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At least 19 recordsLinked to original sources
PubMed1994

Cerebral blood flow in Sneddon syndrome.

Sneddon syndrome is defined as a clinical entity consisting of livedo racemosa generalisata (LRG) and cerebrovascular lesions, which often lead to physical and mental handicaps. Four patients with LRG and the suspected diagnosis of Sneddon syndrome had HMPAO-SPECT studies. The patients underwent CT and/or MR brain imaging and three patients had Duplex sonography of the cerebral arteries (TCD). Brain SPECT was abnormal in all patients, whereas CT/MRI revealed a cerebral lesion in only one patient and all TCD studies were normal. HMPAO-SPECT is valuable in detecting disturbed regional cerebral blood flow before irreversible ischemic insults occur, thus allowing the diagnosis of Sneddon syndrome at an early stage.

Adult↗
PubMed1995

[A case of Sneddon syndrome].

Sneddon syndrome is a clinical entity that is characterized by livedo reticularis and cerebrovascular lesions and is considered to be a subtype of antiphospholipid antibody syndrome. Central retinal artery occlusion is one of the ophthalmic complications of this syndrome and its prognosis of visual acuity is generally poor. We report here a 50-year-old man with Sneddon syndrome who had recurrent central retinal artery occlusion with recovery of visual function after each episode.

Antibodies, Anticardiolipin↗
PubMed2003

Factor V Leiden mutation in Sneddon syndrome.

Sneddon syndrome (SNS) is characterized by the association of ischaemic cerebrovascular events and widespread livedo racemosa. Its pathophysiology is still controversial. The aim of this study was to evaluate the prevalence of factor V Leiden mutation in consecutive patients referred for SNS according to antiphospholipid antibodies (aPL) status. Fifty-three Caucasian patients were enrolled from 1996 to 2001. Diagnosis of SNS was based on the presence of a widespread livedo racemosa and at least one clinical neurologic ischaemic event. The following investigations were performed: detection of antithrombin III, protein C and protein S deficiency, lupus anticoagulant, anticardiolipin and anti-beta2 glycoprotein I antibodies, biologic false-positive test for syphilis, and factor V Leiden mutation by direct genomic analysis. Fisher's test and t-test were used for statistics. Detection of aPL on multiple determinations was negative in 31 patients (group 1) and positive in 22 patients (group 2). Factor V Leiden mutation was detected in six patients (11.3%), heterozygous in all. The frequency of this mutation was statistically higher in group 1 (6/31, 19.3%) than in group 2 (0/22; P = 0.035). Within aPL-negative SNS, the comparison of patients with versus without factor V Leiden mutation showed no difference for clinical data or familial history of thrombosis. A high prevalence of heterozygous factor V mutation was found in aPL-negative patients with SNS. This finding adds further arguments to consider SNS as a heterogeneous entity.

Adolescent↗
PubMed1995

Sneddon syndrome.

Sneddon syndrome--cerebrovascular lesions and livedo racemosa--is a distinctive and uncommon disorder delineated by Sneddon in 1965. The clinical hallmarks are generalized livedo racemosa and central nervous system ischemia. Cutaneous vascular changes begin with intimal endothelial proliferation and fibromucinous matrix formation, leading to obstruction and obliteration of the vessel. The disorder is slowly progressive. No effective treatment is available, but platelet-inhibiting agents or newer antithrombotic agents may offer some hope in preventing or minimizing serious sequelae of this disease.

Arterioles↗
PubMed1994

[Sneddon syndrome].

Sneddon's syndrome is a rare disease characterised by cerebrovascular ischaemic attacks and generalised livedo. Since previous observations, other symptoms were described: involvement of heart, of kidney, arterial hypertension, complicated obstetric or gynaecologic history in women. Prognosis is highly variable, depending on extent and speed of progression of cerebrovascular changes, which can lead to severe permanent mental deterioration. In livedo, histopathology shows pathological changes of small to medium-sized dermal arteries in a distinct time sequence: an early phase localized in endothelium followed by a late fibrotic phase. No specific laboratory findings are found. Recently some cases were reported in association with antiphospholipids antibodies. The etiopathogeny of Sneddon's syndrome is still unknown and could result from different processes: progression to an autoimmune disease such as lupus erythematosus, primitive endarteritis obliterans, or a new clinical expression of the antiphospholipid antibodies syndrome. At present, none of the therapeutic modalities provides significant improvement.

Cerebrovascular Disorders↗
PubMedDate not supplied

[Antiphospholipid antibodies in a female patient with the Sneddon syndrome].

Sneddon was the first one to describe the association of cerebrovascular insult (CVI) and skin changes by livedo reticularis type. Angiography of this patient revealed normal brain blood vessels, occlusive changes or the changes similar to those in moya-moya disease. Computed tomography (CT) of the brain most frequently revealed ischemic lesions and/or diffuse atrophy of the brain, although the normal finding was also possible. Considering the frequent finding of the increased titer of anticardiolipin antibodies (ACA) in those patients, those antibodies could be the most important in the pathogenesis of Sneddon's syndrome. A case of female patient, aged 66 years, with livedo reticularis and recurrent CVI was presented in this study. By panangiography were revealed normal blood vessels of the brain, by scintigraphy two ischemic lesions and by CT ischemic lesions with pronounced cortical and subcortical reductive changes. In laboratory findings were observed increased immunocomplexes and the presence of lupus-like anticoagulants, increased ACA titer, the increase in fibrinogen value associated with increased ESR. The result of bone marrow examination was normal, while the borreliosis test was negative. Vasculitis was excluded by skin biopsy. Solid improvement was noticed after the immunosuppresive therapy was administered, but in the last disease exacerbation with new CVI, the outcome was lethal 3 years since the disease onset.

Aged↗
PubMed1998

[Cerebral hemorrhage in Sneddon syndrome].

INTRODUCTION: Sneddon syndrome is the association of livedo retricularis and cerebrovascular lesions. It is a vasculopathy of vessels of small and medium calibre which affects young people and is of unknown origin. It is often associated with arterial hypertension and repeated ischemic cerebrovascular accidents which lead to intellectual deterioration. CLINICAL CASE: We describe a 49 year old man who, from the age of 23, had had repeated transient or established episodes compatible with ischemic cerebrovascular accidents. These were followed by various neurological sequelae and by intellectual deterioration. Finally he died of a cerebral hemorrhage whilst on anticoagulant treatment with Synthron. We show the neuroimaging findings (CT, MR and angiography) and the neuropathological studies (cerebral hemorrhage, fibrosis of the intima of the vessels of the circle of Willis, absence of inflammation, bilateral unsymmetrical leukoencephalopathy of the cerebral hemispheres and multiple cortical infarcts). CONCLUSION: We wish to draw attention to the occurrence of this complication, its possible relationship to the vasculopathy mentioned, bursting of one of the arteries of the revascularization complex, arterial hypertension or anticoagulant treatment, and also to add one more case to the few described as having hemorrhage and neuropathology in Sneddon syndrome.

Brain↗
PubMed1997

Atrial myxoma syndrome mimicking Ehrmann-Sneddon syndrome.

Livedo racemosa with cerebrovascular lesions has been described as Ehrmann-Sneddon syndrome. The etiopathogenetic factors provoking the vascular lesions, however, are of high diversity reaching from mechanical to autoimmune causes. We present a male patient with typical livedo racemosa, muscle pain and feeling of coldness of the forearms. By dermatohistopathology and magnetic resonance tomography of the brain, Ehrmann-Sneddon syndrome could be confirmed. At this time a chronic streptococcal infection could be diagnosed. Antibiotics, anticoagulants and vascularity-supplying therapy improved the clinical and subjective symptoms. Six months later, the patient developed dizziness, vision disorder, hypesthesia of the right forehead, malaise and weight loss. A further diagnostic workup including echocardiography revealed a myxoma of the left atrium. This report illustrates the association of Ehrmann-Sneddon syndrome with cardiac myxoma and points out that cardiac diagnostic examination should be included when dealing with small-vessel involvement of the brain.

Adult↗
PubMed1999

Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients.

Sneddon syndrome is characterized by the association of livedo reticularis and cerebral ischemic arterial events (stroke or transient ischemic attack). Reported prevalence of antiphospholipid antibodies is highly variable. We conducted this study to compare the clinical and pathologic features of patients with Sneddon syndrome according to the presence or absence of antiphospholipid antibodies. Forty-six consecutive patients with Sneddon syndrome were analyzed. All were examined by the same dermatologist who classified the livedo of the trunk according to the regularity of the fishnet reticular pattern and according to the thickness of the fishnet reticular pattern (> or = 10 mm = large; < 10 mm = fine). Skin biopsies were systematically performed, from both the center and the violaceous netlike pattern in 38 patients. Antiphospholipid antibodies-positive Sneddon syndrome was defined by the presence of lupus anticoagulant or abnormal titers of anticardiolipin antibodies on repeated determinations. Group I consisted of 27 antiphospholipid antibodies-negative patients and Group II, of 19 antiphospholipid antibodies-positive patients. All patients except I in Group II had irregular livedo reticularis. Large livedo racemosa was more frequently observed in Group I (89%) than in Group II (21%, p < 0.001). On skin biopsy, arteriolar obstruction was detected in only 8 patients (4 in each group). The following parameters were not statistically different between the 2 groups: gender, mean age at detection of livedo, mean age at first clinical cerebral event, hypertension, Raynaud phenomenon, patients with extracerebral and extracutaneous arterial or arteriolar thrombosis or stenosis, patients with venous thrombosis, and women with 2 fetal losses or more. In contrast, seizures (11% in Group I versus 37% in Group II, p < 0.05), mitral regurgitation on echocardiogram (19% versus 53%, p = 0.02), and thrombocytopenia < 150,000/muL (0% versus 42%, p < 0.005) were more frequently observed in Group II. The number of events per year of follow-up was lower with antiplatelet therapy (0.08 versus 0.5) in Group I, but was not different with anticoagulation (0.056 versus 0.06). Antiphospholipid antibodies-negative and -positive patients with Sneddon syndrome belong to close but different subsets of Sneddon syndrome.

Adolescent↗
PubMed1992

[2 cases of Sneddon syndrome].

Two cases of Sneddon syndrome (S.s.) in a 33 and 53-year-old women who developed arterial hypertension, cerebral ischaemic signs and who have livedo reticularis or livedo racemosa, are reported. The authors describe clinical, radiological and biological features of this rare disease, as well as diagnostic investigations including the measurements of the antiphospholipid antibodies (APA). The possible role of APA in the pathogenesis of S.s. is discussed.

Adult↗
PubMed1991

[Sneddon syndrome. Presentation of 2 cases].

Sneddon syndrome is a systemic non inflammatory arteriopathy characterized by the association of livedo reticularis, thrombotic cerebrovascular disease, coronary artery disease and hypertension. Middle age females are usually affected. It should be considered in the differential diagnosis of acute ischemic attacks in younger patients. It has been related to the antiphospholipid antibody syndrome. Treatment is based on control of hypertension and cardiovascular risk factors along with antiplatelet agents. Two cases meeting the clinical criteria outlined above are reported.

Adult↗
PubMed2003

[Perioperative management of a patient with Sneddon syndrome--a case report].

Sneddon's syndrome is a rare combination of generalised livedo reticularis and cerebrovascular accidents. Its clinical presentation varies widely and its aetiology is still not known. 60 to 80% of patients are female. First symptoms of the syndrome are mostly repetitive cerebral strokes, but reduced perfusion of the skin, seen as blue or red-brown mottling, precedes the strokes. The vascular disease is generalised and often accompanied by arteriosclerosis, systemic arterial hypertension, valvular heart disease and the presence of antiphospholipid antibodies. The diagnostic procedures are complicated and have to exclude other autoimmunological diseases. Therapeutic options are anticoagulatory therapy with warfarin, ASS or heparin, reduction of endothelial proliferation with ACE-inhibitors, and improvement of microvascular perfusion with prostaglandine. The increased anaesthesiological risk with these patients is due to the acute risk of thromboembolism and ischaemic cerebral and cardiovascular insults. The anaesthetic management must provide stable perfusion pressures for cerebral and myocardial arteries and avoid increasing risk factors for thromboembolism such as increased blood viscosity or stasis due to improper positioning of the patient. The choice of anaesthetic drugs is dependent on good controllability for haemodynamic stability. The high risk of patients with Sneddon's syndrome justifies a more invasive haemodynamic monitoring and postoperative surveillance on an intensive care unit. This case report describes the anaesthesiological considerations for, and management of, a patient with Sneddon's syndrome who was admitted to hospital for vaginal hysterectomy.

Adult↗
PubMed2000

Sneddon syndrome, arylsulfatase A pseudodeficiency and impairment of cerebral white matter.

We describe a 11 year-old-boy with Sneddon syndrome, confirmed by skin biopsy, and MR evidence of diffuse cerebral hyperintensity of white matter; he also suffered from pre-perinatal hypoxic-ischemic distress. Arylsulfatase A activity was found reduced because of arylsulfatase A pseudodeficiency. We suggest that the association of pre-perinatal distress, Sneddon syndrome and arylsulfatase A pseudodeficiency is responsible for the diffuse impairment of cerebral white matter, never reported in Sneddon syndrome and similar to described cases of delayed posthypoxic demyelination and arylsulfatase A pseudodeficiency.

Cerebral Cortex↗
PubMed2001

Sneddon syndrome with multiple cerebral infarctions 12 years after the onset of livedo vasculitis: a possible involvement of platelet activation.

Sneddon syndrome is characterized by livedo reticularis and multiple cerebral infarctions. Skin and central nervous system symptoms usually have a synchronous onset and at times initial symptoms affect one of them, the other lagging several years behind. We here report a patient with Sneddon syndrome who developed multiple cerebral infarctions more than 10 years after the onset of livedo reticularis. While the neurological symptoms were apparent, the patient did not display active skin manifestations. Laboratory findings excluded collagen diseases, antiphospholipid antibody syndrome, and inherited quantitative deficiency of protein C, protein S and antithrombin III. Abnormal findings included extremely elevated levels of beta-thromboglobulin and platelet factor-4 in the blood, although these acute phase markers of thrombosis were examined several years after the onset of cerebral infarctions. Platelet activation may have caused Sneddon syndrome in the present case.

Adult↗
PubMed1991

Sneddon syndrome: cerebral perfusion studies by Tc99m HM-PAO and SPECT.

Sneddon syndrome is a non-inflammatory, non-atherosclerotic disease involving small and medium-sized arteries of the brain and of the skin. The arteriographic examination is often negative despite progressive impairment of the neurological status. In 3 patients with Sneddon syndrome cerebral perfusion was assessed with single photon emission computerized tomography (SPECT) and technetium99 m-hexamethylpropylenamineoxime (Tc99 HM PAO). A correlation between clinical and SPECT finding was found, with significant focal reduction of regional cerebral blood flow (rCBF) in the two more severely affected patients.

Adult↗
PubMed1998

[Sneddon syndrome--case report].

We report a case of Sneddon syndrome in a 39-year-old woman who developed recurrent cerebral ischaemic events associated with livedo racemosa. We describe clinical and radiological features of this rare vasculopathy and laboratory findings essential for differential diagnosis.

Adult↗
PubMed2005

[Sneddon syndrome with anti-mitochondrial type M5 antibodies].

INTRODUCTION: Antimitochondrial type M5 antibodies (AMA-M5) are among the immunological abnormalities associated with Sneddon syndrome. CASE: A 45 year-old woman, hospitalized for diplopia and with a 20-year history of obstetrical accidents, internuclear ophthalmoplegia and livedo, was diagnosed with Sneddon syndrome associated with primary antiphospholipid syndrome (APS) aggravated by the presence of AMA-M5. DISCUSSION: AMA-M5 are immunological markers of APS to the same extent as antiphospholipid antibodies. This case demonstrates the interest of screening for AMA-M5 in cases of strong clinical suspicion of APS when the anticoagulant lupus test is normal and no anti-cardiolipin, anti-b2 glycoprotein I or antiprothrombin antibodies are found.

Antiphospholipid Syndrome↗