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Atypical Posterior Reversible Encephalopathy Syndrome with Spinal Cord Involvement (PRES-SCI)

•Background:

  • PRES rarely manifests in the spinal cord. In case series, imaging of atypical PRES includes isolated deep gray nuclear, brainstem, cerebellar, and, rarely, spinal cord involvement without cerebral hemispheric involvement.

•Clinical Presentation:

  • There are few reported cases of PRES-SCI in the literature. Clinical presentations have been similar to PRES. Patients may present with headaches, blurry vision, nausea, and vomiting, but infrequently with seizures. A multitude of medical conditions and drugs are associated with PRES. PRES-SCI has most often been described in men presenting with severe hypertension and kidney failure. Despite impressive imaging changes, the neurologic exam is normal or near normal. Formal ophthalmologic assessment may reveal hypertensive retinopathy.

•Key Diagnostic Features:

  • In the few reported cases of PRES-SCI, spinal cord expansile T2 hyperintensities were located at the cervical medullary junction and spanned 4 levels. Less frequently, there is thoracic spinal cord involvement. Lower brainstem and spinal cord involvement may occur in isolation. Gray and white matter changes have been described. With treatment, imaging will improve in 1–2 weeks and is normal months later.

•Differential Diagnosis:

  • Neuromyelitis optica spectrum disorder (NMSD): This disorder is most common in women. Clinical manifestations include a single or recurrent episode of symptoms including bilateral optic neuritis, myelitis, or an area postrema syndrome (hiccups, nausea, vomiting). Blood tests are positive for aquaporin 4 IgG. Brain MRI may be normal or have T2-weighted periventricular hyperintensities. Orbital MRI often demonstrates bilateral posterior optic nerve contrast enhancement. Spinal MRI demonstrates a T2-bright longitudinal lesion that is 3 or more vertebral levels.
  • Myelin oligodendrocyte glycoprotein (MOG) antibody disease: MOGAD has a female preponderance and most commonly occurs after a viral illness or, less frequently, after immunization. Clinical manifestations may include bilateral optic neuritis, acute disseminated encephalomyelitis (ADEM), myelitis, and overlap with NMSD. Blood tests are positive for MOG-IgG. Brain MRI reveals ADEM-type white matter, deep gray matter, and/or brainstem T2 hyperintensities that are often fluffy/confluent. Orbital MRI may show bilateral anterior optic nerve enhancement. MRI spinal cord may have multiple longitudinal T2 hyperintense lesions, especially involving the conus.
  • Acute disseminated encephalomyelitis (ADEM): This most commonly occurs in children following an illness or vaccination. Clinical manifestations include fever, headache, nausea and vomiting, encephalopathy, coma, and seizures. Brain MRI shows multiple fluffy white matter and deep gray T2-weighted hyperintensities. Spinal cord MRI will have longitudinal extensive lesions.
  • Infectious myelitis: Many different viral infections can involve the spinal cord, with more common ones including West Nile virus and, nowadays, SARS-CoV-2. Patients with viral myelitis are typically severely myelopathic on exam. Imaging manifestations include edema, variable involvement of gray and white matter, and irregular postgadolinium enhancement. Many viral infections also result in diffusion restriction.

•Treatment:

  • Hypertension control
January 5, 2023

A young woman presents with 1 month of blurry vision and intermittent headaches. She is hypertensive (BP 230/130). Fundoscopic examination shows bilateral blurred disc margins, cotton wool spots, macular edema, mild arteriole attenuation, hemorrhages, and choroidal infarcts. Neurologic exam notes poor visual acuity, red color desaturation, and symmetric hyperreflexia. Initial blood tests reveal elevated BUN and creatinine. All serum autoimmune tests and CSF are normal. She improves after treatment.

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