smotic demyelination syndrome (ODS) was first described in 1959 by Adams and Victor, who reported "pontine myelinolysis" in alcoholic patients (1). On pathophysiological considerations, and as an increasing number of manifestation sites in addition to the pons were detected, central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) were combined into "osmotic demyelination syndrome". Exact epidemiological data are still lacking, as there is not always a clear distinction between ODS and a prior or underlying disease. Furthermore, ODS is not always detected radiologically. As a result, the introduction of magnetic resonance imaging (MRI) led to increasing incidences of ODS, with the demonstration of oligosymptomatic as well as asymptomatic manifestations. Overall, ODS accounts for 0.4% to 0.56% of all neurological admissions to tertiary referral hospitals and 0.06% of all medical hospital admissions (2-4). MRI-based studies describe
SummaryBackground: Osmotic demyelination syndrome (ODS), which embraces central pontine myelinolysis (CPM) and extrapontine myelinosis (EPM), is often underdiagnosed in clinical practice, but can be fatal. In this article, we review the etiology, pathophysiology, clinical features, diagnosis, treatment, and prognosis of ODS.Methods: Pertinent publications from the years 1959 to 2018 were retrieved by a selective search in PubMed.Results: The most common cause of ODS is hyponatremia; particular groups of patients, e.g., liver transplant recipients, are also at risk of developing ODS. The pathophysiology of ODS consists of cerebral apoptosis and loss of myelin due to osmotic stress. Accordingly, brain areas that are rich in oligodendrocytes and myelin tend to be the most frequently affected. Patients with ODS often have a biphasic course, the first phase reflecting the underlying predisposing illness and the second phase reflecting ODS itself, with pontine dysfunction, impaired vigilance, and movement disorders, among other neurological abnormalities. The diagnostic modality of choice is magnetic resonance imaging (MRI) of the brain, which can also be used to detect oligosymptomatic ODS. The current mainstay of management is prevention; treatment strategies for manifest ODS are still experimental. The prognosis has improved as a result of MRI-based diagnosis, but ODS can still be fatal (33% to 55% of patients either die or remain permanently dependent on nursing care).
Conclusion:ODS is a secondary neurological illness resulting from a foregoing primary disease. Though rare overall, it occurs with greater frequency in certain groups of patients. Clinicians of all specialties should therefore be familiar with the risk constellations, clinical presentation, and prevention of ODS. The treatment of ODS is still experimental at present, as no evidence-based treatment is yet available.