CAPLAN CH12: Non-atherosclerotic vasculopathies

Question 1

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

Answer 1

Hypersensitivity vasculitides

Question 2

Characteristic findings of FMD in arteriography

Answer 2

String-of-beads appearance

Question 3

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

Answer 3

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

Question 4

What is the most common artery affected by arterial dissections?

Answer 4

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

Question 5

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

Answer 5

Plasma levels of matrix metalloproteinase-2

Question 6

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

Answer 6

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

Question 7

What happens when ICA dissection extends to the carotid siphon?

Answer 7

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

Question 8

Characteristic ultrasound finding of a dissection?

Answer 8

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

Question 9

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

Answer 9

Diminished intracranial velocieties in the ICA siphon and MCA

Question 10

Most common angiographic finding in patients with dissections?

Answer 10

String sign

Question 11

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

Answer 11

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

Question 12

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

Answer 12

Pain in the posterior neck or occiput and generalized headache

Question 13

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

Answer 13

infarctions in the PICA distribution and lateral medullary infarction

Question 14

Doppler findings of ECVA dissections?

Answer 14

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

Question 15

Most common locations of ICA dissections in the anterior circulation?

Answer 15

Supraclinoid ICA
Maintem of the MCA

Question 16

Usual duration of anticoagulants in patients with dissections?

Answer 16

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

Question 17

Indications for angioplasy in patients with dissections?

Answer 17

• 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

Question 18

Population wherein FMD is more common?

Answer 18

Middle-aged women

Question 19

Finding in ICA FMD?

Answer 19

• 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

Question 20

Most common form of FMD affects which layer?

Answer 20

Media

Question 21

Possible mechanisms of stroke in patients with FMD?

Answer 21

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

Question 22

Treatment modality for FMD

Answer 22

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

Question 23

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

Answer 23

Always check the renal arteries.

Question 24

Gronblad-Starndberg disease

Answer 24

Pseudoxanthoma elasticum

Question 25

Gene affected by Pseudoxanthoma elasticum

Answer 25

ABCC6 gene
- a transmembrane transporter family of proteins

Question 26

Dermatologic findings of pseudoxanthoma elasticum

Answer 26

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

Question 27

ophthalmologic findings of PSE?

Answer 27

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

Question 28

Other complications of PSE?

Answer 28

1. Gastrointestinal bleeding
2. Cardiac manifestations

Question 29

Arterial findings of PXE?

Answer 29

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)

Question 30

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

Answer 30

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

Question 31

Non-neurological features of ED:

Answer 31

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

Question 32

Important and frequent cerebrovascular complications

Answer 32

Carotid-cavernous fistulas
Arterial dissections

Question 33

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

Answer 33

ICA

Question 34

Inheritance pattern of Marfan’s syndrome? And gene affected

Answer 34

Autosomal dominant
FBN1
encodes fibrillin-1
found in chromosome 15

Question 35

Phenotype of Marfan’ syndrome that is easily recognizable

Answer 35

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

Question 36

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

Answer 36

cardiac and aortic manifestations of the condition

Question 37

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

Answer 37

Loeys-Dietz syndrome

Question 38

Gene affected in Loey Dietz syndrome?

Answer 38

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

Question 39

Most frequent location of dilatative arteriopathy

Answer 39

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

Question 40

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

Answer 40

Warfarin

Question 41

Lobes usually affected by amyloid angiopathy?

Answer 41

Parietal and occipital

Question 42

Structure of affected arteries in CAA?

Answer 42

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

Question 43

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

Answer 43

gamma-trace protein

Question 44

Mechanism of Ab-related angiitis?

Answer 44

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

Question 45

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

Answer 45

15-20%
typically pneumococcal meningitis

Question 46

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

Answer 46

Listeria monocytogenes

Question 47

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

Answer 47

TRUE

Question 48

Sensitivity and Specificity of CSF TB PCR?

Answer 48

48-90% Sn
100 Sp

Question 49

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

Answer 49

Mucor
Aspergillus

Question 50

The most common clinical vascular syndrome in VZV?

Answer 50

Delayed brain infarction causing hemiplegia contralateral to herpes zoster ophthalmicus

Question 51

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

Answer 51

trigeminovascular connections

Question 52

Most frequent parasitic infection of the CNS

Answer 52

Plasmodium falciparum

Question 53

Angiography and TCD in children with P falciparum infection

Answer 53

focal stenosis of the basal intracranial arteries

Question 54

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

Answer 54

Trypanosoma cruzi (Chagas disease)

Question 55

Mechanism of strokes in patients with Chagas disesase?

Answer 55

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

Question 56

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

Answer 56

Lemierre’s syndrome

Question 57

Polyarteritis nodosa affects which arteries?

Answer 57

small-medium sized arteries especially at branch points

Question 58

Most common neurological signs of PAN relate to?

Answer 58

mononeuritis multiplex

Question 59

Pulmonary involvement
Asthma
Eosinophilia

Answer 59

Churg-Strauss syndrome

Question 60

Are strokes common in hypersensitivity vasculitides?

Answer 60

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

Question 61

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

Answer 61

Wegener’s granulomatosis

Question 62

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

Answer 62

1. Lupus anticoagulant
2. Antiphospholipid antibody

Question 63

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

Answer 63

von Willebrand Factor