Week 2 - Neuro 1.2 Flashcards

1
Q

What 3 things should be examined when suspecting optic atrophy?

A

• Comparison of colour of 2 discs
- Subtle Pallor; watch if pseudophakia
• Evaluation of surface vasculature of disc
- Thin or absent capillary net
• Assessment of retinal nerve fibre layer
- dropout may precede atrophy, loss of normal glistening appearance, most likely superior than inferior first

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2
Q

What is optic atrophy? And how long for it to appear? What separates unilateral from bilateral?

A

• Pale (chalky white) disc appearance with sharp margins
• Damage to retinal ganglion cells at any level
• Lesion in optic pathway anterior to lateral geniculate body
- Anterior to chiasm - unilateral; Posterior to chiasm - bilateral

• 4 to 6 weeks to appear from time of axonal damage

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3
Q

What are the two types of optic atrophy?

A

• Primary Vs secondary

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4
Q

What is Primary optic atrophy and its causes?

A

No adjacent swelling of ON head
Causes:
• Retrobulbar neuritis
• Compression by tumour or aneurysm

• Hereditary optic neuropathies
• Toxic and nutritional optic neuropathy

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5
Q

What is secondary optic atrophy?

A

Proceeded by swelling of ON head

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6
Q

How is Primary and Secondary optic atrophy managed?

A

Primary:
• Retrobulbar neuritis; needs scan for MS
• Compression by tumour or aneurysm; emergency referral
• Hereditary optic neuropathies; H&S to check if any in family has it, referral
• Toxic and nutritional optic neuropathy; Depends on problem, different for diet/drugs/alcohol, GP/referral
Secondary:
• Swelling of ON head; depends on cause or swelling on ON head

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7
Q

What is Optic Disc Oedema?

A

• Swelling of nonmyelinated nerve fibres from impaired axoplasmic flow
- Evidence of acute / evolving pathology
• Blurred disk margins

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8
Q

What terms can be used to describe the appearance on record card?

A

• Elevated appearance of nerve head
• Filling of cup
• Retinal vessels drape over disc margin
• Blurring of margin
• RNFL oedema - greyish appearance obscuring vessels
• Hyperaemia
• Retinal venous dilation and toruosity
• Peripapillary haemorrhages/exudates

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9
Q

What are the different types of peripapillary haemorrhages and exudates?

A

• Dot-Blot haemorrhages
• Flame shaped haemorrhages
• Exudates
• Cotton wool spots

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10
Q

What are the differences between the two retinal haemorrhages?

A

Flame shaped haemorrhages:
• from superficial pre-capillary arterioles
• in NFL
Dot-blot haemorrhages:
• from venous end of capillaries
• in middle retinal layers

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11
Q

What are the differences between the two main types of Oedema?

A

• Oedema
- Diffuse: caused by extensive leakage
- Localised: focal leakage
Between OPL and INL, may later involve IPL and NFL

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12
Q

What are hard exudates?

A

• Caused by oedema
- Chronic localised oedema
• Located at junction of normal and oedematous tissue
• Lipoprotein and macrophages
• Mainly in OPL
- Get spontaneously absorbed as oedema recovers

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13
Q

What are cotton wool spots?

A

• Accumulation of neuronal debris in NFL
• Disruption of axial flow of neurons

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14
Q

What are the 4 causes of optic oedema, and their subcategories?

A

• Papilloedema (acute and 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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15
Q

What is Papilloedema? What is physiological cause?

A

• Raised intracranial pressure
• Pressure is transmitted to the optic nerve
• Optic nerve sheath acts as a band to impede axoplasmic transport so there is a build up of material at the lamina cribrosa
• BILATERAL

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16
Q

What causes raises intracranial pressure?

A

• Relates to Cerebrospinal fluid - CSF
- produced in centre of brain; choroid plexus, to provide cushion/buffer for brain
- occupies subarachnoid space
- if problem along that pathway; this will raise the CSF, causing papilloedema

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17
Q

What can cause a disruption of CSF and therefore raised intracranial pressure?

A

• Intracranial mass
• Hydrocephalus (dilation of ventricles)
• Central nervous system infection e.g Meningitis
• Trauma
• Infiltration e.g. leukaemia, sarcoidosis
• Benign intracranial hypertension

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18
Q

What is Benign intracranial hypertension?

A

• Raised ICP in the absence of an intracranial mass, lesion or hydrocephalus and normal CF constituents
• Not life threatening but permanent, often severe, visual damage (fields more than VA)

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19
Q

What is the most common symptom of papilloedema, especially in the early stages?

A

• Occasionally asymptomatic

• Headaches
- “muzzy headed” at start, then develop into extreme pain; hospital at 6 weeks
- Characteristic in morning, waking up px
- generalised or localised
- Worse when pressure increases: moving head, bending over, coughing etc
• Very rarely is a headache absent

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20
Q

Apart from headaches, what are other systemic symptoms of papilloedema?

A

• Nausea and vomiting
- Often projectile
- May partially/temporarily relieve headache
- Could occur at time of headache onset or earlier (up to few months)

• Deterioration of consciousness
- From slight (drowsy) to dramatic

• Pulsatile tinnitus

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21
Q

What are the visual symptoms of papilloedema?

A

• Visual symptoms often absent
• Transient visual loss
• Horizontal diplopia (6th nerve palsy)
• Constriction of visual field
• Altered colour perception
• Reduced VA in later stages

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22
Q

What are the four stages if Papilloedema?

A

• Acute/early
• Acute/established
• Chronic
• Atrophic

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23
Q

What are the symptoms and examinations if acute/early papilloedema?

A

• Symptoms
- Headache
- Nausea and vomiting
- Vision normal and no visual symptoms

• Examination
- VA usually normal
- Colour vision usually normal
- Pupil responses normal

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24
Q

How might the disc appear in acute/early papilloedema and what terms might describe it?

A

• Hyperaemia
• Dilatation of capillary net
• Oedematous retinal nerve fibre layer seen as obscuration of disc edge and vessels
• Mild elevation

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25
What absent spontaneous venous pulsation?
• If SVP present, papilloedema unlikely • absence of SVP does not confirm papilloedema as 20% of normal individuals don't have this • Loss of previous SVP more indicative of papilloedema
26
What are the symptoms and examinations of acute/established papilloedema?
Symptoms As early stage but includes: • Transient visual disturbance, lasting seconds Examination As early stage but includes: • Visual fields: enlargement of blind spot
27
How does the fundus appear for acute/established papilloedema?
As early, but includes: • Severe hyperaemia • Moderate elevation, enlarged optic nerve head and retinal folds •Peripapillary flame shaped haemorrhages and cotton wool spots • Hard exudates in macular fan with temporal part missing
28
What are the symptoms and examinations of chronic papilloedema?
Symptoms • Vision variable, fields restricted Examination • Gradual deterioration in optic nerve function • VA variable • Visual fields: - nasal loss - arcuate defect - generalised depression • central loss a late finding
29
What is the disc appearance of chronic papilloedema?
• Pale due to axonal loss • Marked elevation of discs • Absence of cotton wool spots and haemorrhages • Optociliary shunt vessels / collateral vessels - Pre-existing venous channels - Chronic central retinal vein obstruction - Refractile bodies at disc due to chronic lipid rich exudation • High water marks
30
What are the symptoms/examinations and optic disc appearance of Atrophic Papilloedema
(secondary optic atrophy) • Symptoms/examination VA, fields etc severely reduced • Optic disc appearance Pale/grey optic nerve Possibly mild elevation, indistinct margins
31
Management if Papilloedema?
• Same day referral to ophthalmologist • Treatment of cause
32
What are the differential diagnosis for raised discs?
• Compression • Infiltration • Congenital optic neuropathies (Leber's optic neuropathy) • Toxic optic neuropathies (methanol poisoning) • Traumatic optic neuropathy
33
What other conditions could cause APPEARANCE of a raised disk (optic neuropathy without swelling)
• Optic disc drusen • Tilted optic disc • Myelinated nerve fires • Hypermetropic crowded disc
34
What Intraocular diseases could cause APPEARANCE of a raised disk (optic neuropathy without swelling)
• Central retinal vein occlusion • Posterior uveitis • Posterior scleritis • Hypotony
35
What is anterior ischaemic optic neuropathy? (AION)
• Most common optic neuropathy over 50 years • Represents ischaemic damage to optic nerve head
36
What is the carotid artery and its divisions?
• comes from heart • External carotid artery supplies face • Internal carotid artery supplies brain/eyes etc
37
What are the divisions of the internal carotid?
• Ophthalmic artery divides into multiple branches: - Central retinal artery - posterior ciliary artery : PCA, feeds choroid. Around 6-12 in total.
38
What type of vision loss do you get with anterior ischaemic optic neuropathy? (AION)
Visual loss (both types) • Painless, monocular visual loss over hours to days • Altitudinal defects most common • Reduced central vision Relative afferent pupillary defect Optic disc oedema present from onset
39
What are the two subtypes of Anterior ischaemic optic neuropathy?
• Arteritic anterior ischaemic optic neuropathy (AAION) • Non-Arteritic Anterior ischaemic optic neuropathy (NAION)
40
what is Arteritic anterior ischaemic optic neuropathy (AAION) caused by?
• 5-10% of AION cases • Occurs in older patients • Caused by Giant Cell Arteritis (GCA) - Inflammatory and thrombotic occlusion of short posterior ciliary arteries causing optic nerve head infarction
41
What percentage of (AAION) have systemic symptoms, and how is it related to GCA?
• Variable, no systemic symptoms in 20% • Giant Cell Arteritis 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
42
What are the AAION systemic symptoms?
• Usually 60-80years old • Tender, hardened, non pulsatile temporal artery • Scalp tenderness, especially on brushing hair • Jaw claudication (pain on speaking or chewing, almost pathognomonic) • Proximal muscle weakness (typically shoulders), may occur 1st
43
What affects on health might AAION cause to a patient?
• Reduced appetite - Unexplained weight loss - Unexplained lethargy, malaise, depression As a result of all the systemic conditions
44
What are the visual symptoms of AAION?
• Sudden, profound, visual loss - Usually unilateral (initially) • May be proceeded by transient visual obsurcations, flashing lights • Periocular pain
45
What will you find during the fundus examination AAION?
• Severe visual loss • Pale swollen disc • Cotton wool spots (retinal ischaemia) • Over 1-2 months, swelling resolves leaving optic atrophy
46
How is AAION managed in practice?
• If in community, immediate (same day) referral to ophthalmologist • Treatment aimed at preventing blindness of 2nd eye
47
How is AAION managed in ophthalmology?
• Immediate therapy is essential • Confirmed with immediate blood results ( • Temporal artery biopsy confirms diagnosis • Usually overnight stay in hospital, High dose systemic steroids (IV usually) • Blood tests aid in tapering of steroids which is done in response to serial blood tests and symptoms • Patients may remain on oral steroids for years (average 1-2yrs)
48
What is the prognosis of AAION?
• Visual loss usually permanent • Prompt administration of steroids may allow partial visual recovery In 25% of cases 2nd eye affected despite treatment, this could be within days
49
What is Non-Arteritic Anterior Ischaemic Optic Neuropathy (NAION) and its risk factors?
• 90% of AION cases • Occlusion of short posterior ciliary arteries causing infarction of optic nerve head • Typically in 55-70yrs (average 60yrs), younger compared to AAION • Structural "crowding" of the disc when cup is small/absent Common risk factors include: • diabetes • hypertension • high cholesterol • smoking
50
What are NAION symptoms?
• Sudden, painless loss of vision • Unilateral • Visual impairment on wakening (nocturnal hypotension?) • Lack of systemic symptoms
51
What do you find during the examination of NAION?
• VA: Moderate to severe reduction in VA in most px 30% have normal or slightly reduced VA • Visual fields: Commonly inferior altitudinal defect • Dyschromatopsia: Proportional to amount of VA loss Most px have no further visual loss although in a small number, visual loss continues for about 6 weeks
52
What would you find during the Fundus examination of NAION?
• Disc oedema diffuse or segmental • Disc hyperaemic with focal telangiectasia on disc surface • Often a few peripapillary splinter-shaped haemorrhages • Atrophy within 3-8 weeks of onset • Contralateral eye usually small with absent cup - “disc at risk”
53
How is NAION managed?
• In community, difficult to differentiate from other causes of swollen disc and AAION • Treat underlying cause • Prophylaxis ? Aspirin is frequently given but does not appear to reduce risk in fellow eye
54
What is the NAION prognosis, progression and risk factors for progression?
• Most px: no further visual loss, some Px 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 • Risk factors for other eye are: - poor VA in 1st eye - diabetes • Pseudo-Foster Kennedy syndrome if contralateral eye affected - Unilateral disc swelling with contralateral optic atrophy in absense of mass compressing nerve
55
What are the differences in Arteritic vs non/arteritic for: age, sex, associations, VA, Disc, ESR, CRP, fluorescein angiography, prognosis and treatment?
• age: Mean 70 vs 60 yrs • sex : F > M vs F=M • associations: Headaches, tenderness vs none • VA: 60% of cases - <6/60 vs >6/60 • Disc: Pale vs hyperaemic disc odema • ESR: Mean 70 vs mean 20-40 • CRP: Elevated vs normal • prognosis: Rarely improves vs 31% improves - Fellow eye >50% vs 10-20% • treatment: systemic steroids vs none
56
What is posterior ischaemic optic neuropathy and its causes?
• Much rarer than AION • Obstruction of plial artery/capillary plexus leading to ischaemia to retrolaminar part of the optic nerve Causes: • After surgical procedure, e.g. of spine - Arteritic (similar to AAION) - Non-arteritic (similar to NAION) Diagnosis after ruling out other causes (compression, inflammation)
57
What are 3 causes optic neuritis?
• Infection of the optic nerve • Inflammation of the optic nerve (non-infectious) • Demyelination of the optic nerve (most common)
58
What are some causes of infection of optic nerve? (optic neuritis)
• local infection e.g. sinus, syphillis, Lyme disease, Herpes zoster, cat-scratch disease • viral infection e.g. chicken pox, whooping cough, glandular fever, measles, mumps • Following an immunisation
59
What are some causes of inflammation of optic nerve? (non-infectious)
• Sarcoid • Autoimmune
60
What is Demyelination of optic neuritis and some causes?
• Demyelination disrupts nerve conduction within brain, brainstem and spinal chord sparing peripheral nerves Causes: • Multiple sclerosis (most common) • Isolated optic neuritis with no other demyelination, but that may subsequently develop • Devic disease or Schilder disease, both rare and bilateral optic neuritis
61
What other symptoms may brain stem demyelination lesions cause?
• cranial nerve palzies • gaze palsies • facial nerve palsies • nystagmus
62
What is multiple sclerosis (MS) and its statistics with optic neuritis?
• 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 neurotis : 15-20% MS px • 50% of those with MS with get optic neuritis • If have optic neuritis, over 10yrs MS risk is 38%
63
What is optic neuritis and its symptoms?
• Infection, inflammation or demyelination of the optic nerve Symptoms: • Monocular visual impairment • Subacute: develops over several days to 2 weeks • Discomfort, exacerbated by eye movements which precedes visual loss in majority of cases • Globe tenderness
64
What visual, field and fundus defects can be found during examination of optic neuritis?
Visual: • VA 6/18-6/60 (or worse) • RAPD • Reduced colour vison / red desaturation • Reduced contrast sensitivity Field defects: • Generalised depression • Nerve fibre bundle defects • Central loss Fundus examination • Normal in most cases (retrobulbar neuritis) • Could get swollen disc
65
What are symptoms/signs of neuroretinitis? (type of optic neuritis)
• Acute loss of vision (usually painless) • Disc edema which is diffuse, spreads to involve around fovea at plexiform layer • Star pattern of exudates at macula
66
What are some causes if neuroretinitis?
• Demyelination rare • 66% secondary to cat-scratch disease • Syphilis • Lyme disease • Viruses
67
How is optic neuritis managed? (in practice and in hospital)
• 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 • Corticosteroids have no long term effect on visual outcome • If relapsing-remitting MS, immunomodulatory therapy for reducing morbidity
68
If a patient has optic neuritis, and is suspected for MS, how is it managed?
• If suspect MS, MRI scan: • Look for periventricular white matter lesions Assesses risk of future MS • No lesions: 25% risk at 15 years • >1 lesion: 72% at 15 years • Overall risk if no scan: 50% Unresolving cases with other signs should raise suspicion of other underlying cause •Blood tests - inflammatory markers, infective serum tests • CSF • Chest X-ray
69
What is the prognosis of optic neuritis?
• Recovery between 1 month- 6 months - 75% recover to 6/9 or better • May be permanent loss of colour perception and contrast sensitivity - due to Optic atrophy after optic neuritis • Pseudo-Foster Kennedy syndrome - If contralateral eye affected
70
What is diabetic papillopathy and its causes/associations?
• Optic disc swelling in diabetic patient • Diagnostic and therapeutic challenge • Rare • Pathogenesis unclear: Caused by capillary damage? • Associations with small cup/disc ratio and rapid reduction in glycemia • Vascular leakage and edema of the nerve fibres Causes: • Chronic ischaemia and secondary nerve swelling
71
What are the symptoms and what is found during the examination techniques of diabetic papillopathy?
Symptoms: • Usually asymptomatic or with mild symptoms Examination: Va: • 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
72
What is found during the fundus examination of diabetic papillopathy?
• Mild disc edema with hyperaemia • Telangiectasis (dilated capillaries) which could be confused with neovascularisation (new blood vessels)
73
How is diabetic papillopathy managed and what’s its prognosis/risk to develop into?
Management: • In community, difficult to differentiate from other causes of swollen disc • Little evidence that treatment is successful Possibly corticosteroids/steroids 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 ischaemic optic neuropathy