New type of posterior reversible encephalopathy syndrome

The authors propose a new syndrome named posterior reversible encephalopathy syndrome with spinal cord involvement (PRES-SCI) based upon two of their own cases with PRES signs and symptoms but with clinical and radiologic involvement of the spinal cord, and six additional cases they identified from a literature search.

The average age of the eight patients was 31 years, and five were male. All of the patients had severe acute hypertension and a confluent, expansile central spinal cord T2 hyperintensity spanning at least four spinal segments, originating at the cervicomedullary junction. Seven of the patients had hypertensive retinopathy, a favorable clinical course with only antihypertensive treatment, and resolution of spinal cord lesions on follow-up imaging. Four of the eight patients had symptoms referable to the spinal cord lesions and only one of the eight had a seizure.

The authors say that PRES-SCI shares many clinical, radiographic, and, presumably, pathophysiologic attributes with PRES, but is a unique disorder that also affects the spinal cord of younger, often male, patients, who thus far have presented with severe acute hypertension, as well as headache, nausea/vomiting, encephalopathy, visual disturbance, renal failure and hypertensive retinopathy but not necessarily seizure. Serologic and cerebrospinal fluid analyses are not diagnostic of PRES-SCI but are necessary to rule out infectious and noninfectious etiologies of myelitis. Most patients have a full recovery.

They conclude that 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.