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Therapeutic Effect of Steroids in Osmotic Demyelination of Infancy

Lalit R Bansal1

  • 1Division of Neurology, Children's Mercy Hospital, Kansas City, MO, USA.

Child Neurology Open
|April 25, 2018
PubMed

Insights

Severe hypernatremia in an infant can cause osmotic demyelination, leading to neurological deficits. Early high-dose steroid treatment may improve outcomes in these critical cases.

Area of Science:

  • Neurology
  • Pediatrics
  • Endocrinology

Background:

  • Hypernatremia is a critical electrolyte imbalance in infants, often associated with significant morbidity.
  • Osmotic demyelination syndrome (ODS) can occur due to rapid correction of severe hypernatremia or extreme hypernatremia itself.

Observation:

  • An 11-month-old male presented with acute gastroenteritis, seizures, and altered mental status, found to have a serum sodium of 177 mmol/L.
  • Brain MRI revealed hallmarks of osmotic demyelination, including reduced diffusion in white matter, pontine/midbrain T2 hyperintensities, and hemorrhagic cerebellar infarcts, alongside a stroke.

Findings:

  • The infant was diagnosed with hypernatremia-induced central pontine and extrapontine myelinolysis.
  • Treatment with high-dose methylprednisolone for 5 days led to rapid recovery from encephalopathy and spastic quadriparesis.

Implications:

  • Early steroid administration may be a crucial intervention for mitigating neurological deficits in infants with severe hypernatremia-induced ODS.
  • This case highlights the potential for significant neurological sequelae in infancy due to osmotic demyelination and suggests a therapeutic avenue for favorable outcomes.

Keywords:
central pontine myelinolysisextrapontine myelinolysismethylprednisolone

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