Cognitive and psychiatric changes as first clinical presentation in Sneddon syndrome

• Published in: Dementia & neuropsychologia Vol. 12; no. 2; pp. 216 - 219
• Main Authors: Fabiani, Giorgio, Martins, Filho, Raul, Koppe, Gelson Luis, Demartini, Jr, Zeferino, Gatto, Luana Antunes Maranha
• Format: Journal Article
• Language: English
• Published: Brazil Associação de Neurologia Cognitiva e do Comportamento 01-06-2018; Associação Neurologia Cognitiva e do Comportamento
• Subjects: antiphospholipid syndrome; Case Report; central nervous system vasculitis; CLINICAL NEUROLOGY; GERIATRICS & GERONTOLOGY; HEALTH CARE SCIENCES & SERVICES; MEDICINE, GENERAL & INTERNAL; NURSING; presenile dementia; PSYCHIATRY; PSYCHOLOGY; Sneddon syndrome; vascular dementia; antiphospholipid syndrome; Sneddon syndrome; central nervous system vasculitis; vascular dementia; presenile dementia; síndrome de Sneddon; demência vascular; vasculite do sistema nervoso central; síndrome antifosfolipídica; demência pré-senil tipo Alzheimer
• ISSN: 1980-5764; 1980-5764
• DOI: 10.1590/1980-57642018dn12-020016

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.