Neuro-Ophthalmology
Putu Ayu Wulansari, MD
Optic Neuropathy
VF Defect Pattern
Optic Neuropathy
VF Defect Pattern
A. Cecocentral scotoma OS,
paracentral scotoma OD
B. Central scotoma OD
C. Arcuate scotoma OD
D. Broad arcuate
(altitudinal) OS
E. Nasal arcuate (nasal
step) ODS
F. Enlarged blind spot ODS
(OS>OD)
Optic Neuropathy
General Consideration: Etiology
Optic Neuropathy
Papilledema
• ONH edema resulting from increased ICP, eg: intracranial mass,
hydrocephalus, meningeal condition, increased venous pressure,
idiopathic intracranial hypertension
• Papilledema looks the same like any other ONH edema hiperemis,
dilatasi vaskuler, telangiectasia
• Increased ICP additional symptoms headache, nausea, vomiting
• DD: optic disc drusen
ONH edema Optic Disc Drusen
Optic Neuropathy
Idiopathic Intracranial Hypertension a.k.a Pseudotumor Cerebri
• Symptoms and sign of increased ICP + diplopia ec CN VI palsy, CN VII
dysfunction, tinnitus
• Incidence peaks in 3rd decade
• FR: women, obese, vitamin A use/retinoic acid,
tetracycline/minocycline/doxycycline, pregnancy, sleep apnea
• Rule out masses by MRI imaging first
Optic Neuropathy
Idiopathic Intracranial Hypertension a.k.a Pseudotumor Cerebri
Enlarged blind spot on perimetry
• Tx: self limited, weight loss for obese px, acetazolamide (1-4 g/day),
corticosteroid should be avoided, optic nerve sheath fenestration
(ONSF)
Optic Neuropathy
Inflammatory Disease
• Optic neuritis papillitis (ONH edema, more common in infectious
etiology), retrobulbar neuritis (ONH normal)
• Acute inflammatory demyelinating ON (associated with MS)
typically in young female, acute onset, periorbital pain incited by eye
movement, RAPD, retrobulbar form >>, red-green dyschromatopsia,
central scotoma on perimetry, spontaneous resolution in 1 month
Enhancement of optic nerve
Optic Neuropathy
Inflammatory Disease
• Typical ON is associated with demyelinating disorder (MRI:
periventricular white matter)
• Atypical features older age, lack of pain, persistent pain/vision loss,
peripapillary hemorrhage/exudate
• In atypical case hematologic/serologic testing (eg: serum MOG-IgG,
ANCA, AQP4-IgG, etc)
• Tx: spontaneous improvement, ONTT (MP IV 250 mg every 6 hours for
3 days, followed by oral prednisione 1 mg/kgBB for 11 days) speed
recovery by 1-2 weeks
Optic Neuropathy
Inflammatory Disease
Optic Neuropathy Anterior Ischemic Optic Neuropathy
Vascular Condition
NAAION
AAION
Pallid ONH edema
Optic Neuropathy Non-Arteritic Anterior Ischemic Optic Neuropathy
(NAION)
Vascular Condition
• More common than AAION. About 90-95% cases
• Associated strongly with “disc at risk” / “crowding disc” phenomena
• FR lain: DM, HT, hiperlipidemia, OSA
• Most common VF pattern defect is altitudinal
• DD: optic neuritis
Optic Neuropathy Optic nerve sheath meningioma
Compressive/Infiltrative Lesion
Opto shunt vessel
• ONSM ≠ intracranial meningioma
involving/affecting the optic nerve tx akan
berbeda (ONSM tidak bisa direseksi but ICM
bisa)
• Characteristically unilateral, middle-aged
woman, trias: painless slow progressive vision
loss, optic atrophy, optociliary shunt vessel
• Tx: streotactic fractionated radiation
Tram track sign
Optic Neuropathy Optic nerve/pathway glioma
Compressive/Infiltrative Lesion
• Most common primary tumor of optic nerve
• 70% detected in 1st decade, 90% in 2nd decade
• Most common signs: proptosis, vision loss, ONH
pallor/edema, strabismus
Optic Neuropathy Leber Hereditary Optic Neuropathy (LHON)
Hereditary Condition
• Caused by mitochondrial DNA mutation (inherited by the mother)
• Affects boys and men aged 15-25 y.o., acute, painless, severe vision
loss <20/200, central/cecocentral VF impairment
• Tx: none proven, Idebenone?
Trias:
ONH hyperemic and looks elevated (pseudoedema)
Peripapil telangiectasia
Turtousity of retinal vessel
Optic Neuropathy
Toxic substance & nutritional def.
• Gradual, progressive, painless vision loss, bilateral & symmetric
• Optic atrophy may eventually develop
• Causative agents: methanol, ethylene glycol, organic solvent, lead,
tobacco, ethambutol, linezolid, amiodarone, disulfiram, cisplatin &
vincristine, interferon and anti TNF
• Deficiency: vitamin B12, copper, folate, thiamine
Optic Neuropathy
Trauma
• TON direct (ON laceration/injury) and indirect (due to transmitted
force of contusion/head trauma)
• Vision loss is typically immediate and severe, RAPD variably present,
ONH normal at onset but becomes pale in 4-6 weeks
• Therapy for TON is controversial
Cranial Nerve Palsy
Third Cranial Nerve Palsy
• Dysfunction of somatic muscle (IR,
MR, IO, LPS) and autonomic
muscle (pupil sphincter)
• Partial palsy > complete palsy
• Most isolated CN III palsy is from
microvascular origin (ischemia)
Cranial Nerve Palsy Pupil-involving 3rd cranial nerve palsy
Third Cranial Nerve Palsy
• Signs: mid-dilated pupil responding poorly to light +/- EOM dysfunction
• Non traumatic CN III palsy with pupillary involvement is assumed
secondary due to aneurysm until proven otherwise cerebrovascular
imaging (CTA is faster or MRA)
• Only pupil dysfunction but normal lid and EOM function usually
benign disorder
Cranial Nerve Palsy Pupil-sparing 3rd cranial nerve palsy
Third Cranial Nerve Palsy
• May be complete or incomplete (depends on LPS or EOM dysfunction
with normal pupil reaction)
• Pupil-sparing complete FR: DM, HT, hiperlipidemia known risk factor
does not warrant neuroimaging
• Pupil-sparing incomplete may be due to compressive lesion (incl.
aneurysm) imaging is indicated
• Suspected microvascular CN III palsy GDS, blood pressure, serum lipid,
ESR and CRP for older adults (GCA screening)
Cranial Nerve Palsy
Fourth Cranial Nerve Palsy
• Usually congenital, head trauma,
acquired onset > 40 y.o. consider
microvascular origin
• Signs: diplopia worse in
contralateral gaze and ipsilateral
head tilt patients usually tilting
contralateral lesion to avoid
diplopia
• Diagnostic: three step test
Cranial Nerve Palsy
Sixth Cranial Nerve Palsy
• Most frequently affected nerve in isolated ocular motor nerve palsy
• Signs: horizontal diplopia worsen on ipsilat gaze, abduction deficit and
ET
• Most common cause: ischemic mononeuropathy (>>), lesion of
cerebellopontine angle (eg: acoustic neuroma or meningioma usually +
N V, VII, VII affected); younger px: look signs for CCF, MS; children:
leukemia and brainstem glioma
• Imaging if no resolution within 3 months or additional symptom develop
Cranial Nerve Palsy Carotid-Cavernous Fistula
Sixth Cranial Nerve Palsy
• Abnormal connection between cavernous sinus & carotid artery
inducing high arterial pressure
• Classified: direct/high-flow (ec head trauma, produce bruit) vs
indirect/low-flow (spontaneously, in older woman)
• Signs: proptosis, elevated ICP, diplopia (CN VI palsy), ischemic optic
neuropathy
• Imaging: enlargement superior ophthalmic vein
Pupillary Abnormality
Anisocoria
Pupillary Abnormality Horner Syndrome
Anisocoria
Anisocoria greater in dim light
• Lesion in oculosympathetic pathway triad: ipsilat miosis, facial
anhidrosis, ipsilat ptosis
• Pharmacologic confirmation testing: apraclonidine or cocaine eye drop
Lesion location
- First order (CNS) lesi pontine, lesi
spinal cord
- Second order (preganglionic)
superior sulcus of lung/Pancoast
tumor, brachial plexus injury
- Third order (postganglionic)
internal carotid artery dissection
(pain!), superior cervical ganglion
lesion
Anisocoria greater in
dim light
Pupillary Abnormality Idiopathic tonic (Adie) pupil
Anisocoria
Anisocoria greater in bright light
• Unilateral in 80% cases, mostly female
• Large pupil poor reaction to light but reacts strong & well to near vision
(light-near dissociation)
• Due to damage of ciliary ganglion / short ciliary nerve (post ganglionic)
• Pharmacologic confirmation testing: pilocarpine eyedrop
Associated with Systemic Disease
Giant Cell Arteritis (GCA)
• Systemic inflammatory granulomatous vasculitis + polymyalgia
rheumatica exclusive for px > 50 y.o. and women more common
• Associated with AAION and CRAO
• Symptoms: headache, scalp tenderness, jaw/tongue claudication
• Diagnosis: ESR, CRP, CBC. Gold standard: temporal artery biopsy
• High suspicion start corticosteroid (MP IV 1 g/day for 3-5 days)
Associated with Systemic Disease
Multiple Sclerosis
• Inflammatory neurodegenerative disorder of CNS more
likely in women, highest 25-40 y.o., FR: vitamin D deficiency
• Associated with typical optic neuritis (presenting symptom
in 25% cases and will occur in about 75% patients)
• Symptoms: ocular complain, cerebellar dysfunction, mental
change, motor & sensory symptom
• Diagnosis: McDonald criteria (history + presentation + MRI:
imaging) periventricular white matter
lesion
- Lab: presence of oligoclonal IgG band in CSF
- Tx: no cure for MS (ON: consider high dose corticosteroid)
Associated with Systemic Disease
Myasthenia Gravis
• Immunologic disorder characterized neuro-ophthalmic abnormalities
• Patfis: presence of antibodies which reduce the number of
acetylcholine receptor
• Signs: fluctuation of fatigability & weakness (worsen in the evening,
improve with rest) incl. ptosis
• Diagnosis: Tensilon (edrophonium chloride) test, neostigmine
methylsulfate test, sleep test, ice-pack test (90% sensitivity). Gold
standard: serum testing for anti-acetylcholine receptor antibodies;
single fiber ElectroMyoGraphy is most sensitive examination currently
Associated with Systemic Disease
Myasthenia Gravis
Tx: pharmacologic anticholinesterase inhibitor such as neostigmine &
pyridostigmine; corticosteroid; immunosuppressant; surgery
thymectomy consider in patients also presenting with thymoma
