Cognitive and psychiatric changes as first clinical presentation in Sneddon syndrome

Case Report: The patient is a 43 years-old man, Latin-American, with high degree education. He attended the medical consultation after his wife started noticing a two years history of progressive forgetfulness, mental confusion, disorientation, difficulty finding the right words, changes in mood (basically from being shy to outgoing person). He also lost his job for poor performance and was abandoned by his friends, as consequence of his new outgoing personality. Simultaneously he started with sleep changes, and apathy mixed with anxiety symptoms. Initially he was treated as suffering from major depression, and latter type II bipolar disorder. The treatments didn’t change his behavior. The physical examination was completely normal, except for multiple skin stains, mainly on the trunk. Laboratory findings showed undetermined anticardiolipin antibodies, besides the weak presence of lupic anticoagulant. The brain MRI showed multiple areas of signal changes on cortico-subcortical transition and on the deep periventricular white matter with Gadolinium enhanced, compatible with brain ischemia. Cerebral angiography showed distal blood flow of cerebral arteries slowing down with decreased vessel diameter and parietal irregularities suggestive of vasculitis.

Discussion: Sneddon syndrome (SS) is a rare progressive non-inflammatory thrombotic vasculopathy affecting small/medium-sized blood vessels with unknown origin. It’s strongly associated with the presence of antiphospholipids antibodies (AA). The presence of livedo reticularis and cerebrovascular disease are the hallmark. It is far more common in young women.

Final Comments: The recognition of skin blemishes preceding strokes should raise the hypothesis of SS. AA are elevated in more than half of the cases, but their role in the pathogenesis or association of positive antibodies and SS remain unclear. Dementia syndrome in young patients must be extensively investigated to rule out reversible situations. Typical skin findings, MRI and angiography may aid in diagnosis. The patient was discharged from the hospital with a diagnosis of SS with the following treatment: acetylsalicylic acid, corticoids and oral anticoagulants.