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The scope of
Neuro-Ophthalmology
Oculomotor system
Eyelids
Saccadic system
Pupils
Pursuit system
Vergence system
Oculomotor
pathway
Supranuclear(UMN)
FEF: horizontal conjugate gaze
Diffuse frontal and occipital:
vertical conjugate gaze
Nuclear (LMN)
Nerve III, IV, VI Nucleus
Internuclear
PPRF, abducen interneuron,
MLF (Horizontal gaze)
riMLF, INC, PC (Vertical gaze)
Infranuclear(LMN)
Fasciculus
Cranial nerve
NMJ
Muscle
Dysconjugate eyes
Diplopia
Monocular
diplopia
Repetitive
images
- Cerebral polyopia
- Non-organic
Binocular
diplopia
Ghosting
image
- Retinal disease
- Refractive error
Misalignment of
the eyes
Nuclear
control
- CN III
- CN IV
- CN VI
Infranuclear
control
- CN palsy
- NMJ disorder
- Muscle disorder
Internuclear
control
Horizontal diplopia
- INO
- PPRF
Vertical diplopia
- INC, riMLF
Infranuclear control
Muscle
Fasciculus
Nerve
NMJ
IO
SR
LR
MR
SO
IO/SR
IR
SR/IO
SO/IR
IR/SO
MR
MR
SR
IO
MR
LR
IR
SO
CONVERGENCE
emedicine.medscape.com/article/
bjo.bmj.com/content/93/5.cover-expansion
III
IV
VI
Nuclear control:
Nucleus III, IV, VI
Horizontal gaze
internuclear control
Vertical gaze
internuclear control
Key features
Nuclear and fascicular lesion
Nerve lesion
Internuclear lesion
NMJ lesion
Muscle lesion
CN III
Part
nuclear complex->fasciculus->basilar>intracavernous->intraorbital
pupillomotor fibres
Webers
syndrome
Contralateral
hemiparesis
Ipsilateral CN III
palsy
+/- contralateral
parkinsonism,
corticobulbar palsy
http://www.cram.com
Benedikts syndrome
(paramedian midbrain
syndrome)
Ipsilateral CN III
palsy
Contralateral
extrapyramidal sign
Oculomotor nuclear
complex lesion
+/- ptosis
CN IV
SO function
Depression
Intorsion
Abduction
SR
IO
IR
SO
Left-Right-Left
Right-Left-Right
Left SO palsy
Right SO palsy
Cause of isolated CN IV in
adult
30% Unknown
20% Ischemic
10% Aneurysm
40% Traumatic
CN VI
Part: nuclear->fasciculus->basilar
(subarachnoidbase od skull, petrous bone)
>intracavernous->intraorbital
1. Foville syndrome
2. Millard-Gubler syndrome
Cross hemiplegia
Basilar portion of CN VI
palsy
1. Acoustic neuroma: hearing
loss+CN VI palsy (first sign is
diminished corneal sensitivity)
2. IICP
3. Nasopharyngeal tumours:
invade the skull
4. Basal skull fracture
5. Gradenigo syndrome: acute
petrositis (CN VI + CN VII
palsy + hearing loss + Pain)
Diagnostic criteria of Gradenigo syndrome
Suppurative otitis media
Pain in the distribution of the trigeminal nerve
Abducens nerve palsy
Isolated CN VI palsy
29
CN 3, 4, 6, V1
30
31
Interneuclear
lesion
Horizontal
a. Normal primary
position
d. Normal convergence
Interneuclear lesion
Vertical
Clinical syndrome
cases
compression
ischemia/hemorrhage
obstructive hydrocephalus
infection
tumour
Syndrome of
ophthalmoparesis
Miller-Fisher syndrome
Wernicke encephalopathy
Wernickes encephalopathy
Triad
ophthalmoparesis/nystagmus
acute confusion
ataxia
Victor M, et al. The Wernicke-Korsakoff Syndrome and Related Neurologic Disorders Due to
Alcoholism and Malnutrition. 2nd ed. 1989.
Treatment regime
Thiamine IV is recommended
IV route
Infranuclear lesion ;
disease of NMJ
disease of ocular muscle
Neuromuscular Junction
Features of NMJ
disorder
Ophthalmoplegia is not consistent with
nerve distribution
Fatigue
Fluctuating course
with other muscle weakness esp. ptosis,
proximal muscle weakness
Upper eyelid
Levator palpebral
superioris(CN 3)
Muller
muscle(sympathetic)
Frontalis muscle(CN 7)
Lower eyelid
Capsulopalpebral
fascia(inferior rectus)
Inferior tarsal
muscle(sympathetic)
Ptosis
Non-neurogenic(mechanical)
ptosis
Neurologic ptosis
Congenital ptosis
Uni-bilateral
Partial-complete
Supranuclear
lesion(cerebral
ptosis)
Contralateral
cerebral hemisphere
Pupil involvement
EOM impairment
LMN
Neuropathic(N,
fascicle, CN)
NMJ
Myopathic
Horners
syndrome
Horners syndrome
miosis
anophthalmos
anhidrosis
Visual loss
Assessment
Visual acuity
Pupillary reflex
Visual field
Fundus
Visual loss
Non-neurological
causes
-refractive error
-corneal problem
-cataract
-glaucoma
-retinal and choroidal
disease
Diplopia
Neurologic causes
Sudden onset
Transient: monocular, binocular
Non-progressive: monocular,
binocular
Progressive : monocular, binocular
Gradual onset
Retinal artery:
vasospasm(migraine),
hypoperfusion(hypotension,
hyperviscosity,
hypercoagulable stage),
vasculitis(GCA),
TIA(TMB, amaurosis fugax;
emboli to retinal circulation)
Disc:
trainsient visual
obscuration(chronic swelling
of optic disc)
Optic nerve:
Uhthoffs phenomenon in ON
Pupillary abnormality
Evaluation of pupillary abnormality
Size and shape of pupil
Reaction of pupil
Light reaction
Direct light reflex
Indirect(consensual) light reflex:
swing flash light test
Near(Accommodation) reflex:
92
Abnormal pupil
Size abnormality
Abnormal pupillary
reflex
Unequal size(anisocoria)
Abnormal equal size:
miosis VS mydriasis
Shape abnormality
Pupillary irregularity
Anisocoria
Opthalmologic
anisocoria
Simple(physiologic
) anisocoria
Neurologic anisocoria
Symp: ptosis,
anhydrosis
Parasymp: ptosis.
EOM
Visual
system
Pupil constriction
pathway abnormality
-Adies pupil
-CN3 palsy
Small pupil
Old age, syphilis, diabetes, long standing Holme
Adies pupil, congenital, drug/toxic
Tonic (Holme-Adie)pupil
Typically: unilateral mydriasis in healthy
young women
Acute: large
Months to years: small
React to light: sluggish or slow reaction to
light and slow(tonic) near response
Cause: postganglionic parasympathetic
denervation
Argyll-Robertson pupil
Pupillary irregularity
Localization of Horners
syndrome
Nystagmus
Nystagmus
Mechanism
Nystagmus may result from dysfunction
Often chronic
Severe vertigo
Minute to Day to weeks duration
Central nystagmus
Mechanism
Jerk nystagmus:
The Neurologist
CMU
The Neurologist
CMU