Posterior reversible leukoencephalopathy syndrome with spinal cord involvement in a 9-year-old girl

Uluç Yiş1, Pakize Karaoğlu1, Semra Hız Kurul1

  • 1Dokuz Eylül University, School of Medicine, Department of Pediatrics, Division of Pediatric Neurology, İzmir, Turkey.

Brain & Development
|July 30, 2015
PubMed

Insights

This study details the youngest pediatric patient diagnosed with posterior reversible encephalopathy syndrome (PRES) affecting the brainstem and spinal cord. Severe hypertension, even with minimal symptoms, can cause spinal PRES, highlighting the need for clinical awareness.

Area of Science:

  • Neurology
  • Pediatrics
  • Nephrology

Background:

  • Posterior reversible encephalopathy syndrome (PRES) is a neurological condition.
  • PRES typically affects the brain's white matter.
  • Spinal cord involvement in pediatric PRES is rare.

Observation:

  • A pediatric patient presented with bicytopenia, renal dysfunction, and visual disturbances.
  • Cerebrospinal fluid analysis revealed elevated protein, IgG index, and cell count.
  • Magnetic resonance imaging showed extensive lesions in the brainstem and spinal cord.

Findings:

  • The patient was diagnosed with severe hypertension secondary to reflux nephropathy.
  • This case represents the youngest pediatric diagnosis of PRES.
  • The lesions were confined to the brainstem and spinal cord, a rare presentation.

Implications:

  • Severe hypertension can precipitate spinal PRES by exceeding autoregulation in the spinal cord's anterior territory.
  • Clinicians must consider spinal PRES in pediatric cases with extensive brainstem and spinal cord lesions.
  • The phenomenon of "clinical radiologic dissociation" (minimal symptoms despite significant imaging findings) is crucial for diagnosis.