Primary craniocerebral vasculitis Takayasu’s arteritis, giant cell arteritis and primary CNS angiitis all have granulomatous angiitis Takayasu’s arteritis: – This affects the aortic arch, main arterial trunks and descending aorta including the carotid and subclavian arteries – Incidence in Japan is 1 in 3000, mainly in younger women aged 15-45 – The affected arteries become fibrotic, rigid and narrowed, which can lead to cerebral ischemia
Primary craniocerebral vasculitis. Takayasu’s arteritis, giant cell arteritis and primary CNS angiitis all have granulomatous angiitis Takayasu’s arteritis: This affects the aortic arch, main arterial trunks and descending aorta including the carotid and subclavian arteries - PowerPoint PPT Presentation
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Primary craniocerebral vasculitis
Takayasu’s arteritis, giant cell arteritis and primary CNS angiitis all have granulomatous angiitis
Takayasu’s arteritis:– This affects the aortic arch, main arterial trunks and
descending aorta including the carotid and subclavian arteries
– Incidence in Japan is 1 in 3000, mainly in younger women aged 15-45
– The affected arteries become fibrotic, rigid and narrowed, which can lead to cerebral ischemia
Giant cell arteritisGiant cell arteritis:– Most common of the three granulomatous arteritides (2 in
10,000 over age 50)– Targets the extracranial arteries of the head and neck– If the superficial temporal artery is affected it may be
thickened and tender– There is an association with polymyalgia rheumatica, very
elevated ESR and a risk of blindness from extension to the opthalmic arteries
– It responds promptly to steroids– Can also affect the carotid and vertebral arteries leading to
cerebral infarcts
Primary CNS angiitis
• Primary CNS angiitis:– Typically affects women aged 30-50– Angiography shows narrowed segments in
intracerebral arteries– Clinical picture is non-specific (eg headaches,
confusion, memory impairment or focal deficits)– Aggressive immunosuppressive therapy can treat
the disease effectively
ABRA
• Amyloid-beta related angiitis• An unusual complication of Aβ-CAA • 5 cases in the last 3 years
ABRA
• ABRA presents at an older age (mean 67) than primary CNS angiitis (mean 43), but younger than non inflammatory Aβ-CAA (mean 76)
• Primary angiitis cannot be distinguished from ABRA except by pathology
• Biopsies showing vasculitis should be routinely examined for Aβ deposits
Vasculitis in systemic diseases• Lupus
– 50-75% of patients have CNS involvement– Immune complex mediated vasculitis may lead to infarcts, vessels
show fibrinoid necrosis, mononuclear inflammation and fibrosis– However, a predisposition to thrombosis is also advanced as the main
cause of the CNS damage, and some authors discount vasculitis entirely
• Polyarteritis nodosa– Causes necrotizing lesions in medium sized to small arteries– In systemic PAN the visceral organs are targeted– In limited PAN both skeletal muscle and peripheral nerve are involved
with late CNS involvement
Vasculitis in systemic diseases• ANCA associated vasculitides– ANCA stands for anti-neutrophil cytoplasmic antibodies– Wegener’s affects lung and kidney; about 30% develop
CNS complications with focal necrotizing or granulomatous vasculitis
– Churg-Strauss has an allergic component (with asthma and eosinophilia). There is a necrotizing vasculitis in medium sized to small arteries with extravascular granulomas and abundant eosinophils; 60-70% develop peripheral neuropathies or CNS involvement
Vasculitis in systemic diseases• Sjögren’s syndrome
– Autoimmune disorder that results in Sicca syndrome by involving the exocrine glands (salivary and lacrimal)
– Involves peripheral nervous system with varying rates of CNS involvement
– Vasculitis/vasculopathy has been described in venous vessels in CNS white matter and/or the leptomeninges