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August 2008

Reversible Posterior Leukoencephalopathy (and Vasculopathy?) Syndrome

Arch Neurol. 2008;65(8):1135-1137. doi:10.1001/archneur.65.8.1135-a

There is probably no less aptly named neurological disorder than reversible posterior leukoencephalopathy syndrome (RPLS), which, as Lee and coauthors1 report, is frequently irreversible, rarely limited to the posterior regions of the brain, and often located in gray matter and cortex as well as white matter. Another frequently noted feature of this disorder, however, is the occurrence of a variable cerebral arteriopathy similar in some respects to the reversible cerebral vasoconstriction syndrome.2,3 The two disorders may overlap in many cases, presumably because of a common disturbance in autoregulatory capacity.2 This accompanying vasculopathy may be responsible for some of the irreversible injury, presumably ischemic, seen in RPLS. It would be helpful to know whether arteriopathy was seen in this large series and particularly whether the presence of arteriopathy was associated with the irreversible injury seen in some of the cases. Future studies of RPLS should include vascular imaging and perfusion studies as well as structural brain imaging if we are to fully understand the clinical spectrum of the disorder. Vascular studies could also help to disentangle the relative contributions of capillary leakage and autoregulatory dysfunction in these patients.

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