[Sneddon's syndrome]. / Le syndrome de Sneddon.

Sneddon's syndrome is still raising some nosological and etiopathogenic questions. The occurrence of ischemic stroke in young adults especially in the presence of livedo racemosa should suggest the diagnosis and encourage to perform a skin biopsy, which could strengthen the diagnosis. Management begins with prevention of vascular risk factors and treatments based primarily on anti-thrombotic. Large series of studies over several years could provide clarification of the etiopathogenesis of this syndrome and pave the way for the development of diagnostic criteria and new effective therapies in order to prevent progression to irreversible cognitive impairment.

Seizures in primary antiphospholipid syndrome: the relevance of smoking to stroke.

OBJECTIVES: To evaluate the frequency of seizures in primary antiphospholipid syndrome (PAPS) and their possible clinical and laboratory associations. METHODS: Eighty-eight PAPS patients (Sydney's criteria) were analyzed by a standard interview, physical examination and review of medical charts. Risk factors for seizures, clinical manifestations, associated comorbidities, and antiphospholipid antibodies were evaluated. RESULTS: Nine (10.2%) patients with seizures were identified, 77.8% had convulsions onset after PAPS diagnosis. Mean age, gender, and race were comparable in groups with or without seizures. Interestingly, a higher frequency of current smoking (44.4 versus 10.1%, P = 0.019) was observed in the first group. Stroke, Sneddon's syndrome, and livedo reticularis were more frequent in PAPS patients with seizures than those without seizures, although not statistically significant (P > 0.05). Comparison between patients with seizures onset after PAPS diagnosis (n = 7) and those without convulsions (n = 79) demonstrated a higher frequency of current smoking (42.9 versus 10%, P = 0.042) and stroke in the first group (71.4 versus 30.4%, P = 0.041). Regression analysis confirmed that smoking (P = 0.030) and stroke (P = 0.042) were independently associated to seizures. CONCLUSION: About 10.2% of PAPS patients had convulsions, predominantly after PAPS diagnosis, and seizures were associated to current smoking and stroke.

[Non-ischemic neurological manifestations in patients with primary antiphospholipid syndrome].

Ischemic disorders of brain blood circulation caused by brain artery thrombosis due to antiphospholipids-induced anticoagulopathy are main neurological appearances of primary antiphospholipid syndrome (PAPS). A number of neurological disorders in patients with PAPS are the result of primary involvement of the brain and peripheral nervous system. We analyzed the spectrum of neurological non-ischemic PAPS manifestations in 125 patients (102 female, 23 male, mean age--37.5 +/- 11.3 years) with definite PAPS. These manifestations included headache (67%), epileptic seizures (23%), chorea (15%), optic neuropathy (9%), peripheral neuropathy (6%), multiple sclerosis like syndrome (MSLS) (8%), acute psychosis (2%), myasthenic syndrome (1%), non-vascular parkinsonism (1%). In the development of non-ischemic PAPS manifestations, antiphospholipids as well as other antibodies produced as a result of immune disregulation (antibodies to acetylcholine receptors in myasthenic syndrome, antineuronal antibodies in MSLS) may have pathogenic significance. In some cases a role of infection involved in PAPS manifestation cannot be ruled out.

Intracerebral hemorrhage associated with Sneddon's syndrome: is ischemia-related angiogenesis the cause? Case report and review of the literature.

Sneddon's syndrome is characterized by livedo reticularis and multiple ischemic infarcts often associated with antiphospholipid antibodies. Intracerebral hemorrhage (ICH) is unusual in Sneddon's syndrome and has not been reported as the presenting complaint. We report a 38-year-old woman with a history of two miscarriages, Raynaud's phenomenon and livedo reticularis who presented acutely with ICH. Angiography showed prominent leptomeningeal and transdural anastomoses (pseudoangiomatosis). Anticardiolipin antibodies were positive. A right frontal brain biopsy failed to reveal vasculitis and a skin biopsy was nonspecific. MRI showed residual intracerebral hemorrhage (ICH), diffuse atrophy, multiple small white matter infarcts and leptomeningeal enhancement. This is the first report of Sneddon's syndrome presenting with an ICH. It shares features with the Divry-van Bogaert syndrome. We discuss the cause of the pseudoangiomatosis pattern and its role in the genesis of the hemorrhage and suggest that cerebral angiography should be done in every patient with Sneddon's syndrome, as it could impact therapy.

[Sneddon's syndrome: 4 cases and a review of the literature]. / Le syndrome de Sneddon: 4 cas et revue de la littérature.

Sneddon syndrome is an association of livedo racemosa and cerebrovascular ischemic events generally occurring in young adults. This is an uncommon chronic progressive arterio-occlusive disorder of unknown cause involving small and medium sized vessels. We report four cases. One case was disclosed by cerebral hemorrhage. One pathogenic hypothesis suggests the involvement of an idiopathic progressive inflammatory arteriopathy or secondary thrombotic disorder comparable with antiphospholipid syndrome.

Livedo reticularis and cerebrovascular accidents (Sneddon's syndrome) as a clinical expression of antiphospholipid syndrome.

We present two patients with livedo reticularis and cerebrovascular accidents (Sneddon's syndrome) and positive anticardiolipin antibodies. We suggest that these antibodies may be pathophysiologically related to the clinical manifestation observed in some patients with this syndrome. When Sneddon's syndrome is associated with positive anticardiolipin antibodies it could be regarded as antiphospholipid syndrome and fall into this category of nosological entity.

[Sneddon syndrome new clinical and immunological data]. / Novye klinicheskie i immunologocheskie dannye o sindrome Sneddona.

In 27 of 47 patients with Sneddon's syndrome (33 females, 14 males age 40 years) enzyme immunoassay has detected IgG-antibodies to prothrombin (aPT)--one of cofactor proteins responsible for binding of antiphospholipid antibodies (aPL) to phospholipids. Other aPL were also found: antibodies to cardiolipin (aCL), lupus anticoagulant (LA) in 14 and 27 patients, respectively. 37 (79%) patients had at least one of the studied aPL suggesting that such patients belong to patients with primary antiphospholipid syndrome. A correlation exists between aPT and LA: LA is detectable in 67% of aPT-positive patients compared to 45% of aPT-negative patients (p < 0.05). This is in agreement with the fact that prothrombin is a cofactor for most aPL registered as LA. Comparison of two subgroups of aPL patients different by dominant antigenic specificity (18 patients with aPT but free of ACL and 6 patients with aCP but free of aPT) demonstrated that the latter developed disorders of cerebral circulation, head ache, dementia and renal syndrome less frequently. aPT in Sneddon's syndrome seems to be a marker of comparatively low risk of thrombosis and less severe course of the disease.

Classification of Sneddon's syndrome.

BACKGROUND: The combination of generalized broken ("racemose") livedo and cerebrovascular accidents is referred to as "Sneddon's syndrome". Although several pathogenetic factors have been suggested the aetiology of Sneddon's syndrome is unknown. Furthermore, considerable variability 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 generalized broken livedo and cerebrovascular accidents were evaluated by clinical examination, routine diagnostic procedures, MRI of the brain, echocardiography, vascular ultrasound, immunologic and haemostaseologic 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 autoimmune disorder was diagnosed in six patients. A thrombophilic state was detected in six patients. Three patients had preexisting atherosclerosis. One patient suffered from an embolizing atrial myxoma. Extent and kind of cerebral pathology differed between patient groups as did the kind of cardiac involvement. CONCLUSION: Sneddon's syndrome is not a homogeneous disease entity. Patients should be classified as "primary Sneddon's syndrome" if no aetiologic factor can be detected. On clinical grounds, this from differs from several varieties of "secondary Sneddon's syndrome" which occurs mainly as part of an autoimmune disorder or in a thrombophilic state.

[Endothelial antibodies in Sneddon's syndrome]. / Antitela k éndoteliiu pri sindrome Sneddona.

Endothelial antibodies (EAB) were determined by enzyme immunoassay in 39 patients with Sneddon syndrome. EAB were detected in 12 patients (31%). Antibodies to phospholipids occurred more frequently in EAB-positive patients. Clinically, EAB-positive and EAB-negative patients were similar except renal syndrome which in the form of proteinuria was more frequent in EAB-positive subjects.