Posterior Reversible Encephalopathy Syndrome With Spinal Cord Involvement

Posterior reversible encephalopathy syndrome (PRES) results from the failure of cerebrovascular autoregulation with subsequent extravasation of intravascular fluid into the cerebral interstitial space. PRES may rarely affect the spinal cord, termed PRES with spinal cord involvement (PRES-SCI).

A 34-year-old woman with untreated treated systemic lupus with lupus nephritis presented with altered mental status, blurred vision, and seizures. Initial blood pressure was 189/120 mm Hg. The neurological exam was notable for encephalopathy, but otherwise there were no focal motor or sensory deficits. Brain magnetic resonance imaging (MRI) showed posterior predominate T2-hyperintense lesions in a pattern suggestive of PRES. Spine MRI revealed a nonenhancing longitudinally extensive lesion involving most of the cervical and thoracic spinal cord. Investigations for alternative causes of myelitis, including serum antimyelin oligodendrocyte glycoprotein and antiaquaporin-4 antibodies, were negative. The patient's encephalopathy, blurred vision, and seizures gradually resolved with blood pressure control.

PRES-SCI is a relatively rare but likely under-recognized variant of PRES. This diagnosis should be considered in patients with severe acute hypertension and nonenhancing longitudinally extensive T2-hyperintense spinal cord lesions. Patients may exhibit signs and symptoms of myelopathy or, conversely, may demonstrate minimal myelopathic features despite striking MRI abnormalities. Early recognition of PRES-SCI may obviate the need for further testing or empiric myelitis treatment.