Makindo Medical Notes.com

• The inflammation of blood vessels can lead to changes in blood vessel structure and function as well as stasis and thrombosis.
• There are many different types of patterns of pathologies that affect blood vessels and some of these only happen with intracranial vessels.
• Diagnosis is difficult as no specific clinical features nor a classical clinical course, and no blood or imaging
• Contrast catheter cerebral angiography is neither specific nor sensitive

Aetiology

• Possibly due to an immune reaction to an antigenic stimulus
• There is deposition of immune complexes in the vessel walls
• Genetic, environmental and other mechanisms

A size based classification approach is often used.

Summaries of individual characteristics

Clinical

• Elicit a history of connective tissue disease e.g. joint pain and swelling, myalgia
• Both haemorrhagic or ischaemic stroke may ensure
• Inflammatory changes lead to seizures, headache and encephalopathy.
• Coma may develop due to widespread oedema and raised ICP.
• Look for 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

Management

• Management requires the use of toxic immunosuppressive therapy so all attempts must be made to establish a reliable diagnosis and assess if the treatment is more harmful than the vasculitis.
• Standard therapies include steroids and pulse cyclophosphamide as induction treatment.
• Alternative option is the use of the anti-CD20 antibody rituximab.
• Other agents include Methotrexate, azathioprine, and mycophenolate mofetil are recommended as alternatives to Cyclophosphamide once remission is achieved.

References

• Diagnosis and treatment of cerebral vasculitis, Peter Berlit. Ther Adv Neurol Disord (2010) 3(1) 2942