CH11. Primary Angiitis of the CNS

Question 1

Most frequent form of vasculitis involving the CNS

Answer 1

Primary Angiitis

Question 2

What are other terms for PACNS?

Answer 2

granulomatouus angiitis of the CNS
giant cell granulomatous angiitis of the CNS
cerebral granulomatous angiitis

Question 3

What are the different pathologic pattern of PACNS?

Answer 3

• infiltrations of the vascular wall with mononuclear cells
• occasional fibrinoid necrosis
• frequent granulomas with epithelioid cells and giant Langerhans cells

Question 4

What is the rule in pathologic picture of PACNS? Preservation of what?

Answer 4

Preservation of the MEDIA

Question 5

What are the different histologic patterns?

Answer 5

• granulomatous inflammatory
• lymphocytic pattern
• acute necrotizing pattern

Question 6

Which arteries are primarily involved by PACNS?

Answer 6

small and middle-sized arteries

Question 7

What is activated in the pathogenesis of PACNNS that contribute to reduction of regional vascular blood flow?

Answer 7

brainstem and noradrenergic and trigeminovascular responses

Question 8

What are the different mechanisms of angitis in the background of lymphoma?

Answer 8

• malignant lymphoproliferative infiltration
• reactivation of some remove viral infection
• non-specific inflammatory mechanisms

Question 9

What is the difference between cerebral amyloid angiopathy and amyloid-beta-related angiitis?

Answer 9

Cerebral amyloid angiopathy is when inflammation around the fibrils is seen described as perivasculitis.

When destruction of the wall due to vasculitic proocess is seen, it is termmed amyloid-beta-related angiitis.

Question 10

What are the demographics of patients with PACNS?

Answer 10

males than females
occurs after 40 years of age

Question 11

What are the most common presenting symptoms of PACNS?

Answer 11

Headache and focal neurologic deficits

Question 12

What is the mechanism of bleeds in patients with PACNS?

Answer 12

secondary to vessel wall weakening resulting from transmural inflammation

Question 13

What percentage of patients with PACNS present with fever?

Answer 13

15%

Question 14

What is the difference between PACNS and RCVS (angiography)?

Answer 14

RCVS are mainly located on medium-sized cerebral arteries and spontaneously resolve within weeks to months, although ischemic or hemorrhagic strokes may occasionally develop.

Question 15

In clinical terms, what is the difference between PACNS and RCVS?

Answer 15

RCVS usually have
- identified triggering condition for vasocontriction
- severe thunderclap headaches
- rapid improvement after nimodipine or other CCB treatment

Question 16

What is the common finding in imaging of patients with PACNS?

Answer 16

Multi-gerritorial bilateral distal acute stroke lesions following a small or middle-sized arteries distribution.

Question 17

What are the combined abnormalities that can make you lean toward PACNS?

Answer 17

• multi-territorial ischemiic strokes
• small hemorrhages and microbleeds
• leukoencephalopathy
• parenchymal and leptomeningeal contrast-enhancement

Question 18

What is still regarded as the gold standard by most clinicians when CNS vasculitis is suspected?

Answer 18

Conventional angiography

Question 19

What is the angiographic semiology of PACNS?

Answer 19

Multifocal and segmental arterial stenosis associated with ectasia and arterial occlusions.

Question 20

What should be included in the brain biopsy of patients with PACNS?

Answer 20

Leptomeninges because its involvement is the dominating pathologic feature in PACNS.

Question 21

What are the preferred biopsy sites?

Answer 21

Prefrontal area or the temporal tip of the non-dominant hemisphere.

Question 22

What is the diagnostic strategy of Moore in PACNS?

Answer 22

1. headaches and multiple neurolgoic deficits that persist for at least 6 months
2. segmental arterial stenosis
3. exclusion of any infectioius or inflammatory cause
4. Inflammatory lesions of the vascular wall on cerebral and/or leptomeningeal biopsy

Question 23

True or False? The classical sausage-like segmental stenosis seem to be more frequent in RCVS than in PACNS?

Answer 23

True.