Conclusion Inspite of most previous reports, which indicate ischemic stroke as an usual central nervous system manifestation, SS could be presented with hemorrhagic stroke even in the initial phase of presentation. [rcm.mums.ac.ir]

Intracerebral hemorrhage (ICH) is unusual in Sneddon's syndrome and has not been reported as the presenting complaint. [ncbi.nlm.nih.gov]

The case is presented of a woman admitted with an ischaemic neurological disease, hypertension, vascular problems, and skin lesions. The skin biopsy was classified as surface perivascular lymphocytic dermatitis, suggestive of occlusive lesion. [scielo.org.co]

• Inflammation

  Among heart disease-related deaths, the most usual cause is inflammation of the heart muscle caused by the high level of eosinophils, although some are deaths due to inflammation of the arteries that supply blood to the heart or pericardial tamponade. [en.wikipedia.org]

  Superficial thrombophlebitis Superficial thrombophlebitis is inflammation of the veins just under your skin, usually in your leg. The symptoms are similar to DVT, but aren't usually as severe. [nhs.uk]

  Enthesitis is inflammation at the insertion of tendons and ligaments into bones. [physio-pedia.com]

  Inflammation mediated tissue injury in pregnancy Therapy should be against inflammation Ann N Y AcadSci 2005 ;1051:413-20 Mouse model of APS---heparin prevents complement activation induced by APL. [slideshare.net]

  A 3-mm punch biopsy showed endothelial inflammation, subendothelial myointimal hyperplasia, and partial occlusion (H&E, ×200). Fig. 2 The ulcers with livedo reticularis. This photograph shows the wound on the right pretibial area. [e-aps.org]

• Multiple Congenital Anomalies

  Semin Neurol 12:385–393 Google Scholar Golabi M, Ito M, Hall BD (1984) A new X-linked multiple congenital anomalies/mental retardation syndrome. [link.springer.com]

  COG8-CDG (CDG-IIh) Schisis association Multiple congenital anomalies-hypotonia-seizures syndrome type 2 Dihydrolipoamide dehydrogenase deficiency Lyme disease Spinocerebellar ataxia 10 B4GALT1-CDG (CDG-IId) Meige syndrome Vici syndrome Familial encephalopathy [checkrare.com]

• Nausea

  […] both eyes Sudden trouble walking Sudden dizziness, loss of balance or coordination Sudden, severe headache with no known cause The effects of an acute ischemic stroke may cause additional symptoms in women including: Face, arm or leg pain Hiccups or nausea [cedars-sinai.edu]

  Case Report A 33-year-old woman admitted to our neurology polyclinic with the complaints of headache, nausea, vomiting, blurred vision lasting for years. [ijdvl.com]

  Case report Here we describe the case of a 64-year-old woman presenting with a 4-year history of a de-novo throbbing, bilateral, parieto-occipital headache associated with facial pain, but without any other accompanying symptom (phono-, photo-phobias, nausea [thejournalofheadacheandpain.springeropen.com]

• Vomiting

  Case Report A 33-year-old woman admitted to our neurology polyclinic with the complaints of headache, nausea, vomiting, blurred vision lasting for years. [ijdvl.com]

  When to see a doctor Contact your doctor if you have unexplained bleeding from your nose or gums; an unusually heavy menstrual period; vomit that is bright red or looks like coffee grounds; black, tarry stool or bright red stool; or unexplained abdominal [mayoclinic.org]

  Here we describe the case of a 64-year-old woman presenting with a 4-year history of a de-novo throbbing, bilateral, parieto-occipital headache associated with facial pain, but without any other accompanying symptom (phono-, photo-phobias, nausea nor vomiting [thejournalofheadacheandpain.springeropen.com]

  The person may experience: Nausea Vomiting Jaundice (yellow skin) Dark urine Pale stool Swelling of the abdomen Kidneys. Blood clots that affect the kidneys can cause kidney damage and blood in the urine. Skin. [drugs.com]

• Hypertension

  The case is presented of a woman admitted with an ischaemic neurological disease, hypertension, vascular problems, and skin lesions. The skin biopsy was classified as surface perivascular lymphocytic dermatitis, suggestive of occlusive lesion. [scielo.org.co]

  Hypertension is the only risk factor significantly associated with a more severe course of the disease; no medication proved effective. [ncbi.nlm.nih.gov]

  Clinical manifestations may include hypertension and associated coagulopathies. The underlying pathophysiology of this syndrome remains obscure, yet increasing recognition of this entity may further ongoing investigations. [medlink.com]

• Vascular Disease

  Sneddon's syndrome is a rare vascular disease affecting mainly skin and brain arterioles leading to their occlusion due to excessive endothelial proliferation. [ncbi.nlm.nih.gov]

  Capillary vessel disease Skin disease Complex syndrome Nervous system diseases Vascular disease Keyword (es) Hombre Livedo reticularis Medicina Sneddon síndrome Aparato circulatorio patología Capilar sanguíneo patología Piel patología Síndrome complejo [pascal-francis.inist.fr]

  Mechanisms of kidney disease in Sneddon’s syndrome: case report and literature review. [3] F. Macário, M.C. Macário, A. Ferro, F. Gonçalves, M. Campos, A. Marques. Sneddon’s syndrome: a vascular systemic disease with kidney involvement?. [revistanefrologia.com]

  Abstract Vasospastic diseases are peripheral vascular disorders caused by vasospasm, which means a reversible localized or diffuse vasoconstriction of arteries or smaller blood vessels. [link.springer.com]

  The Journal of Headache and Pain 2009 10 :109 https://doi.org/10.1007/s10194-009-0109-3 © Springer-Verlag 2009 Received: 7 December 2008 Accepted: 4 February 2009 Published: 14 March 2009 Abstract Sneddon’s syndrome is a rare vascular disease affecting [thejournalofheadacheandpain.springeropen.com]

• Stroke

  Conclusion Inspite of most previous reports, which indicate ischemic stroke as an usual central nervous system manifestation, SS could be presented with hemorrhagic stroke even in the initial phase of presentation. [rcm.mums.ac.ir]

  CONCLUSION: AECA were frequently found in patients with Sneddon's syndrome, in contrast to the patients with stroke without livedo. [ncbi.nlm.nih.gov]

  The association between migraine and juvenile stroke: a case–control study. Headache 2003 ; 43: 90 – 5. Google Scholar | Crossref | Medline | ISI 9. [doi.org]

• Headache

  The proportion with headache in this second group is 58% (102 individuals), with headache described as migraine in 28 (27.5%) of the headache subjects, including six with migraine with aura. [ncbi.nlm.nih.gov]

  Sneddon syndrome is a rare non-inflammatory obliterative vasculopathy, characterised by the association of cardiovascular (arterial hypertension, intermittent claudication, and coronary artery disease) and neurological events (ischaemic stroke, headache [scielo.org.co]

  Headache: Headache. 2002;42:352-355. Bottin L, Frances C, De Zuttere D, et al. Sneddon Syndrome without Antiphospholipid Antibodies in 55 Cases: Clinical, Radiological and Pathophysiological Features (S43. 005). [rcm.mums.ac.ir]

• Seizure

  Neurologic Dermatologic Cerebrovascular accident Livedo reticularis Transient ischemic attack Leg ulcers Severe migraine Necrotizing purpura Chorea Distal cutaneous ischemia/ Seizures gangrene Multi-infarct dementia Widespread cutaneous necrosis Ischemic [medical-dictionary.thefreedictionary.com]

  Thus, anti-GluR3B Ab's are not crossreactive with aPL, and not produced as a non-specific consequence of seizures on the one hand, or autoimmune-diseases on the other. [ncbi.nlm.nih.gov]

  Rare neurologic symptoms include seizures, chorea, or myelopathies. [uniprot.org]

• Dizziness

  Sneddon syndrome is a rare non-inflammatory obliterative vasculopathy, characterised by the association of cardiovascular (arterial hypertension, intermittent claudication, and coronary artery disease) and neurological events (ischaemic stroke, headache, dizziness [scielo.org.co]

  Nonspecific prodromal symptoms (headache, dizziness) frequently (80%) precede livedo racemosa for 3.5 and (multi)focal neurological symptoms of fully developed disease for 9 years followed by progressive cognitive impairment (60%) 10 years later. [ncbi.nlm.nih.gov]

  In the natural course of patients with SS, neurological events such as headache (62%) and dizziness (54%) have been recorded. [elsevier.es]

  Non-specific prodromal symptoms (headache, dizziness) often precede livedo reticularis by several years and this is followed by the development of focal neurological symptoms and then progressive cognitive impairment. [skin-disorders.net]

  Dizziness. Strokes and TIAs. Changes in your personality, and your ways of thinking and behaving. Kidney problems (rare). The signs and symptoms don’t usually appear all at once. [my.clevelandclinic.org]

• Chorea

  […] encephalopathy Peripheral neuropathy Obstetric Myasthenia gravis Recurrent fetal loss Sneddon's syndrome Intrauterine growth retardation Preeclampsia Cardiovascular Chorea gravidarum Myocardial infarction Postpartum syndrome Valvular lesions, mitral [medical-dictionary.thefreedictionary.com]

  Of nine patients with Sneddon's syndrome, three had rheumatic heart disease (mitral valve stenosis, regurgitation, or both) due to rheumatic fever or Sydenham's chorea. [ncbi.nlm.nih.gov]

  Rare neurologic symptoms include seizures, chorea, or myelopathies. [uniprot.org]

  Features can involve skin (livedo reticularis with no infiltration, and affecting the limbs, trunk, and sometimes the face), central nervous system (cerebrovascular accidents, epilepsy, vertigo, and sometimes Pseudobulbar Syndrome, chorea, episodes of [accessanesthesiology.mhmedical.com]

IgM and IgG, anti-beta-2-glycoprotein antibodies IgM and IgG) (3) thorough workup for conditions associated with hypercoagulability (4) echocardiography of the heart References: Schellong SM, Weissenborn K, et al. [meducator3.net]

Skin biopsy was nonspecific.1 Extensive immunologic, hematologic, and infectious workup was unremarkable.2 The patient was diagnosed with Sneddon syndrome and discharged on aspirin. [n.neurology.org]

The patient’s age, livedo and middle cerebral artery infarct, as well as her nonrevealing workup on repeat antiphospholipid antibody labs and hypercoagulable studies, were concerning for Sneddon syndrome. [the-rheumatologist.org]

"Goodpasture Syndrome Workup". Medscape Reference. WebMD. Retrieved 14 March 2014. ^ a b c d e Kathuria, P; Sanghera, P; Stevenson, FT; Sharma, S; Lederer, E; Lohr, JW; Talavera, F; Verrelli, M (21 May 2013). Batuman, C (ed.). [en.wikipedia.org]

moderate (4-5, 4% risk), high (6-7, 8% risk) stroke in 48hrs MRI showing DWI lesion increases risk of re-stroke, if also vessel occlusion then 30% risk of restroke EXPRESS trial – 2007 ( UK ) Historical control compared to last few years of rapid workup [emilytam.com]

The success of warfarin in the treatment of antiphospholipid syndromes and the failure of immunosuppression and aspirin in the treatment of Sneddon's syndrome argue that warfarin anticoagulation may be the most appropriate intervention currently available [ncbi.nlm.nih.gov]

Management and treatment The most widely accepted treatment is anticoagulation with warfarin. [orpha.net]

While sensorimotor deficits after stroke in these patients had a good prognosis, neuropsychological deficits persisted. Of the 21 patients, 14 were incapable of gainful employment, 10 because of severe cognitive dysfunction. [ncbi.nlm.nih.gov]

The objectives were to assess the clinicoradiological pattern of SS without APL (SSAPL−) and its midterm prognosis. [onlinelibrary.wiley.com]

Prognosis Some symptoms tend to resolve over a very variable time course. Prevention of further vascular events with anticoagulation is the key to a good prognosis. [patient.info]

Prognosis SS is a chronic, intermittent or progressive disease leading to major morbidity. Dementia occurs in 50% of patients resulting in early retirement. The documents contained in this web site are presented for information purposes only. [orpha.net]

Sneddon syndrome - a cerebral arteriopathy of unknown etiology, characterized by noninflammatory intimal hyperplasia of medium-size vessels. [medical-dictionary.thefreedictionary.com]

Sneddon's syndrome is a peculiar clinicopathological condition, probably with several etiologies, but is distinct from primary antiphospholipid syndrome. [ncbi.nlm.nih.gov]

Future therapy should identify the diverse treatment modalities for different etiological subgroups. [elsevier.es]

Notch 3 gene mutation associated with Sneddons syndrome- a complex disorder of unknown etiology with variable clinical features overlapping the phenotype of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy [CADASIL [ashg.org]

Summary Epidemiology SS has an estimated annual incidence of approximately 1/250,000. The disease predominantly affects women in young adulthood. [orpha.net]

You might also be interested in Vascular medicine Occurrence/Epidemiology This section has been translated automatically. Incidence is 0.4/100,000; Etiopathogenesis This section has been translated automatically. [altmeyers.org]

Epidemiology Incidence is estimated at four cases per million population per year, but the true incidence may be higher because some patients are not diagnosed. [patient.info]

We suggest that these antibodies may be pathophysiologically related to the clinical manifestation observed in some patients with this syndrome. [ncbi.nlm.nih.gov]

Gain fresh perspectives and up-to-date insights from the world’s leading authorities on the pathophysiology, diagnosis, and management of stroke. [books.google.de]

Pathophysiology May be a particular form of obliterating vasculitis or of an Antiphospholipid Antibodies Syndrome. Diagnosis Clinically evocated in patients combining livedo reticularis and neurological signs. [accessanesthesiology.mhmedical.com]

Prevention of coronary heart disease with pravastatin in men with hypercholesterolemia. ‎ [books.google.de]

Aspirin was not effective in preventing thrombosis. After the onset of oral anticoagulant therapy, no recurrences were seen. [ncbi.nlm.nih.gov]