|Neurological History taking
| Causes of Stroke
Sneddon syndrome is characterized by livedo reticularis associated with cerebrovascular disease.
- A somewhat rare and progressive syndrome
- 4 per million, affects young women 20-40 predominantly
- Described by Sneddon in 1966 - Cerebrovascular lesions and livedo reticularis
- Non-inflammatory thrombotic vasculopathy
- Eventually leads to dementia for half
- Thrombotic occlusions, arteriovenous malformation, and moya-moya type anastomoses
- Heterozygous missense mutations in the CECR1 gene
- Systemic lupus erythematosus
- Antiphospholipid syndrome
- Behcet disease
- Mixed connective tissue disease
- Patients may have transient "episodes" or "spells", chronic headaches
- Stroke/TIA events: hemiplegia, aphasia, hemianopia and hemianesthesia
- Subarachnoid haemorrhage, spinal stroke
- Livedo racemosa : violaceous-cyanotic, mottled discolouration of the skin affecting primarily the legs and arms, but also involving the buttocks and the trunk, and that is exacerbated by cold or pregnancy.
- Livedo reticularis: affects extremities and seen mainly when cold
- Headache, dizziness, high BP, cognitive issues, seizures, chorea
- Other organs: kidneys, heart, eyes and peripheral nerves
- Reversible cerebral vasoconstriction syndrome
- MELAS syndrome
- Vascular dementia
- Cerebral angiitis.
- Immunological studies are generally negative.
- Anti-DNA, anti-SSA, anti-SSB, anti-Sm and anti-RNP and anti RF are all negative.
- Occasionally ANA, cryoglobulins, APL antibodies, anticardiolipin, lupus anticoagulant or anti-beta2GPI antibodies can be detected
- MRI: white matter disease, infarcts, microbleeds or atrophy.
- Antiphospholipid antibodies may be positive in about 60% of cases.
- Cerebral angiography can be normal
- Skin biopsy: inflammatory changes of small- to medium-sized arteries and subendothelial proliferation and fibrosis.
- Anticoagulation may be considered.
- Antiplatelets for lower risk e.g. APL negative
- ACEI may reduce endothelial proliferation
- Immunosuppression e.g. rituximab may be effective in aPL-positive patients.