Neurology: Cerebral Venous Thrombosis & Temporal Arteritis Flashcards

1
Q

What is cerebral venous thrombosis (CVT)?

A

It is a rare cause of cerebral infarction resulting from thromboembolic occlusion of cerebral veins and sinuses.

It is a neurological emergency requiring urgent neuroimaging and intervention.

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2
Q

Mean age of CVT onset?

A

37 years (typically affects younger individuals when compared to arterial strokes).

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3
Q

Risk factors for CVT?

A

1) Females (3x): likely as a result of increased risk of thromboembolisms in pregnancy, COCP use.

2) Prothrombotic condition:
- Genetic thrombophilias e.g. Factor V Leiden mutation
- Acquired thrombophilias e.g. pregnancy

3) Infection

4) Trauma & surgery

5) Chronic inflammatory diseases e.g. IBD, SLE

6) Haematological disorders e.g. sickle cell disease

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4
Q

What is CVT caused by?

A

Caused by partial or total occlusion of the cerebral veins and sinuses by a thrombus.

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5
Q

What are some genetic thrombophilias that can predispose to CVT?

A

1) antiphospholipid syndrome

2) antithrombin III deficiency

3) protein C deficiency

4) protein S deficiency

5) Factor V Leiden mutation

6) Hyperhomocysteinemia

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6
Q

What are some acquired thrombophilias that can predispose to CVT?

A

1) pregnancy and the puerperium
2) COCP
3) malignancy

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7
Q

What is the most common cause of infection causing CVT?

A

Staph. aureus - spead from infections of the sinuses.

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8
Q

Give 3 examples of infections causing CVT

A

1) Staph. aureus from sinusitis

2) Meningitis

3) Subdural empyema

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9
Q

What is most common trauma causing CVT?

A

Trauma to the head or neurosurgical procedures.

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10
Q

Give 2 procedures that can cause CVT

A

1) jugular venous cannulation

2) lumbar punctures

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11
Q

Give some examples of chronic inflammatory diseases that can cause CVT

A

1) SLE
2) Behcet disease
3) granulomatosis with polyangiitis
4) sarcoidosis
5) inflammatory bowel disease

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12
Q

What medication increases the risk of CVT?

A

Steroids (and they are commonly used in chronic inflammatory conditions)

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13
Q

What haemological disorders can increase the risk of CVT?

A

1) paroxysmal nocturnal haemoglobinuria

2) thrombotic thrombocytopenic purpura

3) sickle cell disease

4) polycythemia

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14
Q

Describe the venous drainage of the CNS

A

1) the brains venous blood enters numerous superficial and deep cerebral veins

2) these drain into the dural venous sinuses.

3) these eleven sinuses eventually drain into the internal jugular vein and allow venous blood to return to the heart.

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15
Q

What is the 1ary mechanism of injury in CVT?

A

1) a cerebral vein or sinus becomes partially or totally occluded by a venous thrombus

2) deoxygenated blood will begin to pool within the brain parenchyma

3) this causes an increase in cerebral venous pressure

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16
Q

What are the 3 effects of an increase in cerebral venous pressure?

A

1) increased intravascular pressure and reduced capillary pressure which results in decreased cerebral perfusion –> ischaemic parenchymal injury and cytotoxic oedema (movement of fluid into the intracellular space)

2) disruption of the blood-brain barrier resulting in vasogenic oedema (leakage of blood plasma into the interstitial space)

3) cerebral vein and capillary rupture causing parenchymal haemorrhage

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17
Q

What is the 2ary mechanism of injury in CVT?

A

1) Obstruction of the superior sagittal, jugular or lateral venous sinuses

2) Causes decreased CSF reabsorption.

3) Results in raised ICP, and worsening of the venous hypertension, ischaemia and oedema.

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18
Q

Resulting complications from CVT?

A

1) cerebral ischaemia

2) oedema

3) haemorrhage

4) raised ICP

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19
Q

Symptoms of CVT?

A

1) headache

2) seizures

3) focal neurological deficits:
- hemiparesis
- fluent aphasia
- sensory/visual field defects

4) changes in metnal state:
- encephalopathy

Symptoms will vary depending upon the site and extent of thrombosis.

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20
Q

What is the most common site of CVT?

A

Sagittal sinus thrombosis (60%)

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21
Q

How does sagittal sinus thrombosis typically present?

A

Most commonly presents with bilateral motor deficits and seizures

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22
Q

What is the 2nd most common site of CVT?

A

Transverse sinus thrombosis.

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23
Q

How does transverse sinus thrombosis typically presnet?

A

Isolated headache and papilloedema (isolated raised Intracranial pressure).

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24
Q

Which sinus is most likely to be affected by septic CVT?

A

Cavernous sinus - with infection spreading from nearby sinuses, nose and middle ear

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25
Q

How does cavernous sinus thrombosis typically present?

A

Most commonly presents with orbital pain, chemosis, proptosis and CN III, IV, V & VI nerve palsies

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26
Q

How does straight sinus thrombosis typically present?

A

Typically presents with severe symptoms with altered mental state, bilateral motor deficits and reduced GCS.

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27
Q

How does jugular vein thrombosis typically present?

A

Patients present with neck pain, pulsating tinnitus and cranial nerve palsies

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28
Q

CVT can be classified into what two categories depending upon its aetiology?

A

1) aspectic CVT

2) septic CVT

29
Q

What is aseptic CVT?

A

An embolism arising from a non-infective cause, often due to underlying hypercoagulable state but has a variety of causes.

This is the most common type of CVT

30
Q

Where does aspetic CVT typically occur?

A

Most frequently affects the superior sagittal sinus, but may also affect the deep or superficial cerebral veins and other venous sinuses

31
Q

What is septic CVT?

A

An embolism arising from a primary source of infection, most commonly sinusitis, osteomyelitis, otitis media or rarely septicaemia.

32
Q

Where does septic CVT typically occur?

A

1) Most commonly affects the cavernous sinus as a result of facial or orbital infection.

2) Rarely may affect the lateral sinus if infection spreads from the middle ear or mastoid bone.

33
Q

What is the most frequent symptom experienced by CVT patients?

A

Headache

34
Q

1st line investigation in suspected CVT?

A

Urgent neuroimaging –> MRI 1st line

35
Q

What is the most sensitive neuroimaging method for diagnosis of CVT?

A

MRI (T2 weighted) in combination with MR venography.

36
Q

If an MRI is not available, what investigation can be performed in CVT?

A

A cranial CT

37
Q

Other investigations in CVT?

A

1) d-dimer (elevated)

2) lumbar puncture (useful in patients with isolated intracranial hypertension to exclude meningitis)

3) thrombophilia screen

38
Q

Management of CVT involves what 3 key elements?

A

1) acute antithrombotic therapy

2) acute symptom management

3) long term management

39
Q

What is used for acute antithrombotic therapy in CVT?

A

LMWH or UH

40
Q

What does acute symptom management involve in CVT?

A

1) Raised ICP:
- bed elevated
- osmotic therapy (mannitol or hypertonic saline)
- brain herniation may need emergency decompressive surgery

2) Seizures: anticonvulsants

3) Infection/inflammation: Abx, steroids

41
Q

Long term management of confirmed CVT?

A

1) Long-term anticoagulation with warfarin –> INR or 2.5

2) Women taking the COCP will need advice regarding non-oestrogen methods of contraception such as the progesterone-only pill.

3) Women who developed an CVT whilst pregnant will need prophylactic anticoagulation during future pregnancies.

42
Q

What is long-term anticoagulant of choicei n CVT?

A

Warfarin

43
Q

INR target in patients on warfarin in CVT?

A

2.5

44
Q

How long is warfarin continued for in CVT?

A

Provoked CVT –> 3-6 months

Unprovoked CVT –> 6-12 months

45
Q

What are some acute complications of CVT?

A

1) Seizures
2) Hydrocephalus
3) Pulmonary embolism
4) Coma
5) Death

46
Q

What are some chronic complications of CVT?

A

1) Residual epilepsy
2) Psychiatric disease (mostly depression)
3) Visual impairment
4) Residual neurological deficits

47
Q

What is temporal arteritis (also known as giant cell arteritis)?

A

A systemic vasculitis of unknown cause that affects the medium and large arteries.

48
Q

What is the main complication of temporal arteritis?

A

Vision loss (permanent)

49
Q

What condition does temporal arteritis heavily overlap with?

A

Polymyalgia rheumatica (PMR)

50
Q

What age is temporal arteritis more common in?

A

> 50 (70 is most common)

51
Q

Clinical features of temporal arteritis?

A

1) Headache:
- unilateral
- severe
- around temple and forehead

2) Scalp tenderness (e.g., noticed when brushing the hair)

3) Jaw claudication

4) Blurred or double vision

5) Loss of vision if untreated
- may be temporary (amaurosis fugax) or permanent

52
Q

What is the most common presenting symptom in temporal arteritis?

A

Headache

53
Q

How may the temporal artery feel in temporal arteritis?

A

The temporal artery may be tender and thickened to palpation, with reduced or absent pulsation.

54
Q

What is a key investigation in temporal arteritis patients?

A

Vision testing

55
Q

Which artery is typically affected in temporal arteritis that causes vision loss?

A

Occlusion of the posterior ciliary artery (a branch of the ophthalmic artery) –> ischaemia of the optic nerve head.

56
Q

Describe the onset of temporal arteritis

A

Sudden onset (<1 month)

57
Q

what are some associated features that may be seen in temporal arteritis?

A

1) Symptoms of PMR e.g. shoulder and pelvic girdle pain and stiffness)

2) Systemic symptoms e.g. weight loss, fatigue and low-grade fever, anorexia, night sweats)

3) Muscle tenderness

4) Carpel tunnel syndrome

5) Peripheral oedema

58
Q

What is diagnosis of temporal arteritis based on?

A

1) clinical presentation

2) raised inflammatory markers, particularly ESR (usually more than 50 mm/hour)

3) temporal artery biopsy (showing multinucleated giant cells)

4) Duplex US (showing the hypoechoic “halo” sign and stenosis of the temporal artery)

59
Q

What will a temporal artery biopsy show in temporal arteritis?

A

Multinucleated giant cells.

Skip lesions may be present.

60
Q

What would a duplex US show in temporal arteritis?

A

The hypoechoic “halo” sign and stenosis of the temporal artery.

61
Q

Investigations in temporal arteritis?

A

1) raised inflammatory markers
- ESR > 50 mm/hr (note ESR < 30 in 10% of patients)
- CRP may also be elevated

2) temporal artery biopsy

62
Q

Typical ESR level in temporal arteritis?

A

Typically >50 mm/hr

63
Q

Differentials for temporal arteritis?

A

1) migraine

2) central retinal artery occlusion

3) acute glaucoma

4) trigeminal neuralgia

5) MS

64
Q

What is the mainstay of management of temporal arteritis?

A

Urgent high dose glucocorticoids (steroids).

There is usually a rapid and significant response to steroid treatment.

65
Q

When should steroids be started in temporal arteritis?

A

Immediately (before confirming diagnosis) to reduce risk of vision loss.

66
Q

Describe initial steroid treatment in temporal arteritis:

a) with no visual symptoms or jaw claudication

b) with visual symptoms or jaw claudication

A

a) 40-60mg oral prednisolone daily

b) 500mg-1000mg IV methylprednisolone daily

Once the diagnosis is confirmed and the condition is controlled, the steroid dose is slowly weaned over 1-2 years.

67
Q

What 3 other medications may be indicated in temporal arteritis management?

A

1) Aspirin 75mg daily: decreases vision loss and strokes

2) PPI e.g. omeprazole: for gastroprotection while on steroids

3) Bisphosphonates and calcium and vitamin D for bone protection while on steroids

68
Q

Complications of temporal arteritis?

A

1) vision loss

2) steroid related complications (e.g. weight gain, diabetes and osteoporosis)

3) Cerebrovascular accident (stroke)

69
Q
A