UNDERSTANDING NEUROLOGICAL EYE DISORDER FOR OPTOMETRY STUDENTS

1. Neurologycal eye disease
Simi Afroz
Assistant professor
Department of Optometry
School of medical and allied sciences
G D GOENKA UNIVERSITY

2. ● Optic nerve
● Optic chiasma
● Optic tract
● Lateral geniculate body
● Optic radiations
● Visual cortex
Anatomy of visual pathway

3. Optic
nerve
Optic
chiasma
Optic tract
Lateral
geniculate
body
Optic
radiation
Visual
cortex

5. Visual pathway
Rod and cones
Optic nerve
Optic chiasma
Optic tract
Lateral
geniculate body
Optic radiation
Visual cortex

6. Lesions of visual pathway

7. Pupillary reflex
Pupillary
reflex
Afferent Efferent

13. RAPD
● Positive (defect in afferent)
● Reflects retina and optic nerve
disease.
● Checked by swing light test.
● Interpretation:
○ There is equal constriction of
pupil irrespective of which eye is
stimulated by light.
○ there is less pupil constriction in
the eye with the retinal or optic
nerve disease

15. ● Include ischaemic optic neuropathy
● Optic neuritis
● Optic nerve compression (orbital tumours or dysthyroid
eye disease)
● Trauma
● Asymmetric glaucoma.
● Less common such causes include infective, infiltrative,
carcinomatous, or radiation optic neuropathy
Causes of RAPD

16. ● Microcoria
● Megalocoria
● Anisocoria
● Polycoria
● Correctopia
Anomalies of pupil

17. ● Argyll Robertson pupil
● Reverse argryll robertson pupil 3-Adie’s tonic pupil
● 4-Horner’s syndrome
● 5-(Marcus gunn pupil) RAPD
● Amaurotic pupil
● Hutchinson’s pupil
● 8-Paradoxical pupil
● Hippus
Anomalies of pupil

18. ● Hallmark of neurosyphilis ,No reaction to light but brisk response to near ,Pupil is small &
irregular in shape
Argyll Robertson pupil

19. ● In this accommodation and
reflex on the pupil is absent
● causes;- Diptheria, tumors
at carpora quadrigemina
Reverse argryll robertson pupil

20. ● occurs mainly due lesion in the ciliary ganglions.
● This lesion is thought to be caused by denervation of the post ganglionic nerve
supply to the sphincter and ciliary muscle
● affected eye is dilated and reacts poorly to light (poor direct and consensual
response)
Adie’s tonic pupil

21. ● Horner's syndrome is due to damage or blockage of the path of the sympathetic nerve which
travels through the neck and brain from the affected eye.
● total or partial interruption of the sympathetic pathway.
● It consist of anisocoria miosis, ptosis & enophthalmus and decrease in the facial sweating on the
affected side.
● In congenital form there may be associated heterochromia of iris .
Horner’s syndrome

22. ● It is due to the conduction defect of the optic nerve- particularly the
afferent fibers are defective
● In this, both the pupils will dilate a little when the abnormal eye is
stimulated.
● The affected eye constricts less & redilates more than a normal eye.
The effected eye has a greater consensual response coming from
the normal eye than direct.
(Marcus gunn pupil) RAPD

23. ● A pupil in an eye that is blind because of ocular or optic nerve disease and
that contracts in response
● to light only when the normal eye is stimulated with light white pupillary reflex
indicates abnormal red reflex indicates normal.
➢Causes:
➢congenital cataract,
➢ retinoblastoma,
➢ ROP,
➢ toxocaris
Amaurotic pupil (leucocoria)

24. ● Irregular rythamatic visible pupillary oscillations 2mm / more in amplitude irregular dilating &
constricting movements are observed Also called as pupillary athestosis
Normal pupils, particularly those of young people, sometimes show slight fluctuation in size (of
less than 1 mm) even when the light shining into the eye is constant. This is called hippus and
it can make eliciting a RA P D more difficult.
Hippus

25. Optic nerve disease

26. ● Swelling, oedema or accumulation of excess fluid in and around the
nerve
● Ischemia, by affecting the blood supply
● Inflammation within or around the nerve
● Degeneration or atrophy of the axons by direct compression or toxic
effects
● Direct injury by penetrating trauma or indirect injury by concussional
and rotational forces
● Abnormal embryogenesis in utero leading to congenital anomalies
Etiopathogenesis of optic nerve disease

27. ● Whether one or both eyes are affected
● The pattern of visual field loss
● The appearance of the optic nerve head or optic disc
Differential diagnosis

28. Signs of optic nerve disfunction
Reduced VA
RAPD
Dichromatopsia
Reduced brightness sensitivity
Reduced contrast sensitivity
Visual field defect (vertical)

29. Idiopathic
Demyelinating Disorders
• Isolated
• Associated with multiple sclerosis
• Neuromyelitis optica
Associated with Infections
• Local
• Endophthalmitis
• Orbital cellulitis
• Sinusitis
• Contiguous spread from meninges, brain, base of skull
• Systemic
• Viral—influenza, measles, mumps, chickenpox, herpes zoster, infectious mononucleosis, cytomegalovirus
• Bacterial—tuberculosis, syphilis (perineuritis), Lyme disease (borreliosis)
• Fungal—cryptococcosis, histoplasmosis
• Protozoal—toxoplasmosis, malaria (Plasmodium), pneumonia (Pneumocystis carinii)
• Parasitic—cysticercosis
Immune-Mediated Disorders
• Local
• Uveitis
• Sympathetic ophthalmitis
• Systemic
• Sarcoidosis
• Wegener granulomatosis
• Acute disseminated encephalomyelitis
Metabolic Disorders
• Diabetes
• Anaemia
Classification of optic neuropathy

30. ● It is defined as swelling of the optic disc.
● Causes:
● Increased ICP
● Trauma / head injury
● Tumor (brain, orbital)
● Sub Dural or sub arachnoid hemorrhage
● Meningitis
● Encephalitis
Papilloedema

31. Signs & Symptoms
Symptoms
○ Headache
○ Nausea & vomiting Mild
decrease in vision
○ Paralysis of extra ocular muscles.
Signs:
○ APD or RAPD
○ Optic disc swelling
Hyperemia Blurred
margins
○ Loss of venous pulsations
Hemorrhages on the disc margin.
Investigation
• Ophthalmoscopy
• Pupillary reflex
• CT Brain
• Visual Fields
• ICT
Treatment:
• Directed towards the cause.

32. ● Optic neuritis” is an inflammation of the optic nerve.
● Causes:
○ Malnutrition. E.g. vit B complex deficiency.
○ Infections such as viruses (especially in children), measles, meningitis,
syphilis, sinusitis, tuberculosis, and human immunodeficiency virus (HIV)
○ Tumours
○ Chemicals or drugs such as tobacco, lead, methyl alcohol, ethambutol,
chloroquine, arsenic, and certain antibiotic
○ Certain autoimmune diseases such as multiple sclerosis
○ Intra ocular inflammation (uveitis)
○ Rare causes include diabetes, hypertension, anemia, Grave's disease, bee
stings, vaccinations, and injuries. However, the cause of optic neuritis is
often unknown.
Optic neuritis
Optic
neuritis
Papillates
Retro bulbar
neuritis

33. It is the inflammation of intraocular part of optic nerve ( optic disc).
● Symptoms
○ Loss of vision
○ Complete or partial blindness
○ Loss of some or all color vision (red green)
○ Pain behind the eye.
○ Painful eye movements
○ Increased dark adaptation time
○ Disc = hyperemic, Margins = blurred., Edematous
○ Ill sustained constriction to light
1. Papillitis

34. • It the inflammation of the posterior part of the optic nerve, behind the globe.
• There is a common saying:
‘PATIENT SEES NOTHING & THE DOCTOR SEES NOTHING’
Signs and symptoms:
• Loss of vision
• Pain behind the eye.
• RAPD
• Complete recovery may take place, but usually optic atrophy.
● Diagnostic test:
Ophthalmoscopy
Pupillary reflex
Visual field
Color vision
CT Scan Brain
MRI Brain
● Treatment: usually resolves by its own if not-Systemic I/V steroids—3 days
2. Retrobulbar neuritis

35. Fundus photographs and visual fields of patient with ON.
(a) Fundus photographs showing mild swelling of both optic discs (left, right eye;
right, left eye).
(b) Visual fields showing bilateral central scotoma and blind spot enlargement
(left, left eye; right, right eye).

36. Observed clinically as pallor of the disc:
•PRIMARY - disc pale & white, margins are distinct
•SECONDARY - disc pale & white, margins indistinct
such cases result from previous oedema or inflammation of
the nerve head.
Cause are same as optic neuritis.
Optic atrophy

37. ● Signs and symptoms:
○ Loss of vision
○ Loss of brightness & color discrimination Diminished or absent Pupillary reflex
○ V. F. defect
○ Blindness ( end result)
● Treatment:
Optic nerve can’t regenerate, so visual loss is irreversible.

38. ● Intrinsic - e.g. gliomas, melanocytomas and meningiomas originating
from nerve tissue.
● Extrinsic e.g. meningiomas of sphenoidal ridge or olfactory groove,
pituitary adenomas and some metastatic tumors.
Tumors

39. Optic nerve glioma
• Optic nerve glioma (also known as optic pathway glioma) is the
most common primary neoplasm of the optic nerve.
Signs & symptoms:
•
•
•
•
Problem in ocular motility
Proptosis
Defective vision if they affect the ON conductive system
Nystagmus
Diagnosis:
•
•
CT
MRI

40. a) Axial orbit CT scan shows enhancing glioma involving the left intraorbital optic nerve (b) Fundus
photograph Image shows marked swelling of the left optic disc. (c) Patient was treated with an excision of the
orbital optic glioma of the left eye. Photograph shows the excised specimen. (d) Fundus photograph 3 months
after the optic nerve excision. Image shows optic disc pallor and tractional retinal detachment.

41. • Optic nerve hypoplasia (small+ poorly developed disc)
• Optic nerve pit (small deep hole in centers of optic disc)
• Optic disc coloboma
Congenital defects

42. Colobomas
• Incomplete closure of fetal cleft thus appearing inferiorly. May
be associated with choroidal /iris coloboma.
• Optic nerve coloboma (medical condition): A hole in the eye
structure called the optic nerve which is responsible for sending
visual information from the eye to the brain. Severity of
symptoms is determined by the size of the defect.
•
•
•
Impaired vision
V.F. defect
Field defect corresponds to the area of projection of the missing
fibers.

43. Ischemic optic neuropathy

44. Ischemic
optic
neuropathy
Anterior
Arteritic Non arteritic
Posterior
Arteritic
Non-
arteritic
Post
operative

46.  Blood flow
 Autoregulation
 Arterial blood pressure
 Intraocular pressure
Opticnerveischemiamostfrequentlyoccursat the optic nerve head, where
structural crowding of nerve fibers and reduction of the vascularsupply
may combine to impair perfusion to acritical degree and produce optic
discedema.Themostcommonsuchsyndromeistermed anterior ischemic
opticneuropathy(AION).
FACTORS INFLUENCING THE BLOOD FLOW IN THE
OPTIC NERVE HEAD

47. ● Pathogenesis:
 Due to transient non-perfusion
or hypoperfusion of the ONH
circulation.
 Due to embolic lesions of
the arteries/arterioles
feeding the ONH
Anterior ischemic optic neuropathy
Optic nerve
ischaemia /
hypoxia
Axoplasmic flow
stasis in the
optic nerve
fibers
Axonal swelling
Asymtopmatic
optic disc
edema
compression of
the intervening
capillaries

48. CHARACTERISTIC ARTERITIC NON-ARTERITIC
AGE Over 70 yrs 60-70 yrs
SEX Females no
PRECEDING SYSTEMIC
FEATURES
Jaw claudication,headache,scalp
tenderness
no
PRECEDING OCULAR SYMPTOMS Highly suggestive Rare
VISUAL LOSS Highly suggestive 20% cases
PAIN Common Rare
SECOND EYE
INVOLVEMENT
75% within days or weeks 40% in mths or yrs
DISC APPEARANCE Chalky white swollen disc Sectoral or pallid
FFA Choroidal filling defect Normal
ESR Raised Normal
CRP Raised Normal
TEMPORAL ART
BIOPSY
Positive Negative
RESPONSE TO definite Nil

49.  Sudden painless deterioration of vision
 Usually discovered on waking in the morning
 Visual field defects
 Photophobia
 Normal visual acuity doesn’t rule out NA-ION
 Might have improvement up to 6 months
 RAPD
 Acute: disc oedema ; Chronic: disc pallor
 Simultaneous bilateral onset of NA-AION is extremely rare, except in patients who
develop sudden, severe arterial hypotension, e.g. during haemodialysis or surgical
shock.
Non arteritic anterior ischemic optic neuropathy
Treatment :
 Optic nerve sheath decompression
 Aspirin
 Systemic corticosteroid therapy
 Intravitreal triamcinolone
 Intravitreal bevacizumab
 Reduction risk factors

50. NA-AION

51. ● Signs and symptoms:
 Amaurosis fugax
 Visual loss
 Visual field defects
 GCA symptoms
 Extraocular motility disorders
 RAPD
 Optic disc changes. ODE, compared to NA-AION, usually has a diagnostic
appearance in A-AION, i.e. chalky white colour.
 High ESR and CRP
Arteritic ischemic anterior optic neuropathy
 Management of A-AION is actually management
of GCA
 Prime medical emergency
 STEROID!

52. AAION

53. ● Signs and symptoms:
 Sudden loss of vision may involve central vision.
 Optic nerve related visual field defects
 RAPD
 Normal fundus
 No other ocular, orbital or neurological abnormality to explain the
visual loss
 Development of optic disc pallor, usually within 6–8 weeks
 Surgical PION: dramatic visual loss noticed as soon as the patient is
alert enough after a major surgical procedure
Posterior ischaemic optic neuropathy
Management:
 Similar to AION
 Prognosis:
- NA PION: good with
steroid
- A PION: steroid could
prevent further visual
deterioration
- Surgical PION: poor
prognosis

54. • Within hours to days
• Mainly spinal.cardiac bypass sx
PERIOPERATIVE
• Associated with GCA
• Poor visual prognosis
ARTERITIC
• Same risk factor of NAION
• Not associated with crowed optic
disc
NONARTERITIC
TYPES PION

55. ● LHON is caused by genetic mutations in the
mitochondrial DNA.
● X-linked disease.
● PATHOGENESIS:
Leber hereditary optic neuropathy
Impair
glutamate
transport
Increase
reactive
oxygen
species
production.
leads to
retinal
ganglion cell
through
apoptosis
Atrophy and
demyelination
is
subsequently
noted in the
optic nerves,
chiasm and
tracts.

56. ● Signs and symptoms:
○ Typically affects young males.
○ Typically begins as a unilateral progressive optic
neuropathy with sequential involvement of the
fellow eye months to years later.
● Management:
○ Use of antioxidant supplements.
○ Gene therapy trials are currently underway

57. Herniation of a dysplastic
retina into the subarachnoid
space through a defect in the
lamina cribrosa at the pit has
also been described.
Optic disc pit

58. Toxic optic neuropathy
Tobacco induced
Ethyl or methyl
alcohol
Quinine
neuropathy.
Ethambutol Chloroquine Amiodarone

59. Thank you