The document discusses the neurological examination of vision and the ocular system. It describes the dual organization of the optic system with two afferent cranial nerves (optic and trigeminal), two sets of eye muscles, two areas of the retina, and two pathways in the optic nerve and tract. It also summarizes examination techniques for vision, eye movements, pupils, and the optic disc. Common causes of eye movement disorders and multiple cranial nerve palsies involving the eyes are outlined including cavernous sinus syndrome, superior orbital fissure syndrome, and Tolosa-Hunt syndrome.

1. Neurological examination
series
1- How to examine vision and ocular system?
By / Lobna Ahmed

2. Dual organization of the optic system
2 afferent CN
• Optic
• Trigeminal
2 set of
muscles
• Intra-ocular
• Extra-ocular
2 areas of the
retina
• Central
• peripheral
2 pathways in
ON
• Nasal
• Temporal
2 pathways in
optic tract
• Visual
• Non visual
2 banks on the
calcarine cortex
• Upper
• lower
ON
• Vision
• pupilloconstriction
Trigeminal
• General sensation (ocular pain –
tearing reflex-corneal reflex-
proprioception of EOM).
DeMyer’s neurological examination 6th edition

3. Optic nerve
Optic disc: posterior ciliary
arterioles, pial arteriole plexus, and
choroid.
Intra-orbital: perforating branches
of the ophthalmic artery.
Intracanalicular and intracranial:
ophthalmic, internal carotid.
The optic disc
(intraocular) is
the only
structure of the
entire tract that
has not myelin.

4. 2 afferent CN
• Optic
• Trigeminal
2 motor
systems
• Intra-ocular
• Extra-ocular
2 areas of the
retina
• Central
• peripheral
2 pathways in
ON
• Nasal
• temporal
2 pathways in
optic tract
• Visual
• Non visual
2 banks on the
calcarine cortex
• Upper
• lower
DeMyer’s neurological examination 6th edition

5. DeMyer’s neurological examination 6th edition

8. 2 afferent CN
• Optic
• Trigeminal
2 set of
muscles
• Intra-ocular
• Extra-ocular
2 areas of the
retina
• Central
• peripheral
2 pathways in
ON
• Nasal
• temporal
2 pathways in
optic tract
• Visual
• Non visual
2 banks on the
calcarine cortex
• Upper
• lower
DeMyer’s neurological examination 6th edition

9. Retina
2
receptors
Cones Rods
2 field of
vision
Central Peripheral
Unipolar
(rod+cone
neurons)
Bipolar
neurons
Multipolar
neurons
Optic
nerve
DeMyer’s neurological examination 6th edition

10. 2 afferent CN
• Optic
• Trigeminal
2 set of
muscles
• Intra-ocular
• Extra-ocular
2 areas of the
retina
• Central
• peripheral
2 pathways in
ON
• Nasal
• temporal
2 pathways in
optic tract
• Visual
• Non visual
2 banks on the
calcarine cortex
• Upper
• lower
Ipsilateral
temporal &
nasal
ON Ipsilateral
temporal &
contralateral
nasal
OT
DeMyer’s neurological examination 6th edition

11. Optic tract
Visual
Lateral geniculate
nucleus oh the
thalamus
Retino-geniculate
tract
Primary visual
cortex
Non-visual
Pretectum of
midbrain
Retino-pretectal
tract
Pupil constriction
to light
Hypothalamus
Retion-
hypothalamic
Sleep-
wakefulness cycle

12. The primary visual cortex
forms dual
upper and lower banks
along the calcarine fissure
on the medial surface of
the occipital lobe
(area 17 of Brodmann).
DeMyer’s neurological examination 6th edition

14. Visual field nomenclature
Quarter / half
Complete/
incomplete
Homonymous
/heteronymous
Left/ right
Superior/
inferior

16. Scotoma
Central
Centro-cecal
Paracentral
Peripheral
Site
central scotoma
connected
to the blind spot

17. Examination of vision
Visual acuity
• Snellen chart (6m – 3m –
1m).
• Counting fingers.
• Hand motion.
• Perception of light.
Color vision (Ishihara plates)

18. Visual field

19. Complete left superior
temporal quadrantanopia
Complete temporal
hemianopia
Complete right
homonymous hemianopia
Incomplete left superior
homonymous quadrantanopia
complete superior
bitemporal quadrantanopia
incomplete superior
bitemporal quadrantanopia
complete bitemporal
hemianopia
Central
scotoma
Complete right homonymous hemianopia with
left superior quadrant paracentral scotoma

20. Examination of the eye
General inspection
• Inspectbothof theeyes assessing thefollowing:
Peri-orbitalregions
Eyelids
Eyes(including pupils)
• Noteanyabnormalities such as:
Swelling
Redness
Discharge
Prominenceoftheeyes
Abnormaleyelid position:ptosis
Abnormalpupillaryshape,size and/orasymmetry

21. Range of eye movements

22. Cover-uncover test for malalignment
Diplopia Heterotropia

23. The heterotropia (aka squint)
• Named according to the direction of deviation
of the errant eye:
 Exotropia: eye deviates outward (laterally).
 Esotropia: eye deviates inward (medially).
 Hypertropia: eye deviates upward.
 Hypotropia: eye deviates downward.

24. Classification of
heterotopias
Paralytic
Neuro-muscular
lesion
Non-paralytic
Lesions that impair
central vision in one eye
therefore impairing
fixation
Cause
-Refractive errors.
-Opacification of
the cornea or lens
(refracting media).
-Macular lesions.
To avoid diplopia, the Pt
tends to compensate for
a paretic eye muscle by
turning or tilting the
head.
A face turn =
horizontally acting
muscle palsy.
Chin elevation or
depression = vertically
acting muscle palsy.
Head tilt = torsional
acting muscle palsy.

25. 3rd Out
down
4th Out
Up
6th Inward

26. Types of conjugate eye movements
Saccadic
Fast conjugate eye
movements to fixate
both eyes on a novel
target.
Pursuit
smooth pursuit eye
movements maintain
both foveas conjugately
on a slowly moving
visual target
Vestibulo-
ocular reflex
Eye movements to
stabilize a retinal image
during head movement.
Cold caloric testing
directly tests the
horizontal VOR.
Optokinetic
nystagmus
A slow pursuit eye
movement followed by
a fast corrective
saccade
When viewing repetitive
target (train).

27. Conjugate eye movements
MLF
 Paramedian pontine reticular formation (PPRF) near the
midline of the pons >> fibers to the ipsilateral abducens
nucleus >> cranial nerve VI to the ipsilateral LR .
 The medial longitudinal fasciculus (MLF) >> fibers that
cross to the opposite medial rectus subnucleus >> the
MR.
Thus, stimulation of the right PPRF or right abducens
nucleus will make the eyes deviate conjugately to the right.
INO

28. Frontal eye field
Gaze
palsy

30. Examination of the pupils
• Normal illumination of the room, with no
direct sunlight.
• The patient should gaze at a distant point to
avoid pupillo-constriction from the
accommodation reflex.
• Look for anisocoria. Benign congenital
anisocoria in which both pupils react normally
is relatively common.
• Inspect the limbus for a Kayser-Fleischer ring
or an arcus senilis (may indicate
hyperlipidemia).
• Dim the room lights and inspect the pupils
immediately.
• Normal pupils dilate promptly, within 5 seconds
of dimming the light (activation of the
pupillodilator fibers by the sympathetic nervous
system).
• A dilation lag of seconds to minutes indicates a
lack of sympathetic innervation (Horner
syndrome) or a myotonic pupil (Adie pupil).

31. Pupillary light reflex
• Direct and consensual light
reflex.
• Swinging flash light reflex
(Marcus-Gunn pupil or
relative afferent pupillary
defect))

32. Pupillary syndromes
Horner’s
Ptosis, miosis, anhidrosis,
enophalmos
Interruption of sympathetic
supply
Argyll
Robertson
pupil
Miosis
Loss of light reflex
Retained accommodation
reflex
Usually bilateral
Neurosyphilis
Holmes-Adie
Tonic pupil
Dilated more than normal
Very slow reaction to direct
light
Light-near dissociation
Usually unilateral
More in females
Unilateral
Tendon areflexia
Unknown cause
Afferent Pupillary
Defect
Marcus Gunn Pupil

33. Argyll Robertson Pupil syndrome
 Found In late-stage syphilis, a disease caused
by the spirochete Treponema pallidum.
 Characterized by absence of pupillary light
reflex with retention of pupillary constriction to
near vision.
 A lesion of the rostral midbrain within the
dorsal aspect of the Edinger-Westphal
nucleus can interrupt the pretectal oculomotor
light reflex fibers. However, the more ventrally
located fibers of the Edinger-Westphal nuclei
that control the near reaction are spared.

34. Holmes-Adie syndrome (HAS)
 Unilateral or bilateral tonically dilated
pupils with light-near dissociation and
tendon areflexia.
 In most patients the pupil is larger than
normal (dilated) and slow to react in
response to direct light.
 The symptoms result from autonomic
disturbances, affecting vasomotor and
sudomotor functions.
 It has a female preponderance with absent or
reduced deep tendon reflexes.
 Damage to the ciliary ganglion most
commonly by an inflammatory process.
Pupillary symptoms result from damage to the
postganglionic parasympathetic supply
innervating the ciliary body and iris.
 Damage to the dorsal root ganglion of the
spinal cord, many patients also experience
problems with autonomic control of the body.

35. Miosis
Argyll
Dilated
Holmes

36. Examination of ptosis
Two muscles elevate the eyelid:
 The superior tarsal (Muller’s) muscle>>
smooth muscle, innervated by carotid
sympathetic nerve.
 The levator palpebrae muscle>> skeletal
muscle, innervated by oculomotor.
Severe ptosis, greater than with superior
tarsal ptosis.
Paralysis of lid elevation during upward
gaze.
3rd
nerve
palsy
NM
(MG)
Horner’s

37. Ophthalmoscopy
Papilledema
• Means a blurred or elevated optic papilla (optic nerve head or optic disc)
resulting from edema fluid in the nerve fibers as they cross the disc to
perforate the lamina cribrosa and enter the optic nerve.
• Papilledema is classed as early, fully developed, chronic, and chronic
atrophic.
• Papilledema results from transmission of increased intracranial pressure
into the eye via the subarachnoid space, which extends out along the
optic nerve or direct pressure on the optic nerve from retrobulbar lesions.

38. Papilledema, papillitis, and acute retrobulbar neuritis
• Inflammatory or toxic process affecting optic papilla >> papillitis.
• Inflammatory or toxic or demyelinating process affecting the optic nerve behind the eyeball (where
the optic axons become myelinated) >> acute retrobulbar neuritis ( Pt loses visual acuity and
color vision).
• Early loss of vision >> papillitis vs Late loss of vision >> papilledema.

40. Basic approach to binocular diplopia
Vestibulo-
ocular
reflex
Supra-
nuclear
Inter/infra-
nuclear
doll’s head
maneuver
Intact Affected

41. Supranuclear causes of diplopia
• Progressive Supranuclear Palsy.
• Parkinson Disease.
• Parinaud Syndrome (Dorsal Midbrain Syndrome):
lesions affecting structures in the dorsal midbrain (e.g., infarction,
hemorrhage, tumors, demyelination, inflammation, infection,
trauma, hydrocephalus, and arteriovenous malformations).
The PS triad : up gaze palsy, convergence retraction nystagmus,
and light-near dissociation.

42. Nuclear/internuclear causes of diplopia:
• Nuclear = Oculomotor, Trochlear, and Abducens nuclear
lesions.
• Internuclear = INO, One and half syndrome.

43. Infra-nuclear causes of diplopia
• Cranial neuropathies (mono or multiple).
• Extra-ocular muscle disorders:
Myasthenia gravis
Thyroid orbitopathy
Orbital apex trauma with connective tissue and muscle
entrapment.

44. Causes of eye-associated MCNP
• Cavernous sinus syndrome.
• Superior orbital fissure syndrome.
• Orbital apex syndrome.
• Cerebello-pontine angle syndrome.
• Tolosa-Hunt syndrome.

45. Cavernous sinus syndrome (CSS)
• Ophthalmoplegia, ptosis, and
facial sensory loss,
proptosis, orbital (ocular
and conjunctival) congestion,
sympathetic disturbance and
Horner’s syndrome.
Eye-associated multiple cranial nerve palsies Burak
Turgut,1 Onur Çatak,2 Sabiha Güngör Kobat,

46. Superior orbital fissure syndrome
(SOFS)
• Superior orbital fissure (SOF) lies at the back of the orbit between
the lesser and greater wing of the sphenoid bone.
• retro-orbital paralysis of EOMs (CN 3,4,6) and impairment of the CN V1
without optic neuropathy (the main difference of SOFS from OAS is no
optic nerve involvement in SOFS).
• It is known that the most common cause of SOFS is trauma
(craniomaxillofacial injury).
Eye-associated multiple cranial nerve palsies Burak
Turgut,1 Onur Çatak,2 Sabiha Güngör Kobat,

47. Orbital apex syndrome (OAS)
• The orbital apex (OA) is the most
posterior part of the pyramidal shaped
orbit at the craniofacial junction.
• Characterized by the involvement of CNs
II, III, IV and VI.
• The clinical characteristics of OAS are
vision loss (if CN II involvement is
present), optic neuropathy and
ophthalmoplegia.
• The etiologies of OAS are neoplastic
pathologies, inflammatory causes such
as idiopathic orbital inflammation,
collagen vascular disease, sarcoidosis,
systemic lupus erythematosus,
granulomatosis with polyangiitis, giant
cell arteritis, thyroid disease, iatrogenic
causes.
Eye-associated multiple cranial nerve palsies Burak
Turgut,1 Onur Çatak,2 Sabiha Güngör Kobat,

48. Cerebellopontine angle syndrome
(CPAS)
• CPAS is characterized by MNCP
including CN V, VII, VIII and cerebellar
signs.
• The presentation includes:
 Unilateral hearing loss (most common).
 Speech impediments.
 Imbalance, vertigo, tinnitus, disequilibrium.
 Tremors.
• CPAS is usually due to an acoustic
schwannoma arising from the vestibular
nerve.
• Diplopia due to CN VI palsy,
papilledema due to raised intracranial
pressure, nystagmus, ipsilateral corneal
reflex loss and facial weakness may
associated to above mentioned signs.
Eye-associated multiple cranial nerve palsies Burak
Turgut,1 Onur Çatak,2 Sabiha Güngör Kobat,

49. Tolosa-Hunt syndrome
• Tolosa-Hunt syndrome is a cause of
the painful ophthalmoplegia in the fifth
decade with unknown etiology located
in SOF and anterior CSs.
• The cause of pain is granulomatous
inflammation.
• The good response of the pain to the
steroid treatment within 72 hours is
critical for diagnosis of THS.
• Common diagnostic criteria for THS
are:
severe unilateral peri- or retro-orbital
pain in a perforating manner.
ipsilateral ophthalmoplegia.
optic nerve involvement
episodes with spontaneous remissions.
Eye-associated multiple cranial nerve palsies Burak
Turgut,1 Onur Çatak,2 Sabiha Güngör Kobat,

50. • The paralysis of one or more of the
CNs III, IV, and CN VI occur.
However, CN V1 and V2 may be
affected.
• The most affected CN is CN III.
• If inflammation reaches to OA, CN II
dysfunction may be developed.
• Horner syndrome may sometimes be
associated with THS due to the
involvement of carotid sympathetic
fibers.
• Pupillary dysfunction may occur in
some cases because of the
involvement of parasympathetic fibers of
CN III
Eye-associated multiple cranial nerve palsies Burak
Turgut,1 Onur Çatak,2 Sabiha Güngör Kobat,

51. Optic neuropathy

52. Clinical features
• Monocular vision loss
• Pain (suggest inflammatory
cause)
• RAPD (unilateral lesion)
• Scotomas
• Dyschromatopsia
• Central loss of vision
The presence of RAPD is a required
clinical feature for the diagnosis of
unilateral optic neuropathy.
• Swinging flashlight test evidence the
RAPD and may suppose the most
valuable test in the clinical examination.
• When RAPD is not present, this usually
signifies that there is bilateral involvement
or a previous damage, sometimes
subclinical, of the fellow pregenicular
afferent pathway.
Diagnosis Approach of Optic Neuritis Pérez–Bartolomé
Francisco

53. Worldwide, the principal cause of optic
neuropathy is GLAUCOMA.
• As glaucoma is a bilateral disease, these patients do not usually present a RAPD.
• Caution for drug-induced glaucoma!!
Tricyclic agents
(amitriptyline
and imipramine)
and of the non
TAC’s drugs
(paroxetine,
fluoxetine, e,
fluvoxamine,
venlafaxine,
citalopram, and
escitalopram)
Diazepam,
clotiazepam
and alprazolam
can cause
AACG.
Acetazolamide Topiramate
Anti-
Parkinsonians
like
cabergoline, a
dopamine D2
receptor
agonist

54. Etiology of optic neuropathy
Inflammatory
Ischemic
Compressive
Infiltrative
Hereditary
Post-traumatic
Nutritional/toxic
Post-radiation
ICH

55. Mode
of
onset
Rapid
Over
years
Gradual
over
months
Optic neuritis,
Ischemic optic
neuropathy,
Inflammatory (non-
demyelinating) and
Traumatic optic
neuropathy.
Compressive toxic
/ Nutritional optic
neuropathy.
Compressive and
Hereditary optic
neuropathies.
Clinical approach to optic neuropathies Raed Behbehani

56. In a young patient ..
• History of eye pain associated with eye
movement, prior history of neurological
symptoms such as paresthesia, limb
weakness, and ataxia is suggestive of
demyelinating optic neuritis.
In an elderly ..
• Signs of optic neuropathy, the presence of
preceding transient visual loss, diplopia,
temporal pain, jaw claudication, fatigue,
weight loss and myalgias is strongly
suggestive of arteritic ischemic optic
neuropathy (AION) due to giant cell
arteritis (GCA).
In children ..
• History of recent flu-like illness or
vaccination days or weeks before
vision loss points to a para-
infectious or post-vaccinial optic
neuritis, respectively.
Ex ..
Clinical approach to optic neuropathies Raed Behbehani

57. Increased intra-cranial
pressure.
Transient visual
obscurations (periods of
vision blackouts lasting
seconds caused by
change in body position),
transient diplopia and
headache.
The use of any medications should be carefully
noted since some are either directly or indirectly
toxic to the optic nerve. These include drugs as
ethambutol, amiodarone, alcohol, and
immunosuppressive medications such as
methotrexate and cyclosporine.
History of diabetes, hypertension and
hypercholesterolemia is common in
patients with non-arteritic ischemic
optic neuropathy (NAION).
Patients who are being
treated for or have history
of malignancy may have
infiltrative or para-
neoplastic optic
neuropathy.
Clinical approach to optic neuropathies Raed Behbehani

58. Diagnosis Approach of Optic Neuritis Pérez–Bartolomé
Francisco

59. Diagnosis Approach of Optic Neuritis Pérez–Bartolomé
Francisco

60. Optic neuritis (typical vs atypical)
• It is an inflammatory demyelination of the optic nerve.
• One classic classification of optic neuritis has been anterior (or papilitis) and posterior
(retrobulbar).
Diagnosis Approach of Optic Neuritis Pérez–Bartolomé
Francisco

61. Clinical approach to optic neuropathies Raed Behbehani

62. Demyelinating
ON
Approach to optic neuritis: An update Swati Phuljhele,
Sachin Kedar1, Rohit Saxena

63. Approach to optic neuritis: An update Swati Phuljhele,
Sachin Kedar1, Rohit Saxena

64. Non-arteritic
ischemic optic
neuropathy
(NAION)
 The presence of severe disc edema with hemorrhages is
characteristic of NAION and atypical of optic.
 Patients are usually over 50 years old and have systemic vascular
risk factors such as diabetes, hypertension, and smoking.
 Nevertheless, NAION can occur in young patients (below 45
years) and patients who lack any vascular risk factors.
 In young patients, it has been associated with
hypercholesterolemia and hyper-homocysteinemia.
Clinical approach to optic neuropathies Raed Behbehani

65. Arteritic ischemic
optic neuropathy
(AION)
 In patients over 60-year-old with features of ischemic optic neuropathy,
the ophthalmologist should strongly consider the possibility of giant cell
arteritis (GCA).
 Careful medical history should be obtained, inquiring about temporal
pain, jaw claudication, transient visual or diplopia, fever, weight loss,
myalgias and fatigue.
 Some patients may not have the constitutional symptoms and have only
visual symptoms (occult GCA).
 Laboratory evaluation should include complete blood count, erythrocyte
sedimentation rate (ESR), and C-reactive protein (CRP).
 Steroid treatment should be instituted in patients who are considered at
high risk to have GCA based on the clinical and laboratory features.
 Definitive diagnosis of AION is established by temporal artery biopsy
and histopathological confirmation.
Clinical approach to optic neuropathies Raed Behbehani

66. Inflammatory (non-demyelinating)
optic neuropathy
 Optic nerve is involved by either an ocular or
systemic inflammatory process.
 Optic disc swelling frequently occurs with
posterior uveitis and retinitis.
 MRI of the orbit will show inflammation of the
optic nerve sheath (optic perineuritis).
 ON can be involved in multiple disorders such
as: sarcoidosis, systemic lupus erythematosus,
Behcet’s disease inflammatory bowel disease,
Sjogren’s syndrome Wegener’s granulomatosis,
syphilis, Lyme disease and cat-scratch disease.
 Chronic relapsing inflammatory optic
neuropathy (CRION) is another entity
characterized by recurrences and steroid
responsiveness.
 The syndrome can behave as
granulomatous optic neuropathy and may
require long-term immunosuppressive
therapy.
Clinical approach to optic neuropathies Raed Behbehani

67. Infiltrative optic
neuropathies
 The optic nerve can be infiltrated in systemic
malignancies such as lymphoma, leukemia,
multiple myeloma, and carcinoma.
 MRI of the brain and orbit may show
meningeal and optic nerve enhancement.
Compressive optic
neuropathy
 Gradual progressive visual loss.
 Common causes include orbital and intracranial
meningiomas, pituitary adenomas, intracranial
aneurysms, craniopharyngiomas, and gliomas of
the anterior visual pathway.
 Vision loss, however, can be fast and dramatic in
pituitary apoplexy, or ruptured aneurysm.
 Visual field testing aids in the localization of the
lesion and neuro-imaging with MRI of the brain
and orbit is essential.
 Compressive optic neuropathy can also occur in
thyroid eye disease and can present as
asymmetric progressive visual loss.
Clinical approach to optic neuropathies Raed Behbehani

68. Hereditary optic
neuropathy
 The hereditary optic neuropathies are a broad category
including autosomally inherited diseases (dominant,
recessive, X-linked) and diseases caused by inheritance of
defective mitochondrial genome.
 Leber’s hereditary mitochondrial optic neuropathy
(LHON) classically presents with acute unilateral, painless,
visual loss. However, some cases may stay asymptomatic
or have a chronic course and sequential bilateral
involvement may occur weeks or months later.
Toxic-nutritional optic
neuropathies
 Optic nerve dysfunction can be caused by
various drugs, toxins and nutritional
deficiencies.
 The most common offenders are ethambutol,
amiodarone, methanol, ethanol and
tobacco.
 Other medications which can cause toxic optic
neuropathy include methotrexate,
cyclosporine, vincristine, cisplatin and
chloramphenicol.
 Deficiency of thiamine (B1), riboflavin (B2),
folate, B12 and B6 have all been associated
with optic neuropathy.
Clinical approach to optic neuropathies Raed Behbehani