Neuro 1.2 Flashcards

1
Q

Optic Atrophy

A
  • atrophy - wasting away of the ON
  • generally causes pale coloured nerve
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2
Q

Features of Optic Atrophy

A
  • Pale (chalky white) disc appearance with sharp margins
  • Damage to retinal ganglion cells anywhere along the pathway
  • Lesion anterior to chiasm – unilateral optic atrophy
  • Lesion posterior to chiasm – bilateral optic atrophy
  • Takes around 4-6 weeks to appear from time of axonal damage
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3
Q

Primary and Secondary Optic Atrophy

A
  • Atrophy (paleness of the ONH) with no adjacent swelling of the ONH
  • Atrophy preceded by swelling of the ONH
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4
Q

Management of Optic Atrophy

A
  • Depends on cause
  • Each cause covered in later cards
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5
Q

Optic Disc Oedema

A
  • Swelling of nonmyelinated nerve fibres (at point of ONH) from impaired axoplasmic flow (reduced flow down nerves as swollen)
  • Is evidence of acute/evolving pathology, something happening now to ONH
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6
Q

OD Oedema Appearance

A
  • Elevated appearance of ONH
  • Filling of cup
  • Retinal vessels drape over disc margin
  • Blurring of margin
  • RNFL oedema – greyish appearance that obscures vessels
  • Hyperaemia
  • Retinal venous dilatation & tortuosity
  • Peripapillary haemorrhages & exudates
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7
Q

Causes of Optic Disc Oedema (LEARN!)

A
  • Papilledema (acute & chronic)
  • Ischaemia
    • Arteritic anterior ischaemic optic neuropathy (AION)
    • Non-Arteritic Anterior Ischaemic optic neuropathy (NAION)
    • Posterior Ischaemic optic neuropathy
  • Inflammation
    • Optic Neuritis (demyelinating, infectious, non-infectious)
    • Neuroretinitis
  • Diabetic papillopathy
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8
Q

Causes of a Raised Optic Disc (LEARN!)

A
  • Not swollen but very similar appearance
  • Compression
  • Infiltration
  • Congenital optic neuropathies (Leber’s optic neuropathy)
  • Toxic optic neuropathies (methanol poisoning)
  • Traumatic optic neuropathy
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9
Q

Causes of APPEARANCE of a Raised Optic Disc (LEARN!)

A
  • Optic disc drusen
  • Tilted optic disc
  • Myelinated nerve fibres
  • Hypermetropic crowded disc
  • Intraocular disease
    • CRVO
    • Posterior uveitis
    • Posterior scleritis
    • Hypotony
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10
Q

Causes of Optic Disc Oedema

A
  • Papilledema (acute & chronic)
  • Ischaemia
    • Arteritic anterior ischaemic optic neuropathy (AION)
    • Non-Arteritic Anterior Ischaemic optic neuropathy (NAION)
    • Posterior Ischaemic optic neuropathy
  • Inflammation
    • Optic Neuritis (demyelinating, infectious, non-infectious)
    • Neuroretinitis
  • Diabetic papillopathy
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11
Q

Papilledema

A
  • Swollen OD secondary to raised intracranial pressure - leads to raised CSF
  • Pressure is transmitted to the ON
  • ON sheath acts as a band to impede axoplasmic transport so there is a build-up of material at the lamina cribrosa
  • Swelling of the ONH results
  • BILATERAL, may be asymmetrical
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12
Q

Papilledema - Causes

A
  • Intracranial mass (benign or cancerous tumour)
  • Hydrocephalus (dilation of ventricles)
  • CNS infection (e.g. Meningitis)
  • Trauma
  • Infiltration (e.g. leukaemia, sarcoidosis)
  • Benign intracranial hypertension
    • Raised ICP in the absence of an intracranial mass, lesion or hydrocephalus and normal CSF constituents (absence of all other causes)
    • Not life threatening but permanent, often severe, visual damage (fields more than VA)
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13
Q

Papilledema - Symptoms

A
  • Occasionally asymptomatic
  • Headaches
    • Can be ‘muzzy headed’ at start and then develop into extreme pain, usually presenting at hospital within 6 weeks
    • Characteristically in morning, waking up px
    • Generalised or localised
    • Worse when pressure increases (moving head, bending over, coughing)
    • Very rarely, headache absent
  • Nausea and vomiting
    • Often projectile
    • May temporarily relieve headache
    • Could occur at time of headache or earlier (up to few months)
  • Deterioration of consciousness
    • From slight (drowsy) to dramatic
  • Pulsatile tinnitus (ringing in the ears)
  • Vision
    • Visual symptoms often absent
    • Transient visual loss
    • Horizontal diplopia (6th nerve palsy)
    • Constriction of VF (pressure building up and pressing on nerves)
    • Altered colour perception
    • Reduced VA in later stages
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14
Q

Papilledema - Stages

A
  • Acute/early
  • Acute/established
  • Chronic
  • Atrophic
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15
Q

Acute Papilledema - Examination

A
  • VA usually normal
  • CV usually normal
  • Pupil responses normal
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16
Q

Acute Papilledema - Disc Appearance

A
  • Hyperaemia
  • Dilation of capillary net
  • Oedematous RNFL seen as obscuration of disc edge and vessels
  • Mild elevation
  • Absent spontaneous venous pulsation (SVP):
    • If SVP present, papilledema unlikely
    • Absence of SVP does not confirm papilledema as 20% of normal individuals don’t have this
    • Loss of previous SVP more indicative of papilledema
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17
Q

Established Papilledema - Examination

A
  • VA normal or reduced
  • VF’s - enlargement of blind spot
  • Plus transient visual disturbance (vision goes and comes), lasting seconds
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18
Q

Established Papilledema - Fundus Appearance

A

(AS PREV STAGE BUT ALSO INCLUDES…)

  • Severe hyperaemia
  • Moderate elevation, enlarged ONH and retinal folds
  • Peripapillary flame shaped haemorrhages and cotton wool spots
  • Hard exudates in macular fan (fan-shaped appearance around the macula) with temporal part missing
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19
Q

Chronic Papilledema - Examination

A
  • VA variable
  • Visual fields:
    • Nasal loss
    • Arcuate defect
    • Generalised depression (function of nerves has generally reduced)
    • Central loss a late finding
  • Gradual deterioration in ON function
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20
Q

Chronic Papilledema - Disc Appearance

A
  • Pale due to axonal loss (been there for a while, paleness takes 4-6 weeks)
  • Marked elevation of discs
  • Absence of cotton wool spots and haemorrhages
  • Optociliary shunt vessels/collateral vessels
    • Pre-existing venous channels start dilating up to try and supply blood to damaged areas
    • Chronic CRVO
    • Refractile bodies (yellow lipid exudates) at disc
  • High water mark (like water arriving on beach shore and leaving) - Sclera starts to bend
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21
Q

Atrophic Papilledema - Examination

A
  • VA severely impaired
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22
Q

Atrophic Papilledema - Disc Appearance

A
  • Pale/grey ON
  • Permanent effect on nerves which are damaged
  • Possibly mild elevation, indistinct margins
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23
Q

Papilledema Management

A
  • Same day referral to ophthalmologist
  • Treatment of cause
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24
Q

Anterior Ischaemic Optic Neuropathy (AION)

A
  • Most common optic neuropathy over 50 years
  • Represents ischaemic damage to ONH
25
Q

AION - Symptoms and Signs

A
  • Visual loss (both types)
    • Painless, monocular visual loss over hours to days
    • Altitudinal defects most common
    • Reduced central vision
  • RAPD
  • OD oedema present from onset
26
Q

Arteritic AION

A
  • 5-10% of AION cases
  • Occurs in older px’s
  • Caused by GCA
  • Inflammatory and thrombotic occlusion of short PCA’s causing ONH infarction
  • Variable, no systemic symptoms in 20%
27
Q

Arteritic AION - Giant Cell Arteritis

A
  • Variable, no systemic symptoms in 20%
  • Granulomatous necrotizing arteritis
  • Affects large and medium sized arteries, especially:
    • Superficial temporal artery
    • Ophthalmic artery
    • Posterior ciliary artery
    • Proximal vertebral artery
  • 5-10% of GCA have AAION
28
Q

Arteritic AION - Symptoms

A
  • Systemic
    • Usually 60-80 yrs old
    • Tender, hardened, non-pulsatile temporal artery
    • Scalp tenderness, especially on brushing hair
    • Jaw claudication (pain on speaking or chewing, almost pathognomonic - very indicative of condition)
    • Proximal muscle weakness (typically shoulders), may occur 1st
    • Reduced appetite
    • Unexplained weight loss
    • Unexplained lethargy, malaise, depression
  • Visual
    • Sudden, profound, visual loss
    • Usually unilateral (initially)
    • May be proceeded by transient visual obscuration’s, flashing lights
    • Periocular pain
29
Q

Arteritic AION - Visual Examination

A
  • Severe visual loss
  • Pale swollen OD (may appear pale initially due to blood supply being lost, complete optic atrophy not present until 4-6 weeks after
  • Cotton wool spots (retinal ischaemia)
  • Over 1-2 months, swelling resolves leaving optic atrophy
30
Q

Arteritic AION - Management

A
  • Immediate, same day referral to ophth
  • Treatment aimed at preventing blindness of other eye
  • As px could get a blockage somewhere else and get a stroke

Management by ophth
- Immediate therapy essential
- Confirmed with immediate blood results
- Temporal artery biopsy confirms diagnosis
- Usually overnight stay in hospital
- High dose systemic steroids
- Px’s may remain on oral steroids for years (average 1-2yrs) to prevent future attack

31
Q

Arteritic AION - Prognosis

A
  • Visual loss usually permanent
  • Prompt administration of steroids may allow partial visual recovery
  • In 25% of cases 2nd eye affected despite treatment, could be within days
32
Q

Non-Arteritic AION

A
  • 90% of AION cases
  • Occlusion of short PCA’s causing infarction of ONH
  • Typically in 55-70 yrs, younger compared to AAION
  • Structural ‘crowding’ of the disc when cup is small/absent
33
Q

Non-Arteritic AION - Risk Factors

A
  • Diabetes
  • Hypertension
  • High cholesterol
  • Smoking
34
Q

Non-Arteritic AION - Symptoms

A
  • Sudden, painless loss of vision
  • Unilateral (papilledema would be bilateral)
  • Visual impairment on wakening (nocturnal hypotension - BP reduces overnight, HR slows etc)
  • Lack of systemic symptoms
35
Q

Non-Arteritic AION - Examination

A
  • VA
    • Moderate to severe reduction in VA in most px (6/24, 6/36)
    • 30% have normal or slightly reduced VA (6/12)
  • Visual fields
    • Commonly inferior altitudinal defect
  • Dyschromatopsia - reduced CV
    • Proportional to amount of VA loss
    • After initial visual loss, most px have no further visual loss (although in a small number, visual loss continues for about 6 weeks)
36
Q

Non-Arteritic AION - Fundus Examination

A
  • Disc oedema diffuse or segmental
  • Disc hyperaemic with focal telangiectasia clustered on disc surface (general dilation of capillaries causing them to appear as small, red or clusters - often spidery which attempt to help with blood supply to that area which is lacking)
  • Often a few peripapillary splinter-shaped haemorrhages
  • Atrophy within 3-8 weeks of onset
  • Contralateral eye usually small with absent cup - ‘disc at risk’
37
Q

Non-Arteritic AION - Management

A
  • Refer to ophth
  • In community, difficult to differentiate from other causes of swollen disc and AAION without appropriate blood tests
  • Treat underlying cause
  • Prophylaxis? Aspirin is frequently given but does not appear to reduce risk in fellow eye
38
Q

Non-Arteritic AION - Prognosis

A
  • Most px have no further visual loss although in a small number, visual loss continues for about 6 weeks
  • Some recovery (e.g. 2 lines) in 31% at 2 years
  • Chance of other eye being affected is 15% over 5yrs (lower than arteritic)
  • Risk factors for other eye are:
    • poor VA in 1st eye
    • diabetes
  • Pseudo-Foster Kennedy Syndrome - unilateral disc swelling with contralateral optic atrophy in absence of mass compressing nerve
39
Q

Arteritic vs Non-Arteritic

A

Arteritic…Non-arteritic
Age Mean 70 years…Mean 60 years
Sex F > M…F = M
Associations Headache, tenderness, etc…Usually none
Visual Acuity <6/60 in >60%…>6/60 in >60%
Disc / fundus Pale disc oedema…Hyperaemic disc oedema
ESR Mean 70…Mean 20-40
CRP Elevated…Normal
Fluorescein Angiography Disc and choroid delay…Disc delay
Natural History Rarely improves…31% improve
Fellow eye >50%…Fellow eye 10 – 20%
Treatment Systemic steroids…None

40
Q

Posterior Ischaemic Optic Neuropathy

A
  • Much rarer than AION
  • Obstruction of plial artery/capillary plexus leading to ischaemia to retrolaminar part of the ON
  • After surgical procedure, e.g. of spine
  • Arteritic (similar to AAION)
  • Non-arteritic (similar to NAION)
  • Diagnosis after ruling out other causes (compression, inflammation)
41
Q

Optic Neuritis

A
  • Inflammation, infection or demyelinating process of the ON (Kanski)
    Retrobulbar neuritis - inflammation behind the ONH
42
Q

Optic Neuritis - Causes

A
  • Infection of the ON
    • Caused by local infection e.g. sinus, syphilis, Lyme disease, HZO
    • Following a viral infection e.g. chicken pox, whooping cough, measles, mumps
    • Following an immunisation
  • Inflammation of the ON (non-infectious)
    • Sarcoid
    • Autoimmune
  • Demyelination of the ON (most common)
43
Q

Optic Neuritis - Demyelination

A
  • Demyelination disrupts nerve conduction within brain, brainstem and spinal cord, spares peripheral nerves
44
Q

Optic Neuritis - Demyelination (Causes)

A
  • Multiple sclerosis (most common)
    • Only diagnosed as MS if 3 or more demyelination lesions
  • Isolated optic neuritis with no other demyelination, but that may subsequently develop
  • Devic disease or Schilder disease, both rare and produce bilateral optic neuritis
  • Brain stem demyelinating lesions may also cause:
    • CN palsies
    • Gaze palsies
    • Facial nerve palsies
    • Nystagmus
45
Q

Optic Neuritis - Demyelination (MS)

A
  • MS is an inflammatory, demyelinating disease of the central nervous system (CNS)
  • Multiple exacerbations characterised by variable CNS involvement
  • Exacerbations are separated in time and anatomical location
  • Optic neuritis is the presenting feature in 15-20% of those with MS
  • 50% of those with MS will get optic neuritis at some point
  • If optic neuritis present, the overall 10 year risk of MS is 38%
46
Q

Optic Neuritis - Demyelination (Symptoms)

A
  • Monocular visual impairment
  • Subacute - develops over several days to 2 weeks
  • Discomfort, exacerbated by eye movements which comes before visual loss in most cases
  • Globe tenderness
47
Q

Optic Neuritis - Demyelination (Examination)

A
  • Reduced VA (6/18 - 6/60)
  • RAPD (affects amount of light being transmitted by one eye, that pupil will dilate when same light is shone in other eye)
  • Reduced CV
  • Reduced CS
  • VF defects
    • Generalised depression
    • Nerve fibre bundle defects
    • Central loss
48
Q

Optic Neuritis - Demyelination (Fundus Examination)

A
  • Normal in most cases
    • If px experiencing retrobulbar neuritis (inflammation behind ON)
  • Could get swollen disc
49
Q

Neuroretinitis

A
  • A feature of optic neuritis
  • optic neuritis including macular and ONH involvement
  • macular star often forms
50
Q

Neuroretinitis - Signs and Symptoms

A
  • Acute loss of vision (usually painless)
  • Disc oedema which is diffuse, spreads to involve around fovea at plexiform layer
  • Star pattern of exudates at macula
51
Q

Neuroretinitis - Causes

A
  • Demyelination rare
  • 66% secondary to cat-scratch disease
  • Syphilis
  • Lyme disease
  • Viruses
52
Q

Neuroretinitis - Management

A
  • In community, difficult to differentiate from other causes of swollen disc
  • Most need no medical treatment
  • Intravenous steroids followed by oral course speed recovery by 1-2 weeks
  • Oral steroids alone associated with increased recurrence rate
53
Q

Neuroretinitis - Prognosis

A
  • Recovery within 1 month, lasting up to 6 months
  • 75% recover to 6/9 or better
  • May be permanent loss of colour perception and contrast sensitivity
  • Will have optic atrophy after optic neuritis
  • Pseudo-Foster Kennedy syndrome may be present
    • If contralateral eye affected
    • Unilateral disc swelling with contralateral optic atrophy in absence of mass compressing nerve
54
Q

Diabetic Papillopathy

A
  • OD swelling in a diabetic patient
  • Difficult to diagnose (very rare)
  • Pathogenesis unclear
  • Caused by capillary damage?
  • Associations with small C:D ratio and rapid reduction in glycemia
  • Vascular leakage and oedema of the nerve fibres
  • Chronic ischaemia and secondary nerve swelling
  • Does not result in full blown ischaemic optic neuropathy
  • Usually diagnosed on examination rather than on symptoms
55
Q

Diabetic Papillopathy - Symptoms

A
  • Usually asymptomatic or with mild symptoms
  • Usually diagnosed on examination rather than on symptoms
56
Q

Diabetic Papillopathy - Examination

A
  • Unilateral
  • VA 6/12 or better
  • Mild or no RAPD
  • Visual field
    • Enlargement of blind spot
    • Constriction or altitudinal loss in severe cases
  • Frequently found on fundus examination
57
Q

Diabetic Papillopathy - Fundus Examination

A
  • Mild disc oedema with hyperaemia
  • Telangiectasia (dilated capillaries) which could be confused with neovascularisation (new BV’s)
58
Q

Diabetic Papillopathy - Management

A
  • In community, difficult to differentiate from other causes of swollen disc
  • Emergency referral to ophth
  • Little evidence that treatment is successful
  • Possibly corticosteroids/steroids
59
Q

Diabetic Papillopathy - Prognosis

A
  • Visual prognosis good: 6/12 or better
  • May take 6 months or longer for oedema to resolve
  • 5 to 15% progress to Non-Arteritic AION (diabetes is a risk factor)