The diagnostic challenge of Divry van Bogaert and Sneddon Syndrome: Report of three cases and literature review

Bersano, Anna; Morbin, Michela; Ciceri, Elisa; Bedini, Gloria; Berlit, Peter; Herold, Michèle; Saccucci, Stefania; Fugnanesi, Valeria; Nordmeyer, Henning; Faragò, Giuseppe; Savoiardo, M.; Taroni, Franco; Carriero, Maria Rita; Boncoraglio, Giorgio B.; Perucca, Laura; Caputi, Luigi; Parati, Eugenio; Kraemer, Markus

doi:10.1016/j.jns.2016.03.011

Cited by 22 publications

(18 citation statements)

References 46 publications

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“…Another syndrome, called Divry van Bogaert Syndrome (DBS), is a familial juvenile-onset disorder characterized by livedo racemosa, white matter disease, dementia, epilepsy and angiographic finding of “cerebral angiomatosis”, raising the question as to whether DBS and SS can be considered different entities or indeed different features of the same syndrome. 5 Our patient more closely matched the criteria for the diagnosis of SS.…”

Section: Discussionsupporting

confidence: 74%

“… 3 The incidence of cases is 4 per 1 million per year, and a mortality rate of 9.5% was reported in a mean observation period of 6.2 years. 2 SS is far more common in young women between 20 and 42 years of age 2 - 5 and has a wide spectrum of physical, neurologic, and laboratory findings. The neurological signs include severe cognitive impairment or dementia syndrome and psychiatric changes.…”

mentioning

confidence: 99%

“… 2 , 3 SS can be associated with valvulopathy, a history of spontaneous abortion, renal involvement, vascular dementia, systemic hypertension, acrocyanosis, Raynaud’s phenomenon, secondary headaches, venous thrombosis and seizures. 2 - 5 …”

mentioning

confidence: 99%

“… 2 - 4 There is no gold standard treatment, although antiplatelet therapy or anticoagulation are the most recommended options. 3 - 5 …”

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confidence: 99%

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Cognitive and psychiatric changes as first clinical presentation in Sneddon syndrome

Fabiani¹,

Filho

Koppe³

et al. 2018

Dement. neuropsychol.

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Sneddon syndrome (SS) is a rare progressive non-inflammatory thrombotic vasculopathy affecting small/medium-sized blood vessels of unknown origin. It is strongly associated with the presence of antiphospholipid antibodies (AA). The presence of livedo reticularis and cerebrovascular disease are hallmark features. The condition is far more common in young women. We report a case of SS in a 43 year-old male with a two-year history of progressive cognitive impairment consistent with dementia syndrome, and major personality changes, besides livedo reticularis and cerebral angiographic pattern of vasculitis. AA were borderline. The recognition of skin blemishes that precede strokes should raise the hypothesis of SS. AA are elevated in more than half of cases, but their role in the pathogenesis or association of positive antibodies and SS remains unclear. Dementia syndrome in young patients should be extensively investigated to rule out reversible situations. Typical skin findings, MRI and angiography may aid diagnosis.

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Section: Discussionsupporting

confidence: 74%

mentioning

confidence: 99%

mentioning

confidence: 99%

“… 2 - 4 There is no gold standard treatment, although antiplatelet therapy or anticoagulation are the most recommended options. 3 - 5 …”

mentioning

confidence: 99%

See 2 more Smart Citations

Cognitive and psychiatric changes as first clinical presentation in Sneddon syndrome

Fabiani¹,

Filho

Koppe³

et al. 2018

Dement. neuropsychol.

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“…Cerebral magnetic resonance imaging (MRI) may show acute ischaemic lesions or chronic infarctions located in both the peripheral tributaries and watershed areas [15]. Cerebral angiography, which has not been performed systemically in all reported cases, is usually normal [16]. However, Fabiani et al reported vasculitic changes in some patients [17].…”

Section: Central Nervous System Diseasesmentioning

confidence: 99%

Livedo racemosa in neurological diseases: an update on the differential diagnoses

Mitri

Enk

Bersano

et al. 2020

Euro J of Neurology

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Livedo is a net‐like violaceous skin pattern. It can be classified as physiological or pathological. The physiological livedo reticularis usually appears in cold conditions, whereas the pathological and irregular livedo, which persists in warm temperatures, is often labeled as ‘livedo racemosa’. Some neurological pathologies are associated with livedo, most commonly those with an inflammatory component or those derived from systemic disorders. The present review summarizes the most important central and peripheral neurological diseases in pediatric and adult age groups associated with livedo, providing physicians with an overview of the clinical presentation, etiology, diagnosis and management of these conditions.

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Sneddon syndrome: under diagnosed disease, complex clinical manifestations and challenging diagnosis. A case-based review

2020

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The diagnostic challenge of Divry van Bogaert and Sneddon Syndrome: Report of three cases and literature review

Cited by 22 publications

References 46 publications

Cognitive and psychiatric changes as first clinical presentation in Sneddon syndrome

Cognitive and psychiatric changes as first clinical presentation in Sneddon syndrome

Livedo racemosa in neurological diseases: an update on the differential diagnoses

Sneddon syndrome: under diagnosed disease, complex clinical manifestations and challenging diagnosis. A case-based review

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