• Adam de Havenon, Zachary Joos, Loren Longenecker, Lubdha Shah, Safdar Ansari, and Kathleen Digre.
• From the Department of Neurology (A.d.H.), University of Washington, Seattle; and the Departments of Ophthalmology (Z.J., K.D.), Radiology (L.L., L.S.), and Neurology (S.A., K.D.), University of Utah, Salt Lake City. ahdh@uw.edu.
• Neurology. 2014 Nov 25; 83 (22): 2002-6.

ObjectiveTo characterize a cohort of patients with the signs and symptoms of posterior reversible encephalopathy syndrome (PRES), but with clinical and radiologic involvement of the spinal cord.MethodsWe report 2 cases of PRES with spinal cord involvement and identified an additional 6 cases in the Medline database using various search terms related to "spinal PRES," "spinal reversible posterior leukoencephalopathy syndrome," and "spinal hypertensive encephalopathy." We analyzed the clinical and imaging characteristics of the 8 cases.ResultsAverage age was 31 years, with 5 male and 3 female patients. All patients had severe acute hypertension and a confluent, expansile central spinal cord T2 hyperintensity spanning at least 4 spinal segments, originating at the cervicomedullary junction. Of 8 patients, 7 had hypertensive retinopathy, a favorable clinical course with only antihypertensive treatment, and resolution of the spinal cord lesions on follow-up imaging. A total of 4 of 8 patients had symptoms referable to the spinal cord lesions and only 1 of 8 had a seizure.ConclusionIn light of the already wide definition of PRES, we propose a new syndrome named PRES with spinal cord involvement (PRES-SCI). Clinicians should suspect PRES-SCI when patients with PRES have neurologic signs referable to the spinal cord, extreme elevation in blood pressure, MRI lesions that extend to the cervicomedullary junction, or grade IV hypertensive retinopathy. These clinical scenarios should prompt a cervical spine MRI to help guide patient management decisions and prognostication. When clinicians evaluate longitudinally extensive spinal T2 hyperintensities, they should consider PRES-SCI, which, if diagnosed, would spare patients the morbidity of a standard myelitis workup and empiric treatment.© 2014 American Academy of Neurology.

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