Sneddon Syndrome Presenting With Unilateral Third Cranial Nerve Palsy

Jiménez‐Gallo, David; Albarrán-Planelles, C.; Linares‐Barrios, Mario; González-Fernández, Julio A.; Espinosa-Rosso, Raúl; Báez-Perea, José María

doi:10.1097/wno.0b013e3182a3060d

Cited by 4 publications

(8 citation statements)

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“…Rehany et al reported a young SS patient with internuclear ophtalmoplegia followed by ophthalmic artery occlusion [ 41 ]. Unilateral third cranial nerve palsy has been seen in a patient with SS and this is likely associated with microvascular complication [ 43 ].…”

Section: Introductionmentioning

confidence: 99%

“…Some diseases that can occur with generalized LR (such as SLE, polyarteritis nodosa, cryoglobulinemia, livedoid vasculitis, cold agglutinin disease, arteriosclerosis, cholesterin embolization and others [ 6 , 22 , 59 ]), must be excluded by appropriate clinical and laboratory tests. The differential diagnosis of neurological symptoms include multiple sclerosis, multiple embolization from cardiac source, infectious conditions (such as syphilis and lyme disease), and others [ 6 , 43 ]. Central nervous system (CNS) vasculitis refers to a broad array of diseases that result in inflammation and destruction of the blood vessels of brain, spinal cord and the meninges.…”

Section: Introductionmentioning

confidence: 99%

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Sneddon’s syndrome: a comprehensive review of the literature

Xu²,

Liang³

2014

Orphanet J Rare Dis

116

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Sneddon’s syndrome (SS) is a rare non-inflammatory thrombotic vasculopathy characterized by the combination of cerebrovascular disease with livedo racemosa(LR). The Orpha number for SS is ORPHA820. It has been estimated that the incidence of SS is 4 per 1 million per annum in general population and generally occurs in women between the ages of 20 and 42 years. LR may precede the onset of stroke by years and the trunk and/or buttocks are involved in nearly all patients. The cerebrovascular manifestations are mostly secondary to ischemia (transient ischemic attacks and cerebral infarct). Other neurological symptoms range from headache, cerebral hemorrhage, seizures, cognitive and psychiatric disturbances. The involved internal organs include heart, kidney, and eyes. Histological findings of skin are characteristic and the involved vessels are small to medium-sized arteries at the border of dermis to subcutis with a distinct histopathological time course. The main diagnostic criteria are general LR with typical histopathological findings on skin biopsy and focal neurological deficits. The pathogenesis is related to hypercoagulable state and intrinsic small-vessel vasculopathy. The optimal management remains an unsolved problem and long-term anticoagulation have been recommended for cerebral ischemic events based on the presumed pathogenesis. There are controversial results in treatment of SS with immunomodulatory agents. The aim of this review is to comprehensively discuss this disease.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Sneddon’s syndrome: a comprehensive review of the literature

Xu²,

Liang³

2014

Orphanet J Rare Dis

116

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“…Sneddon syndrome is a non-inflammatory vasculopathy involving small- and medium-sized arteries. Sneddon syndrome involves various clinical features, including neurological and non-neurological symptoms [ 3 , 4 ]. The disease commonly affects women aged 20-40 years.…”

Section: Discussionmentioning

confidence: 99%

“…The disease commonly affects women aged 20-40 years. Multiple isolated familial cases were reported suggesting genetic causes [ 4 ].…”

Section: Discussionmentioning

confidence: 99%

Hidden in the Rash: A Sneddon Syndrome Case Report

Almutawa,

Almalak,

Alotaibi

2023

Cureus

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This clinical case report aims to highlight the unusual presentation of Sneddon syndrome with a possible association with paroxysmal hemicrania. A medical record review was performed at a tertiary hospital in Riyadh, Saudi Arabia. Data collected include clinical evaluations and laboratory and imaging results. Informed consent was obtained. Hereby, we present a 27-year-old female who presented with multiple stroke attacks, along with severe headaches involving right retro-orbital pain with an eight-year history of spotted skin lesions. Initial unenhanced computed tomography (UCT) brain in the emergency showed left insular cortex hypodensity, revealing acute ischemic insult. Subsequent magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) revealed acute ischemic infarct in the territory of the left middle cerebral artery (MCA) involving the insula and frontoparietal lobe. Further investigations were done, including cerebrospinal fluid (CSF) analysis and autoimmune and infectious workup, which were unrevealing. Skin biopsy of the lesions showed subcutaneous fat necrosis with nonspecific scattered fibrinogen positivity and was labeled as livedo reticularis vs. livedo racemosa. A Sneddon syndrome diagnosis can be very challenging, needing a high index of suspicion to direct the diagnostic investigations. Moreover, the presence of a severe headache is an unusual phenomenon that needs further study.

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“…Эти неспецифические симптомы в большинстве случаев предшествуют появлению сетчатого ливедо и серьезных неврологических изменений [14]. В литературе также были описаны экстрапирамидные расстройства в виде хореи или тремора в качестве манифестации синдрома Снеддона [36], а также поражение черепно-мозговых нервов [37].…”

Section: клиническая картина 1 сетчатое ливедоunclassified

A rare angiopathy with lesions of the skin and central nervous system: Sneddon syndrome and disease

Gruzmanov

Мазуренко

2022

Juvenis scientia

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Sneddon syndrome is a rare, progressive disease that affects relatively young people, mainly female, and manifests itself in two main symptoms: livedo reticularis and recurrent cerebral infarctions. First described in 1965 by the English dermatologist Ian Bruce Sneddon, the syndrome named in his honor unites a heterogeneous group of diseases with different pathogenesis and similar clinical manifestations. Skin manifestations of the disease in the form of reticular livedo are benign, cerebrovascular manifestations are reduced to repeated cerebral infarctions, progressive dementia and can lead to the death of patients. The literature review describes the basic concepts of the etiology, pathogenesis of primary and secondary forms of Sneddon syndrome, and proposes the concept of Sneddon’s disease to describe the primary forms of the syndrome. The article contains photographs of patients from the clinical practice of the authors of the article. The review also describes methods of diagnosis and differential diagnosis of the disease and recommended methods of treatment, which is based on the elimination of additional risk factors for vascular damage and thrombosis, blood pressure control, lipid and carbohydrate metabolism, as well as the appointment of anticoagulant and disaggregant therapy. The use of anti-inflammatory or immunosuppressive therapy remains controversial. The main goal of treatment is the prevention of organic brain damage and neuropsychiatric complications. Unfortunately, the neuropsychiatric prognosis of Sneddon syndrome is relatively poor with the development of impaired memory, concentration, and visual-spatial skills. In exceptional cases, the progression of the disease can lead to death.

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Sneddon Syndrome Presenting With Unilateral Third Cranial Nerve Palsy

Cited by 4 publications

References 11 publications

Sneddon’s syndrome: a comprehensive review of the literature

Sneddon’s syndrome: a comprehensive review of the literature

Hidden in the Rash: A Sneddon Syndrome Case Report

A rare angiopathy with lesions of the skin and central nervous system: Sneddon syndrome and disease

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