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Posterior Reversible Encephalopathy Syndrome in a Child With Renovascular Hypertension

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 6-year child presented with headache for 1 month and seizures followed by altered consciousness for 1 day. He had new-onset daily occipital headache for 1 month of mild to moderate severity and pulsating character. Occasionally, the headache became severe accompanied by vomiting, photophobia, and phonophobia. The child had 4 to 5 episodes of generalized tonic-clonic seizures 1 day prior to admission. He remained unconscious for several hours and spontaneously regained consciousness over 24 hours. There was no history of fever, weight loss, limb weakness, vision impairment, or any strokelike illness. His birth and developmental history were unremarkable.

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A 6-year child presented with headache for 1 month and seizures followed by altered consciousness for 1 day. He had new-onset daily occipital headache for 1 month of mild to moderate severity and pulsating character. Occasionally, the headache became severe accompanied by vomiting, photophobia, and phonophobia. The child had 4 to 5 episodes of generalized tonic-clonic seizures 1 day prior to admission. He remained unconscious for several hours and spontaneously regained consciousness over 24 hours. There was no history of fever, weight loss, limb weakness, vision impairment, or any strokelike illness. His birth and developmental history were unremarkable.

On examination, his pulse rate was 80 beats per minute (regular) with all peripheral pulses palpable without any radio-femoral delay. His blood pressure was 170/110 mm Hg with less than 10 mm Hg difference of systolic and diastolic blood pressure in other limbs. Abdominal examination revealed abdominal bruit. Fundus examination revealed papilledema with changes of hypertensive retinopathy. He was not oriented to place and time. The cranial nerves, motor, sensory, and autonomic system examination were unremarkable. Meningeal signs were absent. There was no evidence of neurocutaneous markers. His cranial magnetic resonance imaging revealed bilateral white matter hyperintensity that was more marked in the bilateral parieto-occipital region. Ultrasound of the abdomen revealed a small right kidney and renal Doppler was suggestive of right renal-artery stenosis. His computed tomography angiography revealed short segment (3 cm) and short circumferential thickening of the descending aorta with right renal artery narrowing (Figure 1). His repeat cranial magnetic resonance imaging showed significant reduction in the white matter hyperintensities.

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Article Information

Corresponding Author: Vimal Kumar Paliwal, DM, Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, Uttar Pradesh, 226014 India (dr_vimalkpaliwal@rediffmail.com).

Published Online: June 5, 2023. doi:10.1001/jamaneurol.2023.1501

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

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AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 credit toward the CME [and Self-Assessment requirements] of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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