TY - JOUR
T1 - Expanding the spectrum of subacute diencephalic angioencephalopathy
AU - Graffeo, Christopher S.
AU - Dawson, Elliot T.
AU - Murphy, Meghan E.
AU - Black, David
AU - Parisi, Joseph E
AU - Crum, Brian A.
AU - Meyer, Fredric B.
PY - 2016/1/1
Y1 - 2016/1/1
N2 - 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.
AB - 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.
KW - Encephalopathy
KW - Posterior reversible encephalopathy syndrome
KW - Rare diseases
KW - Subacute diencephalic angioencephalopathy
KW - Vasculopathy
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U2 - 10.1016/j.jocn.2015.06.016
DO - 10.1016/j.jocn.2015.06.016
M3 - Article
C2 - 26314660
AN - SCOPUS:84952637794
VL - 23
SP - 8
EP - 13
JO - Journal of Clinical Neuroscience
JF - Journal of Clinical Neuroscience
SN - 0967-5868
ER -