Neuro-Ophthalmology Flashcards

1
Q

Sixth Nerve Syndromes

A

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)

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2
Q

Light near dissociation ddx

A

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
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3
Q

Miosis ddx

A
  • drugs (pilo, other cholinergics)
  • Horner’s
  • Argyll Robertson
  • Posterior synechiae
  • spasm of near reflex
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4
Q

Mydriasis ddx

A
  • CN3
  • drugs (atropine, scopolamine, sympathetic agonists, anti-cholinergics, anti-histamines)
  • trauma
  • iris sphincter damage after surgery
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5
Q

Ptosis ddx

A

Neurogenic:

  • horner’s
  • CN 3

Myogenic:

  • CPEO (Kearns sayer, OPMD)
  • myotonic dystrophy

NMJ:
- MG

Mechanical (tumors)
Involutional

Pseudo-ptosis ( enophthalmos, contralateral retraction)

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6
Q

Optociliary shunt vessels

A

Optic nerve meningioma, glioma

CRVO

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7
Q

GC axons project to (4)

A
  • LGB
  • hypothalamus
  • superior colliculus
  • EW nucleus
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8
Q

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

A

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)

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9
Q

Where is the temporal crescent on the occipital lobe

A

Posterior tip (?)

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10
Q

Which CN go through what parts of brainstem

A

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

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11
Q

Constricted visual fields

A
Malingering
RP or its variants
Vitamin A deficiency
CSNB
Advanced glaucoma
ONH drusen
PRP
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12
Q

Arcuate VF ddx

A
Glaucoma
ONH drusen
Hemiretinal artery occlusion
Myelinated NFL
Optic nerve coloboma
AION, NAION
Optic neuritis
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13
Q

Bitemporal vf defect ddx

A

Pituitary/sellar lesion
Tilted discs
ONH drusen
Enlarged blind spots

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14
Q

Junctional scotoma - ddx of lesions

A
Pituitary adenoma
Craniopharyngioma
Glioma
Meningioma
AVM, anneurysm
Rathke's pouch cyst
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15
Q

Optic disc edema ddx

A
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
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16
Q

Bilateral central scotoma ddx

A

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

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17
Q

Painful ophthalmoplegia

A
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)
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18
Q

Vertical diplopia ddx

A
CN 4 palsy
Brown's
Skew
DVD
Inferior oblique palsy (rare)
Thyroid, MG
Mass
Myositis, IOI
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19
Q

Pituitary hormones to check for if you suspect a pituitary adenoma

A

FLAT PIG (for anterior), OA for posterior

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

Oxytocin
ADH (check FBS too)

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20
Q

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

A

Pituitary apoplexy

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21
Q

Abduction deficit

A
Duane's type I
CN 6
Thyroid, mass, myositis, IOI
Decompensated strab
Slipped muscle after surgery
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22
Q

Tram track on CT

A

Optic nerve meningioma

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23
Q

Enlarged blind spot

A
Papilledema
Papillitis
ONH drusen
Megalopapillae
MEWDS/IEBSS
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24
Q

Blood supply of LGB

A

anterior & lateral choroidal arteries

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25
Q

Blood supply of optic tract

A

Middle and posterior cerebral arteries

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26
Q

Blood supply to chiasm

A

ICA

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27
Q

Which layers of LGB are crossed vs not

A

CI, IC IC

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

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28
Q

Layers of LGB that are magno vs parvocellular

A
Magno = 1,2 (M=motion, stereo, contrast)
Parvo = 3-6 (parvo=fine detail)
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29
Q

Anneurysms - which are most common

A

85% ICA. Of these, PCOM most common

ACOM can compress ON and chiasm

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30
Q

ONTT - what are the 3 groups

A
  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)
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31
Q

ONTT - 1 year rates of MS

A

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

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32
Q

ONTT - what is IV steroid better for?

A

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

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33
Q

ONTT - why is oral steroid bad

A

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

34
Q

ONTT - rates of MS based on MRI findings

A

5 years:

-

35
Q

ONTT - bad prognostic factors for development of MS

A

previous attacks of optic neuritis
history of episodes of neurologic symptoms

and MRI findings

36
Q

ONTT - protective factors for MS

A

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

37
Q

Where is the lesion - spasmus nutans

A

Rule out chiasmal glioma

38
Q

Where is the lesion - gaze evoked nystagmus

A

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

39
Q

Where is the lesion - rebound nystagmus

A

Cerebellar lesion

40
Q

Where is the lesion - upbeat nystagmus

A

Medulla

41
Q

Where is the lesion - downbeat nystagmus

A

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

42
Q

Where is the lesion - ocular bobbing

A

bilateral pontine hemorrhage

43
Q

Where is the lesion - oculopalatal myoclonus

A

Cerebellar flocculus to medullary olive

44
Q

Where is the lesion - PAN

A

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

45
Q

Where is the lesion - oculomasticatory myoryhtmia

A

Whipple’s dz (duodenal bx)

46
Q

Where is the lesion - convergence retraction nystagmus

A

Lateral medulla

47
Q

Where is the lesion - Brun’s nystagmus

A

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
Q

Where is the lesion - opsoclonus

A

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

49
Q

Where is the lesion - pendular nystagmus

A

Not localising

50
Q

General ddx for nystagmus

A
Lesions (tumor, AVM)
Infarct
MS
Encephalitis
Syphillis
Drugs (anti-convulsants, EToh, lithium)
Nutritional (Wernicke's, parenteral feding)
51
Q

What can you treat nystagmus with

A

Clonazepam, baclofen, gabapentin

same as for muscle spasms/myokymia

52
Q

Features of dorsal midbrain syndrome

A
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
Q

Causes of dorsal midbrain syndrome

A
PINEAL tumor
stroke
AVM
encephalitis
syphillis
drugs (lithium, etoh, anti-convulsants)
nutritional (wernicke's, parenteral feeding)
hydrocephalus
54
Q

Features of congenital nystagmus

A

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
Q

Features of PSP

A

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

56
Q

CN3 syndromes

A

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
Q

What can you see in AR

A
  • either miosis or lid retraction
  • either with adduction or downgaze

(i.e. 4 things that could happen)

58
Q

Causes of AR

A

Tumor
Trauma
AVM
Congenital (marcus-gunn)

NOT ischemic

59
Q

Unique features of CN 4

A
  • only CN to decussate
  • only CN to exit brainstem dorsally
  • longest course (most suspectible to trauma)
60
Q

Features of bilat CN 4 palsy

A

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

61
Q

Features of congenital CN 4 palsy

A

Head tilt on old photos

Large vertical fusional amplitudes (>10D (?))

62
Q

Unique features of CN 6

A

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

63
Q

CN 7 palsy - different causes

A

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
Q

2 types of childhood aberrant regeneration

A
Marcus Gunn jaw wink (CN3/5)
Crocodile tears (CN7 aberrant regen after Bell's)
65
Q

CN’s in marcus-gunn jaw wink

A

CN 3 and 5

66
Q

Pathology affecting cavernous sinus or SOF

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

Cavernous sinus thrombosis
ICA anneurysm
Mets
Pituitary tumors, apoplexy

67
Q

What is Hutchinson’s

A

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

68
Q

DDx of 2nd order Horners

A
Lung tumor (pancoast, neuroblastoma)
Carotid dissection
69
Q

DDx of 3rd order Horners

A

Cavernous sinus lesion, ICA dissection
Orbital apex
Ciliary ganglion

70
Q

DDx of 1st order Horners

A

Hypothalamus lesion
Down through brainstem
Lateral medullary syndrome

71
Q

Features of lateral medullary syndrome (ocular and systemic)

A

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
Q

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

A

Ocular:

  • Decreased EOM
  • Ptosis
  • Xmas-tree cataract
  • Light-near dissociation

Systemic:

  • MR
  • Frontal baldness
  • Testicular atrophy
73
Q

What tests for MG

A
  • Tensilon
  • Ice pack
  • rest
  • ACH- receptor antibodies
  • EMG
  • Mestinon trial
74
Q

What on history for MG

A
  • alternating and variable ptosis
  • variable diplopia
  • symptoms worse at the end of the day
  • symptoms worse with prolonged use
75
Q

Always rule out with MG

A

thymoma (chest CT)

thyroid (TSH, free T3, T4)

76
Q

Hereditary Optic Neuropathies

A

LHON (15-30 yo) - mitochondrial

Behr (

77
Q

Mutations in LHON

A

11778 - most common
14484 - best prognosis
3460

78
Q

You suspect hereditary optic neuropathy. What do you do?

A

FA
Maybe CT. Have to rule out other causes.

Genetics
Avoid toxins (smoking, etoh, etc)
79
Q

Causes of IIH

A
OCP
Vitamin A compounds (e.g. retinoic acid)
Tetracyclines
Steroid use/withdrawal (Addison's dz)
Young, overweight females
Pregnancy
80
Q

How do you treat IIH

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