中枢神经系统血管炎

80% The same pattern of inflammation can be due to other causes
“VASCULITIS” Look-Alikes on Cerebral Angiography
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Prednisolone 1mg/kg daily (max 80mg); Reduce weekly to 10mg/day by 6 months
Differential Diagnosis
1. CVA 2. MS 3. Infection 4. Tumor 5. Specific / systemic vasculitis 6. Toxic 7. Leukodystrophy 8. MERRF, MELAS 9. Hypertensive encephalopathy
HYPERSENSITIVITY VASCULITIDES
-- Henoch-Schonlein purpura -- Drug-induced vasculitides -- Chemical vasculitides -- Essential mixed cryoglobulinemia
MISCELLANEOUS
CONDITION
AUTHOR(S)
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Neoplastic angioendotheliosis
Witt et al.
Spasm after subarachnoid hemnorrhage
ESR, C-reactive protein
Normal ESR for man is age/2, for women is (age +10)/2.
Corrected ESR = ESR – (standard Hct-actual Hct) x 1.75. Standard Hct is 45 for man, 42 for women.
AUTOPSY
BIOPSY
SYMPTOMS OR
CASES
CASES
SIGNS
(N = 45)
(N = 26)
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Altered mentation Headache Hemiparesis Stupor or coma Dysphasia Seizures “Eye signs” Paraparesis Ataxia Fever Papilledema Weight Loss
Initial ESR (n=47)
Less than 20 mm/hr 22
21-40
14
41-60
7
61-80
3
>81
1
47% 30% 15%
6% 2%
Diagnostic studies for CNS vasculitis
TEST CT MRI
ANGIography
BIOPSY
The size is 2-300 micron.
Animal Models
Intrvanous injection of Mycoplasma gallisepticum in turkeys produced similar damage as human vasculitis.
Clinical Presentation
-- Vasculitis associated with neoplasia -- Vasculitis associated with radiation -- Cogan’s syndrome -- Dermatomyositis-polymyositis -- X-linked lymphoproliferative syndrome -- Thromboangiitis obliterans -- Kawasaki syndrome
Central Nervous System Vasculitis
James Jinxing Wang, MD, PhD
Clinical Assistant Professor University of TN Memphis, TN
jjxwangyahoo
Classification of CNS vasculitis
Ferris and Levine
Atherosclerosis
Ferris and Levine
Oral contraceptive use
Irey et al.
Hypertension
with pheochromocayes,
Postpartum
Garner et al.
-- Takayasu’s arteritis -- Temporal (cranial) arteritis
VASCULITIS ASSOCIATED WITH OTHER SYSTEMIC DISEASES
-- Behcet’s disease -- Ulcerative colitis -- Sarcoidosis -- Relapsing polychondritis -- Kohlmeier-Degos disease
Eclampsia
Trommer, Homer, and
Migraine
Schon and Harrison
Postcoital headache (?)
Kapoor, Kendall,
Trauma
Suwanwela and
Surgical manipulation of intracranial arteries
PRIMARY CNS VASCULITIS
History
1922 Harbitz’s first report. 1959 Gravioto and Feigin’s extensive autopsy descriptions 1970s Primary CNS angiitis, Granulomatous angiitis of the CNS, isolated CNS angiitis. 1980s High dose steroid and Cyclophosphamide started. Prognosis is very poor without treatment. Mortality is almost 100% without treatment
INFECTIOUS VASCULITIS
-- Spirochetal (syphilis) -- Mycobacterial -- Fungal -- Rickettsial -- Bacterial (purulent) meningitis -- Viral -- Other organisms
SENSITIVITY
33-50% (even lbiopsy-proven cases)
50-100% (It approaches 100% in histologically confirmed cases, and is lowest in those diagnosed only by angiography)
Pathology of the isolated CNS vasculitis
The essential feature is a giant cell, granulomatous inflammation of the small arteries and veins, which exhibits a nearly constant affinity for the vessels of the leptomeninges and the branches that arise from them to penetrate the cortex.
ESTIMATED SPECIFICITY
Data not available no pathognomonic findings
Data not available no patholognomonic findings
22% Assessed in only one study but may be higher if vasculitis is secondary to other causes are excluded)
NECROTIZING VASCULITIDES
-- Classic polyarteritis nodosa -- Wegener’s granulomatosis -- Allergic Angitis and granulomatosis (Churg-
Strauss) -- Necrotizing systemic vasculitis-overlap syndrome -- Lymphomatoid granulomatosis
30-100% (It is less than 40% in histologically confirmed cases, and 100% in reports not supported by histology)
75% (The negativity can be due to the patchy nature of the disease and small tissue sample
Treatment for CNS vasculitis
CYTOXIC AGENT
CORTICOSTEROIDS
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