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". A similar syndrome including livedo racemosa and cerebrovascular disease, often associated with anticardiolipin antibodies, has been described as Sneddon Syndrome (SS) highlighting the question whether these two conditions have to be considered different entities or indeed different features of a unique syndrome. Herein, we report the clinical, neuroradiological, histopathological findings and follow up of three cases diagnosed as Divry-van Bogaert Syndrome, including an updated review of literature of both DBS and SS cases. Our findings support the assumption that DBS and SS are different disease entities. DBS is characterized by the typical angiographic feature of angiomatosis, a hereditary trait and a juvenile onset of cognitive impairment and leukoaraiosis, whereas SS has less severe manifestations of cerebrovascular disease associated with livedo racemosa but without the characteristic cerebral angiography. The report of our cases and the literature review underline the necessity of a detailed work-up and the collection of larger series to better clarify the DBS and SS phenotype and course.
The diagnostic challenge of Divry van Bogaert and Sneddon Syndrome : report of three cases and literature review / A. Bersano, M. Morbin, E. Ciceri, G. Bedini, P. Berilt, M. Herold, S. Saccucci, V. Fugnanesi, H. Nordmeyer, G. Faragò, M. Savoiardo, F. Taroni, M. Carriero, G.B. Boncoraglio, L. Perucca, L. Caputi, E.A. Parati, M. Kraemer. - In: JOURNAL OF THE NEUROLOGICAL SCIENCES. - ISSN 0022-510X. - 364(2016 May), pp. 77-83.
|Titolo:||The diagnostic challenge of Divry van Bogaert and Sneddon Syndrome : report of three cases and literature review|
BERSANO, ANNA (Corresponding)
|Parole Chiave:||cerebral angiomatosis; diagnosis; Divry van Bogaert syndrome; livedo racemosa; Sneddon syndrome; stroke; neurology (clinical); neurology|
|Settore Scientifico Disciplinare:||Settore MED/34 - Medicina Fisica e Riabilitativa|
|Data di pubblicazione:||mag-2016|
|Data ahead of print / Data di stampa:||5-mar-2016|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1016/j.jns.2016.03.011|
|Appare nelle tipologie:||01 - Articolo su periodico|
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