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• Posterior Reversible Encephalopathy Syndrome: Has the time come for a change in name?
  Sandeep S. Rana
  Published on: 13 April 2016

• Published on: 13 April 2016

  Posterior Reversible Encephalopathy Syndrome: Has the time come for a change in name?

  • Sandeep S. Rana, Physician
  • Other Contributors:
    • Manasi Gahlot, M.D., James Valeriano, M.D, Santosh Ramanathan, MBBS

  Re: Spinal cord involvement in posterior reversible encephalopathy syndrome. B Lapuyade, I Sibon, S Jeanin, V Dousset jnnp 2009;80:35 doi:10.1136/jnnp.2008.154781 Dear Editor, We read with great interest the article, "Spinal cord involvement in posterior reversible encephalopathy syndrome" published by B Lapuyade et al (JNNP January 2009). We recently treated a patient with similar presentation. She was 22 year old female who pre...

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  Re: Spinal cord involvement in posterior reversible encephalopathy syndrome. B Lapuyade, I Sibon, S Jeanin, V Dousset jnnp 2009;80:35 doi:10.1136/jnnp.2008.154781 Dear Editor, We read with great interest the article, "Spinal cord involvement in posterior reversible encephalopathy syndrome" published by B Lapuyade et al (JNNP January 2009). We recently treated a patient with similar presentation. She was 22 year old female who presented with nausea, vomiting, headache, blurred vision and intermittent confusion. Her past medical history was notable for history of lupus induced acute proliferative glomerulonephritis when she was 13 years of age. Subsequently she had been maintained on plaquenil, azathioprine and low dose prednisone. At the time of admission her blood pressure was documented to be 247/150. She was encephalopathic, but moved all extremities equally. Her deep tendon reflexes were globally brisk with extensor planters bilaterally. Her serum creatinine was 6.1 mg/dl which was higher than her baseline of about 2.

  MRI of head revealed hyperintense confluent lesion on T2WI which involved medulla, cerebellum and extended on to the cervical cord. Subsequent MRI of cervical and thoracic cord confirmed the lesion extending to the entire cord (figure 1A, 1B and 1C). Contrast was not administered since the patient had renal impairment. On spinal tap, her cerebrospinal fluid studies were normal, and serum NMO titers were negative. Based on her imaging abnormalities, negative CSF and NMO serology; she was diagnosed with posterior reversible encephalopathy syndrome (PRES). There was notable disparity between imaging studies and clinical examination, i.e., lack of weakness, which typically does not occur with myelitis.

  Patient was treated aggressively with antihypertensive agents and hemodialysis. On renal biopsy she was found to have advanced sclerosing lupus nephritis for which she was given pulse steroids. She improved rapidly and a repeat MRI scan of cervical spine a week later revealed complete resolution of lesions (figure 1D). There are now multiple reports showing involvement of spinal cord in PRES (1, 2, 3). MRI of spinal cord of our patient revealed confluent hyperintense T2WI lesion extending the entire length of the cord, extending to the brain stem and cerebellum, similar to the imaging studies in the case presented by Dr Lapuyade and his colleagues. The disparities of radiological abnormalities to clinical examination(4), resolution of lesions within one to two weeks were also common to both cases. As discussed in the article, the impairment of endothelial cells due to uncontrolled hypertension is considered to be the underlying Pathophysiology leading to vasogenic edema. Our hypothesis is that these factors also involve the spinal cord blood vessels.

  Though the incidence of spinal cord involvement in PRES remains to be studied extensively, with more cases of PRES with spinal cord involvement being identified, it may well be time to consider changing the name from Posterior Reversible Encephalopathy syndrome (PRES) to Posterior Reversible Enchephalo-Myelopathy Syndrome (PREMS). Increased awareness of spinal cord involvement in this syndrome would help avoid misdiagnosis of these patients as having Transverse Myelitis.

  References

  1) Briganti C, Caulo M, Notturno F, Tartaro A, Uncini A. Asymptomatic spinal cord involvement in posterior reversible encephalopathy syndrome. Neurology 2009; 73:1507-1508.

  2) Lapuyade B, Sibon I, Jeanin S, Dousset V. Neurological picture: spinal cord involvement in posterior reversible encephalopathy syndrome. J Neurol Neurosurg Psychiatry 2009; 80:35.

  3) Milia A, Moller J, Pilia G, et al. Spinal cord involvement during hypertensive encephalopathy: clinical and radiological findings. J Neurol 2008; 255:142-143.

  4) Cruz-Flores S, Gondim GAA, Leira EC. Brainstem involvement in hypertensive encephalopathy: clinical and radiological findings. Neurology. 2004; 62(8):1417-1419.

  Figure legends - available upon request.

  .Figure 1: MRI brain T2 weighted (A) axial cut images showing hyperintensity within the medulla and right greater than left cerebellar hemispheres, (B) sagittal section, signal abnormality throughout the cervical spinal cord and extending superiorly to medulla, (C) axial cut, signal abnormality at cervical level with some expansion of the cord and (D) complete resolution of the signal abnormality seen on the prior scan, after 1 week of presentation. Figures available upon request.

  Conflict of Interest:

  None declared

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  Conflict of Interest:
  None declared.

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