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Journal of Neurology

, Volume 265, Issue 9, pp 2143–2145 | Cite as

Sneddon syndrome and non-bacterial thrombotic endocarditis: a clinicopathological study

  • José Berciano
  • Nuria Terán-Villagrá
Letter to the Editors
  • 80 Downloads

Dear Sirs,

Sneddon syndrome (SS) is a non-inflammatory thrombotic arteriopathy characterized by the combination of cerebrovascular disease and livedo racemosa (LR) [1, 2, 3]. SS is now regarded as a common manifestation of different disease entities, namely: (1) idiopathic SS with neither antiphospholipid antibodies (aPL) nor coexisting systemic lupus erythematosus (SLE); (2) primary antiphospholipid syndrome (APS)-related SS; (3) SLE-related SS with or without aPL. Autopsy studies are scanty [4, 5, 6]. Here, we describe the full clinicopathological study of an SS patient with marantic endocarditis, who was included as case 4 in our idiopathic SS series collected between 1977 and 1981 [1].

This right-handed patient was admitted in 1980, at age 53, with a 4-year history of progressive cognitive decline. At ages 39 and 52, she had experienced two sudden and reversible episodes of right hemiparesis. There was generalized LR (Fig. 1a) [7]. She had seven pregnancies all of them ending in...

Notes

Compliance with ethical standards

Conflicts of interest

The authors declare that we have no conflict of interest.

Ethical standards

This study was performed in accordance with ethical standards.

References

  1. 1.
    Rebollo M, Val JF, Garijo F, Quintana F, Berciano J (1983) Livedo reticularis and cerebrovascular lesions (Sneddon’s syndrome). Clinical, radiological and pathological features in eight cases. Brain 106:965–979CrossRefPubMedGoogle Scholar
  2. 2.
    Francès C, Papo T, Wechsler B, Laporte JL, Biousse V, Piette JC (1999) Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients. Medicine (Baltimore) 78:209–219CrossRefGoogle Scholar
  3. 3.
    Wu S, Xu Z, Liang H (2014) Sneddon’s syndrome: a comprehensive review of the literature. Orphanet J Rare Dis 9:215CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Piñol Aguadé J, Ferrándiz C, Ferrer Roca O, Ingelmo M (1975) Livedo reticularis y accidentes cerebro-vasculares. Med Cutan Ibero Lat Am 3:257–265Google Scholar
  5. 5.
    Muerza FM, González G, Ortiz E, Saracibar N (1998) Cerebral hemorrhage in Sneddon syndrome. Rev Neurol 27:74–76PubMedGoogle Scholar
  6. 6.
    Hilton DA, Footitt D (2003) Neuropathological findings in Sneddon’s syndrome. Neurology 60:1181–1182CrossRefPubMedGoogle Scholar
  7. 7.
    Uthman IW, Khamashta MA (2006) Livedo racemosa: a striking dermatological sign for the antiphospholipid syndrome. J Rheumatol 33:2379–2382PubMedGoogle Scholar
  8. 8.
    Bottin L, Francès C, de Zuttere D, Boëlle PY, Muresan IP, Alamowitch S (2015) Strokes in Sneddon syndrome without antiphospholipid antibodies. Ann Neurol 77:817–829CrossRefPubMedGoogle Scholar
  9. 9.
    Fierini F, Barilaro A, Giambene B, Carlucci G, Grandi V, Maio V, Pantoni L (2015) Moyamoya in a patient with Sneddon’s syndrome. Clin Neurol Neurosurg 129:34–36CrossRefPubMedGoogle Scholar
  10. 10.
    Divry P, Van Bogaert L (1946) Une maladie familiale caractérisée par une angiomatose diffuse cortico-méningée non calcifiante et une démyélinisation progressive de la susbtance blanche. J Neurol Nuerosurg Psychiatry 9:41–54CrossRefGoogle Scholar
  11. 11.
    Bersano A, Morbin M, Ciceri E, Bedini G, Berlit P, Herold M, Saccucci S, Fugnanesi V, Nordmeyer H, Faragò G, Savoiardo M, Taroni F, Carriero M, Boncoraglio Giorgio B, Perucca L, Caputi L, Parati Eugenio A, Kraemer M (2016) The diagnostic challenge of Divry van Bogaert and Sneddon syndrome: report of three cases and literature review. J Neurol Sci 364:77–83CrossRefPubMedGoogle Scholar
  12. 12.
    Aquino Gondim Fde A, Leacock RO, Subrammanian TA, Cruz-Flores S (2003) Intracerebral hemorrhage associated with Sneddon’s syndrome: is ischemia-related angiogenesis the cause? Case report and review of the literature. Neuroradiology 45:368–372CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Service of Neurology, University Hospital “Marqués de Valdecilla (IDIVAL)”University of Cantabria, and “Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED)”SantanderSpain
  2. 2.Department of Medicine and Psychiatry, “Edificio Escuela Universitaria de Enfermería (cuarta planta)”University of CantabriaSantanderSpain
  3. 3.Service of Pathology, University Hospital “Marqués de Valdecilla (IDIVAL)”University of CantabriaSantanderSpain

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