Alok Govind Ravishankar Komari1, Abdul Rehman Nasir1, Claire Delpirou Nouh2
1University of Oklahoma Health Sciences Center, 2University of Oklahoma Health Science Center, Department of Neurology
Objective:
To describe a case of Osmotic Demyelination Syndrome (ODS) in context of normonatremia with alternate osmolytes. Our case underscores the wide range o metabolic disturbances resulting in osmotic shifts leading to ODS.
Background:
ODS is a rare, debilitating, and frequently irreversible neurological disorder most commonly linked to rapid correction of severe chronic hyponatremia. The syndrome primarily affects the central pons, although extrapontine regions such as the thalami, hippocampi but also the basal ganglia may also be involved. Recent literature documents ODS occurring in the context of normonatremia and fluctuations in alternative osmolytes and may be underdiagnosed, particularly as initial MRI brain may be negative. We report a case of ODS associated with Glucose fluctuations and chronic alcohol dependence.
Results:
A 52-year-old man with poorly controlled diabetes mellitus type 2 (A1c 11.7), chronic alcohol dependence recently discharged from rehab and reporting relapse, medication non-adherence, presented to the hospital with encephalopathy, gait instability, vision changes and memory loss. He was normonatremic however his glucose level fluctuated widely within 24 hours after presentation. MRI brain without contrast demonstrated central diffusion restriction in the pons suggesting of ODS. Despite acute management including immediate treatment with high-dose IV thiamine and management of all comorbidities, the patient cognitive impairment and episodic memory deficits remained.
This case highlights the need to maintain a high suspicion for ODS in context of any osmotic shifts. Fluctuating glucose and chronic alcohol use can cause demyelination through astrocyte and oligodendrocyte injury, excitotoxicity, and energy-dependent disruption of osmotic homeostasis.
Conclusions:
ODS can result from shifts in osmolytes other than sodium and a high level of suspicion is required for timely recognition. Imaging changes may initially be subtle and repeat MRI should be considered to define the full extent of demyelination including in extrapontine regions like the hippocampi or thalami.
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