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

• Intravenous Administration

  After intravenous administration of cyclophosphamide and the start of oral prednisolone, the anti-cardiolipin antibody level decreased and his physical condition improved. [ncbi.nlm.nih.gov]

• Hypertension

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

• 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]

  The Journal of Headache and Pain 2009 10 :109 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 mainly skin and brain arterioles leading to [thejournalofheadacheandpain.springeropen.com]

  Handbook of clinical neurology, volume 11: vascular diseases, part iii. New York: Elsevier Science, 1989:401–10. Tourbah A, Piette JC, Iba-Zizen MT, et al. The natural course of cerebral lesions in Sneddon syndrome. Arch Neurol1997;54:53–60. [jnnp.bmj.com]

  Bell, Cerebral ischemic events associated with endocarditis, retinal vascular disease, and lupus anticoagulant, The American Journal of Medicine, 90, 3, (299), (1991). J. Dietl, G. Stroppel, M. Poremba and D. [doi.org]

• Systolic Murmur

  On examination, he had livedo reticularis and perniosis and a systolic murmur. [ncbi.nlm.nih.gov]

• Facial Pain

  Here, we describe the case of a 64-year-old woman presenting with a 4-year history of a throbbing, bilateral, parieto-occipital headache associated with facial pain, but without any other accompanying symptom. [ncbi.nlm.nih.gov]

• Cesarean Section

  Delivery by Cesarean section in the 36th week of pregnancy resulted in marked improvement of the neurological signs and symptoms. [ncbi.nlm.nih.gov]

• Stroke

  Considered in the context of increased risk of stroke with migraine, a higher frequency of livedo in migraineurs with stroke, and the association of migraine and livedo reticularis, the question of whether livedo reticularis may be a risk marker for stroke [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]

  J Neurol Neurosurg Psychiatry 74:542–544 PubMed Central PubMed View Article Google Scholar Headache Classification Subcommittee of The International Headache Society (2004) The International Classification of Headache Disorders, 2nd edn. [thejournalofheadacheandpain.springeropen.com]

• Dizziness

  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]

  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]

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

  Nonspecific 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. [patient.info]

• Neuralgia

  The pain, initially misdiagnosed as atypical trigeminal neuralgia, worsened up to chronic daily and such severely disabling headache that she was constrained to bed. [ncbi.nlm.nih.gov]

  In this case the patient has not been properly treated for a 4-year period and an atypical bilateral trigeminal neuralgia was diagnosed. [thejournalofheadacheandpain.springeropen.com]

  1 Infantile spasms broad thumbs Microcephaly glomerulonephritis Marfanoid habitus Laing distal myopathy MOGS-CDG (CDG-IIb) Sideroblastic anemia and mitochondrial myopathy Congenital mirror movement disorder Limb-girdle muscular dystrophy Trigeminal neuralgia [checkrare.com]

• Trigeminal Neuralgia

  The pain, initially misdiagnosed as atypical trigeminal neuralgia, worsened up to chronic daily and such severely disabling headache that she was constrained to bed. [ncbi.nlm.nih.gov]

  In this case the patient has not been properly treated for a 4-year period and an atypical bilateral trigeminal neuralgia was diagnosed. [thejournalofheadacheandpain.springeropen.com]

  neuralgia Spinocerebellar ataxia autosomal recessive 8 Primary orthostatic tremor Spinocerebellar ataxia 18 Spinocerebellar ataxia 14 GAPO syndrome Dopa-responsive dystonia; Segawa syndrome AD Myosin storage myopathy Neurocutaneous melanosis Grubben [checkrare.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]

"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]

• Hemianopia with Macular Sparing

  Visual field testing revealed a left homonymous hemianopia with macular sparing. [ncbi.nlm.nih.gov]

  Sneddons syndrome in a patient with homonymous hemianopia with macular sparing. Bull Soc Belge Ophthalmol. 1996;263:103–107. 13. Rehany U, Kassif Y, Rumelt S. [journals.lww.com]

  Narbay G: Sneddon’s syndrome in a patient with homonymous hemianopia with macular sparing. Bull Soc Belge Ophtalmol. 1996, 263: 103-107. 41. [ojrd.biomedcentral.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]

An additional anticoagulation treatment with heparin and warfarin was started and maintained for the treatment of the disease. The skin ulcers were treated daily with aseptic dressing. [e-aps.org]

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]

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]

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

[…] cognitive impairment / juvenile dementia, severe leukoaraiosis / juvenile stroke cerebral angiogram shows extensive, multifocal occlusions of peripheral small and medium-sized cerebral arteries and a network of small corkscrew-like collaterals Treatment and prognosis [radiopaedia.org]

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]

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]

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

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

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]

Epidemiology [ edit ] Sneddon's syndrome is a rare condition that is usually misdiagnosed. [3] It occurs in families and may be inherited in an autosomal dominant fashion. [en.wikipedia.org]

The pathophysiology of Sneddon's syndrome remains elusive, but various prothrombotic abnormalities have been previously reported in this setting. [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]

Etiology This disease has at least two distinct etiologies, listed in the next section (pathophysiology). [dermatologyadvisor.com]

Treatment with a combination of immunosuppressive agents and warfarin or aspirin prevented further complications and improved renal function. It is important for those in different specialties to be aware of this potentially treatable disorder. [ncbi.nlm.nih.gov]

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