Sneddon's syndrome: it is all in the ectoderm

Rutter-Locher, Zoe; Chen, Zhongbo; Flores, Lorena; Basu, Tanya; Creamer, Daniel; Weeks, R. A.; Arya, Roopen; Nashef, Lina

doi:10.1136/practneurol-2015-001341

Cited by 8 publications

(5 citation statements)

References 2 publications

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“…Skin biopsy may reveal thrombosis of subcutaneous arterioles and compensatory capillary dilation with blood stagnation causing livedo reticularis. [52][53][54] Approximately 40-50% of patients with Sneddon's syndrome present aPL antibodies, suggesting that some patients should be classified as APS. 52 Cognitive dysfunction CD is a decline in prior cognitive performance and is present in several rheumatic diseases.…”

Section: Cerebrovascular Diseasementioning

confidence: 99%

Neurologic manifestations of antiphospholipid syndrome

Ricarte

Dutra

Abrantes

et al. 2018

Lupus

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Neurological involvement in antiphospholipid antibody syndrome (APS) is common, and its occurrence increases morbidity and mortality. Patients may present variable neurological involvement, such as cerebrovascular disease, cognitive dysfunction, headache, seizures, movement disorders, multiple sclerosis-like syndrome, transverse myelitis and ocular symptoms. Most neurological manifestations are associated with thrombosis of the microcirculation or of large vessels; nonetheless, there is compelling evidence suggesting that, in some cases, symptoms are secondary to an immune-mediated pathogenesis, with direct binding of aPL on neurons and glia. Herein we describe clinical characteristics and management of neurological APS manifestations.

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Section: Cerebrovascular Diseasementioning

confidence: 99%

Neurologic manifestations of antiphospholipid syndrome

Ricarte

Dutra

Abrantes

et al. 2018

Lupus

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“…It has been proposed by some authors that the mechanism is linked to the embryonic origin of the skin and the brain in the ectoderm. 7,8 While other authors, consider it to be a systemic dysfunction of the arterial bed.…”

Section: Etiopathogenesismentioning

confidence: 99%

“…[9][10][11] An unusual but recognized neurological involvement is intraventricular hemorrhage, probably secondary to vascular dilatation and transdural anastomosis. 4,8 Other systems that can be affected in SS are the cardiovascular system, characteristically with myocardial infarction and the presence of arterial hypertension. Heart valve thickening and/or Libman-Sacks endocarditis are the most frequent cardiac pathologies in SS.…”

Section: Clinical Findingsmentioning

confidence: 99%

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Sneddon syndrome a case report and literature review

Leon¹,

Sánchez²,

Reyes³

et al. 2022

Int J Res Med Sci

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Sneddon’s syndrome (SS) is characterized by livedo racemosa (LR) or reticularis and recurrent ischemic strokes. At the skin and brain level a non-inflammatory thrombotic vasculopathy is observed. Almost 80% of cases are women around 40 years old. The most accepted etiological proposal is an autoimmune and inflammatory mechanism versus the presence of thrombophilia. Neurological manifestations occur in 3 phases: prodromal symptoms (headache, dizziness, and vertigo), recurrent strokes, and early-onset dementia. Livedo racemosa has been reported to precede strokes by more than 10 years. Treatment is mainly based on secondary prophylaxis preventing a stroke with antiplatelet and antithrombotic agents. The neuropsychiatric prognosis is relatively poor with deficits in concentration, attention, visual perception, and visuospatial skills.

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“…Stroke is one of the diagnostic hallmarks of Sneddon's syndrome. The cerebrovascular manifestations are mostly secondary to ischemia in the superficial and deep territories of middle and posterior artery [2][3][4][5].Hemorrhagic manifestations are 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]. Notably, as far as we know chronical superficial siderosis (cSS) has never been issued in Sneddon's syndrome.…”

Section: Introductionmentioning

confidence: 99%

Remarkable Superficial Siderosis and Microbleed Restricted in Cortex might be Responsible for Atrophy and Cognitive Decline in Sneddon’s Syndrome

Yao

Jiang

et al. 2020

Preprint

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ObjectiveSneddon’s syndrome is a rare non-inflammatory arteriopathy affecting small and medium-sized arteries, characterized by a generalized livedo reticularis and recurrent 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.MethodsClinical data and cerebral MR imaging including T2* sequence of 7 consecutive patients with Sneddon’s syndrome were reviewed.ResultsThe 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，four of whom presented more than 5 microbleeds. 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.ConclusionsThis 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.

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Sneddon's syndrome: it is all in the ectoderm

Cited by 8 publications

References 2 publications

Neurologic manifestations of antiphospholipid syndrome

Neurologic manifestations of antiphospholipid syndrome

Sneddon syndrome a case report and literature review

Remarkable Superficial Siderosis and Microbleed Restricted in Cortex might be Responsible for Atrophy and Cognitive Decline in Sneddon’s Syndrome

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