Common Neuro-ophthalmological Disorder

1. Common Neuro-Ophthalmological
Disorder
Nilufa Akter
Optometrist (B.Optom,ICO,CMU)
Ispahani Islamia Eye Institute and Hospital

2. Neuro-Ophthalmological Disorder
 Neuro-ophthalmology, a subspecialty of both neurology
and ophthalmology, is the treatment of visual problems
that are related to the nervous system; that is, visual
problems that do not come from the eyes themselves.
Common Neuro Ophthalmological Disorders:
A. Optic nerve disease:
1. Congenital
2. Acquired
B. Neuro-Motility disorder
C. Pupillary Disorder(Anisocoria)
D. Compressive optic neuropathy (tumours)
E. Visual Field defect

3. A. Optic Nerve Disease (Optic Neuropathy)
Congenital (Abnormal
development)
1. Tilted disc
2. Optic disc pit
3.Optic disc drusen
4.Optic disc coloboma
5.Morning glory anomaly
6.Optic nerve hypoplasia
7.Myelinated nerve fibres
Acquired
1. Optic Atrophy
2. Inflammatory (optic
neuritis, Papilloedema,)
3. Vascular (ischaemic)
optic neuropathy
• Arteritic (Giant cell
arteritis)-AION or
NAION
4. Compressive optic
neuropathy (tumours)
5. Toxic Amblyopia
6. Toxic optic neuropathy,
e.g. methyl alcohol
7. Drug induced, e.g.
Ethambutol

4. 1.Tilted disc
-Usually bilateral
-Anomaly caused by an oblique entry of the optic
nerve into the globe.
Sign:
-Small, oval or D-shaped disc –directed
inferonasally
-Disc margin is indistinct where the retinal nerve
fibres are elevated
• Associated with high myopia, astigmatism,
• Field defect( superotemporal defect)
• Complication:-Uncommon, CNV and sensory
macular detachment

5. 2.Optic disc pit
Signs:
• Usually unilateral
• Round or oval pit of variable
size is located in the
inferotemporal aspect of the
disc
• Occasinally central
• VA normal in the absence of
complication
• Associated with serous macular
detachment
• Arcuate scotomata & other field
defects can occur

6. 3.Optic disc drusen
-OD drusen (Hyaline bodies) are composed of
hyaline –like calcific material within the subtance
of the optic nerve head.
Signs:
• Congenital, often familial, usually bilateral
• Optic cup absent
• May be buried or exposed on ONH, which
become more prominent with age
• Buried drusen are differential of ONH swelling
• Exposed drusen appear as multiple glistening
bodies
• Autofluorescence
Associated with
• Vitreous haemorrhage
• SRNVM
• Angoid streaks
• Retinitis pigmentosa
Fig1-Optic disc drusen
Fig2-BuriedOptic disc
drusen

7. 4. Optic disc coloboma
-Absence of any part of tissue in the
optic disc as a result of
incomplete closer of embryonic
fissure.
Signs:
-VA is often decreased
-Disc shows a discreate, focal,
glistering, white,bowl-shaped
excavation
• May produce various field defects
• Vision may be normal or reduced

8. 5. “Morning glory” syndrome
Signs:
• Unilateral coloboma (large disc)
with funnel shaped excavation
filled with white tuft of glial
tissue overlies the central portion
& surrounded by pigment
• “Spoke-like” origin of vessels
• Poor vision & associated with
retinal detachment

9. 6. Optic nerve hypoplasia
-Is characterized by a diminished
number of nerve fibres.
Signs:
• Small grey disc (ONH)
surrounded by yellow/pallid
halo of hypopigmentation
caused by concentric
chorioretinal atrophy.
• Poor vision & various field
defects & an RAPD if unilateral
• Associated with aniridia &
microphthalmos

10. 7.Myelinated nerve fibres
• Congenital
• White flame-shaped patches, usually adjacent
to ONH, producing an enlarged blindspot

11. Acquired Optic nerve disease
1.Optic Atrophy
2. Inflammatory (optic neuritis, Papilloedema,)
3. Vascular (ischaemic) optic neuropathy
• Arteritic (Giant cell arteritis)-AION or NAION
4. Compressive optic neuropathy (tumours)
5. Toxic Amblyopia
6. Toxic optic neuropathy, e.g. methyl alcohol
7. Nutritional, e.g. vitamin B12 deficiency
8.Drug induced, e.g. Ethambutol

12. 1. Optic Neuritis
-Inflammatory and demyelinating disorders of the optic nerve.
Etiology:
1. Idiopathic.
2. Hereditary optic neuritis (Leber’s disease)
3. Demyelinating disorders (common cause ):
multiple sclerosis, 70% cases , encephalitis
4. Parainfectious optic neuritis is associated with various viral
infections such as
measles, mumps, chickenpox, whooping cough and glandular fever.
It may also occur following immunization.
5. Infectious optic neuritis may be sinus related (with acute
ethmoiditis) or associated with cat scratch fever, syphilis, lyme
disease and meningitis in patients with AIDS.
6. Toxic optic neuritis (see toxic amblyopias).

13. • Symptoms of Optic neuritis:
- Sudden loss of vision with recovery over 6-12
weeks
- Painful eye movements
• Signs of Optic neuritis:
- Reduced visual acuity
- Impaired colour vision
- RAPD
- Visual field defect – variable
Ophthalmoscopic features
1. Disc edema/swollen and physiological cup
obliteration (2-3 D)
2. Retinal veins congested and tortous
3. Splinter hemorrhages and fine exudates may be
seen on disc
4. macular star formation – neuroretinits
5.. In retobulbar neuritis fundus appears normal ,
ocassionally temporal pallor of disc may be seen
Swollen disc

14. Types of optic neuritis
1. Papillitis
-Swollen hyperaemic ONH with
haemorrhages
2. Retrobulbar neuritis
-Normal ONH
3. Neuroretinitis
-Papillitis with macular star

15. Treatment of Optic neuritis
• Treat the underlying cause
• No effective treatment for idiopathic and hereditary
optic neuritis and that associated with demyelinating
disorders
• CORTICOSTEROID therapy may shorten the period of
visual loss, but will not influence the ultimate level of
visual recovery in patients with optic neuritis.
• Interferon therapy reduces the recurrences in patients
with MS , costly with unknown long term benefits

16. OPTIC NEURITIS TREATMENT TRIAL (ONTT)
1. Oral prednisolone therapy alone is C/I in treatment of
acute ON since it was associated with risk of new attacks
of ON.
2. IV methylprednisolone –
A pt presenting with AON should have brain MRI scan
- if it show lesions supportive of multiple sclerosis regardless
of severity of vision loss, each should receive immediate iv
methyl prednisolone
• IV methylprednisolone
-1g daily for 3 days followed by oral steroid as prednisone
(1mg/kg/day) for 11 days and then tapered over 4 days
• This therapy will delay conversion to clinical MS over the
next 2 years.

17. 2. Toxic Amblyopia
-conditions where in visual loss results from damage to the
optic nerve fibres due to the effects of exogenous
(commonly) or endogenous (rarely) poisons.
A few common varieties of toxic amblyopia are:
-Tobacco amblyopia,
-Ethyl alcohol amblyopia,
- Methyl alcohol amblyopia(Common)
-Quinine amblyopia,
-Ethambutol amblyopia (Drug induced)

18. 18
-May occur in nonsmoker but heavy drinkers suffering from
chronic gastritis
• Clinical picture:
– Characterized by gradually progressive impairment in the
central vision
– Patient complains of fogginess & difficulty in doing near
works
– V.F.-B/L centrocaecal scotoma with diffuse margins, defects
more for red than white
– Fundus: normal/slight temporal pallor
Methyl alcohol amblyopia

19. 19
• Ethambutol amblyopia
– Caused due to anti -tubercular drugs, Used in
doses of 15mg/kg per day,
– usually occurs in patient who have associated
alcoholism & diabetes
– fundus; sign of papillitis
Drug Induced toxic Amblyopia

20. Treatment of Toxic Amblypia
1. Gastric lavage to wash away the methyl alcohol immediately
2. Administration of alkali to overcome acidosis should be done
in early stages.
-Soda bicarb may be given orally or intravenously (500 ml of 5%
solution).
3. Ethyl alcohol. It should also be given in early
stages.. It should be given in small frequent
doses, 90 cc every 3 hours for 3 days.
4. Eliminative treatment by diaphoresis in the form
of peritoneal dialysis is also helpful by washing
the alcohol and formaldehyde from the system.
5. Prognosis is usually poor; death may occur due
to acute poisoning. Blindness often occurs in
those who survive.

21. 3. Anterior Ischaemic Optic Neuropathy (AION)
-Segmental or generalised infarction of anterior part of the optic
nerve.
Clinical Types:
1. NAION (Non-inflamatory cause) 2.AAION(Giant cell arteritis)
Etiology:
1. Idiopathic AION: most common entity, atherosclerotic changes
in the vessels.
2. Arteritic AION:
-association with giant cell arteritis.
3. AION due to miscellaneous causes:
- severe anaemia, collagen vascular disorders, following massive
haemorrhage, papilloedema, migraine and malignant hypertension.

22. Non-Arteritic AION (Non-inflamatory cause)
-is caused by occlusion of the short posterior ciliary arteries
resulting in partial or total infarction of the optic nerve head.
Risk factors:
-Age group affected usually is 45-65 yrs
1. Structural crowding of the ONH
2. Hypertention
3. DM
4. Hypercholesterolema
5. Sudden Hypotensive event
6. Sleep apnoea syndrome
7. Cataract Sx
8. Erectile dysfunction

23. Symptoms:
1.Sudden onset of visual loss
2.Painless , Monocular
3. Visual loss is frequently discovered on awakening
4. Nocturnal hypotention
Signs:
1.VA-often better than 20/100
2. VF- Typically Altitudinal defect , but central, paracentral,
quadantric, and arcuate defect may also be seen.
3.Color vision-may be severely impaired when VA is good
4.Pupil-RAPD+
5.Ophthalmic Exam findings:
-Diffuse or sectorial hyperaemic disc swelling
associated with few peripapillary splintershaped
haemorrhages.
6. Normal ESR and CRP

24. Treatment of NAAION
• No definitive treatment.
-Treat underlying vascular disorders (Hypertension ,
Diabetes,Hyperlipidemia
• Aspirin is effective in reducing systemic vascular
events.
• Aspirin does not reduce risk of involvement of the
fellow eye.

25. Arteritic Anterior Ischaemic Optic
Neuropathy(AAION)
-Is caused by giant cell arteritis.
• Giant cell arteritis causes occlusion of posterior
ciliary arteries of optic nerve
• Untreated can cause sudden bilateral blindness

26. Giant cell arteritis
• Occlusive vasculitis of ophthalmic artery and
its branches
• Elderly
• Symptoms
– Bilateral irreversible visual loss if untreated
– Temporal tenderness
– Jaw claudication
– Scalp tenderness
– Headache
– Constitutional symptoms, eg weight loss, anorexia
• Signs
– Variable visual acuity but often severe vision loss
– Pale optic disc with diffuse edema and
haemorrhages
– later optic atrophy
– Thickened non pulsatile temporal artery

27. Giant cell arteritis
Investigations (urgent)
• ESR raised > 60 mm/hr (normal = half the age for men and
half the age plus 10 for women)
• C reactive protein (CRP) raised
• Temporal artery biopsy - histology confirms diagnosis
Treatment
• Aim is to prevent blindness in the fellow eye
• Initial treatment is with high dose intravenous
methylprednisolone then oral prednisolone 60 mg daily.Taper
oral steroids gradually

28. 4.Papilloedema
-Bilateral optic disc swelling due to raised intracranial
pressure(ICP)
Causes:
-Intracranial space occupying lesions, e.g. tumour, Haemotoma
-Any lesion causing hydrocephalus in adults e.g.. Meningitis and
subarachnoid haemorrhage
- Venous obstruction caused by thrombosis in the venous sinuses
- Benign intra cranial hypertension

29. Symptoms:
Visual acuity usually normal
- May be associated with transient
visual loss
As atrophy sets in-
– Complete blindness
– Pupils are large and immobile
– Headache which is worse in a
recumbent position
– Nausea and vomiting
– Diplopia (Non specific paresis of
the sixth nerve)
Clinical features
- Enlargement of the blind spot
- Swollen discs
- Optic atrophy if chronic
Cotton wool spots
Engorged retinal veins
Flame shaped
haemorrhages
Acute Papilloedema
Pale waxy disc
Chronic Papilloedema

30. Investigations
• B-scan ultrasonography
• CT scan of brain
• MRI of Brain
• Venography
• Lumbar puncture

31. Treatment of Papilloedema
• According to cause
• Neuro-surgical consultation
• Method of reducing ICP
-Mannitol infusion
-CSF withdrawal
-Control hyperventilation
-Decompressive craniotomy
-Removal of mass lesion if present
-Steriods
Shunt technique
-Ventriculoperitoneal shunt
-Lumboperitoneal shunt
-Ventriculoarterial shunt

32. 5.Optic atrophy
- Caused by damage to the nerve fibres at any point
between the ganglion cells of the retina and
lateral geniculate body
- Irreversible loss of vision
Classified as:
1.Primary optic atrophy
2. Secondary optic atrophy
3. Conscequtive optic atrophy
4.Glaucomatous optic atrophy

33. Causes of Optic Atrophy
1. Retinal
Central retinal artery occlusion
Retinitis pigmentosa
2. Optic nerve
Anterior Ischaemic optic neuropathy
Optic neuritis
Glaucoma
Chronic papilloedema
Toxic e.g. Methyl alcohol, ethyl alcohol, tobacco and ethambutol.
Tumour e.g. optic nerve glioma or meningioma
Trauma
Leber’s Hereditary optic neuropathy
3. Chiasm
Any cause of chiasmal compression e.g. pituitary adenoma,
craniopharyngioma

34. Primary optic atrophy
• When the atrophy is due to disease proximal to the disc so
there is no evidence of previous inflammation.
Causes:
-Multiple sclerosis
-Space occupying lesions
-Syphilis
Clinical features :
-Disc is white
-Margins well defined
-Lamina cribrosa seen
-Retina looks normal
-Cupping is shallow
Pale disc and thinned retinal vessels

35. Secondary optic atrophy
• Also called post neuritic optic atrophy
Clinical features:
• Dirty gray colour of the disc
• Margins may be ill defined
• Cup may be obliterated
• Retina may show some evidence of disease

36. Consecutive optic atrophy
• Is a consequence of retinal or choroidal
disease.( Retinitis pigmentosa, Choroidiris,)
• Disc shows a waxy pallor
• Edges are less well defined
• Retinal vessels are contracted

37. Treatment of Optic Atrophy
- Prevention
• Nutrition supplement
• Weaning away from alcohol, smoking
• Rehabilitation

38. Slowly progressive loss of vision.
Can by uni-lateral or bilateral.
Common tumors are:
1.Optic glioma
2. Meningioma of optic nerve
3. Papillary angioma
4. Papillary melanoma
B. Tumor of optic nerve
(Compressive Optic Neuropathy)

39. Papillary melanoma Papillary angioma
Tumor of optic nerve

40. Tumor of optic nerve
Optic glioma

41. C. Neuro-Motility Disorder
Related anatomy and physiology
Six muscles control eye movements:
1. Superior rectus
2. Medial rectus
3. Inferior rectus
4. Inferior oblique
(All the above muscles innervated by the 3rd CN)
5. Superior oblique - innervated by 4th CN
6. Lateral rectus - innervated by the 6th CN
• The oblique muscles move the eye up (Inferior oblique) or down (superior
oblique) when it is turned in
• The superior and inferior recti move the eye up (SR) and down (IR)
• The lateral and medial recti abduct (move out) and adduct (move in) the eye
respectively
• Eye movements are examined in the six different directions of gaze
representing individual muscle action

42. Third nerve palsy
Clinical features:
• Ptosis
• Eye down and out (Exodeviation)
• Limited elevation, adduction and depression
• Pupil sparing or pupil involving (pupil fixed and
dilated)
• Pupil involving third nerve palsy = PCA aneurysm
until proven otherwise. Life threatening
neurosurgical emergency

43. Oculomotor Nerve Palsy

44. Pupil-involving Third Nerve Palsy
UrgentMRI/MRA or MRI/CTA

45. Causes of 3rd CN Palsy:
- Idiopathic
- Microvascular disease (diabetes, hypertension)
- Posterior communicating artery aneurysm
- Head trauma
- Tumours

46. 6th Cranial Nerve Palsy
Clinical features:
• Esotropia (convergent squint)
in the primary position, due
to unopposed action of the
medial rectus muscle
• Marked limitation of
abduction
• Horizontal diplopia (double
vision)

47. Causes of 6th cranial nerve:
• Microvascular disease (diabetes, hypertension)
• Raised intracranial pressure
• Acoustic neuroma (cerebellopontine angle tumour)
• Nasopharyngeal tumours
• Trauma (basal skull fracture)

48. 4th Cranial Nerve Palsy
Clinical features:
• Affected eye is hypertropic, i.e.
at higher position than the
unaffected eye
• Hypertropia increases on tilting
the head to the ipsilateral
shoulder
• Vertical diplopia
• Patient adopts a compensatory
head tilt to the opposite side to
prevent diplopia

49. Causes of 4th Cranial Nerve Palsy:
• Congenital – may not develop until adult life
• Acquired
– Trauma
– Microvascular disease
– Aneurysms and tumours rare

50. D.Visual field defects

51. E. Pupillary disorder:Anisocoria
Pupil disorders:
1. Microcoria(Small pupil)
2.Megalocoria (Large pupil)
3.Anisocoria(Difference between
the size of two pupil).
4. Polycoria(Multiple pupil)
5.Correctopia (Eccentric
displacement of pupil)

52. Pupil anomalies
1-Argyll Robertson pupil
2-Reverse argyll robertson pupil
3-Adie’s tonic pupil
4-Horner’s syndrome
5-(Marcus gunn pupil) RAPD
6-Amaurotic pupil
7-Hutchinson’s pupil
8-Paradoxical pupil
9-Hippus