A Child with Central Variant Posterior Reversible Encephalopathy Syndrome

A Child with Central Variant Posterior Reversible Encephalopathy Syndrome

A 10-year-old girl presented with history of episodic headache and facial flushing for 2 years with recent worsening. On examination, her blood pressure was 260/190 mm Hg. She had left upper motor neuron facial palsy and bilateral papilledema. A magnetic resonance imaging (MRI) of the brain was suggestive of central variant posterior reversible encephalopathy syndrome (PRES; [Fig. 1]). No diffusion restriction was noted. MRI abdomen revealed left suprarenal mass ([Fig. 2]). Urine vanillyl mandelic acid was 50 mg/g creatinine (normal < 7 mg/g). The child underwent laparotomy for suprarenal mass. Postoperative histopathology was suggestive of pheochromocytoma. Postoperative period remained uncomplicated and her hypertension was controlled. At 2 years of follow-up, she is neurologically normal and off antihypertensive medications.

Fig. 1 Magnetic resonance imaging brain axial sections showing T1 hypointensities (A) and corresponding T2/fluid-attenuated inversion recovery (FLAIR, Fig 1C) hyperintensities of the bilateral caudate, putamen, thalamus, left sided posterior limb of internal capsule (B and C), and right sided mid brain at the level of 4th ventricle (D). MRI, magnetic resonance imaging.

Fig. 2 Magnetic resonance imaging abdomen showing T2-hyperintense (A) lobulated retroperitoneal mass (black arrow) of size 5.5 × 4.2 × 3.7 cm at left suprarenal area. Heterogenous contrast enhancement noted in the post contrast image (B).The mass was compressing the left kidney (white arrow).

PRES commonly involves parieto-occipital regions of the cerebral cortex.[1] Central variant PRES is a rare phenomenon described as brainstem, basal ganglia, and thalamic involvement with sparing of subcortical white matter.[2] Pediatric PRES shows typical parieto-occipital area involvement in only one-third of the cases. In a series of pediatric PRES, deep gray matter involvement was noted in 12%, thalamus in 9%, and diffusion restriction in 16% children.[3] Rarely, spinal cord variant of PRES has also been described.[4] Susceptibility weighted imaging may show punctate microhemorrhages or parenchymal hemorrhages.

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An informed consent form was signed by the parents of the patient to approve the use of patient information or material for scientific purposes.

• References

• 1 Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. Lancet Neurol 2015; 14 (09) 914-925
• 2 McKinney AM, Jagadeesan BD, Truwit CL. Central-variant posterior reversible encephalopathy syndrome: brainstem or basal ganglia involvement lacking cortical or subcortical cerebral edema. AJR Am J Roentgenol 2013; 201 (03) 631-638
• 3 Gupta V, Bhatia V, Khandelwal N, Singh P, Singhi P. Imaging findings in pediatric posterior reversible encephalopathy syndrome (PRES): 5 years of experience from a tertiary care center in India. J Child Neurol 2016; 31 (09) 1166-1173
• 4 Gocmen R, Ardicli D, Erarslan Y, Duzova A, Anlar B. Reversible hypertensive myelopathy-the spinal cord variant of posterior reversible encephalopathy syndrome. Neuropediatrics 2017; 48 (02) 115-118