Title

Osmotic Demyelination Syndrome in Children.

Document Type

Article

Publication Date

8-2019

Identifier

DOI: 10.1016/j.pediatrneurol.2019.03.018

Abstract

Osmotic demyelination syndrome is an acute demyelination process that usually occurs several days following an osmotic stress. This syndrome is rare in adults (0.4% to 0.56%) and even more uncommon in children. We performed a review of all reported pediatric osmotic demyelination syndrome patients from 1960 to 2018. Among all 106 cases, 49 presented with isolated central pontine myelinolysis, 30 with isolated extrapontine myelinolysis, and 27 with combined central pontine myelinolysis and extrapontine myelinolysis. There was no gender preponderance, and the highest prevalence was noted between the ages one and five years. Magnetic resonance imaging remains the diagnostic modality of choice, and diffusion tensor imaging is now increasingly used for prognostication in osmotic demyelination syndrome. Sixty percent of the children had a complete neurological recovery. Current management of osmotic demyelination syndrome in children consists of supportive medical care, steroids, and intravenous immunoglobulin. Our review of the literature supports the hypothesis that steroids and immunoglobulins are potentially helpful, although additional controlled studies are needed.

Journal Title

Pediatric neurology

Volume

97

First Page

12

Last Page

17

MeSH Keywords

Adrenal Cortex Hormones; Age of Onset; Alcoholism; Animals; Brain Damage, Chronic; Child; Child, Preschool; Disease Models, Animal; Humans; Hypernatremia; Immunoglobulins, Intravenous; Infant; Magnetic Resonance Imaging; Myelinolysis, Central Pontine; Neuroimaging; Osmotic Pressure; Positron-Emission Tomography; Prevalence; Rats; Recovery of Function; Sodium; Thyrotropin-Releasing Hormone; Water-Electrolyte Imbalance

Keywords

Central pontine myelinolysis; Diffusion tensor imaging; Extrapontine myelinolysis; Fraction anisotrophy; Mean diffusivity; Osmotic demyelination.

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