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

Anna Bersano, Michela Morbin, Elisa Ciceri, Gloria Bedini, Berlit Peter, Herold Michele, Stefania Saccucci, Valeria Fugnanesi, Nordmeier Hannes, Giuseppe Faragò, Savoiardo Mario, Franco Taroni, Maria Rita Carriero, Giorgio Battista Boncoraglio, Laura Perucca, Luigi Caputi, Eugenio Agostino Parati, Kraemer Markus

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)77-83
Number of pages7
JournalJournal of the Neurological Sciences
Publication statusPublished - May 15 2016


  • Cerebral angiomatosis
  • Diagnosis
  • Divry van Bogaert Syndrome
  • Livedo racemosa
  • Sneddon Syndrome
  • Stroke

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology


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