrhage meningitis or encephalitismeningitis or encephalitis acquired hydrocephalitisacquired hydrocephalitis elseelse

PapilledemaPapilledema

increased intracranial pressureincreased intracranial pressure

axonal transpot is blockedaxonal transpot is blocked

edemaedema

PapilledemaPapilledema

clinical features:clinical features: normal vision in most cases for early papillenormal vision in most cases for early papille

demadema 1~2days to occur and 1 week to develop full1~2days to occur and 1 week to develop full

yy severe vision loss for late papilledemasevere vision loss for late papilledema

PapilledemaPapilledema

the early stage

papilledemapapilledema

the middle stage

papilledemapapilledemathe late stage

papilledemapapilledema

FFAFFA

papilledemapapilledemadifferential diagnosis:differential diagnosis:

burid drusen of the optic nerveburid drusen of the optic nerve small hyperopic diskssmall hyperopic disks AIONAION myelinated nerve fibersmyelinated nerve fibers

papilledemapapilledema

treatment:treatment:deal with the underlying causedeal with the underlying cause

Optic atrophyOptic atrophy primary optic atrophyprimary optic atrophy secondary optic atrophysecondary optic atrophy

primaryprimary secondarysecondary

axonal fiber axonal fiber atrophyatrophy

++ ++

proliferation of gliaproliferation of glia -- ++

pale diskpale disk ++ ++

papilledemapapilledema -- ++

blurring of blurring of marginmargin

-- ++

retinal vesselsretinal vessels normalnormal abnormalabnormal

Optic atrophyOptic atrophy

Optic atrophyOptic atrophy

treatment: treatment: to the underlying causeto the underlying cause

optic nerve tumoroptic nerve tumor

glioma of optic nerveglioma of optic nerve

meningioma of optic nervemeningioma of optic nerve

Glioma of optic nerveGlioma of optic nerve

Glioma of optic nerveGlioma of optic nerve

Meningioma of optic nerveMeningioma of optic nerve

Meningioma of optic nerveMeningioma of optic nerve

optic nerve tumoroptic nerve tumor

treatment:treatment: operationoperation

the optic chiasmthe optic chiasm

视交叉病变视交叉病变

the optic chiasm diseasesthe optic chiasm diseases

pituitary adenomapituitary adenoma craniopharyngiomacraniopharyngioma tuberculum sellae meningiomatuberculum sellae meningioma chiasmatic and optic nerve gliomaschiasmatic and optic nerve gliomas

pituitary adenopituitary adenomama

the retrochiasmatic visual paththe retrochiasmatic visual pathwaysways

cortical blindnesscortical blindness macular sparingmacular sparing macular splittingmacular splitting

casescases