Cortical Visual Impairment (CVI): An Atypical Manifestation of Osmotic Demyelination Syndrome in a Child

Abstract

Osmotic Demyelination Syndrome (ODS) is a unique process of selective demyelination and destruction of oligodendrocytes and astrocytes in specific areas of the brain that usually occurs a few days after an osmotic stress [1]. ODS has been described most frequently in association with a rapid correction of hyponatremia, though it may occur with other electrolyte or metabolic abnormalities [1]. ODS is a rather uncommon disorder in childhood [2,3] and encompasses central pontine and extra pontine myelinolysis. ODS may be associated with neurological impairment in the form of ataxia, bulbar dysfunction, gait disturbances, spasticity, seizures, encephalopathy and even coma and death. It may result in long term neurodevelopmental sequelae [2,3].