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Vascular Neurology Masterclass > CAPLAN CH12: Non-atherosclerotic vasculopathies > Flashcards

CAPLAN CH12: Non-atherosclerotic vasculopathies Flashcards

1
Q

Major finding is a rash, often with palpable purpruic skin lesions, especially on the legs

A

Hypersensitivity vasculitides

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2
Q

Characteristic findings of FMD in arteriography

A

String-of-beads appearance

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3
Q

The most impressurve feature of carotid artery dissection in the neck?

A

Pain
Ipsilateral throbbing headache and sharp pain in the neck, jaw, pharynx or face

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4
Q

What is the most common artery affected by arterial dissections?

A

Extracranial ICA
- pharyngeal and distal extracranial segments
- segment above the ICA origin

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5
Q

Levels are higher in patients with cervical artery dissections than controls especially in patients with recurrent dissections.

A

Plasma levels of matrix metalloproteinase-2

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6
Q

In patient’s with ICA dissection, describe the Horner’s ?

A

INCOMPLETE. Facial sweat function is preserved because the sympathetic innervation of the sweat glands travels along the ECA.

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7
Q

What happens when ICA dissection extends to the carotid siphon?

A

Ischemic optic neuropathy can develop as a result of decreased perfusion of arteries supplyiing the optic nerves.

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8
Q

Characteristic ultrasound finding of a dissection?

A
  • High amplitude signal
  • With markedly reduced systolic Doppler frequencies
  • Alternating flow directions over the region of the luminal narrowing
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9
Q

When dissections are in the extracranial ICA, what would the TCD show?

A

Diminished intracranial velocieties in the ICA siphon and MCA

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10
Q

Most common angiographic finding in patients with dissections?

A

String sign

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11
Q

Pharyngeal segment aneursym formation can lead to dysfunction of the lower cranial nerves at the skull base. Usual presentation?

A
  1. Dysgeusia
  2. Horner’s syndrome
  3. Atrophy of the tongue
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12
Q

Where is the usual pain of an a vertebral artery dissection?

A

Pain in the posterior neck or occiput and generalized headache

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13
Q

Most common pattern of ischemic brain damate in patients with ECVA dissections?

A

infarctions in the PICA distribution and lateral medullary infarction

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14
Q

Doppler findings of ECVA dissections?

A

Diminished flow in the high neck at the level of tha tlas
Decreased flow in the intracranial VA shown by TCD

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15
Q

Most common locations of ICA dissections in the anterior circulation?

A

Supraclinoid ICA
Maintem of the MCA

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16
Q

Usual duration of anticoagulants in patients with dissections?

A

6 weeks (or later until luminal stenosis improves to the point that flow is not significantly obstructed)

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17
Q

Indications for angioplasy in patients with dissections?

A
  • part of intra-arterial approach to lysing MCA intra-arterial emboli from ICA dissections when ICA is occluded or nearly occluded
  • in patients with continued hypoperfusion
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18
Q

Population wherein FMD is more common?

A

Middle-aged women

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19
Q

Finding in ICA FMD?

A
  • bilateral (86%)is common
  • usually involves the pharyngeal portion of the artery, extends from C1 proximally 7-8cm
  • sparing of the carotid bifurcation and intracranial carotid artery
  • 20% have coexistend FMD in the VAs of the neck
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20
Q

Most common form of FMD affects which layer?

A

Media

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21
Q

Possible mechanisms of stroke in patients with FMD?

A
  1. Altered contractility
  2. Dilatation with aneurysm formation
  3. Tears with intramural hematomas
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22
Q

Treatment modality for FMD

A

Antiplatelet is mainstay therapy for carotid or vertebral FMD
Surgery is rarely recommended

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23
Q

What to check in patients with FMD and high blood pressure?

A

Always check the renal arteries.

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24
Q

Gronblad-Starndberg disease

A

Pseudoxanthoma elasticum

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25
Q

Gene affected by Pseudoxanthoma elasticum

A

ABCC6 gene
- a transmembrane transporter family of proteins

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26
Q

Dermatologic findings of pseudoxanthoma elasticum

A
  1. First, thickened and grooved skin of the fac, neck, axilla, antecubital, inguinal and periumbilical regions
  2. Yellowsih papules and plaques in the mucosa
  3. Then, skin becomes lax and redundant
27
Q

ophthalmologic findings of PSE?

A
  1. Angioid streaks
    2.Peau d’ orange- speckled, yellowish mottling of the posterior pole of the retina temporal to the macula
28
Q

Other complications of PSE?

A
  1. Gastrointestinal bleeding
  2. Cardiac manifestations
29
Q

Arterial findings of PXE?

A
  1. aortic arch and intracranial arteries involved
  2. tortuosity and ectasia of the neck arteris and angiography
  3. lacunar infarcts and white matter ischemia (Biswanger type)
30
Q

Most common type of Ehlers-Danlos syndrome to have cerebrovascular complications?

A

Type IV
(but note that 80% of EDsyndrome have Type I, II or III)

31
Q

Non-neurological features of ED:

A

Easy bruising
Regions of translucent skin
Vascular rupture
Rupture of gravid uterus

32
Q

Important and frequent cerebrovascular complications

A

Carotid-cavernous fistulas
Arterial dissections

33
Q

Most common site of aneurysm formation in patients with Ehlers Danlos?

A

ICA

34
Q

Inheritance pattern of Marfan’s syndrome? And gene affected

A

Autosomal dominant
FBN1
encodes fibrillin-1
found in chromosome 15

35
Q

Phenotype of Marfan’ syndrome that is easily recognizable

A

Long limbs
Pectus chest deformit
Arachnodactyly
Joint laxity
Ophtha: SUBLUXATION OF THE LENS

36
Q

Most of the cerebrovascular events in pateints with Marfan’s syndrome is related to ___.

A

cardiac and aortic manifestations of the condition

37
Q

Autosomal dominant syndrome
bony and connective tissue abnormalities
aortic and arterial aneurysms and dissections

A

Loeys-Dietz syndrome

38
Q

Gene affected in Loey Dietz syndrome?

A

gene encoding transforming growth factor beta receptor (TGFBR1 or 2)

39
Q

Most frequent location of dilatative arteriopathy

A

posterior fossa where the basilar artery or one or both VAs are involved

40
Q

In patients with recurrent ischemia and thrombi with dolichoectatic arteries, ___ may be used to prevent strokes

A

Warfarin

41
Q

Lobes usually affected by amyloid angiopathy?

A

Parietal and occipital

42
Q

Structure of affected arteries in CAA?

A

Distinctive double-barrel lumen with amyloid found in the outer or inner media

43
Q

The Icelandic variety of CAAhas been attributed to abnormal metabloism of __.

A

gamma-trace protein

44
Q

Mechanism of Ab-related angiitis?

A

Breakage of amyloid-containing vessels with release of Ab amyloid into the subarachnoid space inducing an immunological response

45
Q

Brain infarction develops in about ___%of adults with bacterial meningitis, typically ___.

A

15-20%
typically pneumococcal meningitis

46
Q

This presents a characteristic inflammatory disorder involving predominantly the medulla and pontine tegmentum. Lower cranial nerve palsies also develop.

A

Listeria monocytogenes

47
Q

T/F In Lyme borreliosis, strokes are much less common than peripheral nerve and root sensory symptoms?

A

TRUE

48
Q

Sensitivity and Specificity of CSF TB PCR?

A

48-90% Sn
100 Sp

49
Q

Mucor vs Aspergillus
_ usually spread from paranasal sinuses?
_ reaches the cerebral circulation by hematogenous spread usually without meningitis?

A

Mucor
Aspergillus

50
Q

The most common clinical vascular syndrome in VZV?

A

Delayed brain infarction causing hemiplegia contralateral to herpes zoster ophthalmicus

51
Q

VZV uses this connection to infect the MCA, ACA, SCA, etc.

A

trigeminovascular connections

52
Q

Most frequent parasitic infection of the CNS

A

Plasmodium falciparum

53
Q

Angiography and TCD in children with P falciparum infection

A

focal stenosis of the basal intracranial arteries

54
Q

Intestinal involvement (megaesophagus and megacolon) is also common in patients with this parasitic disease

A

Trypanosoma cruzi (Chagas disease)

55
Q

Mechanism of strokes in patients with Chagas disesase?

A

Embolism from heart.
Note that the involvement of the ANS innervation of the heart rather than direct attack by parasites.

56
Q

This syndrome is characterized by a purulent thrombophlebitis of the jugular vein caused by an amebae.

A

Lemierre’s syndrome

57
Q

Polyarteritis nodosa affects which arteries?

A

small-medium sized arteries especially at branch points

58
Q

Most common neurological signs of PAN relate to?

A

mononeuritis multiplex

59
Q

Pulmonary involvement
Asthma
Eosinophilia

A

Churg-Strauss syndrome

60
Q

Are strokes common in hypersensitivity vasculitides?

A

No. (Usually present with neuropathies, plexopathies, and encephalopathies)

61
Q

Necrotizing, granulomatous vasculitis that affects:
lung
sinuses
upper respiratory tract
kidneys

A

Wegener’s granulomatosis

62
Q

The presence of these two correlates to the clinical hypercoagulability of SLE.

A
  1. Lupus anticoagulant
  2. Antiphospholipid antibody
63
Q

Abnormalities of the metabolic pathway of ___, particularly of its cleaving protease, are thought to be important in the pathogenesis of TTP?

A

von Willebrand Factor

Vascular Neurology Masterclass (6 decks)

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