Abstract
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. Twelve patients (median age 49years, 11 female) with primary Sneddon's syndrome, diagnosed in last 10years, 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). Median interval from the onset of neurological symptoms to diagnosis was 6years. 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. 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.
Highlights
Sneddon’s syndrome is a progressive cerebrocutaneous noninflammatory distal arteriopathy
Livedos precede cerebrovascular involvement by years, and dementia, if occurs, by decades. It is usually misdiagnosed as central nervous system (CNS) vasculitis, primary CNS angiitis (PACNS), CNS vasculopathy, and antiphospholipid antibody syndrome (APLAS), probably due to unfamiliarity to the clinical, neuroimaging, and angiographic characteristics of this syndrome
We evaluated magnetic resonance imaging (MRI) at the time of diagnosis, and almost invariably closest to the time of catheter angiography, but we can say that there was no significant pattern change on follow-up MRIs
Summary
Sneddon’s syndrome is a progressive cerebrocutaneous noninflammatory distal arteriopathy. It was named after Dr Ian. Bruce Sneddon, a British dermatologist who described the syndrome as a separate entity characterized by combination of cerebrovascular disease and livedo racemosa [1]. Neurol Sci completely affects women in their thirties to forties It is usually sporadic, but families have been reported with autosomal dominant inheritance showing an incomplete penetrance pattern [2]. Livedos precede cerebrovascular involvement by years, and dementia, if occurs, by decades. It is usually misdiagnosed as central nervous system (CNS) vasculitis, primary CNS angiitis (PACNS), CNS vasculopathy, and antiphospholipid antibody syndrome (APLAS), probably due to unfamiliarity to the clinical, neuroimaging, and angiographic characteristics of this syndrome. Sneddon’s syndrome is often considered when standard treatments for PACNS fail [3]
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