Vol. 12 nº 2 - Apr/May/Jun de 2018
Case Report Páginas: 216 a 219

Cognitive and psychiatric changes as first clinical presentation in Sneddon syndrome

Authors Giorgio Fabiani1; Raul Martins Filho2; Gelson Luis Koppe3; Zeferino Demartini Jr4; Luana Antunes Maranha Gatto4

PDF

keywords: Sneddon syndrome, central nervous system vasculitis, presenile dementia, vascular dementia, antiphospholipid syndrome.

ABSTRACT:
Sneddon syndrome (SS) is a rare progressive non-inflammatory thrombotic vasculopathy affecting small/medium-sized blood vessels of unknown origin. It is strongly associated with the presence of antiphospholipid antibodies (AA). The presence of livedo reticularis and cerebrovascular disease are hallmark features. The condition is far more common in young women. We report a case of SS in a 43 year-old male with a two-year history of progressive cognitive impairment consistent with dementia syndrome, and major personality changes, besides livedo reticularis and cerebral angiographic pattern of vasculitis. AA were borderline. The recognition of skin blemishes that precede strokes should raise the hypothesis of SS. AA are elevated in more than half of cases, but their role in the pathogenesis or association of positive antibodies and SS remains unclear. Dementia syndrome in young patients should be extensively investigated to rule out reversible situations. Typical skin findings, MRI and angiography may aid diagnosis.

 

Home Contact