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
AION - Symptoms and Signs
- 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
Arteritic AION
- 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
Arteritic AION - Giant Cell Arteritis
- 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
Arteritic AION - Symptoms
- 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
Arteritic AION - Visual Examination
- 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
Arteritic AION - Management
- 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
Arteritic AION - Prognosis
- 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
Non-Arteritic AION
- 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
Non-Arteritic AION - Risk Factors
- Diabetes - Hypertension - High cholesterol - Smoking
34
Non-Arteritic AION - Symptoms
- 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
Non-Arteritic AION - Examination
- 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
Non-Arteritic AION - Fundus Examination
- 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
Non-Arteritic AION - Management
- 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
Non-Arteritic AION - Prognosis
- 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
Arteritic vs Non-Arteritic
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
Posterior Ischaemic Optic Neuropathy
- 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
Optic Neuritis
- Inflammation, infection or demyelinating process of the ON (Kanski) Retrobulbar neuritis - inflammation behind the ONH
42
Optic Neuritis - Causes
- 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
Optic Neuritis - Demyelination
- Demyelination disrupts nerve conduction within brain, brainstem and spinal cord, spares peripheral nerves
44
Optic Neuritis - Demyelination (Causes)
- 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
Optic Neuritis - Demyelination (MS)
- 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
Optic Neuritis - Demyelination (Symptoms)
- 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
Optic Neuritis - Demyelination (Examination)
- 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
Optic Neuritis - Demyelination (Fundus Examination)
- Normal in most cases - If px experiencing retrobulbar neuritis (inflammation behind ON) - Could get swollen disc
49
Neuroretinitis
- A feature of optic neuritis - optic neuritis including macular and ONH involvement - macular star often forms
50
Neuroretinitis - Signs and Symptoms
- 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
Neuroretinitis - Causes
- Demyelination rare - 66% secondary to cat-scratch disease - Syphilis - Lyme disease - Viruses
52
Neuroretinitis - Management
- 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
Neuroretinitis - Prognosis
- 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
Diabetic Papillopathy
- 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
Diabetic Papillopathy - Symptoms
- Usually asymptomatic or with mild symptoms - Usually diagnosed on examination rather than on symptoms
56
Diabetic Papillopathy - Examination
- 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
Diabetic Papillopathy - Fundus Examination
- Mild disc oedema with hyperaemia - Telangiectasia (dilated capillaries) which could be confused with neovascularisation (new BV's)
58
Diabetic Papillopathy - Management
- 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
Diabetic Papillopathy - Prognosis
- 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)