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:
– 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
• 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 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
• 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 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
• 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
• 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 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
Gradient echo MRI scans
CD3
CD68
LCA
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
Diagnosis
• 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
Thanks!
Questions?