CH11. Primary Angiitis of the CNS Flashcards
Most frequent form of vasculitis involving the CNS
Primary Angiitis
What are other terms for PACNS?
granulomatouus angiitis of the CNS
giant cell granulomatous angiitis of the CNS
cerebral granulomatous angiitis
What are the different pathologic pattern of PACNS?
- infiltrations of the vascular wall with mononuclear cells
- occasional fibrinoid necrosis
- frequent granulomas with epithelioid cells and giant Langerhans cells
What is the rule in pathologic picture of PACNS? Preservation of what?
Preservation of the MEDIA
What are the different histologic patterns?
- granulomatous inflammatory
- lymphocytic pattern
- acute necrotizing pattern
Which arteries are primarily involved by PACNS?
small and middle-sized arteries
What is activated in the pathogenesis of PACNNS that contribute to reduction of regional vascular blood flow?
brainstem and noradrenergic and trigeminovascular responses
What are the different mechanisms of angitis in the background of lymphoma?
- malignant lymphoproliferative infiltration
- reactivation of some remove viral infection
- non-specific inflammatory mechanisms
What is the difference between cerebral amyloid angiopathy and amyloid-beta-related angiitis?
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.
What are the demographics of patients with PACNS?
males than females
occurs after 40 years of age
What are the most common presenting symptoms of PACNS?
Headache and focal neurologic deficits
What is the mechanism of bleeds in patients with PACNS?
secondary to vessel wall weakening resulting from transmural inflammation
What percentage of patients with PACNS present with fever?
15%
What is the difference between PACNS and RCVS (angiography)?
RCVS are mainly located on medium-sized cerebral arteries and spontaneously resolve within weeks to months, although ischemic or hemorrhagic strokes may occasionally develop.
In clinical terms, what is the difference between PACNS and RCVS?
RCVS usually have
- identified triggering condition for vasocontriction
- severe thunderclap headaches
- rapid improvement after nimodipine or other CCB treatment
What is the common finding in imaging of patients with PACNS?
Multi-gerritorial bilateral distal acute stroke lesions following a small or middle-sized arteries distribution.
What are the combined abnormalities that can make you lean toward PACNS?
- multi-territorial ischemiic strokes
- small hemorrhages and microbleeds
- leukoencephalopathy
- parenchymal and leptomeningeal contrast-enhancement
What is still regarded as the gold standard by most clinicians when CNS vasculitis is suspected?
Conventional angiography
What is the angiographic semiology of PACNS?
Multifocal and segmental arterial stenosis associated with ectasia and arterial occlusions.
What should be included in the brain biopsy of patients with PACNS?
Leptomeninges because its involvement is the dominating pathologic feature in PACNS.
What are the preferred biopsy sites?
Prefrontal area or the temporal tip of the non-dominant hemisphere.
What is the diagnostic strategy of Moore in PACNS?
- headaches and multiple neurolgoic deficits that persist for at least 6 months
- segmental arterial stenosis
- exclusion of any infectioius or inflammatory cause
- Inflammatory lesions of the vascular wall on cerebral and/or leptomeningeal biopsy
True or False? The classical sausage-like segmental stenosis seem to be more frequent in RCVS than in PACNS?
True.
