Expanding the spectrum of subacute diencephalic angioencephalopathy

Christopher S. Graffeo, Elliot T. Dawson, Meghan E. Murphy, David Black, Joseph E Parisi, Brian A. Crum, Fredric B. Meyer

Research output: Contribution to journalArticle

4 Scopus citations

Abstract

We present a patient with subacute diencephalic angioencephalopathy (SDAE), a poorly understood syndrome of progressive confusion, disorientation, and dementia that rapidly deteriorates to severe encephalopathy and death. This is the seventh and most thoroughly investigated report of this exceedingly rare diagnosis to date, and we present the first evidence of a potential sentinel syndrome that may allow for earlier diagnosis and therapeutic intervention. We also review the relevant literature, and highlight new evidence that suggests that SDAE and the related disorder, subacute brainstem angioencephalopathy (SBAE), represent severe and terminal variants of posterior reversible encephalopathy syndrome (PRES). Taken together, we suggest a new understanding of SDAE/SBAE and PRES as a spectrum of hypertension-associated non-inflammatory vascular encephalopathies that are mediated by a loss of venous autoregulation, marked by cytotoxic edema, and that ultimately produce subacute-to-acute diencephalic, brainstem, and posterior fossa injuries with devastating neurologic consequences.

Original languageEnglish (US)
Pages (from-to)8-13
Number of pages6
JournalJournal of Clinical Neuroscience
Volume23
DOIs
StatePublished - Jan 1 2016

Keywords

  • Encephalopathy
  • Posterior reversible encephalopathy syndrome
  • Rare diseases
  • Subacute diencephalic angioencephalopathy
  • Vasculopathy

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology
  • Physiology (medical)

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  • Cite this

    Graffeo, C. S., Dawson, E. T., Murphy, M. E., Black, D., Parisi, J. E., Crum, B. A., & Meyer, F. B. (2016). Expanding the spectrum of subacute diencephalic angioencephalopathy. Journal of Clinical Neuroscience, 23, 8-13. https://doi.org/10.1016/j.jocn.2015.06.016