OPTIC ATROPHY

INTRODUCTION
DEFINITION:Optic atrophy can be defined
as damage to the optic nerve resulting in a
degeneration or destruction of the optic
nerve.

qFinal endpoint of any disease process

causing axon degeneration
qClinically, manifests as changes in the
color & structure of optic disc
qVariable degrees of visual dysfunction.
qActually a misnomer
qActually,atrophy refers to involution of a
OPTIC NERVE
qIInd cranial nerve
(neurosensory)
qComprises approximately
1.2 million axons
qOriginate at the ganglion
cell layer of the retina.
qThe axons are
myelinated by
oligodendrocytes,
qThe axons do not
regenerate.
qBehaves more like a
white matter tract
 
 Visual pathway
qUnilateral
atrophy
Lesion
involving
anterior to optic
chiasm
qBilateral
atrophy
Lesion
involving
 The optic nerve is
divided into the
following 4 parts:

4. Intraocular part
=optic nerve head (1
mm)
5. Intraorbital part (25
mm)
6. Intracanalicular part
(5 mm)
7. Intracranial part (10
mm)
Blood supply of
optic nerve
INTRAORBITAL
b) Periaxial :ophthalmic
A,Long posterior ciliary
A,Short posterior ciliary
A,Lacrimal A,Central a of
retina
c) Axial : Intraneural br of
Central retinal
A,Collateral br,Central A
of optic N
INTRACANALICULAR
Periaxial system
INTRACRANIAL: Periaxial
Br of int carotid A;Br
from ant cerebral A;small
INTRAOCULAR
1.Surface N. fibre layer
Capillaries from
retinal arterioles
2.Prelamiar region
Short posterior ciliary
A, Choroidal A
3.Laminar cribrosa
Short posterior
ciliary A
Circle of Zinn-Haller
4.Retrolaminar region
FEATURES OF NORMAL OPTIC NERVE
Normal nerve fibre layer Optic nerve atrophy
Hi st opa thol og ic c ha nge s in opt ic a tr oph y
v Shrinkage or loss of both myelin and axis
cylinders
v Widening of the pial septa
v Gliosis
v Deepening of the physiologic cup with barring
of the lamina cribrosa
v Widening of the subarachnoid space with
redundant dura
v Severed nerve leads to bulbous axonal
swellings (Cajal end bulbs)
Path ologic cl assif ica tion
v  Anterograde degeneration (Wallerian
degeneration)
q Deterioration begins in the retina and proceeds
toward the lateral geniculate body (ie, to the
brain).
q Toxic retinopathy, chronic simple glaucoma

v Retrograde degeneration
q Deterioration starts from the proximal portion of
the axon and proceeds toward the optic disc (ie, to
the eye).
q Intracranial tumor

v Trans-synaptic degeneration
Oph th almo sco pic cl assif ica tion

PRIMARY OPTIC ATROPHY

Secondary optic atrophy
 GLAUCOMATOUS/CAVERNOUS OPTIC
CONSECUTIVE OPTIC ATROPHY
ATROPHY
Eti ol ogi c c las si fic ati on
1) Hereditary
1) Congenital or infantile optic atrophy 
2) Behr hereditary optic atrophy
3) Leber optic atrophy
2) Consecutive atrophy
3) Circulatory atrophy
4) Metabolic atrophy :
Thyroid ophthalmopathy,
juvenile diabetes mellitus, nutritional amblyopia, toxic
amblyopia, tobacco, methyl alcohol, and drugs (eg,
ethambutol, sulphonamides).
5) Demyelinatingatrophy :
multiple sclerosis and
Devic disease.
6) Pressure or traction atrophy : glaucoma and papilledema.
7) Postinflammatory atrophy : optic neuritis, perineuritis
secondary to inflammation of the meninges, sinus and
orbital cellulites.
 DIFFUS
ATROPHY E

SECTORAL
Monocular band optic atrophy
qBand or "bow tie" optic atrophy
qHomonymous hemianopia
qInvovement of fibre entering optic disc
nasally & temporally(sparing superior &
inferior portion)
qUnilateral visual defect.
qAssociated with compressive
lesions of the pregeniculate
post-chiasmal pathway (or)
qCongenital malformations of
postgeniculate radiations or
cortex.
qThe optic atrophy is strictly
U/L
CAUSES
qAfter retrobulbar neuritis
qTumour & aneurysms
qHereditary optic neuropathies
qToxic & nutritional optic
 Primary optic atrophy
qDisc is chalky white
qWell defined
margins
qLamina cribrosa is
well defined
qSlight cupping
shallow,saucer
shaped
qSurrounding retina
is normal
qKestenbaum sign
ETIOLOGY OF POA
qIdiopathic
qDemyelination
qPost inflammatory
qToxic
qInflammation of orbit, sinus and
meninges
qCompressive
qNutritional
qHereditary
 www.riogohchennai.ac.inSeptember 2006

POST INFLAMMATORY
TABES & GENERAL PARALYSIS
qInflammation affects pial sheath
resulting in degenerative change
qGradual loss of vision
qContraction of fields for colours

TREATMENT:Penicillin eradicate
infection &arrest optic atrophy

19
Acute retrobulbar neuritis
It frequently affects only temporal ½ of disc
It occurs in
qDisseminated sclerosis
qLoss of blood
qFocal sepsis & catarrhal disease of nasal
sinus
qOrbital inflammation
qInfectiousDiseases:Meningitis/Encephalomy
elitis,
qVaccination
qNutritional disorder
CHRONIC RETROBULBAR NEURITIS

CAUSES
qTobacco(most common)
qMethyl alcohol,ethyl alcohol
qLead
qQuinine
qCarbon di sulphide,iodoform,thallium
qArsenical preparations
 MENINGITIS INCLUDING ARACHNOIDITIS OPTICO
CHIASMATICA
q Type of atrophy:descending

Arachnoiditis optico chiasmatica

q Chronic progressive disease
q affected by direct pressure
from membrane,cords or cysts

SIGN
Optic arachnoiditis on basal
q U/L or B/L loss of vision surface of pons & inter
q Centre or paracentral scotoma peduncular fossa
q Peripheral fields contraction
q Primary /secondary optic atrophy
treatment

Treatment is mainly surgical

qFreeing of adhesions
qLibration of cystic fluid
COMPRESSIVE OPTIC NEUROPATHY

qProgressive
scotoma
qInitially normal
disc
qSigns of atrophy
qDecrease in color
qDecrease in
vessels
TRAUMA (COMPRESSIVE)
ETIOLOGY
qDistension of optic sheaths with blood
under pressure
qBy bony fragments due to fracture of
optic foramen
 TUMOUR AND BONE DISEASES
qTumour in optic nerve,orbit and base of skull
qCauses pressure atrophy.
qGrowth position indicated by clinical findings
& visual fields.

Optocilliary blood
CAUSES vessels
qPituitary tumour
qSuprasellar meningioma
qOsteopetrosis(albers schoenberg’s disease)
qIdiopathic hypercalcemia
 SECONDARY OPTIC ATROPHY
qDisc is grey or dirty
grey
qMargins are poorly
defined
qLamina cribrosa
obscured
qPeripapillary
sheathing of Artery
qTortorous veins
qKestenbaum’s sign
Secondary optic atrophy
q Ischaemic optic neuropathy
q Chronic papilloedema
q Chronic optic neuritis
 Causes of secondary optic atrophy
q Infectious Disorders (Specific q Metabolic, Storage Disorders
Agent) q Tay-Sachs disease
q Syphilis q Gangliosidosis
q General paresis/CNS syphilis dementia, generalized (GM1)
q Infantile ceroid lipofuscinosis
q Meningitis, cryptococcal /Finnish (Santvuori-Haltia)
q Tabes dorsalis q Juvenile ceroid lipofuscinosis
/Batten-M
q Granulomatous, Inflammatory
q Sandhoff disease
Disorders
q Orbital inflammatory disease q Deficiency Disorders
q Neoplastic Disorders q Anemia of malnutrition
q Kennedy syndrome q Malnutrition/Starvation
q Sellar tumor/suprasellar q Congenital, Developmental
extension Disorders
q Meningioma q Craniofacial dysostosis/
q Suprasella tumor Crouzon
q Optic atrophy-ataxia syndrome
q Allergic, Collagen, Auto-
Immune Disorders
q Takayasu's pulseless aortitis
synd.
q Familial, Genetic q Vegetative, Autonomic,
Disorders Endocrine Disorders
q Acrodermatitis q Pseudotumor cerebri
enteropathica /Benign Intracranial Hyperten
q Cockayne syndrome
q Leukodystrophy, Krabbe q Reference to Organ
q Optic atrophy, hereditary, ofSystem
Leber q Optic atrophy, secondary
q Pelizaeus-Merzbacher q Optic neuritis, acute
disease q Pernicious anemia
q Brooks syndrome
q Drugs
q Usage, Degenerative, q Tryparsamide
Necrosis, Age Related Administration/Toxicity
Disorders q Suramin (Germanine/
q Multiple sclerosis Antrypol
q Optic atrophy, primary ) Administration/Toxicity
q Arteriosclerotic, q Khat herbal/intake
Vascular, Venous q Poisoning (Specific Agent)
Disorders q Methanol ingestion/poisoning
GLAUCOMATOUS OPTIC ATROPHY
qMarked excavation of
optic disc
qPosterior bowing of
lamina cribrosa
qColumnar atrophy
qDecrease in number &
size of small blood
vessels
qIncreased IOP recession of lamina
cribrosa
qCupping depends on
qIOP
qToughness of lamina cribrosa.
qAtrophy of the nerve fibers occurs due
to
q IOP
q vascular sclerosis
qThe degeneration is of nerve fibers and
ganglion cells & inner plexiform &
inner nuclear layers.
qGlucomatous excavation is due to
qatrophy of the nerve
qdisappearance of glial tissue
qThe duration of increased IOP
 SCHNABEL’S CAVERNOUS OPTIC ATROPHY
qSchnabel ‘s theory :cupping of
the disc in glaucoma was not
due to IOP
Enlarge to
qSeen in glaucoma fron
t formwithout raised
IOP
Minute cavities
large
cavity
in front &
behind lamina behind Collapse
& form
cup

qProgressive diminution of blood

supply of the nerve
disappearance of nerve fibers.
 CONSECUTIVE OPTIC ATROPHY
qAssociated with lesions of retina
& choroid.
qIn chorioretinitis & primary
pigmentary degeneration of the
retina.
qInflammatory / degenerative
lesions.
qAscending type

OPHTHALMOSCOPICALLY
qYellowish waxy disc
qAttenuated retinal vessels
qChanges in the surrounding
Optic atrophy in Retinitis
pigmentosa
 HEREDITARY

INFANTILE HEREDITARY BEHR’S LEBER’S DISEASE

ATROPHY DISEASE

INFANTILE HEREDITARY OPTIC ATROPHY

qRecessive inheritence
qLoss of vision
qConstricted visual field
qNystagmus
CONGENITAL OPTIC ATROPHY

MOTHER SON
Behr’s optic atrophy

qBegins in infancy
qAutosomal recessive
qIncomplete bilateral optic atrophy with
temporal pallor of both disc
qAssociated with neurological
abnormalities:cerebellar
ataxia,spasticity, Pyramidal tract
abnomality
 Leber’s optic atrophy
qBilateral
qRecessive X-linkage
mitochondrial DNA
mutation
Telangiectic
11778 microangiopathy
qIn adolescent males
qUnilateral visual defect.
qCentral field
defect:pericentral (or)
paracentral scotoma
qBlindness unusual
qColour blindness except
blue & violet Severe optic
Dif fere nti al D iag nosis
qAxial myopia
qOptic nerve hypoplasia
qBrighter-than-normal luminosity
qOptic nerve pit Optic nerve pit
qMyelinated nerve fibers
qScleral crescent
qOptic disc drusen
qTilted disc

Temporal
crescent
Symptoms
qBlurred vision
qAbnomal side vision
qDefective color vision
qDecreased brightness in one eye
relative to the other
WORK-UP OF OPTIC ATROPHY
q Visual acuity
q Colour vision
q Contrast sensitivity test
q Pupillary evaluation
q Pupil size
q RAPD(Relative afferent pupillary
defect)
q Edge-light pupil cycle time
q Photostess recovery test
q Pulfrich phenomenon
q Cranial nerve examination
q Extraocular movements
q Visual field PULFRICH PHENOMENON
q EEG
q Visually evoked response
q Imaging technique
q B-scan,
q CT scan,MRI
q Gadolinium enhanced MRI
MRI in multiple
sclerosis
BILATERAL OPTIC ATROPHY WITH
CENTROCECAL
SCOTOMAS

qHereditary
(dominant, Leber’s)
qToxic (medications,
methanol, heavy
metals)
qNutritional (folate,
B12)
qDemyelinating (optic
neuritis, multiple
sclerosis) CENTROCECAL
OTHER INVESTIGATIONS
q MRI of the brain and orbits with contrast
q CT scanning of the brain and orbits with contrast :space-
occupying lesion [SOL], sinusitis, hyperpneumatized
sinuses, fibrous dysplasia) 
q Blood glucose level
q Blood pressure, cardiovascular examination
q Carotid Doppler ultrasound study
q Vitamin B-12 levels
q Venereal Disease Research Laboratory (VDRL)/Treponema
pallidum hemagglutination (TPHA) tests
q Antinuclear antibody levels
q Sarcoid examination
q Homocysteine levels
q Antiphospholipid antibodies
q Enzyme-linked immunosorbent assay (ELISA) for
toxoplasmosis, rubella, cytomegalovirus, herpes simplex
virus (TORCH panel)
Treatme nt
Medical Care
q No proven treatment exists for optic atrophy.
q Treatment initiated before the development of
optic atrophy can be helpful
q The role of intravenous steroids is proven in a
case of
qoptic neuritis
qarteritic anterior ischemic optic neuropathy.
q Idebenone, a quinone analog is on trial Leber
hereditary optic neuropathy to ameliorate the
net ATP synthesis
q Stem cell treatment :future treatment of
neuronal disorders.
q At present, the best defense is an early
PREVENTION
qEarly detection of inflammations
qSome doctors recommend vitamin C,
vitamin E, coenzyme Q10, or other
antioxidants,
qAvoid tobacco / alcohol
qAvoiding toxin exposure
qAvoid nutritional deficiency
qEarly diagnosis and prompt treatment of
compressive & toxic neuropathies
qGenetic counselling in hereditary disease
Vitamin, Water Soluble
Essential to normal metabolism and
DNA synthesis.

Cyanocobalamin (Nascobal)
qDeoxyadenosylcobalamin and
hydroxocobalamin are active forms of
vitamin B-12.
qVitamin B-12 synthesized by microbes
qRequired for healthy neuronal functions
and normal functions of rapidly growing
cells.
Dosin g
Adult
q Vitamin B-12 deficiency: 1000 mcg PO once a day
Pernicious anemia or other causes that decrease oral absorption,
administer
parenteralinjection of 30 mcg IM/Sc once a day for 5-10 d,
then 100-200 mcg IM/SC once a month
q Intranasal gel: 500 mcg in one nostril once a wk

Pediatric
q Oral administration:
<12 years: Not established
>12 years: Administer as in adults
q
Alternatively,
100 mcg IM/SC once a day for 10-15 d (total dose of 1-1.5 mg),
then 60-100 mcg IM/SC once or twice weekly for several month

q Intranasal gel: Not established

Fur th er O utpatie nt C are
qLow-vision aids for occupational
rehabilitation.

PROGNOSIS
qEarly and intensive treatment of cause
provide patients with near-normal vision
 Y O U
A N K
T H