Characteristic imaging features of neurovascular involvement in primary Sneddon's syndrome: an analysis of 12 cases.

OBJECTIVE: Sneddon's syndrome is a cerebrocutaneous non-inflammatory progressive distal arteriopathy, characterized by livedo racemosa, stroke, and neuropsychiatric symptoms. Our aim was to highlight the characteristic neuroimaging features of Sneddon's syndrome that might be helpful to clinicians in timely diagnosis of this entity. METHODS: Twelve patients (median age 49 years, 11 female) with primary Sneddon's syndrome, diagnosed in last 10 years, were analyzed from the perspective of magnetic resonance imaging (MRI) features. In addition, a novel pseudoangiomatosis score was defined for grading angiographic abnormalities (range: 0 to 6). RESULTS: Median interval from the onset of neurological symptoms to diagnosis was 6 years. Presentation was with acute stroke in 5, seizures in 3, dementia/speech problems in 2, seizures plus cognitive dysfunction in 1, and chronic progressive hemiparesis in 1. All patients had a typical lesion pattern on MRI. This included multiple (median 3) cortical-subcortical supratentorial and cerebellar non-territorial infarcts, accompanied by multifocal cerebral atrophy. Of note, large territorial infarcts due to cerebral parent artery occlusion, an embolic pattern with multi-territorial involvement on diffusion-weighted imaging, small vessel disease features like severe white matter involvement or lacunar infarcts, and cerebral hemorrhage in the absence of anticoagulation were not observed. MRI lesion severity was not correlated with angiographic arteriopathy severity, clinical stage, or presentation symptoms. CONCLUSION: Sneddon's syndrome is characterized by highly typical clinico-radiological features. Brain MRI has diagnostic value. By knowing the characteristics of the syndrome, misdiagnosis and potentially harmful treatment can be prevented in this entity that might pose a diagnostic challenge.

Strokes in Sneddon syndrome without antiphospholipid antibodies.

OBJECTIVE: Sneddon syndrome (SS) is characterized by the association of a livedo reticularis with stroke. Clinicoradiological features of its neurological manifestations, its prognosis, and the frequency of associated cardiac valvulopathy remain poorly known, particularly in the absence of antiphospholipid antibodies (APL). The objectives were to assess the clinicoradiological pattern of SS without APL (SSAPL- ) and its midterm prognosis. METHODS: Clinical data, transthoracic echocardiograms, and brain imaging of 53 consecutive patients (83% women) with SSAPL- , followed up at our institution between 1991 and 2011, were reviewed. RESULTS: Seventy-four strokes were reported; 76% were ischemic strokes (IS), 15% transient ischemic attacks, and 9% hemorrhagic strokes. Heart valve lesions were found in 50% of the cases. Brain imaging showed 177 IS of 3 different types: large territorial (43%), small distal corticosubcortical (14%), and small deep (23%) IS. No significant association was found between the valve involvement and the presence of territorial IS. After a mean follow-up of 7.4 years, 82% of patients had a modified Rankin Scale score ≤ 2. The ischemic event recurrence rate was 20%, with a similar annual rate in the antiplatelet group (3%) compared to the anticoagulation group (2.7%). INTERPRETATION: SSAPL- is not only a neurocutaneous disorder, but is frequently associated with heart valve involvement. The latter does not influence the IS type, which suggests that strokes are caused by vasculopathy of the small and medium-size cerebral arteries. Our results show no progression toward a serious disability in the majority of the cases and a moderate recurrence rate under antiplatelet therapy.

Sneddon's Syndrome presenting with topographic disorientation.

A 32-year-old woman was admitted to our department for a progressive difficulty in topographic orientation, confirmed by an extensive battery of neuropsychological tests. All biochemical and immunological examinations of blood and cerebrospinal fluid and a cerebral MRI were normal, but a technetium-99m-ethyl cysteinate dimer-single photon emission CT (Tc-99m ECD-SPECT) scan showed bilateral parietal hypoperfusion. The patient refused to undergo other examinations, but 14 months later she returned to hospital for diffuse cutaneous livedo reticularis over her trunk and legs. This time the MRI showed small frontoparietal cortical-subcortical abnormalities suggestive of arterial ischemic infarctions. We made a diagnosis of Sneddon's Syndrome (SNS). SNS is characterized by the association of livedo reticularis and cerebrovascular disease; non-dermatological onset is extremely uncommon. To our knowledge, this is the first description of a patient presenting with cognitive impairment only and observed at such an early stage of the disease that a cerebral MRI was normal.

Intraventricular hemorrhage as an unusual presenting form of Sneddon syndrome.

BACKGROUND: Intraventricular hemorrhage, which has a poor prognosis, is an extremely rare presenting symptom of central nervous system vasculitis. Sneddon syndrome, which is a systemic vasculitic disease, generally presents with ischemic stroke and livedo reticularis. Intraventricular hemorrhage is extremely rare in Sneddon syndrome and has not been reported as the presenting complaint. METHODS: We report a 37-year-old woman who presented with acute intraventricular hemorrhage, and on further evaluation her condition was diagnosed as Sneddon syndrome. RESULTS: Patient underwent ventriculoperitoneal shunting operation for hydrocephalus and her condition markedly improved 6 months later; she was independent in her activities of daily living. CONCLUSIONS: In this report, we emphasize the importance of multisystemic evaluation of patients, especially those with obscure angiography findings.

The natural course of Sneddon syndrome: clinical and magnetic resonance imaging findings in a prospective six year observation study.

Sneddon syndrome (SS) is increasingly recognised as a cause of ischaemic stroke in young adults. As the natural course of SS is not well defined, the authors performed a prospective six year clinical and neuroradiological follow up study. Thirteen patients with definite diagnosis of SS (livedo racemosa, characteristic skin biopsy, and history of stroke) entered a follow up programme that consisted of clinical examinations, two magnetic resonance imaging (MRI) investigations, and a comprehensive laboratory follow up protocol. The most frequent clinical findings during follow up had been headache (62%) and vertigo (54%). Seven patients (54%) suffered from transient ischaemic attacks, however, completed stroke has not been obtained during follow up. Progression of white matter lesions detected in MRI were present in 10 of 13 patients. Laboratory follow up protocol revealed transient antiphospholipid antibodies in two subjects. This prospective six year follow up study suggests a low incidence of territorial stroke but outlines progressive leucencephalopathy in patients with SS.

Clinical and neuroradiological aspects of Sneddon's syndrome and primary antiphospholipid antibody syndrome. A follow-up study.

We performed a study to investigate differences and similarities between patients with Sneddon's syndrome and those with primary antiphospholipid syndrome (PAS), by clinical follow-up, magnetic resonance imaging (MRI) and angiography. Nine patients with Sneddon's syndrome and 11 patients with PAS were assessed at diagnosis and followed for a mean of 6 years. The clinical and MRI findings indicated that Sneddon's syndrome and PAS are distinct entities. Patients with Sneddon's syndrome had a progressive clinical course with increasing disability and cognitive deterioration; patients with PAS had a more benign course. Infarcts in territories of the main cerebral arteries were frequent in PAS, while leukoaraiosis and small lacunar infarcts were more common in Sneddon's syndrome. In 3 of 7 women initially diagnosed with PAS, the diagnosis was changed to systemic lupus erythematosus during follow-up. Differential diagnosis of Sneddon's syndrome and PAS is important, as early therapy is effective for the latter, more benign, condition.

Cerebral blood flow-SPECT in a patient with Sneddon's syndrome.

We report a 50-year-old woman diagnosed with Sneddon's syndrome and examined by CBF scintigraphy several times for follow-up of the disease. There were no significant changes in her CBF scintigraphic findings or neurological status during the 6-year follow-up period. Sneddon's syndrome is a slowly progressive disorder in which livedo reticularis precedes cerebrovascular accidents. Because small cortical arteries are predominantly affected in Sneddon's syndrome, MR and conventional angiography often fail to show any abnormal findings, and MR imaging may not visualize decreased CBF in the early stage. Therefore, CBF scintigraphy should be performed in patients who have or are suspected of having Sneddon's syndrome.

[Antiphospholipid antibodies in a female patient with the Sneddon syndrome]. / Antifosfolipidna antitela kod bolesnice sa Sneddonovim sindromom.

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.

[Clinical, biological and neuroradiological aspects of Sneddon's syndrome: 26 cases]. / Aspects cliniques, biologiques et neuroradiologiques du syndrome de Sneddon: 26 observations.

Sneddon syndrome is characterized by the association of livedo reticularis and cerebrovascular ischemic events. Clinical and MRI aspects are described in 26 patients with Sneddon syndrome. MRI features were classified in 6 groups according to the aspect and topography of the lesions. No correlation was found between the presence of vascular risk factors, valvulopathy or antiphospholipid antibodies and the presence of dementia or MRI lesions. There was a significant correlation between MRI extension of the lesions and clinical disability. Clinical course and MRI findings are presented according to the treatment.