Superficial Siderosis and Microbleed Restricted in Cortex Might Be Correlated to Atrophy and Cognitive Decline in Sneddon's Syndrome.

Ming Yao,Jiuliang Zhao,Jun Ni,Libo Li,Nan Jiang

doi:10.3389/fneur.2020.01035

Abstract

Objective: Sneddon's syndrome is a rare non-inflammatory arteriopathy affecting small and medium-sized arteries, characterized by a generalized livedo reticularis and recurrent transient ischemic attack or ischemic stroke. Hemorrhagic stroke was reported in limited cases, but microbleeds and superficial siderosis were rarely issued. We aimed to investigate the hemorrhagic imaging features of Sneddon's syndrome and explore the possible mechanism and clinical relevance.Methods: Clinical data and cerebral MR imaging including T2* sequence of seven consecutive patients with Sneddon's syndrome were reviewed.Results: The most common neurological manifestations were cognitive impairment and stroke attack (71.4%), followed by seizures and movement disorder (28.6%). Cerebral microbleeds were detected in six patients on T2* sequence, all of them presented with cortical microbleeds, only one of them with microbleeds in basal ganglion. More than five microbleeds were observed in four of these six patients. The majority of the microbleeds were predominantly cortical restricted and especially located in the cortical watersheds. Multiple superficial siderosis were identified mainly involving cortical watersheds in five cases. Significant cerebral atrophy with prominent secondary white matter hyperintensities in bilateral cortical watersheds were also observed. Abnormal tortuous and multiple focal occlusion of bilateral distal MCA were shown in one patient by DSA. No stenosis of proximal segment of cerebral arteries was detected in all the patients.Conclusions: This is the first report illustrating abundant cortical microbleeds and superficial siderosis mainly involved the anterior and posterior cortical watersheds in Sneddon's syndrome. The surprisingly identical topographic distribution of hemorrhagic lesions and the obvious atrophy suggest cerebral atrophy might be secondary to the microangiopathy related hemorrhagic lesions and further contribute to the neurological deficit, especially the early cognitive decline in Sneddon syndrome.

Highlights

Sneddon’s syndrome, first reported in 1965 [1], is considered as a rare neurocutaneous syndrome characterized by an association of a widespread livedo reticularis with stroke
Sneddon’s syndrome was defined based on the association of a widespread livedo reticularis involving the trunk and/or the buttock and stroke [5]. Intensive screenings, such as test for coagulation profile, lupus anticoagulant, antinuclear antibody, antineutrophil cytoplasmic antibodies, antiphospholipid antibodies, antiphospholipid antibodies, cryoblobulin, protein C, protein S, antithrombin, APC resistance, tumor markers, virus antibodies for HIV, syphilis and TORCH, and cerebral spinal fluid analysis, were performed to make sure whether the patient accompanied with antiphospholipid syndrome or systematic lupus erythematosus, and to rule out other acquitted and inherited non-inflammatory vasculopathies which might contribute to livedo reticularis and stroke
Cognitive impairment and ischemic stroke attack were found in five patients (71.4%), one of them presents cognitive decline without prior ischemic stroke attack

Summary

Introduction

Sneddon’s syndrome, first reported in 1965 [1], is considered as a rare neurocutaneous syndrome characterized by an association of a widespread livedo reticularis with stroke. An annual incidence of approximately four patients per million worldwide was estimated with a female predominance It is a chronic, progressive, arterio-occlusive disease of unknown etiology involving small-to-medium-size arteries, with the pathologic hallmark of endothelia proliferation [1]. Hemorrhagic manifestations are believed to be unusual in Sneddon’s syndrome and has been reported in limited cases [2, 4,5,6,7,8,9], among which only two studies have evaluated cerebral microbleeds (CMBs) [4, 10]. Among the limited cases of cerebral bleeding manifestations, GRE or T2∗ sequence was applied in only very rare cases [4, 10], which likely leads to an underestimate of hemorrhagic manifestations in patients with Sneddon’s syndrome

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