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CNS VASCULITIS
Dr Sankalp MohanSenior Resident
Neurology GMC, Kota
VASCULITIS The vasculitides are defined by the presence
of inflammatory leukocytes in vessel walls with reactive damage to mural structures.
Compromise of the lumen leads to downstream tissue ischemia and necrosis
Pathogenetic mechanisms – immune complex deposition , invasion of endothelial cells by microorganisms , autoantibodies ,granuloma formation
CLASSIFICATION OF VASCULITIS
•Takayasu•Giant Cell ArteritisLarge vessel
•PAN•Kawasaki s disease•Primary CNS vasculitis
Medium vessel
•Microscopic Polyangiitis•Churg Strauss Syndrome•Wegner s Granulomatosis
Small Vessel
CNS VASCULITIS
CLASSIFICATION
Primary angiitis of the CNS (PACNS) when it is confined to the CNS.
Secondary when associated with various other disorders.
Reversible cerebral vasoconstriction syndromes
PRIMARY CENTRAL NERVOUS SYSTEM VASCULITIS (PACNS)
. Defined as inflammation of the cerebral vasculature without angiitis in other organs
affects small- and medium-sized arteries of the brain parenchyma, spinal cord, and leptomeninges
1. Granulomatous angiitis of the CNS (GACNS)
2. Atypical PACNS
GRANULOMATOUS ANGIITIS OF THE CENTRAL NERVOUS SYSTEM
20% of all patients with PACNS male-predominant (2:1) at any age mean age at diagnosis is
42 years, long prodromal period insidious onset
of symptoms
PATHOLOGY Langerhans or foreign body giant cells,
necrotizing vasculitis, or lymphocytic vasculitis
Atypical PACNS –biopsy is not granulomatous , only lymphocytic infiltrate
vessels to become narrowed, occluded and thrombosed – ischemia
In contrast to systemic vasculitides, the finding of aneurysms in the cerebral arteries is rare
PATHOGENESIS The cause of PACNS is unknown Infection — Infectious agents,
particularly mycoplasma and viruses varicella zoster virus, West Nile virus HIV infection can cause both a CNS
vasculitis and a CNS vasculopathy that is associated with aneurysm formation
Amyloid angiopathy
CLINICAL MANIFESTATIONS characterized by a long prodromal
period Signs and symptoms of systemic
vasculitis, such as, fever, weight loss, or rash, are usually lacking.
highly variable and nonspecific PACNS should be suspected when
strokes, more often recurrent, occur in young patients
& unexplained diffuse neurologic dysfunction
Decreased cognition — 83 percent Headache — 56 percent Seizure — 30 percent Stroke — 14 percent Cerebral hemorrhage — 12 percent Angiitis affecting the spinal cord usually
presents as a myelopathy /spinal cord infarction
PROPOSED THE CRITERIA FOR THE DIAGNOSIS OF PACNS.
The presence of an acquired and otherwise unexplained neurologic deficit and with (a) the presence of either classic
angiographic or histopathologic features of angiitis within the CNS, and
(b) no evidence of systemic vasculitis or any condition that could elicit the angiographic or pathologic features
DIAGNOSTIC TESTS erythrocyte sedimentation rate (ESR) and C-
reactive protein (CRP) are usually normal Serologic evaluation Antinuclear antibodies Rheumatoid factor Antibodies to the Ro/SSA, La/SSB, Sm, and RNP antigens Antibodies to double-stranded DNA Antineutrophil cytoplasmic antibodies (ANCA) Serum C3 and C4 Serum cryoglobulins
Testing for infections appropriate to the clinical circumstances
Lumbar puncture – CSF is abnormal in 80 to 90 percent of
patients with pathologically documented disease. There are no specific abnormalities of the CSF in PACNS.
excluding any infectious or malignant process RCVS – CSF normal
NEUROIMAGING MR imaging = single or multiple, may include infarcts (both
white and gray matter) and hemorrhage, and may be tumor-like
Nonspecific high-intensity T2WI/FLAIR lesions in white matter present in 42 percent of Secondary vasculitis (low specificity )
MR angiography — The resolution of MR angiography (MRA) remains inadequate for the demonstration of vasculitic changes
NEUROIMAGING Angiography positive findings if focal or diffuse areas of
arterial stenosis, occlusion, dilatation, or beading were detected
findings of ectasia and stenosis referred to as "beading", usually in the small arteries
sensitivity of angiography in biopsy proven PACNS cases was only 60 percent
Thus, a negative angiogram cannot be used to exclude the diagnosis of PACNS.
Brain and leptomeningeal biopsy
REVERSIBLE CEREBRAL VASOCONSTRICTION SYNDROMES
group of disorders linked by prolonged but reversible vasoconstriction of the cerebral arteries
severe, acute-onset headaches Ischemic stroke and/or hemorrhage associated
with reperfusion may occur in the setting of RCVS. pheochromocytoma and poorly-controlled
hypertension can also be associated with cerebral vasospasm and clinical presentations that mimic PACNS.
RCVS AND PACNS Symptom onset Clinical setting Reversibility of angiographic findings Cerebral spinal fluid (CSF)
SYSTEMIC VASCULITIS INVOLVING THE BRAIN
systemic vasculitides, connective tissue disease (CTD),
sarcoidosis, infections, and lymphoproliferative diseases.
INFECTIOUS CAUSES 1. VZV -associated cerebral angiitis affects older age groups disease tends to be more localized Cerebral angiographic findings of segmental,
unilateral involvement of the vessels in the distribution of the middle cerebral artery/ICA
Diagnosis -higher antibodies levels of VZV in the CSF than in the serum
positive VZV PCR in the CSF
(35%) of pathologic findings of AIDS-associated CNS disease demonstrate encephalitis, leptomeningitis, and/or vasculitis,
Treponema pallidum – neurosyphilis most common in patients with HIV infections Meningitis and meningovascular disease are
the usual manifestation. May present as stroke
CNS vasculitis associated with hepatitis C virus (HCV) without underlying cryoglobulinemia
SYSTEMIC VASCULITIDES AFFECTING CNS Temporal Arteritis Takayasu s Arteritis Wegener Granulomatosis Henoch Scholein Purpura Polyarteritis Nodosa
TEMPORAL ARTERITIS >50 years age .Women >men Transmural inflammation of arteries .intimal
hyperplasia Branches of ECA – sup temporal most common ICA ,Subclavian ,coronary and intracranial Systemic features fatigue, weight loss , anorexia - Headche (new onset )unilateral localised to
areas of scalp ,Jaw claudication,scalp tenderness -Neuropathy – Median nerve ,TIA and stroke
Cranial neuropathies – AION .23 % of cases Temporary or permanent blindness Posterior ciliary arteritis Diagnosis – anemia ,High Esr ,CRP Angiography – stenotic segments or complet
occlusions Biopsy – Transmural inflammation,occlusion Sensitivity – 70 % Treatment – Prednisolone -40 -80 mg /day >high dose
for a month then taper .50% by one month.1mg/month
POLYARTERITIS NODOSA Medium vessel vasculitis 40 -60 yrs . Asso - Hep B,C ,cryoglobulinemia Inflammation of PMN, Monocytes – Fibrinoid necrosis Most common neurologic manifestation –
Mononeuritis multiplex CNS- 20 % - 40 %.diffuse
encephalopathy ,focal ..infarcts in spinal cord ,brainstem.Rarely ICH,
Optic neuropathy (AION) CNS abnormalities are late -after systemic signs and
PN
Diagnosis – Clinical Multisystem involvement –Skin ,PN,
Renal ,GIT Fever ,weight loss ,anemia raised ESR Angiography – may show saccular or
fusiform aneurysms Treatment – Systemic steroids with
cytotoxic agents – cyclophosphamide azthioprine
TAKAYASU S ARTERITIS 10 -30 yrs of age .women more commonr ,fatigue ,anoreixa Aorta and its branches innominate,common
carotid ,subclavian ,abdominal aorta ,celiac ,mesentric Symptoms – Acute stage – low grade fever Stage of acute inflammation – Vascular Bruits ,diminished
pulses,hypertension Chronic phase- Claudication ,ischemic ulcers Neurologic – Headche ,syncope blurred vision . DIAGNOSIS –Arteriography – occlusion or dilatation/Stenosis
aneurysm of entire aorta or its primary branches TREATMENT – Prednisolone 1mg/kg /d for 3 months –taper In unsuccesful cases cyclophosphamide ,MTX
WEGNER S GRANULOMATOSIS More in men 40 yrs Small and medium sized vessels Necrotizing granulomatous lesion in upper ,lower
respiratory tract , Kidneys 50 % develop neurologic involvement Most have recurrent
mononeuropathies,mononeuritis multiplex, Cranial neuropathies II,VI,VII
Ischemic stroke ,encephalopathy ,granulomatous basilar meningitis , pachnymenigitis – less frequently
Diagnosis –Clinical picture with c-ANCA Radiographically confirmed vasculitis of
the CNS in Wegener’s granulomatosis is rare, because the small vessel
MR findings – Dural thickening and enhancement hyperintense brainstem signals
Treatment – corticosteroids plus cyclophosphamide/azathioprine
BEHCET S DISEASE Oral ulcers ,Genital ulcers and
Uveitis,Skin finding ,(folliculitis and erythema nodosum ),Thrombophlebitis
Neurologic involvement occurs in 6-10 %
CONNECTIVE TISSUE DISORDERS CTDs that can involve the CNS include Sjogren’s syndrome, rheumatoid
arthritis, mixed CTDs, and dermatomyositis
NEUROLOGIC MANIFESTATIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS
seen in 10 to 80 percent of patients Direct involvement of CNS Secondary to complications of
disease /Treatment
PATHOPHYSIOLOGY initially thought to be due to vasculitis. vasculopathy is characterized by a small
to moderate perivascular accumulation of mononuclear cells, without destruction
Accelerated atherosclerosis may contribute to the risk of stroke
Autoantibodies — The demonstration of a number of autoantibodies including APLA
Secondary factors — Prospective studies suggest that from 50 to 78 percent of neurologic episodes are caused by secondary factors [ 2,35 ], including:
Infections associated with immunosuppressive therapy
Metabolic complications of other organ system failure, such as uremia
Hypertension Toxic effects of therapy (particularly corticosteroids)
COMMON CLINICAL SYNDROMES
REFERENCES CNS Vasculitis in Autoimmune Disease: MR Imaging
Findings and Correlation with Angiography -- AJNR 1999 20: 75-85
Central nervous system vasculitis - Rula A. Hajj-Alia and Leonard H. Calabreseb -, Cleveland Clinic, Cleveland, Ohio, USA ; Current Opinion in Rheumatology 2009, 21:10–18
Neurology Clinics –Neurology and systemic Disease . Feb 2010,vol28
Caplan’s – Stroke –A clinical approach 4th edition ,Chapter 11-Non atherosclerotic vasculopathies
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