Central Pontine and Extrapontine Myelinolysis in a Pediatric Patient Following Rapid Correction of Hypernatremia

Abstract

Central pontine and extrapontine myelinolysis are uncommon disorders characterized by distinctive clinical features and typical findings on neuroimaging. Only a few cases are reported in the pediatric age group. We describe the case of a leukemic, malnourished 14-year-old boy with a high serum sodium concentration that gradually increased to 170 mmol/L. During a septic shock episode, hydration with a low sodium concentration at the rate of 104 mL/h for 24 h was administered. A rapid correction of the high serum sodium occurred, exceeding 0.5 mmol/L/h. The following day the patient developed rapid and progressive neurological impairment with clinical features characteristic of central pontine and extrapontine myelinolysis. Magnetic resonance imaging confirmed the diagnosis 11 days later. The patient was treated with steroids and intravenous immunoglobulins. He achieved an almost full neurological recovery and radiological improvement. The reported case demonstrates that central pontine and extrapontine myelinolysis can occur after excessively rapid correction of hypernatremia.

central pontine myelinolysis - extrapontine myelinolysis - hypernatremia - immunoglobulins

References

• 1 Brown WD. Osmotic demyelination disorders: central pontine and extrapontine myelinolysis.  Curr Opin Neurol. 2000;  13 691-697
• 2 Buis CI, Wijdicks EFM. Serial magnetic resonance imaging of central pontine myelinolysis.  Liver Transpl. 2002;  8 643-645
• 3 Chang L, Harrington DW, Milkotic A. et al . Unusual occurrence of extrapontine myelinolysis associated with acute severe hypernatraemia caused by central diabetes insipidus.  Clin Endocrinol. 2005;  63 233-235
• 4 Escribano-Gascón AB, Casanova-Peño LI, Bartolomé-Puras M. et al . Efficacy of intravenous immunoglobulins in central pontine myelinolysis.  Neurologia. 2008;  23 392-394
• 5 Grimaldi D, Cavalleri F, Vallone S. et al . Plasmapheresis improves the outcome of central pontine myelinolysis.  J Neurol. 2005;  252 734-735
• 6 Hagiwara K, Okada Y, Shida N. et al . Extensive central and extrapontine myelinolysis in a case of chronic alcoholism without hyponatremia: A case report with analysis of serial MR findings.  Intern Med. 2008;  47 431-435
• 7 Huq S, Wong M, Chan H. et al . Osmotic demyelination syndromes: central and extrapontine myelinolysis.  J Clin Neurosci. 2007;  14 684-688
• 8 Martin RJ. Central pontine and extrapontine myelinolysis: the osmotic demyelination syndromes.  J Neurol Neurosurg Psychiatry. 2004;  75 22-28
• 9 Menger H, Jorg J. Outcome of central pontine myelinolysis.  J Neurol. 1999;  246 700-705
• 10 Pearce JM. Central pontine myelinolysis.  Eur Neurol. 2009;  61 59-62
• 11 Ruzek KA, Campeau NG, Miller JM. Early diagnosis of central pontine myelinolysis with diffusion-weighted imaging.  AJNR Am J Neuroradiol. 2004;  25 210-213
• 12 Shah B, Tobias JD. Osmotic demyelination and hypertonic dehydration in a 9-year-old girl: changes in cerebrospinal fluid myelin basic protein.  J Intensive Care Med. 2006;  21 372-376
• 13 Snell DM, Bartley C. Osmotic demyelination syndrome following rapid correction of hyponatraemia.  Anaesthesia. 2008;  63 92-95

Correspondence