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Flashcards in Neuro-Ophthalmology Deck (80):
1

Sixth Nerve Syndromes

1- Raymond’s syndrome:
Sixth nerve paresis
Contralateral hemiparesis (pyramidal tract)

2- Millard-Gubler syndrome:
6th & 7th
Contralateral hemiparesis (pyramidal tract)

3- Foville’s syndrome:
CN 5, 6, 7, 8 palsies
Horizontal conjugate gaze palsy
Ipsilateral Horner’s syndrome

4 - Gradenigo:
6th with mastoiditis

5 - Pseudogradenigo:
6th with nasal cancer

6 - Mobius:
not a fascicular but a nuclear 6th & 7th (no contralateral hemiparesis)

2

Light near dissociation ddx

Divide it by location.

Edinger Westphall nucleus:
- syphillis
- dorsal midbrain syndrome

Ciliary ganglion:
- DM
- Adie's tonic pupil
- Aberrant regeneration (CN3)

Long posterior ciliary nerves:
- PRP

Other:
- severe loss of bilateral afferent input (e.g. dense VH)
- Myotonic dystrophy

3

Miosis ddx

- drugs (pilo, other cholinergics)
- Horner's
- Argyll Robertson
- Posterior synechiae
- spasm of near reflex

4

Mydriasis ddx

- CN3
- drugs (atropine, scopolamine, sympathetic agonists, anti-cholinergics, anti-histamines)
- trauma
- iris sphincter damage after surgery

5

Ptosis ddx

Neurogenic:
- horner's
- CN 3

Myogenic:
- CPEO (Kearns sayer, OPMD)
- myotonic dystrophy

NMJ:
- MG

Mechanical (tumors)
Involutional

Pseudo-ptosis ( enophthalmos, contralateral retraction)

6

Optociliary shunt vessels

Optic nerve meningioma, glioma
CRVO

7

GC axons project to (4)

- LGB
- hypothalamus
- superior colliculus
- EW nucleus

8

Macular sparing occipital infarct. Where is the lesion & what vessel

Anterior tip of occiput, which is supplied by middle and posterior cerebral artery. Posterior = more posterior, so if its infarcted all that's left is the macula (supplied by middle cerebral artery)

9

Where is the temporal crescent on the occipital lobe

Posterior tip (?)

10

Which CN go through what parts of brainstem

Midbrain: 3,4
Pons: 5-8
Medulla: 9-12

11

Constricted visual fields

Malingering
RP or its variants
Vitamin A deficiency
CSNB
Advanced glaucoma
ONH drusen
PRP

12

Arcuate VF ddx

Glaucoma
ONH drusen
Hemiretinal artery occlusion
Myelinated NFL
Optic nerve coloboma
AION, NAION
Optic neuritis

13

Bitemporal vf defect ddx

Pituitary/sellar lesion
Tilted discs
ONH drusen
Enlarged blind spots

14

Junctional scotoma - ddx of lesions

Pituitary adenoma
Craniopharyngioma
Glioma
Meningioma
AVM, anneurysm
Rathke's pouch cyst

15

Optic disc edema ddx

ONH drusen
NAION, AION
Optic neuritis
Papilledema (IIH or tumor)
Infiltration (sarcoid, syphillis, TB)
Compressive optic neuropathy
Neuro-retinitis
Diabetic papillitis
HTN papillopathy
Impending CRVO

16

Bilateral central scotoma ddx

Toxic, nutritional
Bilateral optic neuritis
Macular (dystrophy, AMD, bilateral CSR)
Hereditary (LHON, other hereditary optic atrophies)

17

Painful ophthalmoplegia

Thyroid
Myositis/IOI/other orbital inflammations (Wegener, sarcoid, etc)
Orbital apex mass
Tolosa Hunt
Pituitary apoplexy
Optic neuritis
CCF
Cavernous sinus thrombosis
Orbital infections (bacterial, mucor)

18

Vertical diplopia ddx

CN 4 palsy
Brown's
Skew
DVD
Inferior oblique palsy (rare)
Thyroid, MG
Mass
Myositis, IOI

19

Pituitary hormones to check for if you suspect a pituitary adenoma

FLAT PIG (for anterior), OA for posterior

FSH
LH
ACTH
TSH (and free T3, T4)
Prolactin
i
Growth Hormone

Oxytocin
ADH (check FBS too)

20

Painful sudden loss of vision with ophthalmoplegia. What are you worried about.

Pituitary apoplexy

21

Abduction deficit

Duane's type I
CN 6
Thyroid, mass, myositis, IOI
Decompensated strab
Slipped muscle after surgery

22

Tram track on CT

Optic nerve meningioma

23

Enlarged blind spot

Papilledema
Papillitis
ONH drusen
Megalopapillae
MEWDS/IEBSS

24

Blood supply of LGB

anterior & lateral choroidal arteries

25

Blood supply of optic tract

Middle and posterior cerebral arteries

26

Blood supply to chiasm

ICA

27

Which layers of LGB are crossed vs not

CI, IC IC

Crossed: 1, 4, 6
Ipislateral: 2, 3, 5

28

Layers of LGB that are magno vs parvocellular

Magno = 1,2 (M=motion, stereo, contrast)
Parvo = 3-6 (parvo=fine detail)

29

Anneurysms - which are most common

85% ICA. Of these, PCOM most common
ACOM can compress ON and chiasm

30

ONTT - what are the 3 groups

1. nothing
2. oral pred (1 mg/kg x 14 days)
3. IV pred (250 mg IV QID x 3d, then 1 mg/kg oral x 11 d)

31

ONTT - 1 year rates of MS

7.5% in IV group
15% in oral group
17% in placebo group

32

ONTT - what is IV steroid better for?

Shortens duration of current optic neuritis attack
No change in overall Va recovery

Decreased attacks of optic neuritis

Less MS at 2 years, but same as other groups by 3 years

33

ONTT - why is oral steroid bad

More recurrences of optic neuritis at 1 and 5 years. More MS at 1 year, but no difference in rates of MS overall

34

ONTT - rates of MS based on MRI findings

5 years:
-

35

ONTT - bad prognostic factors for development of MS

previous attacks of optic neuritis
history of episodes of neurologic symptoms

and MRI findings

36

ONTT - protective factors for MS

atypical optic neuritis (no pain, + disc edema, only mild Va loss)
no MRI findings

37

Where is the lesion - spasmus nutans

Rule out chiasmal glioma

38

Where is the lesion - gaze evoked nystagmus

If assymetric, rule out cerebellar lesion (stroke, MS, tumor)

39

Where is the lesion - rebound nystagmus

Cerebellar lesion

40

Where is the lesion - upbeat nystagmus

Medulla

41

Where is the lesion - downbeat nystagmus

Cervico-medullary junction (rule out arnold-chiari malformation)

42

Where is the lesion - ocular bobbing

bilateral pontine hemorrhage

43

Where is the lesion - oculopalatal myoclonus

Cerebellar flocculus to medullary olive

44

Where is the lesion - PAN

Always watch any nystagmus for >2 min to see if it becomes PAN
- cerebellar Nodulus (paN = Nodulus)

45

Where is the lesion - oculomasticatory myoryhtmia

Whipple's dz (duodenal bx)

46

Where is the lesion - convergence retraction nystagmus

Lateral medulla

47

Where is the lesion - Brun's nystagmus

Brun's = gaze evoked + vestibular
- when looking towards lesion, its low-freq
- when looking away, its high freq
*think CPA tumor, or CPA infarct

48

Where is the lesion - opsoclonus

Paraneoplastic.
Kids = metastatic neuroblastoma
Adults = breast, lung, ovaries

49

Where is the lesion - pendular nystagmus

Not localising

50

General ddx for nystagmus

Lesions (tumor, AVM)
Infarct
MS
Encephalitis
Syphillis
Drugs (anti-convulsants, EToh, lithium)
Nutritional (Wernicke's, parenteral feding)

51

What can you treat nystagmus with

Clonazepam, baclofen, gabapentin
(same as for muscle spasms/myokymia)

52

Features of dorsal midbrain syndrome

Light-near dissociation
Upgaze palsy
Eyelid retraction (Collier's sign)
Tonic downgaze (setting sun sign)
Convergence retraction nystagmus (with OKN moving up)
Convergence paralysis (?)

53

Causes of dorsal midbrain syndrome

PINEAL tumor
stroke
AVM
encephalitis
syphillis
drugs (lithium, etoh, anti-convulsants)
nutritional (wernicke's, parenteral feeding)
hydrocephalus

54

Features of congenital nystagmus

CONGENITAL
- better with Convergence
- no Opsoclonus
- have a Null point
- same in all positions of Gaze
- Equal in both eyes
- Near vision better than distance (because dampens with converence)
- Inversion of OKN
- head Turn often present
- Absent during sleep
- they have Latent nystagmus

55

Features of PSP

No Bell's, yes VOR (ie yes Doll's head)
Often have progressive dementia
Eventually get frozen globe, can't blink

56

CN3 syndromes

Nothnagel: ipsilateral 3 + ataxia
BenediKT: 3+ Kontralateral Tremor (cerebral peduncle)
Weber: contralateral hemiparesis (red nucleus)
Claude: nothnagel + benedikt (cerebral peduncle)

- don't quote me on these. Different sources define them differently.

57

What can you see in AR

- either miosis or lid retraction
- either with adduction or downgaze

(i.e. 4 things that could happen)

58

Causes of AR

Tumor
Trauma
AVM
Congenital (marcus-gunn)

NOT ischemic

59

Unique features of CN 4

- only CN to decussate
- only CN to exit brainstem dorsally
- longest course (most suspectible to trauma)

60

Features of bilat CN 4 palsy

hyper reverses on lateral gaze and head tilts
>10 degrees of torsion
V-pattern ET (usually ET > 25D)

61

Features of congenital CN 4 palsy

Head tilt on old photos
Large vertical fusional amplitudes (>10D (?))

62

Unique features of CN 6

Most suspectible to compression with high ICP - non-lateralising sign of high ICP
Second most common cause of CN6 palsy = trauma

63

CN 7 palsy - different causes

Idiopathic = Bell's
HSV = Ramsy Hunt (often have facial rash, hearing loss)
Sarcoid = bilateral
Miller-Fisher = ophthalmoplegia + ataxia
Melkerson Rosenthal = children, recurrent facial swelling & CN 7 palsy. Furrowed tongue.

64

2 types of childhood aberrant regeneration

Marcus Gunn jaw wink (CN3/5)
Crocodile tears (CN7 aberrant regen after Bell's)

65

CN's in marcus-gunn jaw wink

CN 3 and 5

66

Pathology affecting cavernous sinus or SOF

Tolosa Hunt
Thyroid
Mucor, bacterial orbital abscess
Retro-orbital hemorrhage
Meningioma


Cavernous sinus thrombosis
ICA anneurysm
Mets
Pituitary tumors, apoplexy

67

What is Hutchinson's

unilateral CN3 palsy (with blown pupil) in a coma due to a supratentorial mass

68

DDx of 2nd order Horners

Lung tumor (pancoast, neuroblastoma)
Carotid dissection

69

DDx of 3rd order Horners

Cavernous sinus lesion, ICA dissection
Orbital apex
Ciliary ganglion

70

DDx of 1st order Horners

Hypothalamus lesion
Down through brainstem
Lateral medullary syndrome

71

Features of lateral medullary syndrome (ocular and systemic)

Ipsilateral Horner's (miosis, ptosis, peri-ocular anhidrosis)
Nystagmus
Skew

Ipisilateral decreased pain and temp to face
Contralateral decreased pain and temp to trunk

Ataxia
Dysphagia, dysphonia, dysarthria

72

Ocular (4) and systemic (3) features of myotonic dystrophy

Ocular:
- Decreased EOM
- Ptosis
- Xmas-tree cataract
- Light-near dissociation

Systemic:
- MR
- Frontal baldness
- Testicular atrophy

73

What tests for MG

- Tensilon
- Ice pack
- rest
- ACH- receptor antibodies
- EMG
- Mestinon trial

74

What on history for MG

- alternating and variable ptosis
- variable diplopia
- symptoms worse at the end of the day
- symptoms worse with prolonged use

75

Always rule out with MG

thymoma (chest CT)
thyroid (TSH, free T3, T4)

76

Hereditary Optic Neuropathies

LHON (15-30 yo) - mitochondrial
Behr (

77

Mutations in LHON

11778 - most common
14484 - best prognosis
3460

78

You suspect hereditary optic neuropathy. What do you do?

FA
Maybe CT. Have to rule out other causes.

Genetics
Avoid toxins (smoking, etoh, etc)

79

Causes of IIH

OCP
Vitamin A compounds (e.g. retinoic acid)
Tetracyclines
Steroid use/withdrawal (Addison's dz)
Young, overweight females
Pregnancy

80

How do you treat IIH

Mild, no vision loss: lose weight and treat headaches
Some vision loss: diamox
Severe vision loss:
- Optic nerve fenestrations
- therapeutic LP's
- VP shunt