Narrating the neuro-ophthalmological cases in points. Part 2

1. Neuro-ophthalmology 2
Samhaa Mohammed Abd El Moneim
Zagazig Ophthalmology Hospital, 2019
Samhaa Mohammed

2. Siteofthe
lesion
Opticnerve(afferent)√
Pupil(efferent)sympatheticandparasympathetic√
Chiasm √
Retrochiasmaticlesions(tract,radiation,cortex)√
Ocularmotornerves3rd,4th,6th(nuclear,fasiculus,basilar,
cavernous,orbital)√
Supranucleardisordersofocularmotility
Ocularmyopathies(MG,CPEO,myotonicdystrophy)
Nystagmus(central,peripheral)
Migraine,neuralagia,facialspasmSamhaa Mohammed

3. Samhaa Mohammed
Pupil pathway
1st
order
2nd
3rd
4th
1st
order
2nd
3rd
Pupillary light reflex
pathway,
parasympathetic
Pupillary
sympathetic
pathway

4. Samhaa Mohammed
Pupil disorders
Afferent
(sensory lesions from ON till
tract or severe retinal dis.)
Absolute APD (Amarautic pupil)
-No PL, No anisocoria.
-Neither pupil constricts while stimulating
affected eye, both pupils constrict while
stimulating normal eye.
-Normal near reflex
RAPD (Marcus Gunn pupil)
-No anisocoria, variable VA.
-With swinging, both pupil constrict while
stimulating normal eye, early dilatation
while shifting to diseased eye.
-Normal near reflex.
Efferent
(motor nerve lesion or iris
abnormality itself)
Anisocoria (narrow pupil is
the affected)
Horner (↑ in dark)
Others: pharmacological,
physiological , mechanical
(synechia), traumatic
Anisocoria (wide pupil is the
affected)
Adie , 3rd n palsy (↑ in light)
Others: pharmacological,
physiological, traumatic,
mechanical

5. Samhaa Mohammed
Horner clinical
presentation
Miosis ↑in dim
light , normal
light reflex
Mild ptosis,
apparent (UL&LL
ptosis)
enophthalmos,
↓IOP
Facial
anhydrosis
(affected fibers
around ECA)
Hypochromic
heterochromia
(congenital),
idiopathic or 2ry
to birth trauma

6. Samhaa Mohammed
Horner causes
1st
order
2nd
3rd
1st order
Brain stem lesion
Syringomalia
Lateral medullary $
Cervical spinal cord lesion
2nd order (preganglionic)
Pancoast tumour
Neck lesion
Carotid dissection, aneurysm
Thoracic spinal cord lesion
3rd order (post ganglionic)
ICA dissection
Nasopharyngeal tumor
CS lesion
Otitis media
Cluster headache

7. Samhaa Mohammed
Horner clinical
presentation
Isolated is
common
But exclude to
complex
Ask for and
examine
Hx of pain (neck,
arm, headache)..
carotid dissection,
pancoast.
Old photo.
Hx of mass
(thyroid, lung,
neck), surgery,
trauma.
CN (2nd, 3rd, 4th,
6th, 5th)
PNS
CS & cerebellum
signs
Demyelination

8. Samhaa Mohammed
Horner clinical
investigation
Cocaine 4%
Prevent NA reuptake at dilator
pupillae, denervation hypersensitivity
No dilatation in + ve Horner
Hydroxyamphetamine 1%
After 48h of cocaine
No release of NA
No dilatation in postganglionic lesion
Dilatation in preganglionic
Adrenaline 0.1%
No dilatation in preganglionic lesion

9. Samhaa Mohammed
Check light reaction
Anisocoria
Good reaction OU
Anisocoria ↑ in dark
Cocaine 10%
(check
dilatation)
-ve cocaine
dilatation
Horner
Hydroxyamphe
-tamine 1%
Dilated
Postganglionic
Not dilated
Preganglionic
+ve cocaine
Physiologic
Poor reaction of one
eye
Anisocoria ↑ in light
Slit lamp of iris
Near
Pilocarpine 0.125%
Torn pupil
Sluggish near
miosis
Traumatic
Round pupil
Poor near
-ve test
Piocarine 1%
-ve
Pharmacologic
-al
+ve
3rd n palsy
Vermiform
Tonic pupil
+ve
Adie

10. Samhaa Mohammed
Adie clinical
presentation
Young female, viral infection,
unilateral
Denervation hypersensitivity at
posganglionic parasympathetic
sphinter& ciliary ms
Light impaired or absent
Tonic pupil for near, tonic accomodation
constrict and dilate slowly
Homes adie syndrome ( hyporeflexia.
Autonomic association: sweating,
orthostatic hypotension )

11. Samhaa Mohammed
Adie investigation &
TTT
1st
order
2nd
3rd
4th
Pupillary light reflex
pathway,
parasympathetic
• Pilocarpine
0.125%: Miosis in
Adie, no effect on
normal.
• Syphilis screeing in
bilateral
Invest.
• Self resolved in
idiopathic.
• Reading glasses for near
• Sungasses, pilocarpine
0.1 for photophobia
TTT

12. Samhaa Mohammed
Light-near
dissociation
1st
order
2nd
3rd
4th
Pupillary light reflex
pathway,
parasympathetic
Unilateral
• APD
• Adie
• Apparent
regeneration 3rd
n.
• HZO
Bilateral
• Neurosyphilis
• Dorsal midbrain
lesion
• Type 1 DM
• Myotonic
dystrophy
• Alcoholism

13. Samhaa Mohammed
Check light reaction
Anisocoria
Good reaction OU
Anisocoria ↑ in dark
Cocaine 10%
(check
dilatation)
-ve cocaine
dilatation
Horner
Hydroxyamphe
-tamine 1%
Dilated
Postganglionic
Not dilated
Preganglionic
+ve cocaine
Physiologic
Poor reaction of one
eye
Anisocoria ↑ in light
Slit lamp of iris
Near
Pilocarpine 0.125%
Torn pupil
Sluggish near
miosis
Traumatic
Round pupil
Poor near
-ve test
Piocarine 1%
-ve
Pharmacologic
-al
+ve
3rd n palsy
Vermiform
Tonic pupil
+ve
Adie

14. Samhaa Mohammed
Dim light
Bright
light
Cocaine
4%
Dim light
Bright
light
Near
Pilocarpine
1%
Physiologic
mydriasis Pharmacologic
mydriasis

15. Siteofthe
lesion
Opticnerve(afferent)√
Pupil(efferent)sympatheticandparasympathetic√
Chiasm √
Retrochiasmaticlesions(tract,radiation,cortex)√
Ocularmotornerves3rd,4th,6th(nuclear,fasiculus,basilar,
cavernous,orbital)√
Supranucleardisordersofocularmotility√
Ocularmyopathies(MG,CPEO,myotonicdystrophy)
Nystagmus
Migraine,neuralagia,facialspasmSamhaa Mohammed

16. Samhaa MohammedOxford 3rd
edition
Supranuclear control
Supranuclear
control of eye
motility
Saccade
Contralateral Frontal
Eye Field
Pursuit
Ipsilateral parieto-
occipital cortex
Vestibular VOR
Contralateral
vestibular nucleus
Horizontal eye
movement
MLF: Ipsilateral MR &
contralateral LR, PPRF
Vertical eye
movement
Rostral interstitial
nucleus of Cajal, MLF
Convergence
Both MR nuclei spare
MLF

17. Samhaa Mohammed Kanski 8th
edition
Horizontal gaze
disorders
INO (ipsilateral MLF)
• Failure of ipsilateral adduction,
nystagmus of contralateral eye in
abduction, abnormal saccade,
preserved convergence in
anterior INO.
One and half (ipsilateral MLF,
PPRF)
• Loss of all horizontal movements
except abduction of contraleral
eye.

18. Samhaa Mohammed Kanski 8th
edition
Horizontal gaze
disorders
WEBINO Wall-Eyed Bilateral
INO (bilateral MLF)
• Loss of adduction of both eyes,
exotropia.
Saccadic, pursuit disorders
(FEF, parieto-occipital cortical
lesion)
• FEF Frontal Eye Feild → lost
contralateral saccade.
• Cortex parieto-occipital→ lost
ipsilateral pursuit (OKN),
contralateral HH.

19. Samhaa Mohammed
Horizontal gaze
disorders
PP
Rt gaze
Lt gaze
Rt gaze
Lt gaze
Convergence
Lt INO WEBINO

20. Samhaa Mohammed
Vertical gaze disorders
Dorsal midbrain Parinaud $
(pretectal, post commisure
area)
• Saccade, pursuit, VOR, light-near
dissociation, convergence retraction
nystagmus, impaired elevation &
convergence, collier sign.
Progressive supranuclear
palsy (PSP) rare
neurodegenerative dis.
• Vertical supranuclear palsy, vertical
saccade, OKN abnormalities.
• Lid apraxia: sustained lid closure and
opening (LPS & OO weakness) →
exposure.

21. Samhaa Mohammed
Vertical gaze disorders
Skew deviation (pons lateral
medulla)
• Eyes are vertically deviated with
torsion (usually comitant, vertical
VOR,.
Pinealoma, dorsal
midbrain lesion
MRI T1 saggital

22. Siteofthe
lesion
Opticnerve(afferent)√
Pupil(efferent)sympatheticandparasympathetic√
Chiasm √
Retrochiasmaticlesions(tract,radiation,cortex)√
Ocularmotornerves3rd,4th,6th(nuclear,fasiculus,basilar,
cavernous,orbital)√
Supranucleardisordersofocularmotility√
Ocularmyopathies(MG,CPEO,myotonicdystrophy)√
Nystagmus (central,peripheral)
Migraine,neuralagia,facialspasmSamhaa Mohammed

23. Myasthenia gravis
Samhaa Mohammed
20-60 y
Younger age F
Variable
Association with Graves dis.
In 5% of cases thymic hyperplasia.
-Ocular is the presenting is 70-90%
-Ocular will become generalized in 80%
within 2y

24. Myasthenia gravis C/P
Samhaa Mohammed
Variable
Fatigability (limbs,
chewing, speech,
swallowing,
breathing). ↑ at
end of day.
Generalized
C/P Variable
Ptosis, diplopia,
abnormal saccade,
Cogan lid twitch,
fatigue test.
Ocular C/P
Fatigue test: looking upgaze for 30 seconds →
dropping of UL.
Cogan lid twitch: looking downward then in PP →
lid twitches.

25. Myasthenia gravis C/P
Samhaa Mohammed
Variable in severity/ time/
affected ms (embarrasing
ptosis/ squint).
Female, adult/middle age.
Pupil always spared.
Associated autoimmune
diseases, thyroid.

26. Myasthenia gravis
investigation
Samhaa Mohammed
Ice-pack test (at clinic) (anticholinestrase)
Measure ptosis – ice in a towel for 2 min – re measure
ptosis → improved. 75% sensitive.
Tesnsilon test (Edrophonium is anticholinestrase)
Resuscitation facilities/ atropine0.5% - measure ptosis/
Hess – edrophonium IV – re measure ptosis/ Hess →
improved. 85-95% sensitive
Serum Abs
ACh Ab (sensitivity 90% in generalized MG, 50% in ocular).
Anti skeletal ms Ab in thymoma, anti thyroid Ab, ANA.
Single fibre EMG
Repititive stimuli leads to reduction in action potential
amplitude in MG.

27. Myasthenia gravis
investigation
Samhaa Mohammed
Edrophonium
test
Ice-pack test

28. Myasthenia gravis
TTT
Samhaa Mohammed
What is
important
Generalized type (fatal) – liaise with neurologist/
CT chest.
Medical TTT – liaise with physician
(Pyridostigmine, immunesuppressive).
Thymemectomy, plasmapharesis.

29. Neuromuscular junction
disorders
Samhaa Mohammed
Lambert-Eaton syndrome
Small cell lung carcinoma, ↓ Ach release, improved with
repeated testing , ↓ lacrimation.
Infantile MG
Purely ocular C/P.
Toxins
Botulism, tick paralysis, Botox. ↓ Ach release.

30. Other myopathies
Samhaa Mohammed
CPEO (kearns syre)
Mitochondrial DNA mutation, bil ptosis, EOM , pigmentary
retinopathy, heart block (ECG), ragged red fibers in ms
biopsy.
Myotonic dystrophy
AD, bilateral ptosis, EOM, christmas tree cat., pigmentary
retinopathy, mournful facies, myotonic grip, ms weakness,
dysphagia, dysphasia, cardiac myopathy (ECG).
Counseling.
Influenza
vaccination.
Refer to
cardiologist,
neurologist,
phsiotherapist,
Avoid GA in
surgery

31. Other myopathies
Samhaa Mohammed
Kearns-syre
Myotonic
dystrophy
Pigmentary
retinopathy

32. Siteofthe
lesion
Opticnerve(afferent)√
Pupil(efferent)sympatheticandparasympathetic√
Chiasm √
Retrochiasmaticlesions(tract,radiation,cortex)√
Ocularmotornerves3rd,4th,6th(nuclear,fasiculus,basilar,
cavernous,orbital)√
Supranucleardisordersofocularmotility√
Ocularmyopathies(MG,CPEO,myotonicdystrophy)√
Nystagmus (central,peripheral) √
Migraine,neuralagia,facialspasmSamhaa Mohammed

33. Nystagmus
Samhaa Mohammed
Nystagmus: initial slow movement away fixation then corrective fast
(jerky) or slow (pendular).
Oscilopia: intitial fast saccade movement away fixation then
corrective fast saccade (immediate).
Intrusion: intitial fast saccade movement away fixation then corrective
fast saccade (delayed).

34. Nystagmus examination
Samhaa Mohammed
DWARFC
Direction: horizontal, vertical, rotatory.
Waveform: pendular, jerky.
Amplitude: high, low.
Rest:
Frequency: slow, fast.
Conjugate, disconjugate.
Cover/uncover (for
latent), motility (gazes,
convergence, saccade)
Ocular examination
(ant/post segment causes)
Associated (systemic)
neurological
(especially in acute)

35. Infantile nystagmus causes
Samhaa Mohammed
Idiopathic: usually horizontal, conjugate, jerky, in early 2 months, may be
squint, may be inherited, ↑ with fixation, ↓ with convergence, mild ↓VA.
With retinal disease (sensory deprivation : roving eye, severe
decrease VA. Retinal diseases with normal looking eyes like CSNB, LCA,
cone-rod dystrophy, achromatopsia. Others like cataract, ROP, corneal
opacity
Latent/manifest: horizontal jerky conjugate with fast phase toward fixing
eye, ↑ with cover non fixing eye, often with infantile ET

36. Infantile nystagmus TTT
Samhaa Mohammed
Treat the cause: if possible (eg. Surgery for cataract).
Treat EOR: glasses, CL.
Prism base-out: to induce convergence.
Surgery: If AHP.
Kestenbaum recess resect horizontal recti for horizontal/ torsional types, recess
resect vertical recti for vertical type.

37. Acquired conjugate
nystagmus causes
Samhaa Mohammed
Peripheral vestibular: away from site of lesion,
Tinnitus, deafness/vertigo,
↓ with fixation.
Improves with labrynthitis, vestibular neuritis improvement.
Central vestibular/brainstem/cerebellar: toward site of lesion,
No decrease with fixation,
No tinnitus/deafness.
PSP (up/down-beat), dorsal midbrain lesion (convergence-
retraction), Arnold-Chiari (down-beat), spinocerebellar brainstem
lesion , drugs cause CNS depression like Barbiturate, Alcohol/Anti-
convulsant.

38. Acquired dis-conjugate
nystagmus causes
Samhaa Mohammed
Ataxic nystagmus: horizontal, occurs in abducted eye. With INO.
See-saw: vertical tortional nystagmus. One eye is elevated
intorted, the other is depressed extorted. Associated with
brainstem lesion.

39. Siteofthe
lesion
Opticnerve(afferent)√
Pupil(efferent)sympatheticandparasympathetic√
Chiasm √
Retrochiasmaticlesions(tract,radiation,cortex)√
Ocularmotornerves3rd,4th,6th(nuclear,fasiculus,basilar,
cavernous,orbital)√
Supranucleardisordersofocularmotility√
Ocularmyopathies(MG,CPEO,myotonicdystrophy)√
Nystagmus(cental,peripheral) √
Migraine,neuralagia,facialspasm√Samhaa Mohammed

40. Blepharospasm types
Samhaa Mohammed
Exclude 1st 2ry causes.
Bilateral fluctuated in
days, involuntary lid
closure, ↑ with stress,
fatigue, social
interaction, ↓ with
relaxation, sleep,
distraction (typical C/P).
Essential
blepharospasm
Blepharitis
Trichiasis/entropion
, ocular surface dis.
Uveitis, glaucoma.
2ry
Meige syndrome: blepharospasm with spill-over of essential type
to involve midfacial ms during speech, eating/drinking.
Hemifacial spasm: unilateral tonic clonic facial spasm, may
occur during sleep. MRI to rule out 7th n compression
Lid apraxia: extrapyramidal diseases, total LPS inhibition, no OO
activation (sustained lid closure, ↓opening & closure).

41. Facial spasm types
Samhaa Mohammed
Hemifacial
spasm
(unilateral)
Meige
syndrome
(bilateral lid &
facial ms)
Essential
type
(bilateral)

42. Facial spasm management
Samhaa Mohammed
Invetigation: if atypical, neurological signs, always unilateral, present at
sleep, young age.
Consult neurologist, MRI, EMG: if atypical.
Treat ocular diseases.
Botulinum injection.
Medical ttt: benzodiazepine

43. Migraine
Samhaa Mohammed
More common in females in adult age, in males in < 15 y
Uncertain cause, environmental factors release serotonin/
norA→ cortical bl vs constriction causes aura, extra-cranial
bl vs dilatation causes headache.
Migraine without aura, with aura (visual like scotoma/
flickering/ shimmering/zigzag, somatosensory, motor,
speech).
Atypical
(MRI, carotid doppler,
echo, vasculitis)
Occipito-
basal
>55y
Neurological
deficit

44. Neuralgias
(ocular, periocular pain)
Samhaa Mohammed
Cluster headache
• Unilateral, oculo-temporal deep sharp excruciating in adult to middle age men
• Horner postganglionic, autonomic symptoms (rhinorrhea, lacrimation, conj injection)
Reader paratrigeminal neuralgia
• Unilateral severe along distribution of ophthalmic nerve
• Horner post-ganglionic , exclude carotid dissection
Herpes zoster ophthalmicus
● 3 days before skin rash, along trigeminal n
Trigeminal neuralgia (triggered by shaving, no autonomic phenomena)
Occipital neuralgia
Paroxysmal hemicrania
SUNCT (Short acting Unilateral Neuralgiform headache with Conjunctival injection & Tearing)

45. Headache causes
Samhaa Mohammed
Life
threating
GCA
OIS
Malignant
HTN
Increase ICP
Subdural/intrac
ranial Hge
CNS
infection,aneur
ysm,tumor,AV
Sight
threating
GCA
AACG
OIS
IICP
Others
Migraine
Cluster
headache
HZO
Tolosa-Hunt
$
Ant uveitis,
accomodative
spasm
Dental, sinus,
cervical,psychol
ogical

46. Serious headache
Samhaa Mohammed
Scalp tenderness, age
>55y, ESR, CRP, ms pain,
malaise, wt loss (GCA)
Fever, stiff neck
ON swelling
Decrease VA
Altered mentation
Neurological signs
Subhyaloid hge

47. Serious headache criteria
Samhaa Mohammed
Recent onset of
headache
Different than usual
Always in same
location
Awaken pt from sleep
No respose to
medication
Nausea, projectile
vomiting
Headache then
migraine-like aura

48. Neuro-ophthalmological DDs according to..
Samhaa Mohammed
Symptoms
Loss of vision
(sudden/gradual?, free/not
fundus?, unilat/bi?,
permenant/transient?,
pain/not?).
Headache
(uni/bi?, swollen
disc/not?, VA?,
recurrent/recent?,
severity?, associated
sinus/dental/HTN?).
Signs
Binocular diplopia
(vertical/horizontal/mixed
?, variable/persistent?,
ptosis?, pupil?).
Anisocoria (dark/light?,
iris on slit lamp?).
Disc swelling, pallor
(VA sudden/gradual/not?,
free/not disc?, age?).
RAPD (ON dysfunction)?.

49. Samhaa Mohammed
Thank Allah
Thank you
Welcome for any
comment
Dr.samhaa@yahoo.co
m
Thanks Allah
Thank you
Welcome for any comment
dr.samhaa@yahoo.com