primary craniocerebral vasculitis n.
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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

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Primary craniocerebral vasculitis

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primary craniocerebral vasculitis
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 arteritis
Giant cell arteritis

Giant 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
  • 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
  • Amyloid-beta related angiitis
  • An unusual complication of Aβ-CAA
  • 5 cases in the last 3 years
  • 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
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 diseases1
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 diseases2
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
  • Behçet’s syndrome
    • Autoimmune disease producing recurrent oro-genital ulcers, uveitis and arthritis
    • 30-40% have CNS involvement typically meningoencephalitis of the brain stem (rhombencephalitis)
    • Vasculitis is reported in venules
vasculitis in systemic diseases3
Vasculitis in systemic diseases
  • Rheumatoid arthritis
    • Vasculitis of the leptomeninges or parenchyma has been reported; this may respond to steroids
  • Hodgkin’s disease
    • Paraneoplastic vasculitis of the CNS is rare with the exception of Hodgkin’s disease
  • Drug induced
    • Vasculitis has been documented in users of cocaine and amphetamines. There is an association between hepatitis B and C and polyarteritis nodosa, which may link vasculitis to drug use
infectious causes
Infectious causes
  • Bacterial
    • Both small and large arteries can be involved in pneumococcal meningitis and tuberculous meningitis, resulting in large infarcts
  • Spirochetes
    • Heubner’s arteritis occurs in meningovascular neurosyphilus; typically involves large extracranial arteries; T.pallidum cannot usually be identified in the vessel wall
    • Lyme disease due to B.burgdorferi which binds to vessel walls and induces vasculitis.
infectious causes1
Infectious causes
  • Viral
    • Herpes zoster infections in immunosuppressed patients can lead to necrotizing/granulomatous/lymphocytic cerebral vasculitis
    • Vasculitis also reported in HIV and after retroviral combination therapy
  • Fungal
    • Commonest infections due to aspergillus, candida, coccidiodes and mucor species
an unusual case of cns vasculitis
An unusual case of CNS vasculitis
  • 50 year old female social worker
  • Past medical history of ulcerative colitis, benign essential hypertension and hypothyroidism
  • Colitis flared up 3-4 months before presentation, treated with prednisolone
  • Flared up 2 weeks before presentation again, treated with prednisolone
case history
Case history
  • Patient underwent investigative colonoscopy 1 day before presenting
  • Presented (Oct 2010) with continuous dull headache present during the work day
  • This had increased in severity by evening
  • Accompanied by slurred speech, unsteady gait and progressive right sided paralysis
case history1
Case history
  • At a local hospital, she was diagnosed with a right sided stroke
  • While in hospital, developed sudden reduction in level of conciousness
  • ‘Grand mal seizure’ lasted 2 minutes
  • Patient was sedated, intubated and transferred to tertiary care hospital





173 involved vessels

88 in grey matter, 85 in white matter

Mean diameter 66.7 ± 3.9µm

Mean ratio of wall thickness to diameter is 0.18 ± 0.01

Vessels < 100µm are venules/veins or arterioles/small arteries

But ratio is ca. 0.1 for venous vessels, ca. 0.5 for arterial, so most are venous

  • Acute vasculitis, mostly venous, in association with ulcerative colitis
  • Predominantly neutrophilic (acute)
  • Venous involvement explains the extensive hemorrhage since tissue is under arterial perfusion pressure