Optic Atrophy

Dr. Amira Elagamy

Definition

 It means degeneration of the axons of the optic nerve fibers and increase
in interstitial connective tissue.

 Types
1. Primary optic atrophy
2. Secondary optic atrophy
3. Consecutive optic atrophy
4. Glaucomatous optic atrophy
 Primary Optic Atrophy
 It is caused by lesions primarily affecting the optic nerve fibers without an antecedent
papilledema.
 Causes
 Central nervous system diseases such as Tabes dorsalis (demyelination by advanced
syphilis infection (tertiary syphilis)), disseminated sclerosis.
 Severe blood loss, cardiac arrest, cardiac surgery.
 Toxic e.g. Tobacco.
 Pressure on the optic nerve by tumors, aneurysm, or space occupying lesion.
Ophthalmoscopic Picture

 The whole disc is chalky white in color.

 The edges of the disc are sharply defined.
 The lamina cribrosa is well seen.
 There is shallow saucer-shaped atrophic cup.
 The retinal blood vessels are not altered.
 The surrounding retina looks normal.
 Secondary Optic Atrophy
 Causes
 Papillitis
 Papilloedema

 Ophthalmoscopic Picture (Post-neuritic)

 The disc is dirty grey white in color.
 The edges of the disc are irregular & ill defined.
 The optic cup is filled with glial tissues.
 The lamina cribrosa is obscured (residual fibrosis resulting from inflammation).
 The retinal veins are engorged and tortuous, and retinal arteries are constricted, both
are sheathed.
 Pigmentary changes at the edges of the disc.
 Consecutive (Yellow) Optic Atrophy

 Causes
 CRA occlusion
 Retinitis pigmentosa
 Chorio-retinal degeneration
 Choroiditis, chorioretinitis
 Ischemic optic neuropathy

 Ophthalmoscopic Picture
 Disc is waxy yellow in color.
 Edges of the disc are well defined.
 Retinal blood vessels are markedly attenuated.
 The retina shows the evidence of the causative disease.
Dr. Amira El-Agamy
Assistant Professor of Ophthalmology
 Glaucomatous Optic Atrophy
 Ophthalmoscopic Picture
 Disc is white or bluish white in color.
 Edges of the disc are overhanging.
 Optic cup is enlarged.
 Vessels are displaced nasally, and broken off.
 Retinal arteries show pulsations.
C/D: 0.4 C/D: 0.5

C/D: 0.6 C/D: 0.7

C/D: 0.8 C/D: 0.9

Post-Glaucomatous Optic Atrophy

 Investigations of Optic Atrophy
 Ophthalmoscopy.
 VF (visual Field).
 MRI.
 Fluorescein Angiography (FA) shows loss of the vessels in superficial and
deep network in optic atrophy.
 Pupil Constriction (Miosis)
1. Physiological causes 2. Drugs
 Light reflex and accommodation  Local miotics (pilocarpine,
reflex eserine, Di-isopropyl
 During sleep fluorophosphates).
 Senile miosis  Opium, morphine and parathion
 Third stage of anesthesia poisoning.
3. Nervous
4. Causes
Local Causes

 Irritative stage of extradural hemorrhage (Hutchinson’s pupil).
Acute iritis

 Horner's syndrome (ptosis, miosis, enophthalmos and anhidrosis).
Hypermetropia
 Argyll-Robertson pupil.
 Traumatic miosis
 Pontine hemorrhage.
 Puncture of anterior chamber
 Argyll-Robertson Pupil

 It is a small irregular pupil (mostly bilateral) which does not react to light but reacts
to accommodation (light-near dissociation). The site of lesion is in the
intercalated neuron near the aqueduct. A lesion in this locality interrupts the
pupillary light reflex and spares the accommodation reflex.The commonest cause is
neuro-syphilis. Less common causes are diabetes and cerebral tumors.
 Hutchinson’s Pupil
 It is a clinical sign in which the pupil on the side of an intracranial mass lesion is
dilated and unreactive to light, due to compression of the oculomotor nerve on that
side. The pupillomotor fibers pass through the periphery of the oculomotor nerve,
and hence are the first to be affected in case of compression of the nerve.
Stages

 Stage 1: The parasympathetic fibers on the side of injury are irritated, leading to
constriction of pupil on that side.
 Stage 2: The parasympathetic fibers on the side of injury are paralyzed, leading to
dilatation of pupil. The fibers on the opposite oculomotor nerve are irritated,
leading to constriction on opposite side.
 Stage 3: The parasympathetic fibers on both sides are paralyzed, leading to
bilateral pupillary dilatation. Pupils become fixed. This indicates grave
prognosis.
 Sympathetic Supply
 First Order: Posterior Hypothalamus
to Ciliospinal centre of Budge (C8-T2)
(Uncrossed in Brainstem).
 Second Order: Ciliospinal centre of
Budge to Superior Cervical Ganglion.
 Third Order: Superior Cervical
Ganglion to dilator pupillae muscle
(Close to ICA and joins V1 intra-
cranially).
 Horner Syndrome
Causes of Horner Syndrome

It is a combination of signs and symptoms

caused by the disruption of the sympathetic
pathway from the brain to the face and eye on
one side of the body. 
 First-order neuron disorder: Central
lesions that involve the hypothalamo-spinal
tract.

 Second-order neuron disorder:

Preganglionic lesions (e.g. compression of the
sympathetic chain by a lung tumor).

 Third-order neuron disorder:

Postganglionic lesions at the level of the internal
carotid artery (e.g. a tumor in the cavernous
sinus).
Classic Signs

 Partial ptosis (drooping of the upper eyelid from loss of sympathetic innervation to the
superior tarsal muscle, also known as Muller’s muscle).
 Upside-down ptosis (slight elevation of the lower lid).
 Anhidrosis (decreased sweating on the affected side of the face).
 Miosis (small pupils) (the degree of anisocoria is greater in dim than in bright light).

 Enophthaloms (the impression that the eye is sunken).

 Loss of ciliospinal reflex.
 Sometimes there is flushing on the affected side of the face due to dilation of blood
vessels under the skin.
 The pupil's light reflex is maintained as this is controlled via the parasympathetic
nervous system.
 In children, Horner's syndrome sometimes leads to heterochromia which is a
difference in eye color between the two eyes. This happens because a lack of
sympathetic stimulation in childhood interferes with melanin pigmentation of the
melanocytes in the superficial stroma of the iris.
 Ciliospinal Reflex
 The (pupillary-skin reflex) consists of dilation of the ipsilateral pupil in response to
pain applied to the neck, face, and upper trunk.
 If the right side of the neck is subjected to a painful stimulus, the right pupil dilates
(increases in size 1-2 mm from baseline).
 This reflex is absent in Horner’s syndrome and lesions involving the cervical
sympathetic fibers
 Pupil Dilatation (Mydriasis)
1. Physiological Causes 2. Drugs
 Withdrawal of light  Local mydriatics (atropine,
 Second and fourth stage of homatropine, hyoscine, cocaine)
anesthesia  Datura poisoning
 Excitement, fear and anger
3. Local Causes 4. Nervous Causes
 High myopia  All causes of coma except pontine

 Acute congestive glaucoma hemorrhage, morphine and parathion

poisoning.
 Traumatic mydriasis
 Paralytic stage of extradural
 Optic atrophy and retinal
hemorrhage (Hutchinson’s pupil).
degeneration
 Third nerve paralysis.
 Central retinal artery occlusion
 Neuro-ophthalmic Field Changes of Brain Lesions

 Optic Nerve Lesions

 Monocular field defects on the same side, normal field on the other side
 Optic Chiasm
Optic nerve fibers passing through the chiasm are arranged as follows:
 Macular fibers decussate throughout the chiasm.

 Lower nasal fibers traverse the chiasma low and anteriorly. They are therefore most
vulnerable to damage from expanding pituitary lesions, so that the upper temporal quadrants
of the visual fields are involved first.
 Upper nasal fibers traverse the chiasm high and posteriorly and therefore are involved first by
lesions coming from above the chiasm (craniopharyngiomas). So, if the lower temporal
quadrants of the visual fields are affected more than the upper, a pituitary adenoma is unlikely.
 Optic Tract Lesions
Contralateral homonymous hemianopia
 Optic Radiation Lesions
 Temporal lobe lesions can lead to
contralateral homonymous superior
quadrantianopia (pie in the sky) with
hemiparesis and receptive dysphasia
(difficulty in comprehension) if the
dominant hemisphere is involved.
 Parietal lobe lesions can lead to
contralateral homonymous inferior
quadrantanopia (pie in the floor) with Left-
right disorientation, Finger agnosia,
Acalculia, Agraphia if the dominant
hemisphere is involved.
 Occipital Cortex Lesions
 Contralateral homonymous hemianopia with macular sparing.
 Causes of macular sparing:
 Dual blood supply of the macular area.
 Bilateral representation of the macular fibers.
 Large area of macular representation in the occipital cortex.