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 Michele, Saccucci Stefania, Fugnanesi Valeria, Nordmeier Hannes, Faragò Giuseppe, Savoiardo Mario, Taroni Franco, Carriero Mariarita, Boncoraglio Giorgio Battista, Perucca Laura, Caputi Luigi, Parati Eugenio Agostino, Kraemer Markus

Research output: Contribution to journalArticle

Abstract

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
Volume364
DOIs
Publication statusPublished - May 15 2016

Keywords

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

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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