Learning objectives
- Learning
- Understand
- Integrate
- Reflect
|
Introduction
- Abnormal blood vessels without inflammation
- Can lead to stasis and thrombosis.
Summaries of individual characteristics
| Vasculopathy |
|---|
| Reversible vasoconstriction syndromes (RVCS) |
| Atherosclerosis |
| Susac's syndrome |
| Radiation vasculopathy |
| Ehlers-Danlos disease |
| Kohlmeyer- Degos disease |
| Fibromuscular dysplasia |
| Fabry's disease |
| Moya-moya disease |
| Amyloid angiopathy |
| CADASIL |
| Pseudoxanthoma elasticum |
| Mitochondrial diseases (MELAS) |
Clinical
- Both haemorrhagic or ischaemic stroke may ensure
- Seizures, headache and encephalopathy.
- Coma may develop due to widespread oedema and raised ICP.
- Absence of vasculitis rashes, eyes for inflammatory changes, retina and even perineum for ulceration.
- Renal urine analysis to exclude a glomerulonephritis.
Investigations
- FBC: WCC, DWCC, CRP, ESR and abnormal LFTs, U&E, Urinalysis. Coagulation. Complement levels fall in vasculitis associated with immune complexes
- Anticardiolipin-antibodies, lupus anticoagulant, electrophoresis, CK, LDH, haptoglobin, ferritin, ACE
- Cryoglobulins, TSH, thyroid antibodies, RF, ANA, Anti ds-DNA, anti-histone, complement, anti-Ro [SS-A] and anti-La [SS-B-], c- and pANCA/MPO [myeloperoxidase], anti-endothelial antibodies
- Drug screening, blood cultures, Syphilis serology, borreliosis, hepatitis B, and C, HIV
- CSF analysis may be useful. The CSF may (it is not always) inflammatory with raised protein and white cells but no evidence of organisms or malignant cells or TB which is important especially before considering immunosuppression. However in primary angiitis of the central nervous system the CSF may be normal.
- MRI: Standard imaging will show the characteristic changes of infarction on DWI and ADC maps and gradient echo sequences will show changes. Some MRI sequences will allow evidence of inflammatory changes. 18-fluorodeoxyglucose positron emission tomography scanning can also be useful in showing increased uptake and inflammatory changes in the vessel walls.
- Angiography: Before considering immunosuppression angiography is important and DSA will provide the most useful findings.
- Biopsies: Targeted biopsies leptomeninges can be useful to show evidence fo an active vasculitis process but in segmental disease may be negative
Differential for "Beading" appearance
- Reversible cerebral vasoconstriction syndromes
- Fibromuscular dysplasia, Vasospasm
- CNS infections, Lymphocytic vasculitis
- Cerebral arterial emboli, Atherosclerosis
Management
- Immunosuppression not generally warranted
References