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NeurovascularMedicine.com
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Cerebral (Non Inflammatory) Vasculopathy


Learning objectives

  • To understand the pathophysiology of vasculopathy
  • To understand the Causes and clinical signs and symptoms
  • To understand the Diagnostic tests
  • To understand the Management

Introduction

  • Abnormal blood vessels without inflammation
  • Can lead to stasis and thrombosis.

Summaries of individual characteristics

VasculopathyPoints
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 vasculitic 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, haptoglobulin, 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

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