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Sneddon's Syndrome

Sneddon Syndrome


Presentation

  • Intracerebral hemorrhage (ICH) is unusual in Sneddon's syndrome and has not been reported as the presenting complaint.[ncbi.nlm.nih.gov]
  • Patients presenting with clinical stroke were excluded. Patients presented with cognitive complaints at an early age and all noted skin lesions from 6 months to 10 years before onset of cognitive symptoms.[ncbi.nlm.nih.gov]
  • The present cases, having repeatedly negative antiphospholipid antibodies, support the classification of Sneddon's Syndrome as an independent nosological entity.[ncbi.nlm.nih.gov]
  • Present knowledge of the pathogenic relationships and the particular anatomical features of the skin were taken into account.[ncbi.nlm.nih.gov]
  • The use of a mixture of phospholipids as antigen could present some advantages in serological studies performed in antiphospholipid syndromes.[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]
Rigor
  • Sneddon's syndrome patients have a worse prognosis and may represent a subgroup of patients who demands more rigorous follow-up.[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]
  • We report a case of Sneddon's syndrome with the triad of livedo reticularis, hypertension, and neurologic symptoms. The procedures for diagnosis and the tests to delineate clotting abnormalities are examined.[ncbi.nlm.nih.gov]
  • Five patients presented with Sneddon's syndrome characterised by cerebral infarction, livedo reticularis, hypertension, epilepsy and a progressive dementia. Two patients had raised anticardiolipin antibody titres.[ncbi.nlm.nih.gov]
  • It is likely that several pathogenic mechanisms may give rise to Sneddon's syndrome, as the condition is associated with a high incidence of generalised atherosclerosis, hypertension, valvular heart disease and the presence of antiphospholipid antibodies[ncbi.nlm.nih.gov]
  • We investigated 2 patients with Sneddon's syndrome, elevated anticardiolipin antibodies and systemic complications, which included stroke, habitual abortions, cardiac valvular lesions, acrocyanosis, hypertension and renal insufficiency.[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.[jnnp.bmj.com]
  • Raynaud’s phenomenon and the vascular disease in scleroderma. Curr Opin Rheumatol. 1995;7:529–534. CrossRef Google Scholar 70. Kiowski W, Erne P, Buhler FR. Use of nifedipine in hypertension and Raynaud’s phenomenon.[link.springer.com]
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]
Panic Attacks
  • Her worsening in the first year was characterized by obsessive-compulsive behaviours, body-image misperceptions and panic attacks, improved for a period using olanzapine.[ncbi.nlm.nih.gov]
Delusion
  • A 24-year-old man with livedo racemosa and psychiatric disturbances, manifesting as low intelligence (IQ 80) and delusions, had anti-cardiolipin antibody and showed shortening of the fingers and toes.[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
  • Sneddon's syndrome (SS) is an uncommon disorder, characterized by the association of ischemic stroke and widespread livedo reticularis.[ncbi.nlm.nih.gov]
  • 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]
  • To review the clinical, psychometric, laboratory, and radiologic findings of 6 patients with Sneddon's syndrome (SS) who presented with cognitive dysfunction rather than stroke. Case series.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
  • 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]
  • In this case, we aimed to present a 28-year-old female patient with diffuse LR, cardiac valve disease and migraine-type headache who had a right hemiplegic attack.[ncbi.nlm.nih.gov]
  • A 51-year-old man gave a 2-year history of worsening mobility, cognitive decline and headaches. He had a history of thromboembolic stroke, recurrent transient ischaemic attacks and a spontaneous intraventricular haemorrhage.[ncbi.nlm.nih.gov]
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]
  • The signs of a stroke are: Sudden numbness or weakness of the face, arm or leg, especially on one side of the body Sudden confusion Sudden trouble speaking Sudden trouble seeing in one or both eyes Sudden trouble walking Sudden dizziness, loss of balance[cedars-sinai.edu]
  • 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]
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]

Workup

  • 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]
  • "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]
  • Sneddon’s syndrome in a patient with homonymous hemianopia with macular sparing. Bull Soc Belge Ophtalmol. 1996; 263 :103–107. [ PubMed ] [ Google Scholar ] 41. Rehany U, Kassif Y, Rumelt S.[ncbi.nlm.nih.gov]

Treatment

  • 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]
  • Treatment to date has been empirical.[ncbi.nlm.nih.gov]
  • Management and treatment The most widely accepted treatment is anticoagulation with warfarin.[orpha.net]
  • 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]
  • Treatment with acetylsalicylic acid led to normalization of hemostatic parameters and resulted in a symptom-free period of more than 10 months. The importance of hemostatic screening in patients with Sneddon's syndrome is discussed.[ncbi.nlm.nih.gov]

Prognosis

  • 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]
  • Sneddon's syndrome patients have a worse prognosis and may represent a subgroup of patients who demands more rigorous follow-up.[ncbi.nlm.nih.gov]
  • Treatment and prognosis There is no effective treatment to control disease progression but long-term antiplatelet therapy and anticoagulation are commonly used in cases with cerebral manifestations. Promoted articles (advertising)[radiopaedia.org]
  • 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]

Etiology

  • 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]
  • […] cerebrovascular thrombosis involving the medium-sized arteries. livedo reticularis as a skin manifestation progressive cerebral strokes and hemorrhage cerebral dysfunction It is characterized by intimal hyperplasia of medium-sized arteries with unknown etiology[radiopaedia.org]

Epidemiology

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

Pathophysiology

  • The pathophysiology of Sneddon's syndrome remains elusive, but various prothrombotic abnormalities have been previously reported in this setting.[ncbi.nlm.nih.gov]
  • We suggest that these antibodies may be pathophysiologically related to the clinical manifestation observed in some patients with this syndrome.[ncbi.nlm.nih.gov]
  • We described three new cases of SS and discuss the pathophysiology of this disease.[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]
  • Pier Luigi Meroni Springer, 20.11.2014 - 267 Seiten This book, part of the series Rare Diseases of the Immune System, offers comprehensive, up-to-date coverage of the pathophysiology and management of the antiphospholipid syndrome (APS).[books.google.de]

Prevention

  • 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]
  • The treatment options for SS to prevent stroke recurrence and future disability include antiplatelet therapy, anticoagulation, or immunosuppression.[ncbi.nlm.nih.gov]
  • 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]
  • It is important to recognize the Sneddon's syndrome, particularly because stroke episodes may be prevented through appropriate treatment.[ncbi.nlm.nih.gov]

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