NEURO-

OPHTHALMOLOGY

Dr Jusuf Wijaya , SpM

FK - UKI
Cawang
 Curriculum Vitae
 1994 : Dokter Umum , Vrije Universiteit Brussel
(Belgia)
 1999 : Dokter Spesialis Mata , Vrije Universiteit
Brussel (Belgia)
 2001 : Fellow Ilmu Bedah , Foundation Eye Care
Himalaya (Belanda – Nepal)
 2002 : Fellow di bidang Glaukoma , Rotterdam
Eye Hospital (Belanda)
 2004 : Adaptasi (penyesuaian) , Universitas Sam
Ratulangi (Manado)
NEURO-OPHTHALMOLOGY

 The Pupil
 Visual field loss

 Ocular motility

 Central nervous system disorders

 The Pupil
Pupillary evaluation involves notation of :
 Pupil size

 Direct and consensual response

 Swinging flashlight test

 (Accommodation)
 The Pupil
Normal size : 2 – 7 mm and equal in size ,
but small differences in diameter are seen in
about 20% of the normal population and are
known as essential anisocoria.

Anisocoria refers to asymmetric pupil sizes

 The pathway of the pupillary light reflex
The pupillary light response begins with the rods and cones
of the retina. Afferent pupillomotor fibers travel through
the optic nerves and semi decussate at the optic chiasm.
They then follow the optic tracts and exit before the lateral
geniculate body to enter the brainstem via the brachium of
the colliculus.
Pupillomotor fibers synapse in the pretectal nuclei, which then
project equally to the ipsilateral and contralateral Edinger-
Westphal nuclei.
The pupillary fibers travel with N III to innervate the iris
sphincter and cause pupillary constriction
 Relative Afferent Pupillary Defect
(RAPD) = Marcus Gunn Pupil
 RAPD is an objective sign of an asymmetrical lesion
of the anterior visual pathway (retina, optic nerve,
chiasm or optic tract)
 An RAPD is seen with major retinal lesions or
neurological lesions of the anterior visual pathway
 Opacities in the ocular media, such as cataract, do not
produce an RAPD but small RAPDs may be seen with
a dense vitreous haemorrhage or dense amblyopia
 Relative Afferent Pupillary Defect
(RAPD)
Thus, the presence of an RAPD in the absence of gross
ocular disease indicates a neurological lesion in the
anterior visual pathway and the importance of this
physical sign cannot be overemphasized
 The Pupil
The smooth muscle of the pupil is innervated by :
- The Sympathetic system → dilator pupillae
- The Parasympathetic system → constrictor pupillae

Both pupils are normally of the same size, but small

differences in diameter are seen in about 20% of the
normal population and are known as essential
anisocoria.
 The Pupil
Pathological anisocoria is caused by lesions affecting the
sympathetic or parasympathetic pathways or by local
iris disease

Lesions of the afferent visual system do not produce

anisocoria, i.e. sectioning one optic nerve does not
alter pupillary size in that eye.
 Etiology of Anisocoria
 Physiologic anisocoria (essential anisocoria)
 Horner’s syndrome
 Structural / pharmacologic miosis
 Adie’s tonic pupil
 IIIrd nerve palsy
 Pharmacologic mydriasis
 Iris damage
 Horner’s Syndrome
 Horner’s syndrome is the result of a lesion in the
sympathetic pathway to the eye and may be due to a
lesion in the central (1st order), pre- (2nd order) or
post-ganglionic (3rd order) neuronal pathways
 The features are :
- Miosis
- Ptosis
- Enophthalmos
 Causes of Horner Syndrome
1st order Brain stem stroke
Demyelination
Tumor
2nd order Apical lung tumor (Pancoast)
Thyroid mass
Central line placement
Neck trauma or surgery
Lymphadenopathy
Spinal anesthesia
3rd order Cluster headache
Carotid artery dissection
Cavernous sinus tumor
Idiopathic
 Horner’s Syndrome

 Preganglionic lesions are often sinister and may

accompany lesions such as Pancoast tumour of
the lung
Horner’s syndrome shows greater anisocoria in the dark than in the light
as the affected eye fails to dilate. This can be useful as a simple bedside
diagnostic test.
 Adie’s Tonic Pupil
 Adie’s Tonic Pupil is a defect in the
parasympathetic innervation of the pupil
 The clinical findings are :
- Dilated pupil that is usually slightly irregular
- Segmental iris constriction at the slit lamp
NEURO-OPHTHALMOLOGY

 The Pupil
 Visual field loss

 Ocular motility

 Central nervous system disorders

 Visual Field
The temporal retinae is responsible for the nasal visual
field & the nasal retinae is responsible for the for the
temporal visual field
The nasal fibers (temporal field) cross in the chiasm ,
which is why lesions of the chiasm causes
bitemporal hemianopia
Posterior of the chiasm, nasal fibers from the
contralateral eye and temporal fibers from the
ipsilateral eye travel together
 Visual Field Defect
Visual field defect that respect the vertical
meridian(termed Hemianopia) are secondary to
lesions at or behind the chiasm

Visual field characteristics are helpful in localization of

lesions

Monocular field defects are typically due to lesions in

front of the chiasm
 Visual Field Defect

Binocular hemianopia that are on the same side of the

vertical meridian are termed homonymous
(hemianopia)

Binocular hemianopia that are on the opposite side of

the vertical meridian are termed binasal or
bitemporal (hemianopia)
Normal Visual field
 Visual Field Loss
 Prechiasmal Field Defects
Prechiasmal field defects are due to lesions of the Optic
nerve
Lesions of the Optic nerve in turn, may be either be:
- a demyelinating disease (e.g. Multiple Sclerosis)
- a compression
- ischemic
- inflammatory
- toxic / nutritional
- or due to trauma
 Visual Field Loss
 Chiasmal Field Defects
Fifty percent of visual fibers from each eye decussate at
the chiasm
Field defects produced by chiasmal lesions are usually
due to either :
- pituitary adenoma wit suprasellar extension
- craniopharyngioma
- suprasellar meningioma
- aneurysm
 Bitemporal hemianopia
The hallmark of chiasmal compression is a bitemporal
hemianopic visual field defect . This is due to damage
done onto the crossing fibers of the nasal retinae
from each eye
The most common disorder of the chiasm is from
compression :
- pituitary tumor
- meningioma
- craniopharyngioma
- aneurysm
Bitemporal hemianopia
 Visual Field Loss
 Retro-Chiasmal Field Defects
It may be due lesion in either :
- Optic tract
- Optic radiations
- Visual cortex
Right Homonymous Hemianopia
NEURO-OPHTHALMOLOGY

 The Pupil
 Visual field loss

 Ocular motility

 Central nervous system disorders

 Ocular motility
 Signals which control ocular movement are
initiated in the cerebral hemispheres
(supranuclear pathways) in a manner analogous
to other motor neuronal pathways
 They are then transmitted to the gaze
centres and ocular motor nuclei in the midbrain
and pons, and leave the brain in the IIIrd, IVth
and VIth cranial nerves
The major inputs to the vertical and horizontal gaze centers are
Saccadic & Pursuit commands from the cerebral hemispheres and
the Vestibular Ocular Reflexes from the vestibular nuclei in the
medulla.
 Ocular motility
Disturbances in ocular motility causes binocular
diplopia
Diplopia is a symptom in which the patient perceives
two images of a single object
Binocular diplopia represents misalignment between
the eyes , and are due to :
- Nerve palsy (Neuropathic)
- Extraocular muscle dysfunction or restriction
(Myopathic)
- Neuromuscular junction disorders (e.g.
Myasthenia Gravis)
 Third Nerve (Oculomotorius) Palsy

2 sub-types :
 Pupil involvement

 Pupil sparing

Common causes :
- (posterior communicating artery) aneurysma
- Ischemia
- Compressive lesion
 Right Third Nerve (Oculomotorius) Palsy

The IIIrd nerve innervates the ipsilateral superior rectus,

levator palpebrae, medial rectus, and inferior oblique
muscle
IIIrd nerve palsy results in poor elevation, adduction, and
depression of the eye and ptosis
 Fourth Nerve (Trochlear) Palsy
The IVth nerve has the longest intracranial course and
is the only cranial nerve to exit dorsally from the
brain stem

Common causes of IVth nerve palsy :

- Trauma
- Ischemia
- Decompensation of a congenital palsy
- Idiopathic
 Left Sixth Nerve (Abducens) Palsy

Common causes :
- Ischemia
- Intracranial tumors
- Increased intracranial pressure
- Trauma
- Idiopathic
NEURO-OPHTHALMOLOGY

 The Pupil
 Visual field loss

 Ocular motility

 Central nervous system disorders

 Supranuclear gaze control
Conjugate eye movements – versions i.e.
movements of both eyes in the same direction as
yoked pair and mediated through supranuclear
neuronal pathways) can be divided into :
 Saccadic movement

 Pursuit movement

 Vestibular movement

Disconjugate eye movements - vergences i.e.

movements of the eyes in the opposite directions
 Convergence

 Divergence
The major inputs to the vertical and horizontal gaze centers are
Saccadic & Pursuit commands from the cerebral hemispheres and
the Vestibular Ocular Reflexes from the vestibular nuclei in the
medulla.
 Supranuclear gaze control
 Saccadic movements are rapid and relocate
fixation of gaze, either reflexly or voluntarily.
They are initiated in the contralateral premotor
frontal cortex and, once initiated, the movement
is irrevocable and ocular position cannot again
be modified until the saccade has been
completed
 Saccades are tested clinically by commanding the
patient to look at a target, or to look right or left
 Supranuclear gaze control
 Pursuit movements are slower and are
concerned with keeping ocular fixation locked
on target. They appear to be generated in the
ipsilateral occipital cortex. The movement is
smooth and is continuously modified according
to the speed of the target; if the pursuit
movement lags behind the target position, a
corrective saccade is inserted to keep up
 Pursuit movements are tested by asking the
patient to follow a slowly moving target
 Supranuclear gaze control
 Vestibular ocular movements are initiated in the
semicircular canals by changes in head posture.
They serve to maintain ocular fixation,
independent of head and neck posture. Thus,
vestibular ocular reflexes keep the horizon
steady as we walk (the eyes moving in the
opposite direction to that of the orbits)
 Vestibular reflexes may be tested either by a
“doll’s head” manoeuvre or by caloric stimuli
 NEURO-OPHTHALMOLOGY
Supranuclear gaze control

Conjugate gaze palsies :

- Horizontal Supranuclear Palsy
- Vertical Gaze Palsy
- Progressive Supranuclear Palsy (Steele-Richardson
Syndrome)

Internuclear Ophthalmoplegia
 Conjugate gaze palsies
Horizontal Supranuclear Palsy
It results in an inability to make a conjugate ocular
movement to one side and may result from a
supranuclear or pontine lesion
 Conjugate gaze palsies
Vertical Gaze Palsy
Vertical gaze palsies are caused by lesions in the area of
the upper midbrain and are less common
They produce a characteristic triad of signs known as
Parinaud’s syndrome or Dorsal Midbrain syndrome :
- Loss of vertical gaze
- Loss of pupillary light reflex with preservation
of the of the near reflex
- Convergence retraction nystagmus
 Conjugate gaze palsies
Progressive Supranuclear Palsy (Steele-Richardson
Syndrome)
This is a Parkinsonian-like syndrome in which there is :
- extra pyramidal rigidity
- pseudo-bulbar palsy
- dysarthria
- dementia
 Conjugate gaze palsies
Progressive Supranuclear Palsy (Steele-Richardson
Syndrome)
An early and diagnostic feature of this syndrome is :
- difficulty in making downgaze saccades with
- preservation of horizontal saccadic movements and
- doll’s head reflexes
This patient can make an upward saccadic movement but
downgaze saccades are lost. Downgaze is present on doll’s
head movement. Horizontal movement are intact
 Internuclear Ophthalmoplegia
Internuclear Ophthalmoplegia are caused by lesions in
the medial longitudinal fasciculus (MLF) and produce :
- Poor adduction of the eye on the affected side
- Abducting nystagmus in the contralateral eye
- Normal convergence
 This hypertensive patient shows a right-sided lower motor
neuron seventh nerve palsy, internuclear ophthalmoplegia and
partial sixth palsy following a pontine infarct.