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Abstract
Objective: To 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.
Methods: We 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.
Results: Average 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.
Conclusion: In 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.
GLOSSARY
- FLAIR=
- fluid-attenuated inversion recovery;
- HE=
- hypertensive encephalopathy;
- PRES=
- posterior reversible encephalopathy syndrome;
- PRES-SCI=
- posterior reversible encephalopathy syndrome with spinal cord involvement
Footnotes
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Editorial, page 1996
- Received March 12, 2014.
- Accepted in final form June 30, 2014.
- © 2014 American Academy of Neurology
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