Therapeutic Perspectives in Sneddon’s Syndrome

Sneddon’s syndrome is a rare but severe progressive chronic disease, characterized by multiple discoloration skin patches called Livedo racemosa and recurrent cerebrovascular events. It mainly affects women aged around 40. Considering the two main forms, antiphospholipid (APS) positive and negative, the available treatments are directed at either one of them. The idiopathic form (APS negative) is associated with a more severe prognosis as chronic oral anticoagulant therapy (COA) is more difficult to manage. One therapeutic perspective in controlling disease progression in these patients is by understanding the protein Z deficiency in these patients as a deciding factor in the success of the COA therapy.

Sneddon’s Syndrome: a case report

Context: Sneddon’s Syndrome is a small and medium caliber arteries vasculopathy, characterized by concomitant occurrence of cerebrovascular disease and livedo reticularis. It’s a rare disorder, more prevalent in women. In up to 80% of cases, positive antiphospholipid’s antibodies are found. Case report: A 28-year-old woman was admitted to Hospital das ClínicasUFTM on May 21th, 2020, referred under suspicion of stroke with ictus on May 19th, 2020. The exam revealed left hemiparesis, anomic afasia and livedo reticularis on her thighs and thorax. She denied having comorbidities or previous episodes of thromboembolism. She had taken combined oral contraceptive for eleven years, having changed medication a year ago. In the cranial angioresonance exam, acute ischemia was confirmed in addition to moderate microangiopathy and an area of encephalomalacia. In the etiological investigation, HEP-2 positive antibody (antinuclear factor in a fine dotted nuclear pattern) was detected. She was discharged with a prescription of 100 mg of acetylsalicylic acid daily, maintained as a form of secondary prophylaxis. Conclusions: The case illustrates the importance of a thorough physical examination and anamnesis in cerebrovascular disease patients, in order to get a accurate aetiological diagnosis of these diseases, enabling a more effective prognostic evaluation and secondary prophylaxis.

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

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.