Sneddon's Syndrome Patients Research Articles

Sneddon Syndrome: Livedo Reticularis and Ischemic Stroke

Sneddon syndrome(SS) is a rare, progressive vasculopathy with recurrent ischemic attacks. There is no specified treatment recommendation, various treatment methods such as anticoagulants, antiaggregants, and immunosuppressions are recommended. A 44-years-old woman who applied with a temporary numbness on her right side and speech disorder. In her neurological examination, there was central facial asymmetry on the right and mild dysarthria in his speech. In cranial diffusion MR, there was an acute ischemic change in the left lentiform nucleus and at the head of the caudate nucleus. In cranial MR, there were perivascular ischemic gliotic changes and diffuse cerebral cortical atrophy. The patient underwent a skin biopsy to have livedo reticularis-like lesions on both legs. Skin biopsy was compatible with vasculopathy. Due to the ischemic attack, periventricular ischemic changes, and livedoreticularis, the patient was accepted as SS. Since the antiphospholipid antibodies (APL) were negative, that she was followed up with dual antiplatelet therapy. There was no new attack within a year with dual therapy. Dual antiplatelet therapy may be used in SS patients with recurrent ischemic events and negative APL.

Sneddon syndrome: a comprehensive clinical review of 53 patients

BackgroundThe presence of livedo reticularis in patients with ischaemic stroke is associated with Sneddon syndrome (SS). Our objective was to present the clinical features of SS patients and to assess the role of antiphospholipid antibodies (APL).MethodsConsecutive patients, diagnosed with SS between 1996 and 2017, were retrospectively reviewed for their demographic, neurological, dermatological, cardiac and extracerebral vascular features. Diagnosis of SS was made only if other causes of stroke were excluded. Patients with and without APL were included and compared for their clinical features.ResultsFifty-three patients (79% female) were included, of whom 14 patients were APL-positive. Median age at diagnosis was 40 years. Approximately 60% of the patients had ≥ 3 cardiovascular risk factors. There were 129 previous vascular events (66 ischaemic strokes, 62 TIAs and 1 amaurosis fugax) during a median period of 2 years between the first event and diagnosis of SS. Skin biopsy was positive for SS in 29 patients (67%), mostly showing a thickened vessel wall with neovascularization in the deep dermis. After a median follow-up of 28 months, 4 patients, either on antiplatelet or oral anticoagulation therapy, had a recurrent stroke. There were few statistically significant differences between APL-negative and APL-positive patients, including the number of vascular events before diagnosis.ConclusionsSS predominantly affects young women with a relatively large number of cardiovascular risk factors. Clinical features of SS are comparable across different studies. We found no differences in the main clinical features between APL-positive and APL-negative patients.

Echocardiographic features in antiphospholipid-negative Sneddon's syndrome and potential association with severity of neurological symptoms or recurrence of strokes: a longitudinal cohort study.

Sneddon's syndrome (SS) may be classified as antiphospholipid positive (aPL+) or negative (aPL- SS). An association between Libman-Sacks (LS) endocarditis and strokes has been described in aPL+ patients. To describe cardiac involvement in aPL- SS and assess the potential association between LS endocarditis and severity or recurrence of neurological symptoms. This longitudinal cohort study included aPL- SS patients followed in our departments between 1991 and June 2018. All patients underwent transthoracic 2D and Doppler echocardiography at diagnosis. Follow-up echocardiography was performed annually and the potential relationship between LS endocarditis development and neurovascular relapse as well as long-term cardiac worsening was prospectively assessed. We included 61 patients [52 women; median age 45 (range 24-60)]. For valvular involvement, 36 (59%) patients showed leaflet thickening; 18 (29.5%) had LS endocarditis at baseline. During a median follow-up of 72 months, LS endocarditis developed in eight (17.4%) patients, and 13 (28.3%) showed significant worsening of their cardiac status, including two who needed valvular replacement. After adjusting for baseline antithrombotic treatment regimen, neither the presence of LS endocarditis at baseline nor development during follow-up was associated with neurological relapse [hazard ratio (HR): 1.06, 95% confidence interval (CI): 0.33-4.74, P = 0.92] and [HR: 0.38, 95% CI: 0.02-1.89, P = 0.31], respectively. A long-term follow-up is needed to detect cardiac complications in aPL- SS. No change in neurological relapse was observed in patients presenting LS endocarditis occurrence during follow-up without any modification in antithrombotic treatment. Further research is necessary to assess the usefulness of treatment escalation in these patients.

Treatment of Refractory Lower Extremity Ulcer Associated with Sneddon's Syndrome

Treatment of Refractory Lower Extremity Ulcer Associated with Sneddon's Syndrome

Une nouvelle toxicité à ne pas méconnaître : la sclérose cutanée algique induite par le pemetrexed (Alimta®)

Sneddon syndrome (SS) is an episodic or chronic, slowly progressive disorder and characterized by generalized livedo racemosa (patchy, violaceous, skin discoloration) and recurrent cerebrovascular events. The histopathology of skin and brain is remarkable for a noninflammatory thrombotic vasculopathy involving medium- and small-sized dermal and cerebral arteries, respectively. Approximately 80% of the SS patients are women with a median age of diagnosis at 40 years. However, the onset of the disease during childhood have been reported. Etiopathogenesis of SS is unknown with 2 primary mechanisms proposed – autoimmune/inflammatory versus thrombophilia. SS is primarily classified as antiphospholipid positive or negative type. Neurological manifestations usually occur in 3 phases: (1) prodromal symptoms such as headaches, dizziness, and vertigo, (2) recurrent strokes, and (3) early onset dementia. Livedo racemosa precedes the onset of recurrent strokes by more than 10 years, but in many instances, the significance of the skin lesion is recognized only after the appearance of the stroke. The involvement of the heart valves, systolic labile hypertension, and retinal changes are also commonly associated with this syndrome. Treatment of SS is primarily based on anecdotal reports. Antiplatelet and antithrombotic agents are used for secondary stroke prophylaxis, and a recent study showed a relatively lower stroke recurrence rate with the universal use of antiplatelet/antithrombotic agents. Routine use of anti-inflammatory or immunosuppressive therapies is controversial. Neuropsychiatric prognosis of SS is relatively poor with predominant deficits in the concentration, attention, visual perception, and visuospatial skills.

Sneddon's syndrome: case report and review of its relationship with antiphospholipid syndrome

The Sneddon's syndrome is a rare disorder characterized by the occurrence of cerebrovascular disease associated with livedo reticularis. The antiphospholipid syndrome is the most frequent type of acquired thrombophilia, defined by the occurrence of thrombosis or pregnancy morbidity in the presence of persistently positive antiphospholipid antibodies. Approximately 80% of Sneddon's syndrome patients have an antiphospholipid antibody marker. These antibodies may play a pathogenetic role in some cases of Sneddon's syndrome, and many authors consider these two syndromes as the same entity. Although clinical features of antiphospholipid syndrome and Sneddon's syndrome may overlap, there is a distinction between clinical and laboratory evidence suggesting that these two entities are different diseases. A recent finding of coagulopathies, including elevated levels of coagulation factor VII, decreased levels of protein S, and activated protein C in Sneddon's syndrome patients suggested a possible biological link between the vasculopathy and a primary coagulopathy. Moreover, the clinical course seems to be progressive in Sneddon's syndrome patients and includes increase of disability and cognitive deterioration, more arterial involvement, and the antiphospholipid syndrome shows a more benign course. Both syndromes share clinical and laboratory features, and whether Sneddon's syndrome represents a spectrum of antiphospholipid syndrome remains unclear. Sneddon's syndrome patients have a worse prognosis and may represent a subgroup of patients who demands more rigorous follow-up. It is important to recognize the Sneddon's syndrome, particularly because stroke episodes may be prevented through appropriate treatment.

Primary antiphospholipid syndrome with and without Sneddon’s syndrome

The main objective of this study was to compare clinical and laboratory data obtained from patients with primary antiphospholipid syndrome (PAPS) with and without Sneddon's syndrome (SS). A transverse study with 54 (85.2% female) PAPS patients (Sapporo criteria) was performed. Demographic, drug use, and antiphospholipid antibodies data were evaluated, as well as clinical and laboratory findings of SS. Patients were subdivided into one of two groups: PAPS with SS and PAPS without SS. Both groups were similar with respect to age (p=0.05), gender (p=0.34), race (p=0.31), weight (p=0.93), height (p=0.27), and body mass index (p=0.75); however, the SS group exhibited higher disease duration (96.0±54.9 vs. 55.2±52.0months, p=0.01). By definition, all PAPS with SS patients suffer from stroke, an arterial event; the frequency of stroke events (28.5 vs. 7.5%, p=0.04), as well as of limb ischemia (100 vs. 30.0%, p<0.0001) was higher in this group than in the PAPS without SS group. On the other hand, patients in the PAPS without SS group had more venous events, such as deep venous thrombosis, than those in the PAPS with SS group (80.0 vs. 50.0%, p=0.03). In conclusion, an understanding of the relationship between APS and SS is important in order to identify a subgroup for which more rigorous accompaniment and therapy may be necessary.

Antibodies to prothrombin in patients with Sneddon's syndrome.

Antiprothrombin antibodies (aPT), a new serologic marker of antiphospholipid syndrome, were studied in 46 patients randomly selected from 73 with Sneddon's syndrome and 20 matched normal controls. aPT were elevated in 26 patients (57%) and were not found in any of the controls. The addition of aPT data increased the proportion of Sneddon's syndrome patients with at least one type of antiphospholipid syndrome marker from 65 to 78%. The finding that aPT are common in Sneddon's syndrome supports the hypothesis that Sneddon's syndrome is a form of antiphospholipid syndrome.

Sneddon's syndrome: a clinical and neuropsychological study on 21 patients

We systematically investigated the cognitive performances of a large series of patients with Sneddon's syndrome (SS) and their relation to clinical or instrumental parameters. We evaluated twenty‐one patients with idiopathic generalized livedo reticularis and neurological symptoms and signs consistent with cerebrovascular disease, comparing their cognitive performances with those of the same number of matched normal controls. The patients underwent dermatological evaluation with skin biopsy, biochemical tests including anticardiolipin antibodies, neurological and neuroimaging investigations. Only three patients were classifiable as demented, but all the others showed multiple or few cognitive disorders (nine patients for each group, respectively). Both patients and controls were examined by neuropsychological tests investigating mental deterioration, executive functions, memory and instrumental abilities. The group with few cognitive disorders performed significantly worse with respect to the controls in copy design, verbal fluency, immediate visual memory and forward and reverse digit span. In the whole group of SS patients, cognitive involvement was correlated with neurological focal signs and neuroradiological focal alterations. These data suggest a constant cognitive involvement in SS, to varying degrees, which is more often mild and subclinical. The prominent visual–constructive, attentional and working memory dysfunctions which were detected in our series point to a subcortical pattern.