CLASSIFICATION OF SNEDDONS-SYNDROME

Background: The combination of generalized broken (''racemose'') lived o and cerebrovascular accidents is referred to as ''Sneddon's syndrome ''. Although several pathogenetic factors have been suggested the aeti ology of Sneddon's syndrome is unknown. Furthermore, considerable vari ability of patient characteristics gives rise to the question whether ''Sneddon's syndrome'' denotes a homogeneous disease entity at all. We hypothesized that the diagnosis ''Sneddon's syndrome'' can be broken down into different subgroups according to possible aetiologic factors . Patients and methods: Thirty-two patients with the combination of ge neralized broken livedo and cerebrovascular accidents were evaluated b y clinical examination, routine diagnostic procedures. MRI of the brai n, echocardiography, vascular ultrasound, immunologic and haemostaseol ogic testing. Patient groups were formed, depending on (1) whether or not an additional feature with a possibly aetiologic role for Sneddon' s syndrome was present, and (2) which kind of feature it was. Results: In 16 out of 32 patients, diagnostic features with an implication for the pathogenesis of Sneddon's syndrome could be identified. An autoim mune disorder was diagnosed in six patients. A thrombophilic state was detected in six patients. One patient suffered from an embolizing atr ial myxoma. Extent and kind of cerebral pathology differed between pat ient groups as did the kind of cardiac involvement. Conclusion: Sneddo n's syndrome is not a homogeneous disease entity. Patients should be c lassified as ''primary Sneddon's syndrome'' if no aetiologic factor ca n be detected. On clinical grounds, this form differs from several var ieties of ''secondary Sneddon's syndrome'' which occurs mainly as part of an autoimmune disorder or in a thrombophilic state.