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A very rare progressive disorder affecting blood vessels and characterized by multiple cerebrovascular accidents and the presence of idiopathic livedo reticularis.

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Livedo Reticularis and Cerebrovascular Accidents; Sneddon-Champion Syndrome.

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A genetic disorder first described by Ian Bruce Sneddon, British dermatologist in 1965.

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Autosomal dominant.

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May be a particular form of obliterating vasculitis or of an antiphospholipid antibodies syndrome.

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Clinically evocated in patients combining livedo reticularis and neurological signs. Skin biopsies can be contributive (arterial intimal hyperplasia).

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Onset possible in childhood. A delay from months to years is possible between skin and neurological lesions. Features can involve skin (livedo reticularis with no infiltration, and affecting the limbs, trunk, and sometimes the face), central nervous system (cerebrovascular accidents, epilepsy, vertigo, and sometimes pseudobulbar syndrome, chorea, episodes of amnesia, or transient amaurosis), and cardiovascular system (occlusive noninflammatory arteriopathy; arteriography can show multiple occlusions in medium-sized arteries; CT scan can show defect corresponding to infarct). Laboratory investigations can find antiphospholipid antibodies, anticardiolipin antibody, lupus anticoagulant.

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Evaluate neurological function (clinical, history, EEG, CT/MRI scan). The cardiovascular function must be systematically evaluated to eliminate the presence of systemic arteries obliteration that have been described.

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Blood pressure control is necessary and may require invasive monitoring. Regional anesthesia is not contraindicated but benefit (particularly of central blockade) has to be clearly established.

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Consider interaction between antiepileptic treatment and anesthetic drugs.

Boesch SM, Plorer AL, Auer AJ, et al: The natural course of Sneddon syndrome: Clinical and magnetic resonance imaging findings in a prospective six-year observation study. J Neurol Neurosurg Psychiatry 74(4):542, 2003.
Bruyn RPM, van der Veen JPW, Donker AJM, et al: Sneddon's syndrome: Case report and literature review. J Neurol Sci 79:243, 1987.  [PubMed: 3612175]
Heesen M, Rossaint R: Anaesthesiological considerations in patients with Sneddon's syndrome. Paediatr Anaesth 10(6):678, 2000.

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