 Optic atrophy refers to degeneration of

the optic nerve, which occurs as an end

result of any pathological process that
damages axons in the anterior visual
system, i.e. from retinal ganglion cells to
the lateral geniculate body.
 Primary optic atrophy
 Simple degeneration of nerve fibres
without any complicating process within
the eye. Eg syphilitic optic atrophy of
tabes dorsalis.
 Secondary optic atrophy
 Any pathological process which
produces optic neuritis or papilloedema.
 Ascending or anterograde optic atrophy
(Wallerian degeneration)
 It follows damage to ganglion cells or
nerve fibre layer due to diseases of the
retina or optic disc.
 Degeneration ascends from eyeball
towards geniculate body.
 Itproceeds from optic tract or chiasma or
posterior portion of the optic nerve
towards the optic disc.
 Damage beyond the laternal geniculate
nucleus does not cause optic atrophy as
second order neurons (axons of ganglion
cells )synapse in LGN.
 Primary (simple) optic atrophy
 Consecutive optic atrophy
 Glaucomatous optic atrophy
 Post neuritic optic atrophy
 Vascular (ischaemic) optic atrophy
 Degeneration is associated with
attempted but unsuccessful regeneration.
 Proliferation of astrocytes and glial
tissue.
 Degree of loss of nerve tissue and gliosis
 Consecutive and postneuritic optic
atrophy.
 The proliferating astrocytes arrange
themselves in longitudinal columns
replacing the nerve fibres (columnar
gliosis)
 Seen in primary optic atrophy.
 Progessive decrease in blood supply.
 Also called cavernous (schnabel’s) optic
atrophy.
 Features of glaucomatous and ischaemic
(vascular) optic atrophy.
 Primary optic atrophy- results from lesions
proximal to the optic disc.
 Multiple sclerosis
 Retrobulbar neuritis
 Leber’s hereditary optic atrophy
 Intracranical tumors pressing directly on the
anterior visual pathway(pituitary tumour)
 Traumatic severance or avulsion of the optic
nerve
 Toxic amblyopias
 Degeneration of the ganglion cells
secondary to degenerative or
inflammatory lesions of choroid or retina.
 Diffuse chorioretinitis
 Retinitis pigmentosa
 Pathological myopia
 CRAO
 It
is secondary optic atrophy which
develops as a sequelae to long standing
papilloedema or papillitis.
 Effect
of long standing raised intraocular
pressure.
 Resultsfrom conditions (other than
glaucoma) producing disc ischaemia.
 Giant cell arteritis
 Severe haemmorhage
 Severe anaemia
 Quinine poisoning
 Loss of vision – sudden or gradual onset
and partial or total depending upon the
degree of atrophy.
 Pupil is semi dilated and direct light
reflex is sluggish or absent. Swinging
flash light test depicts marcus gunn pupil
(RAPD).
 Visual field loss – Peripheral in systemic
infections
 Central in focal optic neuritis
 Eccentric when the nerve or tracts are
compressed.
 Pallorof the disc – not due to atrophy of
nerve fibres but due to loss of
vasculature.
 Decrease in number of small blood
vessels (Kastenbaum index)
•Chalky white optic disc
•Margins are sharply
outlined
•Slight recession of the
entire optic disc
•Lamina cribrosa is
clearly seen at the
bottom of the
physiological cup
•Major retinal vessels
and surrounding retina is
normal.
•Yellow waxy optic
disc
•Margins are not well
defined as in primary
•Vessels are
attenuated.
•Surrounding retina
pathology may be
seen.
•Optic disc appears dirty
white in colour.
•Edges are blurred due to
gliosis.
•Cup is obliterated.
•Lamina cribrosa is not
visible.
•Retinal vessels are
attenuated and
perivascular sheathing is
often present.
•Deep and wide
cupping.
•Nasal shift of the blood
vessels.
•Pallor of the optic
disc.
•Marked
attenuation of the
vessels.
 Non pathological pallor of optic disc –
axial myopia, infants, elderly with
sclerotic changes.
 Pathological causes – hypoplasia,
congenital pit, coloboma
 Field of vision: In partial optic atrophy, the
central vision is depressed with concentric
contraction of the visual field, according to the
cause.
 Pupil may be semidilated & direct light reflex is
very sluggish or absent. Swinging flash light test
depicts Marcus Gunn Pupil (RAPD).
 Fluorescein angiography of the optic nerve head.
 Visual Evoke response (VEK) is useful specially
in children.
 Total neurological evaluations like, X-ray of skull,
CT Scan, MRI of brain and optic nerve etc.
 Treatment is according to cause.
However, high dose of vitamin B1, B6, B12
is given.
 Hydroxocobalamine (vitamin-B12) 1000
µg is administered for atrophy due to
toxic optic neuritis.
 Characterised by bilateral optic atrophy.
 Dominant optic atrophy (Kjer type
optic atrophy)
 Inheritance is autosomal dominant (AD);
this is the most common hereditary optic
atrophy with an incidence of around 1 : 50
000; it is frequently due to a mutation in
the OPA1 gene which causes
mitochondrial dysfunction.
 Presentation is typically in childhood
with insidious visual loss. There is usually
a family history.
 Optic atrophy may be temporal or
diffuse. There may be enlargement of the
cup.
 Prognosis is variable (final VA 6/12–6/60)
with considerable differences within and
between families. Very slow progression
over decades is typical.
 Systemic abnormalities. Twenty per cent
develop sensorineural hearing loss.
 Inheritance is AR. Heterozygotes may
have mild features.
 Presentation is in early childhood with
reduced vision.
 Optic atrophy is diffuse.
 Prognosis is variable, with moderate to
severe visual loss and nystagmus.
 Systemic abnormalities include spastic
gait, ataxia and mental handicap.
 Wolfram syndrome is also referred to as
DIDMOAD (diabetes insipidus, diabetes mellitus,
optic atrophy and deafness).
 Inheritance – 3 genetic forms are recognized,
caused by a variety of mutations in WFS1, which
gives Wolfram syndrome 1, CISD2 – Wolfram
syndrome 2 – and probably a form caused by a
mitochondrial DNA mutation, with inheritance
being AR, AD or via the maternal mitochondrial
line.
 Presentation is usually between the ages of 5 and
21 years. Diabetes mellitus is typically the first
manifestation, followed by visual problems.
 Optic atrophy is diffuse and severe and
may be associated with disc cupping.
 Prognosis is typically poor (final VA is
<6/60).
 Systemic abnormalities (apart from
DIDMOAD) include anosmia, ataxia,
seizures, mental handicap, short stature,
endocrine abnormalities and elevated
CSF protein. Life expectancy is usually
substantially reduced.